Sickle cell disease

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of the family are avoided. The stigmatization and social isolation people with sickle cell disease tend to experience is often the consequence of popular misconceptions that people with SCD should not socialize with those free from the disease. This mentality robs people with SCD of the right to freely participate in community activities like everyone else SCD-related stigma and social isolation in schools, especially, can make a life for young people living with sickle cell disease extremely difficult. For school-aged children living with SCD, the stigma they face can lead to peer rejection. Peer rejection involves the exclusion from social groups or gatherings. It often leads the excluded individual to experience emotional distress and may result in their academic underperformance, avoidance of school, and occupational failure later in life. This social isolation is also likely to negatively impact people with SCD's

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and consequently, high blood-flow velocity is correlated with narrowing of the arteries. In 2002, the National Institute of Health (NIH) issued a statement recommending that children with sickle cell get the Transcranial Doppler ultrasound screen annually, and in 2014, a panel of experts convened by the NIH issued guidelines reiterating the same recommendation. One review of medical records, by hematologist Dr. Julie Kanter at the University of Alabama at Birmingham, showed that on average only 48.4 per cent of children with sickle cell get the recommended ultrasound test.

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This means that 66 million people are at risk of having a child who has sickle cell disease. It is also estimated that about 25,000 Ugandans are born each year with SCD and 80% of those people do not live past five years old. SCD also contributes 25% to the child mortality rate in Uganda. The Bamba people of Uganda, located in the southwest of the country, carry 45% of the gene which is the highest trait frequency recorded in the world. The Sickle Cell Clinic in Mulago is only one sickle cell disease clinic in the country and on average sees 200 patients a day.

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Due to many adults in high-risk groups not knowing if they are carriers, pregnant women and both partners in a couple are offered screening so they can get counselling if they have the sickle cell trait. In addition, blood donors from those in high-risk groups are also screened to confirm whether they are carriers and whether their blood filters properly. Donors who are found to be carriers are informed and their blood, while often used for those of the same ethnic group, is not used for those with sickle cell disease who require a blood transfusion.

663:. Another influence of hemolytic crises in sickle cell disease is oxidative stress on the erythrocytes, leukocytes, and platelets. When there is not enough red blood cell production in the bone marrow, the oxygen that the body receives, processes, and transports is unbalanced with the body's antioxidants. There is an imbalance in the oxygen reactive species in the cells, which leads to more production of red blood cells that are not properly oxygenated or formed. Oxidative stress may lead to anemia because of the imbalance of oxygen in the tissue. 3183:

environmental factors to place blame on the mother reflects many Ugandan's poor knowledge of how the disease is acquired as it is determined by genetics, not environment. Mothers of children with sickle cell disease are also often left with very limited resources to safeguard their futures against the stigma of having SCD. This lack of access to resources results from their subordinating roles within familial structures as well as the class disparities that hinder many mothers' ability to satisfy additional childcare costs and responsibilities.

947: 3158:. Other misconceptions about SCD include the belief that it is caused by environmental factors but, in reality, SCD is a genetic disease. There have been efforts throughout Uganda to address the social misconceptions about the disease. In 2013, the Uganda Sickle Cell Rescue Foundation was established to spread awareness of sickle cell disease and combat the social stigma attached to the disease. In addition to this organization's efforts, there is a need for the inclusion of sickle cell disease education in preexisting community 3272:. The researchers used a viral vector to make the mice—which have essentially the same defect that causes human sickle cell disease—express production of fetal haemoglobin (HbF), which an individual normally ceases to produce shortly after birth. In humans, using hydroxyurea to stimulate the production of HbF has been known to temporarily alleviate sickle cell disease symptoms. The researchers demonstrated that this gene therapy method is a more permanent way to increase therapeutic HbF production. 12597: 955: 2951:, a study published in 1998 that covered about 56,000 people in hospitals in Bahrain found that 2% of newborns have sickle cell disease, 18% of the surveyed people have the sickle cell trait, and 24% were carriers of the gene mutation causing the disease. The country began screening of all pregnant women in 1992, and newborns started being tested if the mother was a carrier. In 2004, a law was passed requiring couples planning to marry to undergo free 12627: 12637: 437: 927: 915: 3132:. Failure to provide the public with information about sickle cell disease results in a population with a poor understanding of the causes of the disease, symptoms, and prevention techniques. The differences, physically and socially, that arise in those with sickle cell disease, such as jaundice, stunted physical growth, and delayed sexual maturity, can also lead them to become targets of bullying, rejection, and stigma. 678:, presenting as early as six months of age, and may occur in children with sickle cell trait. The crisis can last up to a month. Given that pneumonia and sickling in the lung can both produce symptoms of acute chest syndrome, the patient is treated for both conditions. It can be triggered by painful crisis, respiratory infection, bone-marrow embolisation, or possibly by atelectasis, opiate administration, or surgery. 935: 12617: 12607: 429: 12663: 3847: 56: 1441:, and close observation. In the absence of high quality evidence regarding the effectiveness of antibiotics for acute chest syndrome in people with sickle cell disease, there is no standard antibiotic treatment as of 2019. It is recommended that people with suspected acute chest syndrome should be admitted to the hospital with worsening A-a gradient an indication for ICU admission. 1076: 2940:, about 4.2% of the population carry the sickle cell trait and 0.26% have sickle cell disease. The highest prevalence is in the Eastern province, where approximately 17% of the population carry the gene and 1.2% have sickle cell disease. In 2005, Saudi Arabia introduced a mandatory premarital test including HB electrophoresis, which aimed to decrease the incidence of SCD and 1468:, probably reduces the frequency of painful episodes and the risk of life-threatening illness or death but there is currently insufficient evidence regarding the risk of adverse effects. Hydroxyurea and phlebotomy combined may be more effective than transfusion and chelation combined in terms of pain, life-threatening illness and risk of death. 1534:

the process' success. However, there are still some drawbacks to hematopoietic stem cell transplantation (HSCT), such as the requirement for precise details to inhibit the susceptible system and problems from the new cells not taking hold. However, a successful HSCT can result in a long-term cure from sickle cell anemia for the patient.

3008:(1861–1954), in 1910. Irons saw "peculiar elongated and sickle-shaped" cells in the blood of a man named Walter Clement Noel, a 20-year-old first-year dental student from Grenada. Noel had been admitted to the Chicago Presbyterian Hospital in December 1904 with anaemia. Noel was readmitted several times over the next three years for " 1298:, provides not only a method of early detection for individuals with sickle cell disease but also allows for the identification of the groups of people who carry the sickle cell trait. Genetic counselors can help individuals of colour and their families tackle the racial and ethnic disparities that exist in healthcare. 3217:

failure to inform the hospital haematology team and poor pain management had caused deaths. Specialist haematology staff preferred to work in bigger, teaching hospitals, leading to shortages of expertise elsewhere. In 2021, the NHS initiated its first new treatment in 20 years for Sickle Cell. This involved the use of

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aspects of life including social and psychological well-being. Studies have shown that those with SCD frequently feel as though they must keep their diagnosis a secret to avoid discrimination in the workplace and also among peers in relationships. In the 1960s, the US government supported initiatives for workplace

1153:. However, the African American community is descended from several African and non-African ethnic groups including American slaves. Thus, a degree of genetic dilution via crossbreeding with non-African people and high health-selective pressure through slavery (especially the slave trade and the frequently deadly 3170:

The deeply rooted stigma of SCD from society causes families to often hide their family members' sick status for fear of being labeled, cursed, or left out of social events. Sometimes in Uganda, when it is confirmed that a family member has sickle cell disease, intimate relationships with all members

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promotes red blood cell sickling and repeated episodes of sickling damage the cell membrane and decrease the cell's elasticity. These cells fail to return to normal shape when normal oxygen tension is restored. As a consequence, these rigid blood cells are unable to deform as they pass through narrow

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Sickle cell gene mutation probably arose spontaneously in different geographic areas, as suggested by restriction endonuclease analysis. These variants are known as Cameroon, Senegal, Benin, Bantu, and Saudi-Asian. Their clinical importance is because some are associated with higher HbF levels, e.g.,

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pattern of inheritance from parents. The types of haemoglobin a person makes in the red blood cells depend on what haemoglobin genes are inherited from her or his parents. If one parent has sickle cell anaemia and the other has sickle cell trait, then any given child has a 50% chance of having sickle

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or focal abnormality, respiratory symptoms, or hypoxemia. It is the second-most common complication and it accounts for about 25% of deaths in patients with SCD. Most cases present with vaso-occlusive crises, and then develop acute chest syndrome. Nevertheless, about 80% of people have vaso-occlusive

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SCD patients do not experience a crisis until five years of age, or older. Some of these patients, and others who have other genotypes, may only present with symptoms under conditions of moderate to severe hypoxia, and thus may be unaware that they have the disease until later in life. Among children

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The data compiled on sickle cell disease in Uganda has not been updated since the early 1970s. The deficiency of data is due to a lack of government research funds, even though Ugandans die daily from SCD. Data shows that the trait frequency of sickle cell disease is 20% of the population in Uganda.

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surrounding SCD that discourage people with SCD from receiving necessary care. These stigmas mainly affect people of African American and Latin American ancestries, according to the National Heart, Lung, and Blood Institute. People with SCD experience the impact of stigmas of the disease on multiple

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is about 100,000 (one in 3,300), mostly affecting Americans of sub-Saharan African descent. In the United States, about one out of 365 African-American children and one in every 16,300 Hispanic-American children have sickle cell anaemia. The life expectancy for men with SCD is approximately 42 years

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were affected by sickle cell anaemia, giving a total of 150,000 affected children born every year in Nigeria alone. The carrier frequency ranges between 10 and 40% across equatorial Africa, decreasing to 1–2% on the North African coast and <1% in South Africa. Studies in Africa show a significant

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About 90% of people survive to age 20, and close to 50% survive beyond age 50. In 2001, according to one study performed in Jamaica, the estimated mean survival for people was 53 years for men and 58 years for women with homozygous SCD. The life expectancy in much of the developing world is unknown.

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Transcranial Doppler ultrasound (TCD) can detect children with sickle cell that have a high risk for stroke. The ultrasound test detects blood vessels partially obstructed by sickle cells by measuring the rate of blood into the brain, as blood flow velocity is inversely related to arterial diameter,

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In 2017, twelve clinical trials were focusing on gene therapy to treat sickle cell anemia. Of those 12 trials, four of them replaced the mutated HBB gene with a healthy one. Three trials used Mozobil, a medication used to treat types of cancer, to determine whether the increase of stem cells can be

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In 2021 many patients were found to be afraid to visit hospitals, such was the level of ignorance among staff, so purchased pain relief to treat themselves outside the NHS.They were often waiting a long time for pain relief, and sometimes suspected of "drugs-seeking" behaviour. Delays to treatment,

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million are carriers of the sickle cell trait. Most infants with SCD born in the United States are identified by routine neonatal screening. As of 2016 all 50 states include screening for sickle cell disease as part of their newborn screen. The newborn's blood is sampled through a heel-prick and is

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The treatment for sickle cell anemia implicitly involves hematopoietic stem cell transplantation (HSCT). This entails replacing the dysfunctional stem cells in the case with healthy bones from a well-matched donor. Finding the ideal client—typically a stock or someone almost matched—is essential to

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have proven effective in children; they are the only known cure for SCD. However, bone marrow transplants are difficult to obtain because of the specific HLA typing necessary. Ideally, a close relative (allogeneic) would donate the bone marrow necessary for transplantation. The close relative needs

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is indicated. The latter involves the exchange of a significant portion of the person's red cell mass for normal red cells, which decreases the level of haemoglobin S in the patient's blood. However, there is currently uncertain evidence about the possible benefits or harms of blood transfusion for

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In 2010, there was significant consideration and debate in the US surrounding comprehensive screening of athletes for SCD. In 2012, the American Society of Hematology concluded that they do not support testing or disclosure of sickle cell trait status as a prerequisite for participation in athletic

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of gene therapy for sickle cell disease in humans were started in 2014. The clinical trials assessed the safety of lentiviral vector-modified bone marrow for adults with severe sickle cell disease. A case report for the first person treated was published in March 2017, with a few more people being

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launched a campaign called "Can you tell it's sickle cell?". The campaign had twin aims. One was to increase awareness of the key signs and symptoms of the blood disorder so that people would be as alert to signs of a sickle cell crisis as they are to an imminent heart attack or stroke. The second

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In the United Kingdom, between 12,000 and 15,000 people are thought to have sickle cell disease with an estimated 250,000 carriers of the condition in England alone. As the number of carriers is only estimated, all newborn babies in the UK receive a routine blood test to screen for the condition.

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by sufferers. The treatment can be accessed, via consultants, at any of ten new hubs set up around the country. In the same year, however, an All-Party Parliamentary Group produced a report on Sickle Cell and Thalassaemia entitled 'No-one is listening'.Partly in response to this, on 19 June 2022,

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by invading the red cell precursors and multiplying in and destroying them. Parvovirus infection almost completely prevents red blood cell production for two to three days. In normal individuals, this is of little consequence, but the shortened red cell life of SCD patients results in an abrupt,

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of SCD, especially if SCD is not present in earlier generations, due to the suspicion that the child's poor health may have been caused by the mother's failure to implement preventative health measures or promote a healthy environment for her child to thrive. The reliance on theories related to

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The protective effect of sickle cell trait does not apply to people with sickle cell disease; in fact, they are more vulnerable to malaria, since the most common cause of painful crises in malarial countries is infection with malaria. People with sickle cell disease living in malarial countries

3338:(M/Met) start codon in protein amino acid position numbering. Current nomenclature calls for counting the methionine as the first amino acid, resulting in the glutamic acid residue falling at position 7. Many references still refer to position 6 and both should likely be referenced for clarity. 1137:

parasite unable to reproduce. Further, the polymerization of Hb affects the ability of the parasite to digest Hb in the first place. Therefore, in areas where malaria is a problem, people's chances of survival actually increase if they carry sickle cell traits (selection for the heterozygote).

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is 40 to 60 years. It often gets worse with age. All the major organs are affected by sickle cell disease. The liver, heart, kidneys, gallbladder, eyes, bones, and joints also can suffer damage from the abnormal functions of the sickle cells, and their inability to flow through the small blood

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The first modern report of sickle cell disease may have been in 1846, where the autopsy of an executed runaway slave was discussed; the key finding was the absence of the spleen. Reportedly, African slaves in the United States exhibited resistance to malaria, but were prone to leg ulcers. The

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exists for those with sickle cell disease because of the lack of general knowledge of the disease. The general gap in knowledge surrounding sickle cell disease is noted among adolescents and young adults due to the culturally sanctioned secrecy about the disease. While most people have heard

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activities due to lack of scientific evidence, inconsistency with good medical practice, and inconsistency with public health ethics. They recommended universal interventions to reduce exertion-related injuries and deaths effective for all athletes irrespective of their sickle cell status.

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The stigma around the disease is particularly bad in regions of the country that are not as affected. For example, Eastern Ugandans tend to be more knowledgeable of the disease than Western Ugandans, who are more likely to believe that sickle cell disease resulted as a punishment from

67:. Figure (B) shows abnormal, sickled red blood cells sticking at the branching point in a blood vessel. The inset image shows a cross-section of a sickle cell with long polymerized sickle haemoglobin (HbS) strands stretching and distorting the cell shape to look like a crescent moon. 1157:) may be the most plausible explanations for the lower prevalence of sickle cell anaemia (and, possibly, other genetic diseases) among African Americans compared to West Africans. Another factor that limits the spread of sickle cell genes in North America is the relative absence of 595:

shock. Sequestration crises are considered an emergency. If not treated, patients may die within 1–2 hours due to circulatory failure. Management is supportive, sometimes with blood transfusion. These crises are transient; they continue for 3–4 hours and may last for one day.

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Since 2000, neonatal screening of SCD has been performed at the national level for all newborns defined as being "at-risk" for SCD based on ethnic origin (defined as those born to parents originating from sub-Saharan Africa, North Africa, the Mediterranean area (South Italy,

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A 1994 NIH study showed that children at risk for strokes who received blood transfusions had an annual stroke rate of less than 1 per cent, whereas those children who did not receive blood transfusions had a 10 per cent stroke rate per year. (Also see 1998 study in the

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Treatment involves a number of measures. While it has been historically recommended that people with sickle cell disease avoid exercise, regular exercise may benefit people. Dehydration should be avoided. A diet high in calcium is recommended but the effectiveness of

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The HbS gene can be found in every ethnic group. The highest frequency of sickle cell disease is found in tropical regions, particularly sub-Saharan Africa, tribal regions of India, and the Middle East. About 80% of sickle cell disease cases are believed to occur in

1619:. Migration of substantial populations from these high-prevalence areas to low-prevalence countries in Europe has dramatically increased in recent decades and in some European countries, sickle cell disease has now overtaken more familiar genetic conditions such as 1294:. During the first trimester of pregnancy, chorionic villus sampling (CVS) is also a technique used for SCD prenatal diagnosis. Since taking a blood sample from a fetus has greater risks, the latter test is usually used. Neonatal screening sometimes referred to as 974:(HbF), consisting of two alpha and two gamma chains in their bodies. Of these three types, haemoglobin F dominates until about 6 weeks of age. Afterwards, haemoglobin A dominates throughout life. In people diagnosed with sickle cell disease, at least one of the 1427:

hydroxyurea can reduce the risk of irreversible organ and brain damage. Guidelines from NIH published in 2014, state that all children and adolescents should take hydroxyurea, as should adults with serious complications or three or more pain crises in a year.

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The loss of red blood cell elasticity is central to the pathophysiology of sickle cell disease. Normal red blood cells are quite elastic and have a biconcave disc shape, which allows the cells to deform to pass through capillaries. In sickle cell disease, low

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work with families to discuss the benefits, limitations, and logistics of genetic testing options as well as the potential impact of testing and test results on the individual. During pregnancy, genetic testing can be done on either a blood sample from the

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The usual characteristics of the first event leading to a diagnosis of SCD depend on the age of the child. When the disease presents within the first year of life, the most common problem is an episode of pain and swelling in their hands and feet, known as

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older than 2 years, the most frequent initial presentation is a painful event of generalized or variable type, and a slightly less common version appears as an event of acute chest pain. Dactylitis rarely or never occurs in children older than 2 years.

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used for gene therapy. One trial focused on analyzing bone marrow samples from patients with sickle cell anemia. Another trial experimented with using umbilical cord blood from babies both with and without sickle cell anemia to develop gene therapy.

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saying this shows racial discrimination or the role of wealth in health care advocacy. Overall, without considering race, approximately 1.5% of infants born in the United States are carriers of at least one copy of the mutant (disease-causing) gene.

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It was the first approved drug for the treatment of sickle cell anaemia, and was shown to decrease the number and severity of attacks in 1995 and shown to possibly increase survival time in a study in 2003. This is achieved, in part, by reactivating

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cell disease and a 50% chance of having sickle cell trait. When both parents have the sickle cell trait, any given child has a 25% chance of sickle cell disease; a 25% chance of no sickle cell alleles, and a 50% chance of the heterozygous condition.

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This condition is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.

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or cell function may be restored in cells that have the disease by a normal copy of the gene that is mutated, may be a good candidate for gene therapy treatment. The risks and benefits related to gene therapy for sickle cell disease are not known.

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gene. A number of researchers have considered the ethical implications of SCD being one of the first potential applications of CRISPR technology, given the historical abuses and neglect of the African American community by the medical field.

1104:, more specifically 11p15.5. A person who receives the defective gene from both father and mother develops the disease; a person who receives one defective and one healthy allele remains healthy, but can pass on the disease and is known as a 734:

causes no immediate symptoms, but is associated with damage to the brain. Silent stroke is probably five times as common as symptomatic stroke. About 10–15% of children with SCD have strokes, with silent strokes predominating in the younger

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Not only does the abandonment experienced by these women cause emotional distress for them, but this low level of parental support can be linked to depressive symptoms and overall lower quality of life for the child once they are born.

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sent to a lab for testing. The baby must have been eating for a minimum of 24 hours before the heel-prick test can be done. Some states also require a second blood test to be done when the baby is two weeks old to ensure the results.

3924:"Global, regional, and national incidence, prevalence, and years lived with disability for 301 acute and chronic diseases and injuries in 188 countries, 1990–2013: a systematic analysis for the Global Burden of Disease Study 2013" 8052: 3190:

often face extreme discrimination and discouragement in Uganda. These women are frequently branded by their peers as irresponsible for having a baby while living with sickle cell disease or even engaging in sex while living with

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are often used in the management of sickle cell disease in acute cases and to prevent complications by decreasing the number of red blood cells (RBCs) that can sickle by adding normal red blood cells. In children, preventive RBC

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Most people with sickle cell disease have intensely painful episodes called vaso-occlusive crises. However, the frequency, severity, and duration of these crises vary tremendously. Painful crises are treated symptomatically with

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The malaria parasite has a complex lifecycle and spends part of it in red blood cells. In a carrier, the presence of the malaria parasite causes the red blood cells with defective haemoglobin to rupture prematurely, making the

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in 1922, the name "sickle cell anemia" is first used. Childhood problems related to sickle cells disease were not reported until the 1930s, despite the fact that this cannot have been uncommon in African-American populations.

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generally about the disease, a large portion of the population is relatively misinformed about how SCD is diagnosed or inherited. Those who are informed about the disease learned about it from family or friends and not from

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The terms "sickle cell crisis" or "sickling crisis" may be used to describe several independent acute conditions occurring in patients with SCD, which results in anaemia and crises that could be of many types, including the

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and sub-Saharan Africa to mainland France, sickle cell disease has become a major health problem in France. SCD has become the most common genetic disease in the country, with an overall birth prevalence of one in 2,415 in

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Management is similar to vaso-occlusive crisis, with the addition of antibiotics (usually a quinolone or macrolide, since cell wall-deficient bacteria are thought to contribute to the syndrome), oxygen supplementation for

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Colah, R. B., Gorakshakar, A. C., & Nadkarni, A. H. (2011). Invasive & non-invasive approaches for prenatal diagnosis of haemoglobinopathies: experiences from India. The Indian Journal of Medical Research, 134(4),

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for genetic diseases in an attempt to be protective towards people with SCD. By having this screening, it was intended that employees would not be placed in environments that could potentially be harmful and trigger SCD.

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Extra fluids, administered either orally or intravenously, are a routine part of treatment of vaso-occlusive crises but the evidence about the most effective route, amount and type of fluid replacement remains uncertain.

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Signs of sickle cell disease usually begin in early childhood. The severity of symptoms can vary from person to person, as can the frequency of crisis events. Sickle cell disease may lead to various acute and chronic

1557:. High quality, randomized, controlled trials are needed to assess the most effective treatment option and determine if a combination of physical therapy and surgery is more effective than physical therapy alone. 11164: 11149: 11118: 3063:

Large-scale natural history studies and further intervention studies were introduced in the 1970s and 1980s, leading to widespread use of prophylaxis against pneumococcal infections amongst other interventions.

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Mirre E, Brousse V, Berteloot L, Lambot-Juhan K, Verlhac S, Boulat C, et al. (March 2010). "Feasibility and efficacy of chronic transfusion for stroke prevention in children with sickle cell disease".

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Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, Hassell KL, James AH, et al. (September 2014). "Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members".

3044:, a prolific researcher into sickle cell disease, first introduced the distinction between sickle cell disease and trait in 1933, although until 1949, the genetic characteristics had not been elucidated by 4616:

Mekontso Dessap A, Leon R, Habibi A, Nzouakou R, Roudot-Thoraval F, Adnot S, et al. (March 2008). "Pulmonary hypertension and cor pulmonale during severe acute chest syndrome in sickle cell disease".

1017:(V/Val) at position 6 (E6V substitution). Haemoglobin S with this mutation is referred to as HbS, as opposed to the normal adult HbA. This is normally a benign mutation, causing no apparent effects on the 10402: 978:

subunits in haemoglobin A is replaced with what is known as haemoglobin S. In sickle cell anaemia, a common form of sickle cell disease, haemoglobin S replaces both β-globin subunits in the haemoglobin.

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Splenic sequestration crises are acute, painful enlargements of the spleen, caused by intrasplenic trapping of red cells and resulting in a precipitous fall in haemoglobin levels with the potential for

3642:"Global, regional, and national life expectancy, all-cause mortality, and cause-specific mortality for 249 causes of death, 1980–2015: a systematic analysis for the Global Burden of Disease Study 2015" 3195:

The criticism and judgement these women receive, not only from healthcare professionals but also from their families, often leaves them feeling alone, depressed, anxious, ashamed, and with very little

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has the fifth-highest sickle cell disease burden in Africa. One study indicates that 20 000 babies per year are born with sickle cell disease with the sickle cell trait at 13.3% and with disease 0.7%.

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Gene editing platforms like CRISPR/Cas9 have been used to correct the disease-causing mutation in hematopoietic stem cells taken from a person with the condition. In July 2019 the gene-editing tool

3074:, depicted the story of the parents of a child with sickle cell disease. The 1990s had the development of hydroxycarbamide, and reports of cure through bone marrow transplantation appeared in 2007. 648:), and the rapid turnover of red cells leads to the drop in haemoglobin. This crisis takes four to seven days to disappear. Most patients can be managed supportively; some need a blood transfusion. 8207:

National Academies of Sciences, Engineering, and Medicine, Health and Medicine Division, Board on Population Health and Public Health Practice, Committee on Addressing Sickle Cell Disease (2020).

3588:"Global, regional, and national incidence, prevalence, and years lived with disability for 310 diseases and injuries, 1990–2015: a systematic analysis for the Global Burden of Disease Study 2015" 283:-like shape under certain circumstances. Problems in sickle cell disease typically begin around 5 to 6 months of age. A number of health problems may develop, such as attacks of pain (known as a 1201:

attempts to compensate by creating new red cells, it does not match the rate of destruction. Healthy red blood cells typically function for 90–120 days, but sickled cells only last 10–20 days.

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Green NS, Fabry ME, Kaptue-Noche L, Nagel RL (October 1993). "Senegal haplotype is associated with higher HbF than Benin and Cameroon haplotypes in African children with sickle cell anemia".

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and 1 in 375 are born with the disease. Patient advocates for sickle cell disease have complained that it gets less government and private research funding than similar rare diseases such as

6523:"BestBets: Does routine urinalysis and chest radiography detect occult bacterial infection in sickle cell patients presenting to the accident and emergency department with painful crisis?" 1240:. The presence of sickle haemoglobin can also be demonstrated with the "sickle solubility test" (also called "sickledex"). A mixture of haemoglobin S (HbS) in a reducing solution (such as 6828: 5817:

Powars DR, Elliott-Mills DD, Chan L, Niland J, Hiti AL, Opas LM, et al. (October 1991). "Chronic renal failure in sickle cell disease: risk factors, clinical course, and mortality".

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Thuret I, Sarles J, Merono F, Suzineau E, Collomb J, Lena-Russo D, et al. (June 2010). "Neonatal screening for sickle cell disease in France: evaluation of the selective process".

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Wong WY, Powars DR, Chan L, Hiti A, Johnson C, Overturf G (March 1992). "Polysaccharide encapsulated bacterial infection in sickle cell anemia: a thirty year epidemiologic experience".

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is frequently affected in sickle cell disease, as the sickle-shaped red blood cells cause narrowing of blood vessels and reduced function in clearing the defective cells. It is usually

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Balgir RS (March 2012). "Community expansion and gene geography of sickle cell trait and G6PD deficiency, and natural selection against malaria: experience from tribal land of India".

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Mothers of children with sickle cell disease tend to receive disproportionate amounts of stigma from their peers and family members. These women will often be blamed for their child's

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Richard-Lenoble D, Toublanc JE, Zinsou RD, Kombila M, Carme B (1980). "" [Results of a systematic study of drepanocytosis in 1,500 Gabonese using hemoglobin electrophoresis].

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devices are commonly used in this setting. Vaso-occlusive crisis involving organs such as the penis or lungs are considered an emergency and treated with red blood cell transfusions.

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Aidoo M, Terlouw DJ, Kolczak MS, McElroy PD, ter Kuile FO, Kariuki S, et al. (April 2002). "Protective effects of the sickle cell gene against malaria morbidity and mortality".

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As of 2015, about 4.4 million people have sickle cell disease, while an additional 43 million have sickle cell trait. About 80% of sickle cell disease cases are believed to occur in

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There is no strong medical evidence to determine the risks and potential benefits related to treating people with sickle cell disease with hematopoietic stem cell transplantations.

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count (as the bone marrow compensates for the destruction of sickled cells by producing more red blood cells). In other forms of sickle cell disease, Hb levels tend to be higher. A

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Most pregnant women with SCD also go on to be single mothers as it is common for them to be left by their male partners who claim they were unaware of their partner's SCD status.

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prophylaxis is the most commonly used treatment during childhood, with some haematologists continuing treatment indefinitely. Patients benefit today from routine vaccination for

401:(sub-Saharan) living in other parts of the world. In 2015, it resulted in about 114,800 deaths. The condition was first described in the medical literature by American physician 10509:

Pawliuk R, Westerman KA, Fabry ME, Payen E, Tighe R, Bouhassira EE, et al. (December 2001). "Correction of sickle cell disease in transgenic mouse models by gene therapy".

6939: 1170: 3052:

described the unusual chemical behaviour of haemoglobin S, and attributed this to an abnormality in the molecule itself. The molecular change in HbS was described in 1956 by

822:

In eyes, background retinopathy, proliferative retinopathy, vitreous haemorrhages, and retinal detachments can result in blindness. Regular annual eye checks are recommended.

7879:... teams report that two strategies for directly fixing malfunctioning blood cells have dramatically improved the health of a handful of people with these genetic diseases. 6627: 499:

that appears when a large number of red cells undergo hemolysis. Most episodes of sickle cell crises last between five and seven days. "Although infection, dehydration, and

8710:

Edwards QT, Seibert D, Macri C, Covington C, Tilghman J (November 2004). "Assessing ethnicity in preconception counseling: genetics—what nurse practitioners need to know".

6858:

Martin C, Pialoux V, Faes C, Charrin E, Skinner S, Connes P (February 2018). "Does physical activity increase or decrease the risk of sickle cell disease complications?".

8753: 3231:

aim was to set up a new training programme to help paramedics, Accident and Emergency staff, carers and the general public to care effectively for sufferers in crisis.

10356: 6736:

Acharya K, Benjamin HJ, Clayton EW, Ross LF (November 2011). "Attitudes and beliefs of sports medicine providers to sickle cell trait screening of student athletes".

9035: 8089:

Wierenga KJ, Hambleton IR, Lewis NA (March 2001). "Survival estimates for patients with homozygous sickle-cell disease in Jamaica: a clinic-based population study".

1111:

Due to the adaptive advantage of the heterozygote, the disease is still prevalent, especially among people with recent ancestry in malaria-stricken areas, such as

6901: 6573: 5523:

Smith WR, Penberthy LT, Bovbjerg VE, McClish DK, Roberts JD, Dahman B, et al. (January 2008). "Daily assessment of pain in adults with sickle cell disease".

1509:

shows abnormal cerebral blood flow. In those who have sustained a prior stroke event, it also reduces the risk of recurrent stroke and additional silent strokes.

1141:

In the United States, with no endemic malaria, the prevalence of sickle cell anaemia among people of African ancestry is lower (about 0.25%) than among people in

861:. 21% of children and 30% of adults have evidence of pulmonary hypertension when tested; this is associated with reduced walking distance and increased mortality. 3028:

with the same title, "Peculiar Elongated and Sickle-Shaped Red Blood Corpuscles in a Case of Severe Anemia." This article is based on a patient admitted to the

1379:; pain management requires opioid drug administration at regular intervals until the crisis has settled. For milder crises, a subgroup of patients manages on 1041:

and forms fibrous precipitates because the deoxy form of haemoglobin exposes a hydrophobic patch on the protein between the E and F helices (Phe 85, Leu 88).

7529:"Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia" 3586:

Allen C, Arora M, Barber RM, Bhutta ZA, Brown A, Carter A, et al. (GBD 2015 Disease and Injury Incidence and Prevalence Collaborators) (October 2016).

1145:(about 4.0%) and is falling. Without endemic malaria, the sickle cell mutation is purely disadvantageous and tends to decline in the affected population by 3820: 1480:

agent, and some concern exists that long-term use may be harmful, but this risk is either absent or very small and the benefits likely outweigh the risks.

9269:

Awasthy N, Aggarwal KC, Goyal PC, Prasad MS, Saluja S, Sharma M (2008). "Sickle cell disease: Experience of a tertiary care center in a nonendemic area".

7862: 1127:. Malaria was historically endemic to southern Europe, but it was declared eradicated in the mid-20th century, with the exception of rare sporadic cases. 8046: 5964: 2850: 659:

are acute accelerated drops in haemoglobin level. The red blood cells break down at a faster rate. This is particularly common in people with coexistent

9174: 12698: 2862: 8977: 7019: 4228:"Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease [see comments]" 3248:

can potentially cure the condition, a suitable donor is available in only 10% of people. About 7% of people also die as a result of the procedure and

63:

Figure (A) shows normal red blood cells flowing freely through a blood vessel. The inset shows a cross-section of a normal red blood cell with normal

7259: 7349:

Aldrich TK, Nagel RL (1998). "Pulmonary Complications of Sickle Cell Disease.". In Reynolds HY, Bone RC, Dantzker DR, George RB, Matthay RA (eds.).

9318: 9300: 3922:

Vos T, Barber RM, Bell B, Bertozzi-Villa A, Biryukov S, Bolliger I, et al. (Global Burden of Disease Study 2013 Collaborators) (August 2015).

1345:

review of its use found "the effect of supplementation on anaemia and any symptoms of anaemia remains unclear" due to a lack of medical evidence.

8955: 3795: 1537:

When treating avascular necrosis of the bone in people with sickle cell disease, the aim of treatment is to reduce or stop the pain and maintain

511:

The vaso-occlusive crisis is caused by sickle-shaped red blood cells that obstruct capillaries and restrict blood flow to an organ, resulting in

9110:"Six-year outcome of the national premarital screening and genetic counseling program for sickle cell disease and β-thalassemia in Saudi Arabia" 6836: 3561: 3491: 8559: 7286:"Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography" 3640:

Wang H, Naghavi M, Allen C, Barber RM, Bhutta ZA, Carter A, et al. (GBD 2015 Mortality and Causes of Death Collaborators) (October 2016).

3306:

gene editing tool was approved by UK regulators for the treatment of sickle cell disease and also for the blood disorder transfusion-dependent

2871: 1521:

to have to same blood type as the patient. Some gene therapies are under development that would alter the patient's own bone marrow stem cells

8663: 6654:"Framing the research agenda for sickle cell trait: building on the current understanding of clinical events and their potential implications" 523:, and often organ damage. The frequency, severity, and duration of these crises vary considerably. Painful crises are treated with hydration, 9271: 5934: 3456: 3056:. The late 1940s and early 1950s saw further understanding in the link between malaria and sickle cell disease. In 1954, the introduction of 1667:

decrease in infant mortality rate, ages 2–16 months, because of the sickle cell trait. This happened in areas of predominant malarial cases.

1337:

daily, due to the immature immune system that makes them more prone to early childhood illnesses, is recommended. Dietary supplementation of

10591: 7653: 5133: 3376: 2963:

Sickle cell disease is common in some ethnic groups of central India, where the prevalence has ranged from 9.4 to 22.2% in endemic areas of

11451: 8384: 11656: 8855:"Prevalence and cost of sickle cell disease in France: real-world analysis using data from the Echantillon Généraliste des Bénéficiaires" 3201:

Burden of sickle cell trait and disease in the Uganda Sickle Surveillance Study (US3): a cross-sectional study - The Lancet Global Health

1357:

Possible advantage of being heterozygous for sickle cell anemia disease (A) vs. normal blood cell response (B) when infected with malaria

9247: 5357:

Chrouser KL, Ajiboye OB, Oyetunji TA, Chang DC (April 2011). "Priapism in the United States: the changing role of sickle cell disease".

4791:"Human parvovirus B19: general considerations and impact on patients with sickle-cell disease and thalassemia and on blood transfusions" 12126: 11807: 11309: 8365: 7991:"Effectiveness of a home-based therapeutic exercise program on lower back pain and functionality in Sickle Cell Disease (SCD) patients" 3421: 6701:

Bonham VL, Dover GJ, Brody LC (September 2010). "Screening student athletes for sickle cell trait--a social and clinical experiment".

5774:

Rai P, Niss O, Malik P (November 2017). "A reappraisal of the mechanisms underlying the cardiac complications of sickle cell anemia".

1606:

In 1975, about 7.3% of people with SCD died before their 23rd birthday; while in 1989, 2.6% of people with SCD died by the age of 20.

12467: 11759: 11581: 10044:"Knowledge, perception and practices towards sickle cell disease: a community survey among adults in Lubaga division, Kampala Uganda" 9540: 8464:"Knowledge, perception and practices towards sickle cell disease: a community survey among adults in Lubaga division, Kampala Uganda" 8078:(Professional Edition: Expert Consult – Online (Robbins Pathology) ed.). Elsevier Health. pp. Kindle Locations 33530–33531. 4321:(Professional Edition: Expert Consult – Online (Robbins Pathology) ed.). Elsevier Health. pp. Kindle Locations 33498–33499. 1499: 874:

caused by fibrosis or scarring of cardiac tissues. This also contributes to pulmonary hypertension, decreased exercise capacity, and

9672:

Ingram VM (October 1956). "A specific chemical difference between the globins of normal human and sickle-cell anaemia haemoglobin".

9021: 11651: 9990:"To Join Or Not To Join? A Case Of Sickle Cell Clubs, Stigma And Discrimination In Secondary Schools In Butambala District, Uganda" 6367: 3791: 10853:"A CRISPR focus on attitudes and beliefs toward somatic genome editing from stakeholders within the sickle cell disease community" 10183:"'All her children are born that way': gendered experiences of stigma in families affected by sickle cell disorder in rural Kenya" 12066: 11591: 5447: 4491: 3520: 695: 462:

or "hand-foot syndrome." Pallor, jaundice, and fatigue can also be early signs due to anemia resulting from sickle cell disease.

371: 6508: 12688: 6341: 1260: 1176:

showing a mixture of red blood cells, some with round normal morphology, some with mild sickling showing elongation and bending

405:

in 1910. In 1949, its genetic transmission was determined by E. A. Beet and J. V. Neel. In 1954, the protective effect against

8611: 4752:(Professional Edition: Expert Consult – Online (Robbins Pathology) ed.). Elsevier Health. pp. Kindle Location 33329. 12708: 11085: 11062: 11041: 11020: 9949: 8218: 7570:"Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment" 7358: 6461: 6434: 6408: 5080: 4722: 7944:"Physical Therapy in Pediatric and Young Adult Patients With Sickle Cell Disease: Assessing Potential Benefits and Barriers" 535:

drug administration at regular intervals until the crisis has settled. For milder crises, a subgroup of patients manages on

12366: 11468: 10385: 9165: 7729:"Effect of chronic red cell transfusion therapy on vasculopathies and silent infarcts in patients with sickle cell disease" 4705:

Sysol JR, Machado R (2016). "Sickle Cell Disease and Acute Chest Syndrome: Epidemiology, Diagnosis, Management, Outcomes".

2902: 810:

and Gram-negative enteric bacilli perhaps because intravascular sickling of the bowel leads to patchy ischaemic infarction.

536: 10328: 9942:

Medical apartheid : the dark history of medical experimentation on Black Americans from colonial times to the present

9043: 8685: 11623: 11179: 7041: 5986: 694:

Increased risk of severe bacterial infections is due to loss of functioning spleen tissue (and comparable to the risk of

8168:"Sickle cell disease in the United States: looking back and forward at 100 years of progress in management and survival" 8042:"MHRA authorises world-first gene therapy that aims to cure sickle-cell disease and transfusion-dependent β-thalassemia" 4974: 1068:. Carriers have symptoms only if they are deprived of oxygen (for example, while climbing a mountain) or while severely 12502: 11844: 6909: 6581: 3094:

issued a Policy Interpretation Ruling providing background information on sickle cell disease and a description of how

9456:

Herrick JB (1 November 1910). "Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia".

6526: 4295: 3200: 3000:

abnormal characteristics of the red blood cells, which later lent their name to the condition, was first described by

11632: 11603: 8143: 6313: 4032: 3012:" and "bilious attacks" but completed his studies and returned to the capital of Grenada (St. George's) to practice 2979:

of Nepal and India; however, they have a sevenfold lower rate of malaria despite living in a malaria infested zone.

1476:

production in place of the haemoglobin S that causes sickle cell anaemia. Hydroxyurea had previously been used as a

1026: 11663: 11535: 4548: 1018: 5442: 4053:

Savitt TL, Goldberg MF (January 1989). "Herrick's 1910 case report of sickle cell anemia. The rest of the story".

2991:, 10% of the population carry the sickle cell gene, making it the most prevalent genetic disorder in the country. 1646:; humans with one of the two alleles of sickle cell disease show less severe symptoms when infected with malaria. 1525:

which can then be transplanted back into the patient after chemotherapy eliminates the original unmodified cells.

1259:

with sickling (HgbSC)—the two most common forms—can be identified from there. The diagnosis can be confirmed with

1022: 12693: 10978: 10133:"Knowledge gaps, attitude and beliefs of the communities about sickle cell disease in Eastern and Western Uganda" 8999: 5488:

Elagouz M, Jyothi S, Gupta B, Sivaprasad S (July 2010). "Sickle cell disease and the eye: old and new concepts".

3245: 3095: 1108:

or heterozygote. Heterozygotes are still able to contract malaria, but their symptoms are generally less severe.

826: 8515:"Burden of sickle cell trait and disease in the Uganda Sickle Surveillance Study (US3): a cross-sectional study" 7680:"Blood transfusion usage among adults with sickle cell disease – a single institution experience over ten years" 1581:, that aim to complement current medical treatments, require further research to determine their effectiveness. 1406:

was approved in the United States to reduce the frequency of vaso-occlusive crisis in those 16 years and older.

12620: 12492: 11926: 11596: 11302: 9416: 7167:

Carroll CP (January 2020). "Opioid treatment for acute and chronic pain in patients with sickle cell disease".

3091: 1002: 11617: 11525: 8369: 5956: 3193:

Burden of sickle cell trait and disease in the Uganda Sickle Surveillance Study (US3): a cross-sectional study

1277:

People who are known carriers of the disease or at risk of having a child with sickle cell anemia may undergo

11690: 11642: 10361: 9160: 5629:"Estimated pulmonary artery systolic pressure and sickle cell disease: a meta-analysis and systematic review" 5474: 2906:

Percentage of newborns screened regionally and overall for sickle cell disease in Metropolitan France in 2018

417: 327:, and high altitude. A person with a single abnormal copy does not usually have symptoms and is said to have 10997: 9517:

Washburn RE (1911). "Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia".

7329: 3205:

Unprepared and Misinformed Parents of Children with Sickle Cell Disease: Time to Rethink Awareness Campaigns

503:(all of which favor sickling) can act as triggers, in most instances, no predisposing cause is identified." 339:, and some countries test all babies at birth for the disease. Diagnosis is also possible during pregnancy. 12683: 12472: 11547: 8969: 7011: 4380:

Glassberg J (August 2011). "Evidence-based management of sickle cell disease in the emergency department".

3826: 1638:

sub-Saharan regions where malaria is or was common. Where malaria is common, carrying a single sickle cell

1173: 1060:

for HbS, the presence of long-chain polymers of HbS distort the shape of the red blood cell from a smooth,

839:

Chronic pain: Even in the absence of acute vaso-occlusive pain, many patients have unreported chronic pain.

548: 488: 10699: 12116: 11699: 11573: 11437: 11392: 8277:"Sickle cell disease as a paradigm of immigration hematology: new challenges for hematologists in Europe" 3736: 1578: 1311: 1248: 17: 11275: 4662:

Paul RN, Castro OL, Aggarwal A, Oneal PA (September 2011). "Acute chest syndrome: sickle cell disease".

1353: 12653: 12096: 11637: 11463: 3070: 3057: 1594: 9314: 8947: 3787: 3761: 3192: 12703: 12525: 11564: 11554: 11542: 11295: 8459: 3553: 3483: 3249: 2841:

Sickle cell anemia is the most common genetic disorder among African Americans. Approximately 8% are

1659: 1274:

urinary tract infection, and chest X-ray to look for occult pneumonia should be routinely performed.

1270:

An acute sickle cell crisis is often precipitated by infection. Therefore, a urinalysis to detect an

1255:

on which the various types of haemoglobin move at varying speeds. Sickle cell haemoglobin (HgbS) and

8633: 8555: 7057:

twice-daily prophylactic penicillin beginning in early infancy and continuing through at least age 5

5537: 2866:

Percentage of newborns screened for sickle cell disease within Metropolitan France from 2006 to 2018

724:, which can result from a progressive narrowing of blood vessels, prevents oxygen from reaching the 12317: 12239: 12234: 12154: 11914: 11530: 11190: 10649:

Ribeil JA, Hacein-Bey-Abina S, Payen E, Magnani A, Semeraro M, Magrin E, et al. (March 2017).

10279:"Depression and quality of life in children with sickle cell disease: the effect of social support" 8659: 6426:

Revolutionizing Tropical Medicine: Point-of-Care Tests, New Imaging Technologies and Digital Health

4631: 3090:

Sickle cell disease is frequently contested as a disability. Effective 15 September 2017, the U.S.

1590: 813: 700: 10636:

for "Collection and Storage of Umbilical Cord Stem Cells for Treatment of Sickle Cell Disease" at

9841: 9819: 7918:"Unravelito the Quest: Is There a Cure for Sickle Cell Anemia? Introduction to Sickle Cell Anemia" 7917: 7812:

Walters MC, Patience M, Leisenring W, Eckman JR, Scott JP, Mentzer WC, et al. (August 1996).

5926: 4149: 3448: 12297: 12271: 11735: 11586: 10726:

Dever DP, Bak RO, Reinisch A, Camarena J, Washington G, Nicolas CE, et al. (November 2016).

10587: 8853:

Beillat M, Durand-Zaleski I, Pirenne F, Bénard S, Chillotti L, Galacteros F (21 September 2023).

6055: 5727:"Association between diffuse myocardial fibrosis and diastolic dysfunction in sickle cell anemia" 5097: 4573:

Khatib R, Rabah R, Sarnaik SA (January 2009). "The spleen in the sickling disorders: an update".

3368: 3227: 1342: 886: 446: 113: 4265: 4227: 11889: 11837: 11726: 11387: 10333: 8513:

Ndeezi G, Kiyaga C, Hernandez AG, Munube D, Howard TA, Ssewanyana I, et al. (March 2016).

6210:

Ponçon N, Toty C, L'Ambert G, Le Goff G, Brengues C, Schaffner F, et al. (February 2007).

5532: 4626: 4243: 4022: 3275: 3222: 3029: 1643: 1517: 1391:). For more severe crises, most patients require inpatient management for intravenous opioids. 1064:-like shape to ragged and full of spikes, making it fragile and susceptible to breaking within 842: 706: 580: 142: 11230: 11131: 11127: 11012:

Comprehensive handbook of childhood cancer and sickle cell disease: a biopsychosocial approach

9866: 9586:

Mason VR (October 1985). "Landmark article Oct. 14, 1922: Sickle cell anemia. By V.R. Mason".

6424: 6163:"How malaria has affected the human genome and what human genetics can teach us about malaria" 5901: 547:. For more severe crises, most patients require inpatient management for intravenous opioids; 432:

Sickle cells in human blood - both normal red blood cells and sickle-shaped cells are present.

12540: 12426: 12399: 12372: 12324: 12278: 11973: 11706: 11473: 11143: 11139: 9294: 9239: 8133: 7568:

Steinberg MH, Barton F, Castro O, Pegelow CH, Ballas SK, Kutlar A, et al. (April 2003).

5468: 2952: 1486:

was approved in the United States in 2019, to increase hemoglobin in people with SS disease.

871: 806: 757: 480: 126: 11135: 9478:"Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia. 1910" 8780: 8391: 5546: 4226:

Gill FM, Sleeper LA, Weiner SJ, Brown AK, Bellevue R, Grover R, et al. (15 July 1995).

12560: 12520: 12387: 12201: 12196: 12176: 12111: 11963: 11716: 11711: 11519: 11405: 10739: 10518: 9681: 9630: 9345: 7678:

Drasar E, Igbineweka N, Vasavda N, Free M, Awogbade M, Allman M, et al. (March 2011).

6612:

Lee, C., Davies, S.,& Dezatoux, C. (2000). Neonatal Screening for sickle cell disease.

5098:"Management of children with sickle cell disease: a comprehensive review of the literature" 3413: 3024:

in the north of Grenada. Shortly after the report by Herrick, another case appeared in the

1503: 1449: 1237: 1210: 1161:. In polygamous societies, affected males may father many children with multiple partners. 679: 608: 604: 552: 440:

Normal blood cells next to a sickle blood cell, coloured scanning electron microscope image

9536: 3869:"UK regulator approves "groundbreaking" gene treatment for sickle cell and β thalassaemia" 857:; typical symptoms are shortness of breath, decreased exercise tolerance, and episodes of 8: 12640: 12582: 12565: 12224: 11941: 11765: 11318: 11168: 10421:"Hematopoietic stem cell transplantation for sickle cell disease: The changing landscape" 9469: 9013: 7425:"Blood transfusions for treating acute chest syndrome in people with sickle cell disease" 7212:"Fluid replacement therapy for acute episodes of pain in people with sickle cell disease" 3303: 3283: 3129: 3009: 2892: 2880: 1363: 1325:

use was supported by the FDA starting at the age of five, as it decreases complications.

1252: 983: 919: 466: 292: 102: 10743: 10522: 9685: 9634: 9349: 8881: 8854: 6781:"Sickle Cell Trait Screening of Collegiate Athletes: Ethical Reasons for Program Reform" 6363: 4479: 3204: 12530: 12487: 12477: 12266: 12219: 12186: 12181: 11931: 11909: 11797: 11770: 11375: 10992: 10952: 10927: 10877: 10852: 10833: 10760: 10727: 10704: 10680: 10637: 10619: 10542: 10486: 10461: 10305: 10207: 10182: 10070: 10016: 9917: 9892: 9705: 9654: 9494: 9477: 9368: 9216: 9136: 9085: 8929: 8735: 8723: 8490: 8441: 8342: 8317: 8252: 8235: 8114: 8015: 7990: 7989:

Zanoni CT, Galvão F, Maria RM, Zulli R, Ricciardi JB, Cliquet a J, et al. (2020).

7843: 7794: 7709: 7634: 7501: 7476: 7449: 7424: 7400: 7375: 7334: 7236: 7211: 7192: 7144: 7119: 7095: 7070: 6987: 6962: 6883: 6810: 6761: 6678: 6653: 6286: 6238: 6187: 6162: 6103: 6078: 6036: 5878: 5853: 5799: 5751: 5726: 5702: 5677: 5658: 5604: 5558: 5423: 5334: 5309: 5285: 5260: 5125: 5019: 4922: 4905: 4733: 4687: 4598: 4530: 4436: 4411: 4203: 4003: 3948: 3923: 3904: 3666: 3641: 3612: 3587: 3524: 1616: 1282: 1278: 858: 378: 88: 11173: 10700:"These Patients Had Sickle-Cell Disease. Experimental Therapies Might Have Cured Them" 10561: 10230: 8531: 8514: 8429: 8102: 5501: 4460: 3991: 3939: 3657: 3603: 656: 492: 12626: 12600: 12482: 12452: 12431: 12394: 12377: 12292: 12256: 12246: 12141: 12061: 12005: 11968: 11953: 11894: 11861: 11830: 11802: 11792: 11751: 11746: 11741: 11682: 11514: 11503: 11493: 11427: 11410: 11401: 11383: 11345: 11201: 11081: 11074: 11058: 11037: 11016: 10957: 10882: 10837: 10825: 10809: 10765: 10672: 10534: 10491: 10442: 10310: 10212: 10154: 10075: 10021: 9955: 9945: 9922: 9801: 9762: 9697: 9646: 9603: 9579: 9499: 9438: 9373: 9221: 9141: 9090: 8921: 8886: 8835: 8808: 8800: 8727: 8536: 8495: 8433: 8347: 8298: 8257: 8214: 8189: 8139: 8106: 8020: 7971: 7963: 7835: 7786: 7782: 7750: 7701: 7696: 7679: 7626: 7591: 7550: 7506: 7454: 7405: 7354: 7307: 7284:

Adams RJ, McKie VC, Hsu L, Files B, Vichinsky E, Pegelow C, et al. (July 1998).

7241: 7196: 7184: 7149: 7100: 6992: 6902:"Keeping Well with Sickle Cell Disease - Brent Sickle Cell & Thalassaemia Centre" 6875: 6802: 6753: 6718: 6683: 6500: 6478: 6457: 6430: 6404: 6319: 6309: 6278: 6243: 6192: 6143: 6108: 6028: 5883: 5834: 5791: 5756: 5707: 5650: 5609: 5550: 5505: 5415: 5410: 5393: 5374: 5339: 5290: 5211: 5170: 5117: 5076: 5046: 5023: 5011: 4966: 4962: 4927: 4886: 4847: 4812: 4807: 4790: 4718: 4679: 4675: 4644: 4590: 4522: 4483: 4441: 4389: 4355: 4247: 4195: 4128: 4123: 4106: 4070: 4028: 3995: 3953: 3908: 3896: 3888: 3718: 3710: 3671: 3617: 3287: 3179: 1574: 1570: 1566: 1550: 1494: 1473: 1445: 1438: 1295: 1241: 1230: 1182: 1146: 850: 645: 528: 484: 359: 328: 299:. Long-term pain may develop as people get older. The average life expectancy in the 208: 177: 72: 10546: 9658: 8933: 8607: 8445: 8118: 7863:"CRISPR and another genetic strategy fix cell defects in two common blood disorders" 7847: 7798: 7713: 6887: 6814: 6765: 6479:"Laboratory investigation of hemoglobinopathies and thalassemias: review and update" 6337: 6290: 6040: 5803: 5662: 5562: 5229: 5129: 4691: 4534: 4207: 4007: 3334:

Historic numbering put this glutamic acid residue at position 6 due to skipping the

3268:

In 2001, sickle cell disease reportedly had been successfully treated in mice using

2919:), the Arabic peninsula, the French overseas islands, and the Indian subcontinent). 1236:

Sickling of the red blood cells, on a blood film, can be induced by the addition of

946: 12610: 12302: 12287: 12164: 12039: 11995: 11498: 11483: 11422: 11241: 10947: 10943: 10939: 10872: 10864: 10817: 10755: 10747: 10684: 10662: 10526: 10481: 10477: 10473: 10432: 10300: 10290: 10202: 10194: 10144: 10065: 10055: 10011: 10001: 9912: 9904: 9793: 9709: 9689: 9638: 9595: 9575: 9489: 9465: 9428: 9363: 9353: 9280: 9211: 9201: 9131: 9121: 9080: 9070: 8913: 8876: 8866: 8792: 8739: 8719: 8526: 8485: 8475: 8425: 8337: 8329: 8288: 8247: 8179: 8098: 8010: 8002: 7955: 7825: 7778: 7740: 7691: 7618: 7581: 7540: 7496: 7492: 7488: 7444: 7440: 7436: 7395: 7391: 7387: 7297: 7231: 7227: 7223: 7176: 7139: 7135: 7131: 7090: 7086: 7082: 6982: 6978: 6974: 6867: 6792: 6745: 6710: 6673: 6665: 6652:

Goldsmith JC, Bonham VL, Joiner CH, Kato GJ, Noonan AS, Steinberg MH (March 2012).

6548: 6490: 6394: 6270: 6233: 6223: 6182: 6174: 6135: 6098: 6090: 6020: 5873: 5865: 5826: 5783: 5746: 5738: 5697: 5689: 5640: 5599: 5589: 5542: 5497: 5405: 5366: 5329: 5321: 5280: 5276: 5272: 5201: 5165: 5160: 5148: 5109: 5001: 4958: 4917: 4878: 4839: 4802: 4728: 4710: 4671: 4636: 4602: 4582: 4514: 4475: 4431: 4427: 4423: 4345: 4239: 4187: 4118: 4062: 3987: 3943: 3935: 3880: 3702: 3661: 3653: 3607: 3599: 3307: 3159: 3005: 1465: 1444:

Should the pulmonary infiltrate worsen or the oxygen requirements increase, simple

1085: 1052:

of sickling haemoglobin), the polymerisation problems are minor because the normal

971: 868: 846: 402: 398: 363: 323:

in each haemoglobin gene. An attack can be set off by temperature changes, stress,

257: 81: 10784:"In A 1st, Doctors In U.S. Use CRISPR Tool To Treat Patient With Genetic Disorder" 10632: 10614: 10131:

Okwi AL, Byarugaba W, Ndugwa CM, Parkes A, Ocaido M, Tumwine JK (September 2009).

10042:

Tusuubira SK, Nakayinga R, Mwambi B, Odda J, Kiconco S, Komuhangi A (April 2018).

9908: 8581: 7638: 7376:"Antibiotics for treating acute chest syndrome in people with sickle cell disease" 7069:

Dixit R, Nettem S, Madan SS, Soe HH, Abas AB, Vance LD, et al. (March 2018).

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is rarely performed, as other investigations are highly specific for HbS and HbC.

342:

The care of people with sickle cell disease may include infection prevention with

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programs in order to reduce the stigmatization of sickle cell disease in Uganda.

3107: 3001: 2896: 2884: 2846: 2842: 1624: 1506: 1264: 1150: 1105: 1049: 660: 623:

are instances of an acute worsening of the patient's baseline anaemia, producing

620: 576: 386: 355: 332: 300: 220: 204: 146: 11253: 7830: 7813: 7545: 7528: 7302: 7285: 5869: 5830: 5206: 5189: 5038: 4714: 1045: 12462: 12416: 12229: 12159: 11978: 11694: 11322: 11250: 11247: 11206: 10821: 9621:

Pauling L, Itano HA (November 1949). "Sickle cell anemia a molecular disease".

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Sickle cell disease occurs more commonly among people whose ancestors lived in

1291: 1154: 1038: 902: 882: 864: 738: 636: 632: 276: 260: 11287: 11264: 11244: 10868: 10295: 10278: 10060: 10043: 9334:"Excess risk of maternal death from sickle cell disease in Jamaica: 1998–2007" 8480: 8463: 7609:

Platt OS (March 2008). "Hydroxyurea for the treatment of sickle cell anemia".

6797: 6780: 6628:"Genetic Counselors Of Color Tackle Racial, Ethnic Disparities In Health Care" 6274: 6139: 5310:"Platelet hyperactivity in sickle-cell disease: a consequence of hyposplenism" 4586: 3821:"FDA Approves First Gene Therapies to Treat Patients with Sickle Cell Disease" 3706: 3691:"Dizziness, Falls, and Hearing Loss in Adults Living With Sickle Cell Disease" 1057: 12677: 12545: 12334: 12329: 12191: 12131: 12034: 10928:"Hematopoietic stem cell transplantation for people with sickle cell disease" 10149: 10132: 9959: 9599: 8804: 7967: 7586: 7569: 6323: 6126:

Allison AC (October 2009). "Genetic control of resistance to human malaria".

5261:"Treatment for avascular necrosis of bone in people with sickle cell disease" 4946: 4866: 4251: 3892: 3851: 3714: 3218: 3124: 3119:

Uganda has the 5th highest sickle cell disease (SCD) burden in the world. In

3102: 3098:

evaluates the disease during its adjudication process for disability claims.

3053: 3049: 3045: 2829: 1554: 1546: 1542: 1399: 1256: 1116: 1101: 1089: 967: 854: 834: 790:(bacterial bone infection), the most common cause of osteomyelitis in SCD is 787: 764: 742: 731: 316: 307:

Sickle cell disease occurs when a person inherits two abnormal copies of the

11158: 10530: 9285: 9206: 9189: 9126: 9109: 9075: 9058: 8917: 8796: 7943: 4191: 3868: 3690: 3221:, a drug given via transfusion drips, which reduces the number of visits to 728:. Cerebral infarction occurs in children and cerebral haemorrhage in adults. 12550: 12535: 12361: 12086: 11936: 11921: 11867: 11337: 10961: 10886: 10829: 10769: 10676: 10538: 10495: 10446: 10314: 10216: 10158: 10079: 10025: 9926: 9805: 9701: 9650: 9503: 9442: 9377: 9225: 9145: 9094: 8925: 8890: 8839: 8812: 8754:"Sickle Cell Patients Endure Discrimination, Poor Care And Shortened Lives" 8731: 8540: 8499: 8437: 8351: 8302: 8293: 8276: 8261: 8193: 8110: 8024: 7975: 7790: 7754: 7705: 7630: 7595: 7510: 7458: 7409: 7245: 7188: 7153: 7104: 6996: 6879: 6806: 6757: 6722: 6687: 6504: 6389:

Kruzliak P (19 July 2012), "Hematologic manifestations of celiac disease",

6247: 6228: 6211: 6196: 6147: 6112: 6024: 5887: 5795: 5760: 5711: 5654: 5613: 5554: 5509: 5419: 5378: 5294: 5215: 5174: 5121: 5113: 5050: 5015: 4890: 4851: 4816: 4683: 4648: 4594: 4518: 4445: 4393: 4359: 4199: 4132: 4066: 3999: 3957: 3900: 3722: 3675: 3621: 3269: 3041: 2976: 2972: 2937: 2875: 1477: 1424: 1222: 1214: 963: 926: 890: 768: 698:). These infections are typically caused by encapsulated organisms such as 641: 394: 367: 169: 118: 10667: 10650: 10255: 9797: 9766: 9607: 7839: 7622: 7554: 7330:"These Sisters With Sickle Cell Had Devastating, and Preventable, Strokes" 7311: 7012:"Press Announcements – FDA approves new treatment for sickle cell disease" 6961:

Soe HH, Abas AB, Than NN, Ni H, Singh J, Said AR, et al. (May 2020).

6282: 6032: 5838: 5343: 4970: 4931: 4526: 4074: 607:

is defined by at least two of these signs or symptoms: chest pain, fever,

575:

before the end of childhood in individuals with sickle cell anaemia. This

12409: 12354: 12251: 12106: 12081: 11899: 11488: 11446: 11259: 11225: 10585: 10006: 9989: 9867:"Sickle Cell Disease | National Heart, Lung, and Blood Institute (NHLBI)" 8210:

Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action

6829:"Statement on Screening for Sickle Cell Trait and Athletic Participation" 6714: 5325: 4487: 3978:

Rees DC, Williams TN, Gladwin MT (December 2010). "Sickle-cell disease".

3172: 3033: 2941: 1635: 1620: 1461: 1322: 1198: 1197:, the destruction of the red cells, because of their shape. Although the 1142: 1124: 1069: 894: 875: 725: 628: 592: 556: 555:, a technique to encourage deep breathing to minimise the development of 343: 324: 272: 192: 64: 10751: 10728:"CRISPR/Cas9 β-globin gene targeting in human haematopoietic stem cells" 6549:"National Society of Genetic Counselors : About Genetic Counselors" 1502:

has been shown to reduce the risk of first stroke or silent stroke when

12497: 12382: 12349: 12121: 12071: 12056: 12051: 12044: 12029: 12019: 11457: 7651: 6094: 5096:

Kavanagh PL, Sprinz PG, Vinci SR, Bauchner H, Wang CJ (December 2011).

4990:"How I treat acute chest syndrome in children with sickle cell disease" 4461:"Sickle cell anemia and severe infections due to encapsulated bacteria" 3335: 3155: 3065: 2833:

of age while women live approximately six years longer. An additional 2

1403: 1384: 1338: 1334: 1244:) gives a turbid appearance, whereas normal Hb gives a clear solution. 1218: 1133: 995: 934: 798:

Salmonella typhimurium, Salmonella enteritidis, Salmonella choleraesuis

792: 778: 760:) of the hip and other major joints may occur as a result of ischaemia. 750: 711: 675: 572: 540: 459: 421: 351: 347: 336: 200: 196: 182: 165: 134: 130: 98: 77: 11110: 8991: 8462:, Nakayinga R, Mwambi B, Odda J, Kiconco S, Komuhangi A (April 2018). 8364: 8236:"Inherited haemoglobin disorders: an increasing global health problem" 8184: 8167: 7745: 7728: 6669: 6306:

Pathophysiology : introductory concepts and clinical perspectives

6212:"Biology and dynamics of potential malaria vectors in Southern France" 5787: 5645: 5628: 5443:"Hyperbaric oxygen therapy in the treatment of sickle cell leg ulcers" 4350: 4333: 3884: 3762:"Sickle Cell Disease - How Sickle Cell Disease May Affect Your Health" 3521:"Sickle-cell disease and other haemoglobin disorders Fact sheet N°308" 3165: 3004:(1877–1959), intern to Chicago cardiologist and professor of medicine 1423:.) In addition to ultrasounds and blood transfusions, the inexpensive 12570: 12312: 12307: 12171: 12149: 12024: 11988: 11236: 9693: 8333: 7891: 3261: 3187: 3017: 3013: 2968: 1483: 1376: 1318: 1213:

reveals haemoglobin levels in the range of 6–8 g/dl with a high

1194: 1187: 1100:

responsible for sickle cell anaemia can be found on the short arm of

1065: 1010: 954: 898: 746: 524: 390: 215: 9842:"Federal Register, Volume 82 Issue 178 (Friday, September 15, 2017)" 9752: 9563: 8553: 3060:

allowed the discovery of particular subtypes, such as HbSC disease.

583:; preventive antibiotics and vaccinations are recommended for those 436: 315:) that makes haemoglobin, one from each parent. This gene occurs in 12076: 11853: 11270: 10357:"Better protection and support for people with sickle cell disease" 10106: 7374:

Martí-Carvajal AJ, Conterno LO, Knight-Madden JM (September 2019).

7373: 6178: 5678:"Cardiomyopathy With Restrictive Physiology in Sickle Cell Disease" 3286:

was used to edit bone marrow cells from a person with SCD to boost

3021: 1631: 1388: 1226: 1158: 1061: 998: 991:

Senegal and Saudi-Asian variants, and tend to have milder disease.

830: 774: 584: 544: 520: 512: 500: 496: 320: 264: 138: 10648: 8852: 8318:"The impact of malaria parasitism: from corpuscles to communities" 6399: 3737:"Sickle Cell Anemia: Symptoms, What It Is, Causes & Treatment" 690:

Sickle cell anaemia can lead to various complications, including:

12555: 12015: 11983: 11876: 7767: 5576:

Lai YC, Potoka KC, Champion HC, Mora AL, Gladwin MT (June 2014).

4615: 3850:

This article incorporates text from this source, which is in the

3260:

Diseases such as sickle cell disease for which a person's normal

2988: 2955:. These programs were accompanied by public education campaigns. 2948: 2870:

As a result of population growth in African-Caribbean regions of

1663: 939: 406: 55: 11184: 9755:

Bulletin de la Société de Pathologie Exotique et de ses Filiales

6779:

Ferrari R, Parker LS, Grubs RE, Krishnamurti L (December 2015).

4832:

Cardiovascular & Hematological Agents in Medicinal Chemistry

914: 11366: 11153: 11122: 10588:"Gene Therapy Corrects Sickle Cell Disease In Laboratory Study" 10231:"Our Vision and Mission – Uganda Sickle Cell Rescue Foundation" 7811: 6453:

Henry's Clinical Diagnosis and Management by Laboratory Methods

4292:"BestBets: How long should an average sickle cell crisis last?" 3689:

Nelson MD, Bennett DM, Lehman ME, Okonji AI (5 December 2022).

3291: 3120: 2916: 2912: 1670: 1639: 1541:

mobility. Current treatment options include resting the joint,

1271: 1112: 1097: 1075: 1053: 1034: 1030: 1014: 930:

Distribution of the sickle cell trait, shown in pink and purple

721: 624: 568: 532: 428: 296: 288: 280: 122: 106: 94: 10562:"Murine Gene Therapy Corrects Symptoms of Sickle Cell Disease" 6778: 6077:

Odièvre MH, Verger E, Silva-Pinto AC, Elion J (October 2011).

5258: 4176: 1658:

Three-quarters of sickle cell cases occur in Africa. A recent

1169: 370:

were approved. A small percentage of people can be cured by a

271:. It results in an abnormality in the oxygen-carrying protein 6209: 6076: 5816: 5627:

Caughey MC, Poole C, Ataga KI, Hinderliter AL (August 2015).

5522: 5153:

Hematology. American Society of Hematology. Education Program

1538: 1380: 1287: 1120: 1006: 782: 666:

Management is supportive, sometimes with blood transfusions.

382: 10850: 10041: 8709: 8458: 7652:

Center for Drug Evaluation and Research (25 November 2019).

7567: 6574:"ABGC – Information for Certified Genetic Counselors | ABGC" 5626: 5487: 5356: 5259:

Martí-Carvajal AJ, Solà I, Agreda-Pérez LH (December 2019).

4549:"Blood Transfusions for Sickle Cell Disease | HealthLink BC" 11822: 10851:

Persaud A, Desine S, Blizinsky K, Bonham VL (August 2019).

8512: 8135:

Sickle Cell Anemia: From Basic Science to Clinical Practice

7892:"Empowering people affected by MS to live their best lives" 7727:

Gyang E, Yeom K, Hoppe C, Partap S, Jeng M (January 2011).

7677: 7071:"Folate supplementation in people with sickle cell disease" 6391:

Celiac Disease – From Pathophysiology to Advanced Therapies

6010: 5987:"Sickle-Cell Anemia: Haplotype | Learn Science at Scitable" 5095: 3921: 3144: 516: 10508: 9268: 8948:"Inheriting sickle cell anaemia – Live Well – NHS Choices" 8903: 8415: 7009: 6735: 4619:

American Journal of Respiratory and Critical Care Medicine

4412:"Psychological therapies for sickle cell disease and pain" 3768:. National Heart, Lung, and Blood Institute. 23 April 2024 3313: 3032:

Hospital on 15 November 1910. In the later description by

11076:

In the blood: sickle cell anemia and the politics of race

10586:

St. Jude Children's Research Hospital (4 December 2008).

10130: 9014:"Give Blood – Resources – Sickle Cell and Blood Donation" 7854: 7260:"FDA approves crizanlizumab-tmca for sickle cell disease" 6651: 3688: 3369:"What Are the Signs and Symptoms of Sickle Cell Disease?" 3151: 1453:

acute chest syndrome in people with sickle cell disease.

1080: 975: 10925: 10725: 10618:

for "Stem Cell Gene Therapy for Sickle Cell Disease" at

9987: 7523: 7477:"Hydroxyurea (hydroxycarbamide) for sickle cell disease" 6857: 5578:"Pulmonary arterial hypertension: the clinical syndrome" 4789:

Slavov SN, Kashima S, Pinto AC, Covas DT (August 2011).

3135: 1597:

were approved for the treatment of sickle cell disease.

1009:

changing to GTG) of the β-globin gene, which results in

970:, which consists of two alpha and two delta chains, and 9414: 8165: 5146: 4788: 4661: 8712:

Journal of the American Academy of Nurse Practitioners

8315: 8274: 8088: 7988: 7474: 6456:(23 ed.). Elsevier Health Sciences. p. 578. 4951:

The Journal of Bone and Joint Surgery. American Volume

4225: 3639: 3585: 12651: 10329:"London Eye: Shouldn't need an improvement programme" 9890: 9331: 9059:"Epidemiology of sickle cell disease in Saudi Arabia" 9028: 8316:

Wellems TE, Hayton K, Fairhurst RM (September 2009).

8073: 7814:"Bone marrow transplantation for sickle cell disease" 7262:. U.S. Food and Drug Administration. 15 November 2019 5724: 5575: 5455:(1): 1–4. Archived from the original on 15 April 2013 4747: 4316: 958:

Base-pair substitution that causes sickle cell anemia

696:

infections after having the spleen removed surgically

644:

counts drop dramatically during the disease (causing

11100: 10651:"Gene Therapy in a Patient with Sickle Cell Disease" 9988:

Tusuubira SK, Naggawa T, Nakamoga V (October 2019).

9893:"Stigma of Sickle Cell Disease: A Systematic Review" 9415:

Ballas SK, Gupta K, Adams-Graves P (November 2012).

9190:"Public awareness of sickle cell disease in Bahrain" 8582:"Data & Statistics on Sickle Cell Disease | CDC" 8074:

Kumar V, Abbas AK, Fausto N, Aster J (28 May 2009).

7941: 7726: 6079:"Pathophysiological insights in sickle cell disease" 5254: 5252: 5250: 4748:

Kumar V, Abbas AK, Fausto N, Aster J (28 May 2009).

4317:

Kumar V, Abbas AK, Fausto N, Aster J (28 May 2009).

901:

and worsened anaemia. If it progresses to end-stage

631:, and fatigue. This crisis is normally triggered by 11033:

Managing Sickle Cell Disease in Low-Income Families

10256:"Background – Uganda Sickle Cell Rescue Foundation" 10180: 9187: 8213:. Washington: National Academies Press. p. 1. 8047:

Medicines and Healthcare products Regulatory Agency

7068: 6963:"Vitamin D supplementation for sickle cell disease" 5073:

Andrews' Diseases of the Skin: clinical Dermatology

3977: 3166:

Social isolation of people with sickle cell disease

1341:had been previously recommended by the WHO. A 2016 966:, which consists of two alpha and two beta chains, 11073: 9983: 9981: 9979: 9977: 9975: 9973: 9971: 9969: 9036:"Why is Blood from Afro-Caribbean Donors Special?" 8825: 7283: 7042:"Evidence-Based Management of Sickle Cell Disease" 6364:"Sickle Cell Anemia: eMedicine Emergency Medicine" 5675: 4867:"The Role of Oxidative Stress in Hemolytic Anemia" 4864: 4707:Hematologic Abnormalities and Acute Lung Syndromes 4572: 4504: 4334:"How I manage priapism due to sickle cell disease" 4331: 3815: 3813: 3239: 3020:in 1916 and is buried in the Catholic cemetery at 9944:(1st paperback ed.). New York: Harlem Moon. 9391:Lebby R (1846). "Case of absence of the spleen". 8778: 8385:"Sickle-cell anaemia – Report by the Secretariat" 7475:Rankine-Mullings AE, Nevitt SJ (September 2022). 7422: 7353:(6th ed.). St. Louis: Mosby. pp. 1–10. 7120:"Malaria chemoprophylaxis in sickle cell disease" 6932:"Nutrition for the Child with Sickle Cell Anemia" 6416: 6303: 5307: 5247: 319:. Several subtypes exist, depending on the exact 12675: 10926:Oringanje C, Nemecek E, Oniyangi O (July 2020). 10459: 10181:Marsh VM, Kamuya DM, Molyneux SS (August 2011). 8940: 7942:Callaway D, Chawla A, Sprinz P (November 2020). 7654:"FDA approves voxelotor for sickle cell disease" 7323: 7321: 6700: 1627:. In 2015, it resulted in about 114,800 deaths. 497:a yellowish colour of skin or whites of the eyes 381:. It also occurs to a lesser degree in parts of 11317: 9966: 9891:Bulgin D, Tanabe P, Jenerette C (August 2018). 9588:The Journal of the American Medical Association 9568:The Journal of the American Medical Association 9315:"Life with sickle cell – Nation – Nepali Times" 8826:Bardakdjian J, Wajcman H (September 2004). "". 8233: 8200: 7574:The Journal of the American Medical Association 6449: 5906:NORD (National Organization for Rare Disorders) 4375: 4373: 4371: 4369: 4180:The Journal of the American Medical Association 4100: 4098: 4096: 4094: 4092: 4090: 4088: 4086: 4084: 4055:The Journal of the American Medical Association 3810: 3754: 1662:report estimated that around 2% of newborns in 1193:The actual anaemia of the illness is caused by 1056:can produce half of the haemoglobin. In people 674:One of the earliest clinical manifestations is 562: 449:, several of which have a high mortality rate. 10403:"NHS launches lifesaving sickle cell campaign" 10176: 10174: 10172: 10170: 10168: 9410: 9408: 9406: 8984: 8166:Prabhakar H, Haywood C, Molokie R (May 2010). 8076:Robbins and Cotran Pathologic Basis of Disease 7209: 5391: 5036: 4906:"Dactylitis in a child with sickle cell trait" 4750:Robbins and Cotran Pathologic Basis of Disease 4319:Robbins and Cotran Pathologic Basis of Disease 4052: 4027:(2 ed.). Berlin: Springer. p. 2950. 3077:Some old texts refer to it as drepanocytosis. 2899:(one in 5,014) for the same reference period. 1529:Hematopoietic Stem Cell Transplantation (HSCT) 1247:Abnormal haemoglobin forms can be detected on 1084:gene (responsible for sickle cell anaemia) is 749:production and precipitation due to prolonged 11838: 11303: 10810:"CRISPR's "magnificent moment" in the clinic" 10566:The Scientist – Magazine of the Life Sciences 9272:Annals of Tropical Medicine and Public Health 9101: 9050: 8409: 7561: 7318: 7117: 6960: 6476: 6443: 6422: 5851: 4944: 4903: 4266:"Sickle Cell Disease - Symptoms | NHLBI, NIH" 4144: 4142: 4048: 4046: 4044: 3478: 3476: 3474: 3443: 3441: 3439: 3408: 3406: 3404: 3402: 3400: 3398: 3396: 3394: 3363: 3361: 3359: 3357: 3355: 3302:In November 2023, a gene treatment using the 2828:The number of people with the disease in the 1186:capillaries, leading to vessel occlusion and 942:(no longer endemic in Europe), shown in green 11452:Glucose-6-phosphate dehydrogenase deficiency 10418: 9994:Adolescent Health, Medicine and Therapeutics 9620: 9299:: CS1 maint: DOI inactive as of June 2024 ( 9161:"Features of sickle-cell disease in Bahrain" 9107: 8779:Vlachadis N, Vrachnis N (15 November 2022). 8574: 8082: 7805: 7517: 7348: 6470: 6154: 6119: 5773: 5767: 5718: 5669: 5516: 5481: 5350: 4981: 4823: 4782: 4704: 4655: 4566: 4366: 4081: 3581: 3579: 1328: 11336: 10932:The Cochrane Database of Systematic Reviews 10466:The Cochrane Database of Systematic Reviews 10414: 10412: 10276: 10165: 9782:"Contested Disability: Sickle Cell Disease" 9403: 9332:Asnani MR, McCaw-Binns AM, Reid ME (2011). 8370:"Sickle cell disease: MedlinePlus Genetics" 8131: 7481:The Cochrane Database of Systematic Reviews 7470: 7468: 7429:The Cochrane Database of Systematic Reviews 7380:The Cochrane Database of Systematic Reviews 7216:The Cochrane Database of Systematic Reviews 7124:The Cochrane Database of Systematic Reviews 7075:The Cochrane Database of Systematic Reviews 6967:The Cochrane Database of Systematic Reviews 6160: 6004: 5858:Cold Spring Harbor Perspectives in Medicine 5854:"The switch from fetal to adult hemoglobin" 5810: 5308:Kenny MW, George AJ, Stuart J (July 1980). 5301: 5265:The Cochrane Database of Systematic Reviews 5147:Adams RJ, Ohene-Frempong K, Wang W (2001). 5070: 4938: 4609: 4416:The Cochrane Database of Systematic Reviews 4405: 4403: 4172: 4170: 4150:"Sickle cell disease: MedlinePlus Genetics" 3635: 3633: 3631: 3548: 3546: 3544: 3542: 12127:Reproductive endocrinology and infertility 11845: 11831: 11808:Hereditary persistence of fetal hemoglobin 11310: 11296: 9939: 9779: 9417:"Sickle cell pain: a critical reappraisal" 9384: 9158: 8608:"September is Sickle Cell Awareness Month" 8586:Centers for Disease Control and Prevention 8554:National Heart, Lung and Blood Institute. 8366:United States National Library of Medicine 8275:Roberts I, de Montalembert M (July 2007). 7602: 7010:Office of the Commissioner (7 July 2017). 7003: 6260: 4244:10.1182/blood.v86.2.776.bloodjournal862776 4139: 4107:"One hundred years of sickle cell disease" 4041: 3471: 3436: 3391: 3352: 1369: 54: 12699:Disorders of globin and globulin proteins 12468:Bachelor of Medicine, Bachelor of Surgery 11760:Mean corpuscular hemoglobin concentration 11582:Warm antibody autoimmune hemolytic anemia 10951: 10876: 10759: 10666: 10485: 10460:Olowoyeye A, Okwundu CI (November 2020). 10436: 10425:Hematology/Oncology and Stem Cell Therapy 10304: 10294: 10250: 10248: 10206: 10148: 10069: 10059: 10015: 10005: 9916: 9493: 9432: 9367: 9357: 9284: 9215: 9205: 9135: 9125: 9084: 9074: 9056: 9006: 8880: 8870: 8530: 8489: 8479: 8341: 8292: 8251: 8240:Bulletin of the World Health Organization 8183: 8036: 8034: 8014: 7829: 7744: 7695: 7585: 7544: 7500: 7448: 7399: 7301: 7279: 7277: 7235: 7143: 7094: 6986: 6796: 6677: 6625: 6494: 6398: 6237: 6227: 6186: 6102: 5877: 5750: 5701: 5644: 5603: 5593: 5536: 5434: 5409: 5394:"Bone involvement in sickle cell disease" 5333: 5284: 5205: 5164: 5071:James WD, Berger TG, et al. 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Beet. 1949 was the year when 1261:high-performance liquid chromatography 331:. Such people are also referred to as 11826: 11365: 11291: 10594:from the original on 13 December 2014 10419:Kassim AA, Sharma D (December 2017). 10086: 10032: 9822:. U.S. Social Security Administration 9820:"Social Security Ruling: SSR 2017-3p" 9585: 9561: 9390: 8958:from the original on 2 December 2014. 8322:The Journal of Clinical Investigation 8055:from the original on 25 November 2023 7608: 6477:Clarke GM, Higgins TN (August 2000). 6338:"How Does Sickle Cell Cause Disease?" 5440: 5187: 4221: 4219: 4217: 3973: 3971: 3969: 3967: 3554:"How Is Sickle Cell Disease Treated?" 3136:Rate of sickle cell disease in Uganda 1348: 579:increases the risk of infection from 495:, and others. The early symptoms are 473: 412: 46:Sickle cell disorder; drepanocytosis 12606: 12367:Physical medicine and rehabilitation 11469:triosephosphate isomerase deficiency 11080:. University of Pennsylvania Press. 9543:from the original on 31 January 2015 9537:"UVa Hospital Celebrating 100 Years" 9470:10.1001/archinte.1910.00050330050003 9177:from the original on 8 October 2016. 9166:Eastern Mediterranean Health Journal 8992:"Who is offered screening and when?" 8660:"Disorder Name: Sickle Cell Disease" 8562:from the original on 2 December 2010 7915: 7351:Pulmonary and Critical Care Medicine 6370:from the original on 4 December 2010 6308:. Philadelphia: F.A. Davis Company. 5957:"Inheritance of Sickle Cell Anaemia" 5234:The Lecturio Medical Concept Library 4910:Canadian Medical Association Journal 3857: 3835:from the original on 8 December 2023 3788:"Learning About Sickle Cell Disease" 2982: 1489: 1409: 651: 612:crises during acute chest syndrome. 537:nonsteroidal anti-inflammatory drugs 409:of sickle cell trait was described. 12616: 11624:paroxysmal nocturnal hemoglobinuria 11592:Donath–Landsteiner hemolytic anemia 11572: 10655:The New England Journal of Medicine 10453: 10107:"Sickle Cell Association of Uganda" 9933: 9884: 8970:"Sickle cell anaemia – NHS Choices" 8051:(Press release). 16 November 2023. 7896:National Multiple Sclerosis Society 7818:The New England Journal of Medicine 7611:The New England Journal of Medicine 7533:The New England Journal of Medicine 7290:The New England Journal of Medicine 7210:Okomo U, Meremikwu MM (July 2017). 6703:The New England Journal of Medicine 6511:from the original on 20 March 2008. 6423:Atkinson K, Mabey D (23 May 2019). 5967:from the original on 8 January 2024 4904:Jadavji T, Prober CG (April 1985). 3798:from the original on 4 January 2017 3211: 1321:supplementation remains uncertain. 796:(especially the atypical serotypes 267:. The most common type is known as 24: 12503:Medical Scientist Training Program 10972: 10277:Sehlo MG, Kamfar HZ (April 2015). 9321:from the original on 24 June 2015. 9188:Al Arrayed S, Al Hajeri A (2010). 8724:10.1111/j.1745-7599.2004.tb00426.x 6860:British Journal of Sports Medicine 6738:Clinical Journal of Sport Medicine 6167:American Journal of Human Genetics 5136:from the original on 4 March 2016. 5075:. Saunders Elsevier. p. 847. 4468:The Journal of Infectious Diseases 4214: 3964: 3459:from the original on 24 March 2016 3449:"What Causes Sickle Cell Disease?" 3085: 2958: 1164: 1033:concentration. However, under low 615: 291:, swelling in the hands and feet, 25: 12720: 11633:Microangiopathic hemolytic anemia 11604:Mixed autoimmune hemolytic anemia 11096: 11051:Serjeant GR, Serjeant BE (2001). 10111:Sickle Cell Association of Uganda 9519:The Virginia Medical Semi-Monthly 9250:from the original on 25 June 2017 8781:"A Review of Sickle Cell Disease" 7022:from the original on 10 July 2017 6529:from the original on 17 June 2010 6304:Capriotti T, Frizzell JP (2016). 5502:10.1016/j.survophthal.2009.11.004 5392:Almeida A, Roberts I (May 2005). 4865:Fibach E, Rachmilewitz E (2008). 4494:from the original on 27 May 2016. 4480:10.1093/infdis/136.Supplement.S25 4298:from the original on 17 June 2010 3564:from the original on 9 March 2016 3494:from the original on 9 March 2016 3424:from the original on 6 March 2016 3379:from the original on 9 March 2016 3255: 3186:Women living with SCD who become 2922: 1333:From birth to five years of age, 12661: 12635: 12625: 12615: 12605: 12596: 12595: 11664:Hemolytic disease of the newborn 10919: 10893: 10844: 10801: 10776: 10691: 10642: 10624: 10606: 10553: 10502: 10395: 10377: 10349: 10321: 10270: 10223: 10124: 9859: 9834: 9812: 9773: 9746: 9716: 9665: 9614: 9580:10.1001/jama.1922.02640160038012 9555: 9529: 9510: 9449: 9325: 9307: 9262: 9232: 9181: 9152: 8897: 8846: 8819: 8772: 8746: 8678: 8652: 8626: 8600: 8556:"Sickle cell anemia, key points" 8547: 8506: 8452: 8376: 8358: 8309: 8268: 8234:Weatherall DJ, Clegg JB (2001). 8227: 8159: 8125: 8067: 7982: 7935: 7909: 7884: 7783:10.1111/j.1600-0609.2009.01379.x 7761: 7720: 7697:10.1111/j.1365-2141.2010.08451.x 7671: 7645: 7416: 7367: 7342: 7252: 7203: 7160: 7111: 7062: 6450:McPherson RA, Pincus MR (2017). 5937:from the original on 15 May 2016 5411:10.1111/j.1365-2141.2005.05476.x 4963:10.2106/00004623-197658080-00024 4808:10.1111/j.1574-695X.2011.00819.x 4676:10.1111/j.1600-0609.2011.01647.x 4124:10.1111/j.1365-2141.2010.08419.x 3867:Wilkinson E (16 November 2023). 3845: 2823: 1565:Psychological therapies such as 899:loss of red blood cells in urine 685: 27:Group of genetic blood disorders 12636: 9897:Issues in Mental Health Nursing 8382: 7771:European Journal of Haematology 7034: 6954: 6924: 6894: 6851: 6821: 6772: 6729: 6694: 6645: 6619: 6606: 6596: 6566: 6541: 6515: 6382: 6356: 6330: 6297: 6254: 6203: 6070: 6047: 5979: 5949: 5919: 5894: 5845: 5620: 5569: 5385: 5222: 5181: 5140: 5089: 5030: 4897: 4858: 4756: 4741: 4698: 4664:European Journal of Haematology 4541: 4498: 4452: 4325: 4310: 4284: 4258: 4024:Textbook of clinical pediatrics 4014: 3915: 3780: 3729: 3328: 3246:umbilical cord blood transplant 3240:Umbilical cord blood transplant 3068:'s Emmy-winning 1972 TV movie, 1609: 1584: 1421:New England Journal of Medicine 1149:, and now artificially through 827:intrauterine growth restriction 452: 372:transplant of bone marrow cells 12493:Doctor of Osteopathic Medicine 11927:Oral and maxillofacial surgery 11597:Paroxysmal cold hemoglobinuria 10944:10.1002/14651858.CD007001.pub5 10478:10.1002/14651858.CD007652.pub7 10365:. October 2022. pp. 16–17 9728:Facts On File History Database 9244:American Society of Hematology 8638:American Society of Hematology 8172:American Journal of Hematology 7733:American Journal of Hematology 7684:British Journal of Haematology 7493:10.1002/14651858.CD002202.pub3 7441:10.1002/14651858.CD007843.pub4 7392:10.1002/14651858.CD006110.pub5 7228:10.1002/14651858.CD005406.pub5 7136:10.1002/14651858.CD003489.pub2 7087:10.1002/14651858.CD011130.pub3 6979:10.1002/14651858.CD010858.pub3 6833:American Society of Hematology 6658:American Journal of Hematology 6161:Kwiatkowski DP (August 2005). 6013:American Journal of Hematology 5633:British Journal of Haematology 5398:British Journal of Haematology 5277:10.1002/14651858.CD004344.pub7 5190:"Big strokes in small persons" 5166:10.1182/asheducation-2001.1.31 4507:American Journal of Hematology 4428:10.1002/14651858.CD001916.pub3 4338:British Journal of Haematology 4111:British Journal of Haematology 3682: 3523:. January 2011. Archived from 3414:"What Is Sickle Cell Disease?" 3092:Social Security Administration 1456: 1003:single-nucleotide polymorphism 950:Modern distribution of malaria 905:, it carries a poor prognosis. 771:(malfunctioning of the spleen) 585:lacking proper spleen function 465:Approximately half of classic 99:swelling in the hands and feet 13: 1: 12689:Autosomal recessive disorders 9909:10.1080/01612840.2018.1443530 9458:Archives of Internal Medicine 9108:Memish ZA, Saeedi MY (2011). 8906:Journal of Clinical Pathology 8532:10.1016/S2214-109X(15)00288-0 8430:10.1016/S0140-6736(02)08273-9 8103:10.1016/s0140-6736(00)04132-5 6785:Journal of Genetic Counseling 6616:. John Wiley & Sons, Ltd. 6128:Current Opinion in Immunology 5595:10.1161/CIRCRESAHA.115.301146 5371:10.1016/j.amjsurg.2010.03.017 5314:Journal of Clinical Pathology 5037:Friend A, Girzadas D (2021). 4410:Anie KA, Green J (May 2015). 4105:Serjeant GR (December 2010). 3992:10.1016/s0140-6736(10)61029-x 3940:10.1016/s0140-6736(15)60692-4 3695:American Journal of Audiology 3658:10.1016/S0140-6736(16)31012-1 3604:10.1016/S0140-6736(16)31678-6 3345: 3175:and overall quality of life. 3026:Virginia Medical Semi-Monthly 1305: 1013:(E/Glu) being substituted by 637:production of red blood cells 358:. Other measures may include 223:40–60 years (developed world) 12709:Hereditary hemolytic anemias 12473:Bachelor of Medical Sciences 12240:Neurosurgical anesthesiology 11852: 11548:Southeast Asian ovalocytosis 11536:Minkowski–Chauffard syndrome 10998:Resources in other libraries 10900:Walker M (15 January 2018). 10808:Zipkin M (6 December 2019). 10698:Kolata G (27 January 2019). 10438:10.1016/j.hemonc.2017.05.008 10199:10.1080/13557858.2010.541903 10137:East African Medical Journal 9643:10.1126/science.110.2865.543 9434:10.1182/blood-2012-04-383430 9359:10.1371/journal.pone.0026281 7960:10.1097/MPH.0000000000001888 7861:Kaiser J (5 December 2020). 7181:10.1016/j.neulet.2019.134534 6872:10.1136/bjsports-2015-095317 6750:10.1097/JSM.0b013e31822e8634 6626:Stallings E (27 July 2019). 6263:Journal of Biosocial Science 5776:Pediatric Blood & Cancer 5743:10.1182/blood-2017-02-767624 5682:JACC. Cardiovascular Imaging 5007:10.1182/blood-2010-11-261834 3827:Food and Drug Administration 2931: 2887:(one in 10,862), congenital 1600: 1204: 1174:Scanning electron micrograph 994:The gene defect is a single 849:) can lead to strain on the 640:life-threatening situation. 563:Splenic sequestration crisis 549:patient-controlled analgesia 489:splenic sequestration crisis 366:(hydroxyurea). In 2023, new 7: 11057:. Oxford University Press. 11036:. Temple University Press. 11015:. Oxford University Press. 10560:Wilson JF (18 March 2002). 10384:Rylatt A (7 October 2022). 9393:Southern J of Med Pharmacol 8132:Costa FF, Conran N (2016). 7916:Dudi A (17 November 2023). 7831:10.1056/NEJM199608083350601 7546:10.1056/NEJM199505183322001 7303:10.1056/NEJM199807023390102 6906:www.sickle-thal.nwlh.nhs.uk 5870:10.1101/cshperspect.a011643 5831:10.7326/0003-4819-115-8-614 5819:Annals of Internal Medicine 5525:Annals of Internal Medicine 5359:American Journal of Surgery 5207:10.1001/archneur.64.11.1567 5149:"Sickle cell and the brain" 4715:10.1007/978-3-319-41912-1_4 4382:Emergency Medicine Practice 3234: 3226:World Sickle Cell Day, the 3058:haemoglobin electrophoresis 2975:. It is also endemic among 2891:(one in 3,132), congenital 1579:psychodynamic psychotherapy 1312:Pain management in children 1249:haemoglobin electrophoresis 982:Sickle cell disease has an 938:Historical distribution of 909: 845:(increased pressure on the 531:; pain management requires 10: 12725: 11657:Drug-induced nonautoimmune 11638:Thrombotic microangiopathy 11464:pyruvate kinase deficiency 10822:10.1038/d41587-019-00035-2 9539:. University of Virginia. 8872:10.3389/fpubh.2023.1215605 8859:Frontiers in Public Health 8007:10.1016/j.htct.2020.05.004 6614:The Cochrane Collaboration 6496:10.1093/clinchem/46.8.1284 5694:10.1016/j.jcmg.2015.05.013 5188:Adams RJ (November 2007). 4883:10.2174/156652408786241384 4871:Current Molecular Medicine 4844:10.2174/187152512799201190 4641:10.1164/rccm.200710-1606OC 4459:Pearson HA (August 1977). 3071:To All My Friends on Shore 2994: 1595:lovotibeglogene autotemcel 1309: 1151:prenatal genetic screening 745:may result from excessive 12591: 12513: 12451: 12212: 12140: 12095: 12004: 11875: 11860: 11785: 11725: 11676: 11643:Hemolytic–uremic syndrome 11563: 11555:Hereditary stomatocytosis 11543:Hereditary elliptocytosis 11436: 11421: 11374: 11358: 11329: 11216: 11104: 10993:Resources in your library 10869:10.1038/s41436-018-0409-6 10296:10.1186/s12888-015-0461-6 10061:10.1186/s12889-018-5496-4 8690:www.stanfordchildrens.org 8519:The Lancet. Global Health 8481:10.1186/s12889-018-5496-4 6798:10.1007/s10897-015-9849-1 6275:10.1017/S0021932000008622 6140:10.1016/j.coi.2009.04.001 5473:: CS1 maint: unfit URL ( 5045:. StatPearls Publishing. 4587:10.1007/s00247-008-1049-9 3707:10.1044/2022_AJA-22-00059 3250:graft versus host disease 3114: 2857: 2033:Central African Republic 1653: 1551:joint-replacement surgery 1364:medication for prevention 1329:Folic acid and penicillin 895:protein loss in the urine 635:, which directly affects 424:in the hands of an infant 279:. This leads to a rigid, 235: 227: 214: 188: 176: 160: 152: 112: 87: 71: 62: 53: 42: 37: 12427:Transplantation medicine 12318:Clinical neurophysiology 12235:Obstetric anesthesiology 12155:Interventional radiology 11915:Digestive system surgery 11531:Hereditary spherocytosis 10906:Sickle Cell Disease News 10150:10.4314/eamj.v86i9.54167 9786:Health & Social Work 9600:10.1001/jama.254.14.1955 9289:(inactive 22 June 2024). 9194:Annals of Saudi Medicine 9114:Annals of Saudi Medicine 9063:Annals of Saudi Medicine 8138:. Springer. p. 35. 7587:10.1001/jama.289.13.1645 7328:Kolata G (24 May 2021). 4947:"Sickle-cell dactylitis" 3321: 1591:exagamglogene autotemcel 1362:should receive lifelong 1088:on the short (p) arm of 814:Acute papillary necrosis 701:Streptococcus pneumoniae 669: 399:people of African origin 12298:Intensive care medicine 12272:Mass gathering medicine 12117:Maternal–fetal medicine 11736:Mean corpuscular volume 11700:Diamond–Blackfan anemia 11652:Drug-induced autoimmune 11587:Cold agglutinin disease 11393:Plummer–Vinson syndrome 10531:10.1126/science.1065806 9286:10.4103/1755-6783.43069 9207:10.4103/0256-4947.65256 9127:10.4103/0256-4947.81527 9076:10.4103/0256-4947.81540 8918:10.1136/jcp.2009.068874 8797:10.1001/jama.2022.16732 7175:. Elsevier BV: 134534. 5931:Genetics Home Reference 5490:Survey of Ophthalmology 4192:10.1001/jama.2014.10517 3276:Phase 1 clinical trials 1518:Bone marrow transplants 887:sickle-cell nephropathy 12694:Chronic pain syndromes 11890:Cardiothoracic surgery 11388:Iron-deficiency anemia 11009:Brown RT, ed. (2006). 10630:Clinical trial number 10612:Clinical trial number 10334:Health Service Journal 10187:Ethnicity & Health 9940:Washington HA (2006). 8662:. New Born Screening. 8294:10.3324/haematol.11474 6429:. Wiley. p. 227. 6229:10.1186/1475-2875-6-18 6025:10.1002/ajh.2830440214 5114:10.1542/peds.2010-3686 5039:"Acute Chest Syndrome" 4988:Miller ST (May 2011). 4764:"Parvovirus B19 | CDC" 4519:10.1002/ajh.2830390305 4067:10.1001/jama.261.2.266 3030:University of Virginia 2907: 2867: 2573:São Tomé and Príncipe 1644:heterozygote advantage 1513:Bone marrow transplant 1358: 1177: 1093: 962:Normally, humans have 959: 951: 943: 931: 923: 889:manifests itself with 843:Pulmonary hypertension 707:Haemophilus influenzae 581:encapsulated organisms 441: 433: 425: 252:), also simply called 143:pulmonary hypertension 12541:Personalized medicine 12400:Reproductive medicine 12325:Occupational medicine 12279:Evolutionary medicine 11707:Pure red cell aplasia 11474:hexokinase deficiency 10668:10.1056/NEJMoa1609677 10386:"No-one is Listening" 9780:Srikanthan S (2023). 9159:Al Arrayed S (1995). 9040:sicklecellsociety.org 8828:La Revue du Praticien 7623:10.1056/NEJMct0708272 6489:(8 Pt 2): 1284–1290. 5927:"sickle cell disease" 5902:"Sickle Cell Disease" 5194:Archives of Neurology 3101:In the US, there are 2953:premarital counseling 2905: 2874:and immigration from 2865: 1561:Psychological therapy 1370:Vaso-occlusive crisis 1356: 1310:Further information: 1221:may show features of 1172: 1078: 1027:quaternary structures 957: 949: 937: 929: 917: 872:diastolic dysfunction 807:Staphylococcus aureus 758:aseptic bone necrosis 507:Vaso-occlusive crisis 481:vaso-occlusive crisis 439: 431: 420: 354:supplementation, and 350:, high fluid intake, 199:, high fluid intake, 127:aseptic bone necrosis 12561:Traditional medicine 12521:Alternative medicine 12388:Addiction psychiatry 12202:Transfusion medicine 12197:Medical microbiology 12112:Gynecologic oncology 11964:Reproductive surgery 11712:Sideroblastic anemia 11520:Hemoglobin C disease 11406:Megaloblastic anemia 10857:Genetics in Medicine 10814:Nature Biotechnology 10007:10.2147/AHMT.S223956 9564:"Sickle cell anemia" 9246:. 16 December 2014. 9240:"Sickle Cell Anemia" 9057:Jastaniah W (2011). 9046:on 30 December 2014. 7169:Neuroscience Letters 6715:10.1056/NEJMp1007639 5582:Circulation Research 5326:10.1136/jcp.33.7.622 4388:(8): 1–20, quiz 20. 3652:(10053): 1459–1544. 3598:(10053): 1545–1602. 3279:treated since then. 3130:health professionals 2895:(one in 19,008) and 1547:pain-relief medicine 1504:transcranial Doppler 1450:exchange transfusion 1432:Acute chest syndrome 1238:sodium metabisulfite 1211:complete blood count 802:Salmonella paratyphi 756:Avascular necrosis ( 680:Hematopoietic ulcers 609:pulmonary infiltrate 605:Acute chest syndrome 600:Acute chest syndrome 553:Incentive spirometry 335:. Diagnosis is by a 293:bacterial infections 103:bacterial infections 12684:Sickle-cell disease 12583:History of medicine 12566:Veterinary medicine 12373:Preventive medicine 12225:Adolescent medicine 12067:Infectious diseases 11511:Sickle cell disease 11265:Sickle Cell Disease 11054:Sickle Cell Disease 10984:Sickle cell disease 10752:10.1038/nature20134 10744:2016Natur.539..384D 10523:2001Sci...294.2368P 10517:(5550): 2368–2371. 9798:10.1093/hsw/hlad014 9686:1956Natur.178..792I 9635:1949Sci...110..543P 9476:Herrick JB (2001). 9350:2011PLoSO...626281A 8976:. 23 October 2017. 8954:. 23 October 2017. 8634:"Sickle Cell Trait" 8424:(9314): 1311–1312. 5961:Sickle Cell Society 4575:Pediatric Radiology 4553:www.healthlinkbc.ca 4021:Elzouki AY (2012). 3986:(9757): 2018–2031. 3831:. 8 December 2023. 3081:Society and culture 3010:muscular rheumatism 2893:adrenal hyperplasia 2881:metropolitan France 1677: 1575:behavioural therapy 1500:transfusion therapy 1253:gel electrophoresis 1231:Howell-Jolly bodies 1037:concentration, HbS 984:autosomal recessive 920:autosomal recessive 467:autosomal recessive 362:and the medication 304:vessels correctly. 246:Sickle cell disease 145:, vision problems, 38:Sickle cell disease 12531:Molecular oncology 12488:Doctor of Medicine 12478:Master of Medicine 12395:Radiation oncology 12267:Emergency medicine 12220:Addiction medicine 12187:Clinical chemistry 12182:Clinical pathology 11974:Transplant surgery 11932:Orthopedic surgery 11910:Colorectal surgery 11798:Sulfhemoglobinemia 11217:External resources 10705:The New York Times 10638:ClinicalTrials.gov 10620:ClinicalTrials.gov 8640:. 8 September 2017 7335:The New York Times 6839:on 26 January 2021 6483:Clinical Chemistry 6095:10.1007/bf00168807 6056:"Genetic mutation" 5108:(6): e1552–e1574. 4474:(Suppl): S25–S30. 3290:by inhibiting the 3040:Memphis physician 2908: 2868: 2849:, with researcher 2173:Equatorial Guinea 1676: 1642:(trait) confers a 1617:Sub-Saharan Africa 1495:Blood transfusions 1359: 1349:Malaria prevention 1283:Genetic counselors 1279:genetic counseling 1178: 1094: 960: 952: 944: 932: 924: 825:During pregnancy, 559:, is recommended. 474:Sickle cell crisis 442: 434: 426: 413:Signs and symptoms 379:Sub-Saharan Africa 285:sickle cell crisis 269:sickle cell anemia 258:hemoglobin-related 231:4.4 million (2015) 209:blood transfusions 12649: 12648: 12483:Master of Surgery 12447: 12446: 12432:Tropical medicine 12378:Prison healthcare 12293:Hospital medicine 12257:Disaster medicine 12247:Aviation medicine 12062:Hospital medicine 11969:Surgical oncology 11954:Pediatric surgery 11948: 11895:Endocrine surgery 11820: 11819: 11816: 11815: 11803:Reticulocytopenia 11793:Methemoglobinemia 11781: 11780: 11672: 11671: 11612: 11611: 11411:Pernicious anemia 11354: 11353: 11346:Polycythemia vera 11285: 11284: 11087:978-0-8122-3471-8 11072:Tapper M (1999). 11064:978-0-19-263036-0 11043:978-1-59213-195-2 11022:978-0-19-516985-0 10979:Library resources 10738:(7629): 384–389. 10405:. 7 October 2022. 10048:BMC Public Health 9951:978-0-7679-1547-2 9871:www.nhlbi.nih.gov 9680:(4537): 792–794. 9629:(2865): 543–548. 9594:(14): 1955–1957. 9574:(16): 1318–1320. 9562:Mason VR (1922). 9427:(18): 3647–3656. 8834:(14): 1531–1533. 8760:. 4 November 2017 8468:BMC Public Health 8397:on 4 January 2011 8220:978-0-309-66960-3 8185:10.1002/ajh.21676 8097:(9257): 680–683. 7746:10.1002/ajh.21901 7617:(13): 1362–1369. 7580:(13): 1645–1651. 7539:(20): 1317–1322. 7360:978-0-8151-1371-3 6912:on 3 October 2019 6670:10.1002/ajh.22271 6463:978-0-323-41315-2 6436:978-1-119-28265-5 6410:978-953-51-0684-5 6054:Clancy S (2008). 5788:10.1002/pbc.26607 5646:10.1111/bjh.13447 5448:J. Hyperbaric Med 5441:Rudge FW (1991). 5200:(11): 1567–1574. 5082:978-0-7216-2921-6 5000:(20): 5297–5305. 4724:978-3-319-41910-7 4351:10.1111/bjh.12199 4270:www.nhlbi.nih.gov 4186:(10): 1033–1048. 3934:(9995): 743–800. 3885:10.1136/bmj.p2706 3766:www.nhlbi.nih.gov 3288:fetal haemoglobin 2983:Caribbean Islands 2851:Elliott Vichinsky 2821: 2820: 1571:cognitive therapy 1567:patient education 1490:Blood transfusion 1474:fetal haemoglobin 1446:blood transfusion 1410:Stroke prevention 1296:newborn screening 1242:sodium dithionite 1147:natural selection 1092:at position 15.5. 741:(gallstones) and 657:Haemolytic crises 652:Haemolytic crisis 646:reticulocytopenia 529:blood transfusion 493:haemolytic crisis 360:blood transfusion 329:sickle cell trait 243: 242: 203:supplementation, 178:Diagnostic method 156:5–6 months of age 93:Attacks of pain, 32:Medical condition 16:(Redirected from 12716: 12704:Health in Africa 12666: 12665: 12664: 12657: 12639: 12638: 12629: 12619: 12618: 12609: 12608: 12599: 12598: 12303:Medical genetics 12288:General practice 12165:Nuclear medicine 12040:Gastroenterology 11996:Vascular surgery 11946: 11873: 11872: 11847: 11840: 11833: 11824: 11823: 11570: 11569: 11484:hemoglobinopathy 11434: 11433: 11372: 11371: 11363: 11362: 11334: 11333: 11312: 11305: 11298: 11289: 11288: 11102: 11101: 11091: 11079: 11068: 11047: 11030:Hill SA (2003). 11026: 10966: 10965: 10955: 10923: 10917: 10916: 10914: 10912: 10897: 10891: 10890: 10880: 10863:(8): 1726–1734. 10848: 10842: 10841: 10805: 10799: 10798: 10796: 10794: 10780: 10774: 10773: 10763: 10723: 10717: 10716: 10714: 10712: 10695: 10689: 10688: 10670: 10646: 10640: 10628: 10622: 10610: 10604: 10603: 10601: 10599: 10590:. ScienceDaily. 10583: 10577: 10576: 10574: 10572: 10557: 10551: 10550: 10506: 10500: 10499: 10489: 10472:(11): CD007652. 10457: 10451: 10450: 10440: 10416: 10407: 10406: 10399: 10393: 10392: 10390: 10381: 10375: 10374: 10372: 10370: 10353: 10347: 10346: 10344: 10342: 10325: 10319: 10318: 10308: 10298: 10274: 10268: 10267: 10265: 10263: 10252: 10243: 10242: 10240: 10238: 10227: 10221: 10220: 10210: 10193:(4–5): 343–359. 10178: 10163: 10162: 10152: 10128: 10122: 10121: 10119: 10117: 10103: 10084: 10083: 10073: 10063: 10039: 10030: 10029: 10019: 10009: 9985: 9964: 9963: 9937: 9931: 9930: 9920: 9888: 9882: 9881: 9879: 9877: 9863: 9857: 9856: 9854: 9852: 9838: 9832: 9831: 9829: 9827: 9816: 9810: 9809: 9777: 9771: 9770: 9750: 9744: 9743: 9741: 9739: 9730:. 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Archived from 6936:www.eatright.org 6928: 6922: 6921: 6919: 6917: 6908:. Archived from 6898: 6892: 6891: 6855: 6849: 6848: 6846: 6844: 6835:. Archived from 6825: 6819: 6818: 6800: 6776: 6770: 6769: 6733: 6727: 6726: 6698: 6692: 6691: 6681: 6649: 6643: 6642: 6640: 6638: 6623: 6617: 6610: 6604: 6600: 6594: 6593: 6591: 6589: 6584:on 19 April 2021 6580:. 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Herrick 2836: 2759:Northern Africa 2719:Northern Africa 2679:Southern Africa 2539:Southern Africa 2499:Northern Africa 2379:Northern Africa 2339:Southern Africa 2219:Southern Africa 2159:Northern Africa 1959:Southern Africa 1919:Northern Africa 1687:Population(2020) 1678: 1675: 1543:physical therapy 1466:hydroxycarbamide 1464:, also known as 1377:pain medications 869:left ventricular 847:pulmonary artery 804:B), followed by 765:immune reactions 682:may also occur. 403:James B. Herrick 364:hydroxycarbamide 295:, dizziness and 256:, is a group of 168:mutation in the 82:medical genetics 58: 35: 34: 21: 12724: 12723: 12719: 12718: 12717: 12715: 12714: 12713: 12674: 12673: 12672: 12662: 12660: 12652: 12650: 12645: 12587: 12576:Chief physician 12509: 12454: 12443: 12437:Travel medicine 12422:Sports medicine 12405:Sexual medicine 12345:Palliative care 12340:Pain management 12284:Family medicine 12262:Diving medicine 12208: 12136: 12098: 12091: 12007: 12000: 11959:Plastic surgery 11905:General surgery 11885:Cardiac surgery 11866: 11864: 11856: 11851: 11821: 11812: 11777: 11721: 11680: 11668: 11608: 11559: 11425: 11417: 11350: 11325: 11323:red blood cells 11316: 11286: 11281: 11280: 11212: 11211: 11113: 11099: 11094: 11088: 11071: 11065: 11050: 11044: 11029: 11023: 11008: 11004: 11003: 11002: 10987: 10986: 10982: 10975: 10973:Further reading 10970: 10969: 10938:(7): CD007001. 10924: 10920: 10910: 10908: 10898: 10894: 10849: 10845: 10806: 10802: 10792: 10790: 10782: 10781: 10777: 10724: 10720: 10710: 10708: 10696: 10692: 10647: 10643: 10629: 10625: 10611: 10607: 10597: 10595: 10584: 10580: 10570: 10568: 10558: 10554: 10507: 10503: 10458: 10454: 10417: 10410: 10401: 10400: 10396: 10388: 10382: 10378: 10368: 10366: 10355: 10354: 10350: 10340: 10338: 10327: 10326: 10322: 10275: 10271: 10261: 10259: 10254: 10253: 10246: 10236: 10234: 10229: 10228: 10224: 10179: 10166: 10129: 10125: 10115: 10113: 10105: 10104: 10087: 10040: 10033: 9986: 9967: 9952: 9938: 9934: 9889: 9885: 9875: 9873: 9865: 9864: 9860: 9850: 9848: 9840: 9839: 9835: 9825: 9823: 9818: 9817: 9813: 9778: 9774: 9751: 9747: 9737: 9735: 9734:on 5 March 2016 9722: 9721: 9717: 9670: 9666: 9619: 9615: 9560: 9556: 9546: 9544: 9535: 9534: 9530: 9515: 9511: 9474:; reprinted as 9454: 9450: 9413: 9404: 9389: 9385: 9330: 9326: 9313: 9312: 9308: 9292: 9291: 9267: 9263: 9253: 9251: 9238: 9237: 9233: 9186: 9182: 9157: 9153: 9106: 9102: 9055: 9051: 9034: 9033: 9029: 9012: 9011: 9007: 8990: 8989: 8985: 8968: 8967: 8963: 8946: 8945: 8941: 8902: 8898: 8851: 8847: 8824: 8820: 8777: 8773: 8763: 8761: 8752: 8751: 8747: 8718:(11): 472–480. 8708: 8704: 8694: 8692: 8684: 8683: 8679: 8669: 8667: 8658: 8657: 8653: 8643: 8641: 8632: 8631: 8627: 8617: 8615: 8606: 8605: 8601: 8591: 8589: 8580: 8579: 8575: 8565: 8563: 8552: 8548: 8511: 8507: 8457: 8453: 8414: 8410: 8400: 8398: 8394: 8387: 8381: 8377: 8363: 8359: 8314: 8310: 8273: 8269: 8232: 8228: 8221: 8205: 8201: 8164: 8160: 8150: 8148: 8146: 8130: 8126: 8087: 8083: 8072: 8068: 8058: 8056: 8040: 8039: 8032: 7987: 7983: 7940: 7936: 7926: 7924: 7914: 7910: 7900: 7898: 7890: 7889: 7885: 7872: 7870: 7859: 7855: 7810: 7806: 7766: 7762: 7725: 7721: 7676: 7672: 7662: 7660: 7650: 7646: 7607: 7603: 7566: 7562: 7522: 7518: 7473: 7466: 7435:(1): CD007843. 7421: 7417: 7386:(4): CD006110. 7372: 7368: 7361: 7347: 7343: 7326: 7319: 7282: 7275: 7265: 7263: 7258: 7257: 7253: 7222:(4): CD005406. 7208: 7204: 7165: 7161: 7116: 7112: 7081:(4): CD011130. 7067: 7063: 7050: 7048: 7044: 7040: 7039: 7035: 7025: 7023: 7008: 7004: 6973:(9): CD010858. 6959: 6955: 6945: 6943: 6942:on 19 June 2020 6930: 6929: 6925: 6915: 6913: 6900: 6899: 6895: 6856: 6852: 6842: 6840: 6827: 6826: 6822: 6777: 6773: 6734: 6730: 6709:(11): 997–999. 6699: 6695: 6650: 6646: 6636: 6634: 6624: 6620: 6611: 6607: 6601: 6597: 6587: 6585: 6572: 6571: 6567: 6557: 6555: 6547: 6546: 6542: 6532: 6530: 6521: 6520: 6516: 6475: 6471: 6464: 6448: 6444: 6437: 6421: 6417: 6411: 6387: 6383: 6373: 6371: 6362: 6361: 6357: 6347: 6345: 6336: 6335: 6331: 6316: 6302: 6298: 6259: 6255: 6216:Malaria Journal 6208: 6204: 6159: 6155: 6124: 6120: 6075: 6071: 6052: 6048: 6009: 6005: 5995: 5993: 5985: 5984: 5980: 5970: 5968: 5955: 5954: 5950: 5940: 5938: 5925: 5924: 5920: 5910: 5908: 5900: 5899: 5895: 5850: 5846: 5815: 5811: 5772: 5768: 5723: 5719: 5674: 5670: 5625: 5621: 5574: 5570: 5538:10.1.1.690.5870 5521: 5517: 5486: 5482: 5466: 5465: 5458: 5456: 5439: 5435: 5390: 5386: 5355: 5351: 5306: 5302: 5257: 5248: 5238: 5236: 5228: 5227: 5223: 5186: 5182: 5145: 5141: 5094: 5090: 5083: 5069: 5065: 5055: 5053: 5035: 5031: 4986: 4982: 4943: 4939: 4902: 4898: 4863: 4859: 4828: 4824: 4787: 4783: 4773: 4771: 4762: 4761: 4757: 4746: 4742: 4725: 4703: 4699: 4660: 4656: 4614: 4610: 4571: 4567: 4557: 4555: 4547: 4546: 4542: 4503: 4499: 4463: 4457: 4453: 4422:(5): CD001916. 4408: 4401: 4378: 4367: 4330: 4326: 4315: 4311: 4301: 4299: 4290: 4289: 4285: 4275: 4273: 4264: 4263: 4259: 4224: 4215: 4175: 4168: 4158: 4156: 4154:medlineplus.gov 4148: 4147: 4140: 4103: 4082: 4051: 4042: 4035: 4019: 4015: 3976: 3965: 3920: 3916: 3865: 3858: 3846: 3838: 3836: 3819: 3818: 3811: 3801: 3799: 3786: 3785: 3781: 3771: 3769: 3760: 3759: 3755: 3745: 3743: 3735: 3734: 3730: 3687: 3683: 3638: 3629: 3584: 3577: 3567: 3565: 3552: 3551: 3540: 3530: 3528: 3527:on 9 March 2016 3519: 3518: 3507: 3497: 3495: 3482: 3481: 3472: 3462: 3460: 3447: 3446: 3437: 3427: 3425: 3412: 3411: 3392: 3382: 3380: 3367: 3366: 3353: 3348: 3343: 3342: 3333: 3329: 3324: 3316: 3258: 3242: 3237: 3214: 3168: 3147: 3138: 3117: 3096:Social Security 3088: 3083: 3002:Ernest E. Irons 2997: 2985: 2961: 2959:India and Nepal 2934: 2925: 2897:cystic fibrosis 2885:phenylketonuria 2872:overseas France 2860: 2847:cystic fibrosis 2834: 2826: 2779:Eastern Africa 2739:Western Africa 2699:Eastern Africa 2659:Eastern Africa 2639:Western Africa 2619:Eastern Africa 2599:Western Africa 2559:Eastern Africa 2519:Eastern Africa 2479:Eastern Africa 2459:Western Africa 2439:Western Africa 2419:Eastern Africa 2399:Eastern Africa 2359:Western Africa 2319:Eastern Africa 2299:Western Africa 2279:Western Africa 2239:Eastern Africa 2199:Eastern Africa 2139:Eastern Africa 2119:Western Africa 2079:Eastern Africa 2019:Western Africa 1999:Eastern Africa 1979:Western Africa 1939:Western Africa 1899:Eastern Africa 1879:Eastern Africa 1859:Eastern Africa 1839:Western Africa 1819:Western Africa 1799:Western Africa 1779:Western Africa 1697:% of Prevalence 1656: 1625:cystic fibrosis 1612: 1603: 1587: 1563: 1515: 1507:ultrasonography 1492: 1459: 1434: 1412: 1372: 1351: 1331: 1314: 1308: 1290:or a sample of 1265:Genetic testing 1207: 1167: 1165:Pathophysiology 912: 851:right ventricle 688: 672: 661:G6PD deficiency 654: 629:fast heart rate 625:pale appearance 621:Aplastic crises 618: 616:Aplastic crisis 602: 565: 509: 485:aplastic crisis 476: 455: 415: 387:Southern Europe 356:pain medication 301:developed world 277:red blood cells 261:blood disorders 221:Life expectancy 205:pain medication 147:kidney problems 33: 28: 23: 22: 15: 12: 11: 5: 12722: 12712: 12711: 12706: 12701: 12696: 12691: 12686: 12671: 12670: 12647: 12646: 12644: 12643: 12633: 12623: 12613: 12603: 12592: 12589: 12588: 12586: 12585: 12580: 12579: 12578: 12568: 12563: 12558: 12553: 12548: 12543: 12538: 12533: 12528: 12523: 12517: 12515: 12514:Related topics 12511: 12510: 12508: 12507: 12506: 12505: 12495: 12490: 12485: 12480: 12475: 12470: 12465: 12463:Medical school 12459: 12457: 12449: 12448: 12445: 12444: 12442: 12441: 12440: 12439: 12429: 12424: 12419: 12417:Sleep medicine 12414: 12413: 12412: 12402: 12397: 12392: 12391: 12390: 12380: 12375: 12370: 12364: 12359: 12358: 12357: 12347: 12342: 12337: 12332: 12327: 12322: 12321: 12320: 12310: 12305: 12300: 12295: 12290: 12281: 12276: 12275: 12274: 12264: 12259: 12254: 12249: 12244: 12243: 12242: 12237: 12230:Anesthesiology 12227: 12222: 12216: 12214: 12210: 12209: 12207: 12206: 12205: 12204: 12199: 12194: 12189: 12184: 12179: 12169: 12168: 12167: 12162: 12160:Neuroradiology 12157: 12146: 12144: 12138: 12137: 12135: 12134: 12129: 12124: 12119: 12114: 12109: 12103: 12101: 12097:Obstetrics and 12093: 12092: 12090: 12089: 12084: 12079: 12074: 12069: 12064: 12059: 12054: 12049: 12048: 12047: 12037: 12032: 12027: 12022: 12012: 12010: 12002: 12001: 11999: 11998: 11993: 11992: 11991: 11981: 11979:Trauma surgery 11976: 11971: 11966: 11961: 11956: 11951: 11950: 11949: 11942:Otolaryngology 11939: 11934: 11929: 11924: 11919: 11918: 11917: 11912: 11902: 11897: 11892: 11887: 11881: 11879: 11870: 11868:subspecialties 11858: 11857: 11850: 11849: 11842: 11835: 11827: 11818: 11817: 11814: 11813: 11811: 11810: 11805: 11800: 11795: 11789: 11787: 11783: 11782: 11779: 11778: 11776: 11775: 11774: 11773: 11768: 11756: 11755: 11754: 11749: 11744: 11731: 11729: 11723: 11722: 11720: 11719: 11714: 11709: 11702: 11697: 11695:Fanconi anemia 11688: 11686: 11674: 11673: 11670: 11669: 11667: 11666: 11660: 11659: 11654: 11648: 11647: 11646: 11645: 11635: 11629: 11628: 11627: 11626: 11613: 11610: 11609: 11607: 11606: 11601: 11600: 11599: 11589: 11584: 11578: 11576: 11567: 11561: 11560: 11558: 11557: 11552: 11551: 11550: 11540: 11539: 11538: 11522: 11517: 11508: 11507: 11506: 11501: 11496: 11479: 11478: 11477: 11476: 11471: 11466: 11454: 11442: 11440: 11431: 11419: 11418: 11416: 11415: 11414: 11413: 11398: 11397: 11396: 11395: 11380: 11378: 11369: 11360: 11356: 11355: 11352: 11351: 11349: 11348: 11342: 11340: 11331: 11327: 11326: 11315: 11314: 11307: 11300: 11292: 11283: 11282: 11279: 11278: 11267: 11256: 11233: 11221: 11220: 11218: 11214: 11213: 11210: 11209: 11198: 11187: 11176: 11161: 11146: 11114: 11109: 11108: 11106: 11105:Classification 11098: 11097:External links 11095: 11093: 11092: 11086: 11069: 11063: 11048: 11042: 11027: 11021: 11005: 11001: 11000: 10995: 10989: 10988: 10977: 10976: 10974: 10971: 10968: 10967: 10918: 10902:"Gene Therapy" 10892: 10843: 10800: 10775: 10718: 10690: 10661:(9): 848–855. 10641: 10623: 10605: 10578: 10552: 10501: 10452: 10431:(4): 259–266. 10408: 10394: 10376: 10348: 10337:. 27 July 2022 10320: 10283:BMC Psychiatry 10269: 10244: 10222: 10164: 10143:(9): 442–449. 10123: 10085: 10031: 9965: 9950: 9932: 9903:(8): 675–686. 9883: 9858: 9833: 9811: 9792:(3): 209–216. 9772: 9761:(2): 200–206. 9745: 9715: 9664: 9613: 9554: 9528: 9525:(21): 490–493. 9509: 9488:(3): 179–184. 9464:(5): 179–184. 9448: 9402: 9383: 9344:(10): e26281. 9324: 9306: 9261: 9231: 9200:(4): 284–288. 9180: 9151: 9120:(3): 229–235. 9100: 9069:(3): 289–293. 9049: 9027: 9005: 8983: 8961: 8939: 8912:(6): 548–551. 8896: 8845: 8818: 8771: 8745: 8702: 8677: 8651: 8625: 8599: 8573: 8546: 8505: 8451: 8408: 8375: 8357: 8308: 8287:(7): 865–871. 8267: 8246:(8): 704–712. 8226: 8219: 8199: 8178:(5): 346–353. 8158: 8144: 8124: 8081: 8066: 8030: 8001:(3): 268–279. 7981: 7954:(8): 463–466. 7934: 7908: 7883: 7867:ScienceMag.org 7853: 7824:(6): 369–376. 7804: 7777:(3): 259–265. 7760: 7739:(1): 104–106. 7719: 7690:(6): 766–770. 7670: 7644: 7601: 7560: 7516: 7464: 7415: 7366: 7359: 7341: 7317: 7273: 7251: 7202: 7159: 7110: 7061: 7033: 7002: 6953: 6923: 6893: 6866:(4): 214–218. 6850: 6820: 6791:(6): 873–877. 6771: 6744:(6): 480–485. 6728: 6693: 6664:(3): 340–346. 6644: 6618: 6605: 6595: 6565: 6540: 6514: 6469: 6462: 6442: 6435: 6415: 6409: 6381: 6355: 6329: 6314: 6296: 6269:(3): 307–313. 6253: 6202: 6179:10.1086/432519 6173:(2): 171–192. 6153: 6134:(5): 499–505. 6118: 6089:(1): 532–537. 6069: 6046: 6019:(2): 145–146. 6003: 5991:www.nature.com 5978: 5948: 5918: 5893: 5864:(1): a011643. 5844: 5825:(8): 614–620. 5809: 5782:(11): e26607. 5766: 5737:(2): 205–213. 5717: 5688:(3): 243–252. 5668: 5639:(3): 416–424. 5619: 5588:(1): 115–130. 5568: 5515: 5496:(4): 359–377. 5480: 5433: 5404:(4): 482–490. 5384: 5365:(4): 468–474. 5349: 5320:(7): 622–625. 5300: 5246: 5221: 5180: 5139: 5088: 5081: 5063: 5029: 4980: 4937: 4916:(7): 814–815. 4896: 4877:(7): 609–619. 4857: 4822: 4801:(3): 247–262. 4781: 4755: 4740: 4723: 4697: 4670:(3): 191–207. 4654: 4632:10.1.1.504.790 4625:(6): 646–653. 4608: 4565: 4540: 4513:(3): 176–182. 4497: 4451: 4399: 4365: 4344:(6): 754–765. 4324: 4309: 4283: 4257: 4238:(2): 776–783. 4213: 4166: 4138: 4117:(5): 425–429. 4080: 4061:(2): 266–271. 4040: 4033: 4013: 3963: 3914: 3856: 3809: 3794:. 9 May 2016. 3779: 3753: 3728: 3681: 3627: 3575: 3538: 3505: 3470: 3435: 3390: 3350: 3349: 3347: 3344: 3341: 3340: 3326: 3325: 3323: 3320: 3315: 3312: 3257: 3254: 3241: 3238: 3236: 3233: 3213: 3212:United Kingdom 3210: 3197:social support 3167: 3164: 3146: 3143: 3137: 3134: 3116: 3113: 3087: 3084: 3082: 3079: 2996: 2993: 2984: 2981: 2965:Madhya Pradesh 2960: 2957: 2933: 2930: 2924: 2923:United Kingdom 2921: 2889:hypothyroidism 2859: 2856: 2825: 2822: 2819: 2818: 2813: 2808: 2806: 2801: 2796: 2790: 2789: 2786: 2783: 2780: 2777: 2774: 2770: 2769: 2766: 2763: 2760: 2757: 2754: 2750: 2749: 2746: 2743: 2740: 2737: 2734: 2730: 2729: 2726: 2723: 2722:0.03883333333 2720: 2717: 2714: 2710: 2709: 2706: 2703: 2700: 2697: 2694: 2690: 2689: 2686: 2683: 2680: 2677: 2674: 2670: 2669: 2666: 2663: 2660: 2657: 2654: 2650: 2649: 2646: 2643: 2640: 2637: 2634: 2630: 2629: 2626: 2623: 2620: 2617: 2614: 2610: 2609: 2606: 2603: 2600: 2597: 2594: 2590: 2589: 2586: 2583: 2580: 2579:Middle Africa 2577: 2574: 2570: 2569: 2566: 2563: 2560: 2557: 2554: 2550: 2549: 2546: 2543: 2542:0.03883333333 2540: 2537: 2534: 2530: 2529: 2526: 2523: 2520: 2517: 2514: 2510: 2509: 2506: 2503: 2500: 2497: 2494: 2490: 2489: 2486: 2483: 2480: 2477: 2474: 2470: 2469: 2466: 2463: 2460: 2457: 2454: 2450: 2449: 2446: 2443: 2440: 2437: 2434: 2430: 2429: 2426: 2423: 2420: 2417: 2414: 2410: 2409: 2406: 2403: 2400: 2397: 2394: 2390: 2389: 2386: 2383: 2380: 2377: 2374: 2370: 2369: 2366: 2363: 2360: 2357: 2354: 2350: 2349: 2346: 2343: 2340: 2337: 2334: 2330: 2329: 2326: 2323: 2320: 2317: 2314: 2310: 2309: 2306: 2303: 2300: 2297: 2294: 2293:Guinea-Bissau 2290: 2289: 2286: 2283: 2280: 2277: 2274: 2270: 2269: 2266: 2263: 2260: 2259:Middle Africa 2257: 2254: 2250: 2249: 2246: 2243: 2240: 2237: 2234: 2230: 2229: 2226: 2223: 2220: 2217: 2214: 2210: 2209: 2206: 2203: 2200: 2197: 2194: 2190: 2189: 2186: 2183: 2180: 2179:Middle Africa 2177: 2174: 2170: 2169: 2166: 2163: 2160: 2157: 2154: 2150: 2149: 2146: 2143: 2140: 2137: 2134: 2130: 2129: 2126: 2123: 2120: 2117: 2114: 2113:Côte d'Ivoire 2110: 2109: 2106: 2103: 2100: 2099:Middle Africa 2097: 2094: 2090: 2089: 2086: 2083: 2080: 2077: 2074: 2070: 2069: 2066: 2063: 2060: 2059:Middle Africa 2057: 2054: 2050: 2049: 2046: 2043: 2040: 2039:Middle Africa 2037: 2034: 2030: 2029: 2026: 2023: 2020: 2017: 2014: 2010: 2009: 2006: 2003: 2000: 1997: 1994: 1990: 1989: 1986: 1983: 1980: 1977: 1974: 1970: 1969: 1966: 1963: 1960: 1957: 1954: 1950: 1949: 1946: 1943: 1940: 1937: 1934: 1930: 1929: 1926: 1923: 1920: 1917: 1914: 1910: 1909: 1906: 1903: 1900: 1897: 1894: 1890: 1889: 1886: 1883: 1880: 1877: 1874: 1870: 1869: 1866: 1863: 1860: 1857: 1854: 1850: 1849: 1846: 1843: 1840: 1837: 1834: 1830: 1829: 1826: 1823: 1820: 1817: 1814: 1810: 1809: 1806: 1803: 1800: 1797: 1794: 1790: 1789: 1786: 1783: 1780: 1777: 1774: 1770: 1769: 1766: 1763: 1760: 1759:Middle Africa 1757: 1754: 1750: 1749: 1746: 1743: 1740: 1739:Middle Africa 1737: 1734: 1730: 1729: 1726: 1723: 1720: 1719:Middle Africa 1717: 1714: 1710: 1709: 1704: 1699: 1694: 1689: 1684: 1655: 1652: 1611: 1608: 1602: 1599: 1589:In 2023, both 1586: 1583: 1562: 1559: 1514: 1511: 1491: 1488: 1458: 1455: 1433: 1430: 1411: 1408: 1371: 1368: 1350: 1347: 1330: 1327: 1307: 1304: 1292:amniotic fluid 1206: 1203: 1183:oxygen tension 1166: 1163: 1155:Middle Passage 968:haemoglobin A2 911: 908: 907: 906: 903:kidney failure 883:kidney failure 879: 865:Cardiomyopathy 862: 853:and a risk of 840: 837: 829:, spontaneous 823: 820: 817: 816:in the kidneys 811: 785: 772: 761: 754: 739:Cholelithiasis 736: 729: 719: 687: 684: 671: 668: 653: 650: 633:parvovirus B19 617: 614: 601: 598: 564: 561: 508: 505: 475: 472: 454: 451: 414: 411: 368:gene therapies 241: 240: 239:114,800 (2015) 237: 233: 232: 229: 225: 224: 218: 212: 211: 190: 186: 185: 180: 174: 173: 162: 158: 157: 154: 150: 149: 116: 110: 109: 91: 85: 84: 75: 69: 68: 60: 59: 51: 50: 44: 40: 39: 31: 26: 9: 6: 4: 3: 2: 12721: 12710: 12707: 12705: 12702: 12700: 12697: 12695: 12692: 12690: 12687: 12685: 12682: 12681: 12679: 12669: 12659: 12658: 12655: 12642: 12634: 12632: 12628: 12624: 12622: 12614: 12612: 12604: 12602: 12594: 12593: 12590: 12584: 12581: 12577: 12574: 12573: 12572: 12569: 12567: 12564: 12562: 12559: 12557: 12554: 12552: 12549: 12547: 12546:Public health 12544: 12542: 12539: 12537: 12534: 12532: 12529: 12527: 12526:Allied health 12524: 12522: 12519: 12518: 12516: 12512: 12504: 12501: 12500: 12499: 12496: 12494: 12491: 12489: 12486: 12484: 12481: 12479: 12476: 12474: 12471: 12469: 12466: 12464: 12461: 12460: 12458: 12456: 12450: 12438: 12435: 12434: 12433: 12430: 12428: 12425: 12423: 12420: 12418: 12415: 12411: 12408: 12407: 12406: 12403: 12401: 12398: 12396: 12393: 12389: 12386: 12385: 12384: 12381: 12379: 12376: 12374: 12371: 12368: 12365: 12363: 12360: 12356: 12353: 12352: 12351: 12348: 12346: 12343: 12341: 12338: 12336: 12335:Oral medicine 12333: 12331: 12330:Ophthalmology 12328: 12326: 12323: 12319: 12316: 12315: 12314: 12311: 12309: 12306: 12304: 12301: 12299: 12296: 12294: 12291: 12289: 12285: 12282: 12280: 12277: 12273: 12270: 12269: 12268: 12265: 12263: 12260: 12258: 12255: 12253: 12250: 12248: 12245: 12241: 12238: 12236: 12233: 12232: 12231: 12228: 12226: 12223: 12221: 12218: 12217: 12215: 12211: 12203: 12200: 12198: 12195: 12193: 12192:Cytopathology 12190: 12188: 12185: 12183: 12180: 12178: 12175: 12174: 12173: 12170: 12166: 12163: 12161: 12158: 12156: 12153: 12152: 12151: 12148: 12147: 12145: 12143: 12139: 12133: 12132:Urogynecology 12130: 12128: 12125: 12123: 12120: 12118: 12115: 12113: 12110: 12108: 12105: 12104: 12102: 12100: 12094: 12088: 12085: 12083: 12080: 12078: 12075: 12073: 12070: 12068: 12065: 12063: 12060: 12058: 12055: 12053: 12050: 12046: 12043: 12042: 12041: 12038: 12036: 12035:Endocrinology 12033: 12031: 12028: 12026: 12023: 12021: 12017: 12014: 12013: 12011: 12009: 12003: 11997: 11994: 11990: 11987: 11986: 11985: 11982: 11980: 11977: 11975: 11972: 11970: 11967: 11965: 11962: 11960: 11957: 11955: 11952: 11945: 11944: 11943: 11940: 11938: 11935: 11933: 11930: 11928: 11925: 11923: 11920: 11916: 11913: 11911: 11908: 11907: 11906: 11903: 11901: 11898: 11896: 11893: 11891: 11888: 11886: 11883: 11882: 11880: 11878: 11874: 11871: 11869: 11863: 11859: 11855: 11848: 11843: 11841: 11836: 11834: 11829: 11828: 11825: 11809: 11806: 11804: 11801: 11799: 11796: 11794: 11791: 11790: 11788: 11784: 11772: 11769: 11767: 11764: 11763: 11762: 11761: 11757: 11753: 11750: 11748: 11745: 11743: 11740: 11739: 11738: 11737: 11733: 11732: 11730: 11728: 11724: 11718: 11717:Myelophthisic 11715: 11713: 11710: 11708: 11704: 11703: 11701: 11698: 11696: 11692: 11689: 11687: 11684: 11679: 11675: 11665: 11662: 11661: 11658: 11655: 11653: 11650: 11649: 11644: 11641: 11640: 11639: 11636: 11634: 11631: 11630: 11625: 11622: 11621: 11620: 11619: 11615: 11614: 11605: 11602: 11598: 11595: 11594: 11593: 11590: 11588: 11585: 11583: 11580: 11579: 11577: 11575: 11571: 11568: 11566: 11562: 11556: 11553: 11549: 11546: 11545: 11544: 11541: 11537: 11534: 11533: 11532: 11529: 11527: 11523: 11521: 11518: 11516: 11512: 11509: 11505: 11502: 11500: 11497: 11495: 11492: 11491: 11490: 11487: 11485: 11481: 11480: 11475: 11472: 11470: 11467: 11465: 11462: 11461: 11460: 11459: 11455: 11453: 11450: 11448: 11444: 11443: 11441: 11439: 11435: 11432: 11429: 11424: 11420: 11412: 11409: 11408: 11407: 11403: 11400: 11399: 11394: 11391: 11390: 11389: 11385: 11382: 11381: 11379: 11377: 11373: 11370: 11368: 11364: 11361: 11357: 11347: 11344: 11343: 11341: 11339: 11335: 11332: 11328: 11324: 11320: 11313: 11308: 11306: 11301: 11299: 11294: 11293: 11290: 11277: 11273: 11272: 11268: 11266: 11262: 11261: 11257: 11255: 11252: 11249: 11246: 11243: 11239: 11238: 11234: 11232: 11228: 11227: 11223: 11222: 11219: 11215: 11208: 11204: 11203: 11199: 11197: 11193: 11192: 11188: 11186: 11182: 11181: 11177: 11175: 11171: 11170: 11166: 11162: 11160: 11156: 11155: 11151: 11147: 11145: 11141: 11137: 11133: 11129: 11125: 11124: 11120: 11116: 11115: 11112: 11107: 11103: 11089: 11083: 11078: 11077: 11070: 11066: 11060: 11056: 11055: 11049: 11045: 11039: 11035: 11034: 11028: 11024: 11018: 11014: 11013: 11007: 11006: 10999: 10996: 10994: 10991: 10990: 10985: 10980: 10963: 10959: 10954: 10949: 10945: 10941: 10937: 10933: 10929: 10922: 10907: 10903: 10896: 10888: 10884: 10879: 10874: 10870: 10866: 10862: 10858: 10854: 10847: 10839: 10835: 10831: 10827: 10823: 10819: 10815: 10811: 10804: 10789: 10785: 10779: 10771: 10767: 10762: 10757: 10753: 10749: 10745: 10741: 10737: 10733: 10729: 10722: 10707: 10706: 10701: 10694: 10686: 10682: 10678: 10674: 10669: 10664: 10660: 10656: 10652: 10645: 10639: 10635: 10634: 10627: 10621: 10617: 10616: 10609: 10593: 10589: 10582: 10567: 10563: 10556: 10548: 10544: 10540: 10536: 10532: 10528: 10524: 10520: 10516: 10512: 10505: 10497: 10493: 10488: 10483: 10479: 10475: 10471: 10467: 10463: 10456: 10448: 10444: 10439: 10434: 10430: 10426: 10422: 10415: 10413: 10404: 10398: 10387: 10380: 10364: 10363: 10358: 10352: 10336: 10335: 10330: 10324: 10316: 10312: 10307: 10302: 10297: 10292: 10288: 10284: 10280: 10273: 10258:. 13 May 2017 10257: 10251: 10249: 10233:. 13 May 2017 10232: 10226: 10218: 10214: 10209: 10204: 10200: 10196: 10192: 10188: 10184: 10177: 10175: 10173: 10171: 10169: 10160: 10156: 10151: 10146: 10142: 10138: 10134: 10127: 10112: 10108: 10102: 10100: 10098: 10096: 10094: 10092: 10090: 10081: 10077: 10072: 10067: 10062: 10057: 10053: 10049: 10045: 10038: 10036: 10027: 10023: 10018: 10013: 10008: 10003: 9999: 9995: 9991: 9984: 9982: 9980: 9978: 9976: 9974: 9972: 9970: 9961: 9957: 9953: 9947: 9943: 9936: 9928: 9924: 9919: 9914: 9910: 9906: 9902: 9898: 9894: 9887: 9872: 9868: 9862: 9847: 9843: 9837: 9821: 9815: 9807: 9803: 9799: 9795: 9791: 9787: 9783: 9776: 9768: 9764: 9760: 9757:(in French). 9756: 9749: 9733: 9729: 9725: 9719: 9711: 9707: 9703: 9699: 9695: 9691: 9687: 9683: 9679: 9675: 9668: 9660: 9656: 9652: 9648: 9644: 9640: 9636: 9632: 9628: 9624: 9617: 9609: 9605: 9601: 9597: 9593: 9589: 9584:Reprinted in 9581: 9577: 9573: 9569: 9565: 9558: 9542: 9538: 9532: 9524: 9520: 9513: 9505: 9501: 9496: 9491: 9487: 9483: 9479: 9471: 9467: 9463: 9459: 9452: 9444: 9440: 9435: 9430: 9426: 9422: 9418: 9411: 9409: 9407: 9398: 9394: 9387: 9379: 9375: 9370: 9365: 9360: 9355: 9351: 9347: 9343: 9339: 9335: 9328: 9320: 9316: 9310: 9302: 9296: 9287: 9282: 9278: 9274: 9273: 9265: 9249: 9245: 9241: 9235: 9227: 9223: 9218: 9213: 9208: 9203: 9199: 9195: 9191: 9184: 9176: 9172: 9168: 9167: 9162: 9155: 9147: 9143: 9138: 9133: 9128: 9123: 9119: 9115: 9111: 9104: 9096: 9092: 9087: 9082: 9077: 9072: 9068: 9064: 9060: 9053: 9045: 9041: 9037: 9031: 9023: 9019: 9015: 9009: 9001: 8997: 8993: 8987: 8979: 8975: 8971: 8965: 8957: 8953: 8949: 8943: 8935: 8931: 8927: 8923: 8919: 8915: 8911: 8907: 8900: 8892: 8888: 8883: 8878: 8873: 8868: 8864: 8860: 8856: 8849: 8841: 8837: 8833: 8830:(in French). 8829: 8822: 8814: 8810: 8806: 8802: 8798: 8794: 8790: 8786: 8782: 8775: 8759: 8755: 8749: 8741: 8737: 8733: 8729: 8725: 8721: 8717: 8713: 8706: 8691: 8687: 8681: 8665: 8661: 8655: 8639: 8635: 8629: 8613: 8609: 8603: 8587: 8583: 8577: 8561: 8557: 8550: 8542: 8538: 8533: 8528: 8524: 8520: 8516: 8509: 8501: 8497: 8492: 8487: 8482: 8477: 8473: 8469: 8465: 8461: 8455: 8447: 8443: 8439: 8435: 8431: 8427: 8423: 8419: 8412: 8393: 8386: 8379: 8371: 8367: 8361: 8353: 8349: 8344: 8339: 8335: 8331: 8327: 8323: 8319: 8312: 8304: 8300: 8295: 8290: 8286: 8282: 8281:Haematologica 8278: 8271: 8263: 8259: 8254: 8249: 8245: 8241: 8237: 8230: 8222: 8216: 8212: 8211: 8203: 8195: 8191: 8186: 8181: 8177: 8173: 8169: 8162: 8147: 8145:9783319067131 8141: 8137: 8136: 8128: 8120: 8116: 8112: 8108: 8104: 8100: 8096: 8092: 8085: 8077: 8070: 8054: 8050: 8048: 8043: 8037: 8035: 8026: 8022: 8017: 8012: 8008: 8004: 8000: 7996: 7992: 7985: 7977: 7973: 7969: 7965: 7961: 7957: 7953: 7949: 7945: 7938: 7923: 7919: 7912: 7897: 7893: 7887: 7880: 7868: 7864: 7857: 7849: 7845: 7841: 7837: 7832: 7827: 7823: 7819: 7815: 7808: 7800: 7796: 7792: 7788: 7784: 7780: 7776: 7772: 7764: 7756: 7752: 7747: 7742: 7738: 7734: 7730: 7723: 7715: 7711: 7707: 7703: 7698: 7693: 7689: 7685: 7681: 7674: 7659: 7655: 7648: 7640: 7636: 7632: 7628: 7624: 7620: 7616: 7612: 7605: 7597: 7593: 7588: 7583: 7579: 7575: 7571: 7564: 7556: 7552: 7547: 7542: 7538: 7534: 7530: 7526: 7520: 7512: 7508: 7503: 7498: 7494: 7490: 7486: 7482: 7478: 7471: 7469: 7460: 7456: 7451: 7446: 7442: 7438: 7434: 7430: 7426: 7419: 7411: 7407: 7402: 7397: 7393: 7389: 7385: 7381: 7377: 7370: 7362: 7356: 7352: 7345: 7337: 7336: 7331: 7324: 7322: 7313: 7309: 7304: 7299: 7295: 7291: 7287: 7280: 7278: 7261: 7255: 7247: 7243: 7238: 7233: 7229: 7225: 7221: 7217: 7213: 7206: 7198: 7194: 7190: 7186: 7182: 7178: 7174: 7170: 7163: 7155: 7151: 7146: 7141: 7137: 7133: 7129: 7125: 7121: 7114: 7106: 7102: 7097: 7092: 7088: 7084: 7080: 7076: 7072: 7065: 7058: 7043: 7037: 7021: 7017: 7013: 7006: 6998: 6994: 6989: 6984: 6980: 6976: 6972: 6968: 6964: 6957: 6941: 6937: 6933: 6927: 6911: 6907: 6903: 6897: 6889: 6885: 6881: 6877: 6873: 6869: 6865: 6861: 6854: 6838: 6834: 6830: 6824: 6816: 6812: 6808: 6804: 6799: 6794: 6790: 6786: 6782: 6775: 6767: 6763: 6759: 6755: 6751: 6747: 6743: 6739: 6732: 6724: 6720: 6716: 6712: 6708: 6704: 6697: 6689: 6685: 6680: 6675: 6671: 6667: 6663: 6659: 6655: 6648: 6633: 6629: 6622: 6615: 6609: 6599: 6583: 6579: 6575: 6569: 6554: 6550: 6544: 6528: 6524: 6518: 6510: 6506: 6502: 6497: 6492: 6488: 6484: 6480: 6473: 6465: 6459: 6455: 6454: 6446: 6438: 6432: 6428: 6427: 6419: 6412: 6406: 6401: 6400:10.5772/31233 6396: 6392: 6385: 6369: 6365: 6359: 6343: 6339: 6333: 6325: 6321: 6317: 6315:9780803615717 6311: 6307: 6300: 6292: 6288: 6284: 6280: 6276: 6272: 6268: 6264: 6257: 6249: 6245: 6240: 6235: 6230: 6225: 6221: 6217: 6213: 6206: 6198: 6194: 6189: 6184: 6180: 6176: 6172: 6168: 6164: 6157: 6149: 6145: 6141: 6137: 6133: 6129: 6122: 6114: 6110: 6105: 6100: 6096: 6092: 6088: 6084: 6080: 6073: 6065: 6061: 6057: 6050: 6042: 6038: 6034: 6030: 6026: 6022: 6018: 6014: 6007: 5992: 5988: 5982: 5966: 5962: 5958: 5952: 5936: 5932: 5928: 5922: 5907: 5903: 5897: 5889: 5885: 5880: 5875: 5871: 5867: 5863: 5859: 5855: 5848: 5840: 5836: 5832: 5828: 5824: 5820: 5813: 5805: 5801: 5797: 5793: 5789: 5785: 5781: 5777: 5770: 5762: 5758: 5753: 5748: 5744: 5740: 5736: 5732: 5728: 5721: 5713: 5709: 5704: 5699: 5695: 5691: 5687: 5683: 5679: 5672: 5664: 5660: 5656: 5652: 5647: 5642: 5638: 5634: 5630: 5623: 5615: 5611: 5606: 5601: 5596: 5591: 5587: 5583: 5579: 5572: 5564: 5560: 5556: 5552: 5548: 5544: 5539: 5534: 5531:(2): 94–101. 5530: 5526: 5519: 5511: 5507: 5503: 5499: 5495: 5491: 5484: 5476: 5470: 5454: 5450: 5449: 5444: 5437: 5429: 5425: 5421: 5417: 5412: 5407: 5403: 5399: 5395: 5388: 5380: 5376: 5372: 5368: 5364: 5360: 5353: 5345: 5341: 5336: 5331: 5327: 5323: 5319: 5315: 5311: 5304: 5296: 5292: 5287: 5282: 5278: 5274: 5270: 5266: 5262: 5255: 5253: 5251: 5235: 5231: 5225: 5217: 5213: 5208: 5203: 5199: 5195: 5191: 5184: 5176: 5172: 5167: 5162: 5158: 5154: 5150: 5143: 5135: 5131: 5127: 5123: 5119: 5115: 5111: 5107: 5103: 5099: 5092: 5084: 5078: 5074: 5067: 5052: 5048: 5044: 5040: 5033: 5025: 5021: 5017: 5013: 5008: 5003: 4999: 4995: 4991: 4984: 4976: 4972: 4968: 4964: 4960: 4956: 4952: 4948: 4941: 4933: 4929: 4924: 4919: 4915: 4911: 4907: 4900: 4892: 4888: 4884: 4880: 4876: 4872: 4868: 4861: 4853: 4849: 4845: 4841: 4837: 4833: 4826: 4818: 4814: 4809: 4804: 4800: 4796: 4792: 4785: 4769: 4765: 4759: 4751: 4744: 4735: 4730: 4726: 4720: 4716: 4712: 4708: 4701: 4693: 4689: 4685: 4681: 4677: 4673: 4669: 4665: 4658: 4650: 4646: 4642: 4638: 4633: 4628: 4624: 4620: 4612: 4604: 4600: 4596: 4592: 4588: 4584: 4580: 4576: 4569: 4554: 4550: 4544: 4536: 4532: 4528: 4524: 4520: 4516: 4512: 4508: 4501: 4493: 4489: 4485: 4481: 4477: 4473: 4469: 4462: 4455: 4447: 4443: 4438: 4433: 4429: 4425: 4421: 4417: 4413: 4406: 4404: 4395: 4391: 4387: 4383: 4376: 4374: 4372: 4370: 4361: 4357: 4352: 4347: 4343: 4339: 4335: 4328: 4320: 4313: 4297: 4293: 4287: 4271: 4267: 4261: 4253: 4249: 4245: 4241: 4237: 4233: 4229: 4222: 4220: 4218: 4209: 4205: 4201: 4197: 4193: 4189: 4185: 4181: 4173: 4171: 4155: 4151: 4145: 4143: 4134: 4130: 4125: 4120: 4116: 4112: 4108: 4101: 4099: 4097: 4095: 4093: 4091: 4089: 4087: 4085: 4076: 4072: 4068: 4064: 4060: 4056: 4049: 4047: 4045: 4036: 4034:9783642022012 4030: 4026: 4025: 4017: 4009: 4005: 4001: 3997: 3993: 3989: 3985: 3981: 3974: 3972: 3970: 3968: 3959: 3955: 3950: 3945: 3941: 3937: 3933: 3929: 3925: 3918: 3910: 3906: 3902: 3898: 3894: 3890: 3886: 3882: 3878: 3874: 3870: 3863: 3861: 3853: 3852:public domain 3834: 3830: 3828: 3822: 3816: 3814: 3797: 3793: 3789: 3783: 3767: 3763: 3757: 3742: 3738: 3732: 3724: 3720: 3716: 3712: 3708: 3704: 3700: 3696: 3692: 3685: 3677: 3673: 3668: 3663: 3659: 3655: 3651: 3647: 3643: 3636: 3634: 3632: 3623: 3619: 3614: 3609: 3605: 3601: 3597: 3593: 3589: 3582: 3580: 3563: 3559: 3555: 3549: 3547: 3545: 3543: 3526: 3522: 3516: 3514: 3512: 3510: 3493: 3489: 3485: 3479: 3477: 3475: 3458: 3454: 3450: 3444: 3442: 3440: 3423: 3419: 3415: 3409: 3407: 3405: 3403: 3401: 3399: 3397: 3395: 3378: 3374: 3370: 3364: 3362: 3360: 3358: 3356: 3351: 3337: 3331: 3327: 3319: 3311: 3309: 3305: 3300: 3296: 3293: 3289: 3285: 3280: 3277: 3273: 3271: 3266: 3263: 3253: 3251: 3247: 3232: 3229: 3224: 3220: 3219:Crizanlizumab 3209: 3206: 3202: 3198: 3194: 3189: 3184: 3181: 3176: 3174: 3163: 3161: 3157: 3153: 3142: 3133: 3131: 3126: 3125:social stigma 3122: 3112: 3109: 3104: 3099: 3097: 3093: 3086:United States 3078: 3075: 3073: 3072: 3067: 3061: 3059: 3055: 3054:Vernon Ingram 3051: 3050:Linus Pauling 3047: 3046:James V. Neel 3043: 3038: 3035: 3031: 3027: 3023: 3019: 3016:. He died of 3015: 3011: 3007: 3003: 2992: 2990: 2980: 2978: 2974: 2970: 2966: 2956: 2954: 2950: 2945: 2943: 2939: 2929: 2920: 2918: 2914: 2904: 2900: 2898: 2894: 2890: 2886: 2882: 2877: 2873: 2864: 2855: 2852: 2848: 2844: 2839: 2831: 2830:United States 2824:United States 2817: 2814: 2812: 2809: 2807: 2805: 2802: 2800: 2799:1,338,826,604 2797: 2795: 2792: 2791: 2787: 2784: 2781: 2778: 2775: 2772: 2771: 2767: 2764: 2761: 2758: 2755: 2752: 2751: 2747: 2744: 2741: 2738: 2735: 2732: 2731: 2727: 2724: 2721: 2718: 2715: 2712: 2711: 2707: 2704: 2701: 2698: 2695: 2692: 2691: 2687: 2684: 2681: 2678: 2675: 2673:South Africa 2672: 2671: 2667: 2664: 2661: 2658: 2655: 2652: 2651: 2647: 2644: 2641: 2638: 2635: 2633:Sierra Leone 2632: 2631: 2627: 2624: 2621: 2618: 2615: 2612: 2611: 2607: 2604: 2601: 2598: 2595: 2592: 2591: 2587: 2584: 2581: 2578: 2575: 2572: 2571: 2567: 2564: 2561: 2558: 2555: 2552: 2551: 2547: 2544: 2541: 2538: 2535: 2532: 2531: 2527: 2524: 2521: 2518: 2515: 2512: 2511: 2507: 2504: 2501: 2498: 2495: 2492: 2491: 2487: 2484: 2481: 2478: 2475: 2472: 2471: 2467: 2464: 2461: 2458: 2455: 2452: 2451: 2447: 2444: 2441: 2438: 2435: 2432: 2431: 2427: 2424: 2421: 2418: 2415: 2412: 2411: 2407: 2404: 2401: 2398: 2395: 2392: 2391: 2387: 2384: 2381: 2378: 2375: 2372: 2371: 2367: 2364: 2361: 2358: 2355: 2352: 2351: 2347: 2344: 2341: 2338: 2335: 2332: 2331: 2327: 2324: 2321: 2318: 2315: 2312: 2311: 2307: 2304: 2301: 2298: 2295: 2292: 2291: 2287: 2284: 2281: 2278: 2275: 2272: 2271: 2267: 2264: 2261: 2258: 2255: 2252: 2251: 2247: 2244: 2241: 2238: 2235: 2232: 2231: 2227: 2224: 2221: 2218: 2215: 2212: 2211: 2207: 2204: 2201: 2198: 2195: 2192: 2191: 2187: 2184: 2181: 2178: 2175: 2172: 2171: 2167: 2164: 2161: 2158: 2155: 2152: 2151: 2147: 2144: 2141: 2138: 2135: 2132: 2131: 2127: 2124: 2121: 2118: 2115: 2112: 2111: 2107: 2104: 2101: 2098: 2095: 2092: 2091: 2087: 2084: 2081: 2078: 2075: 2072: 2071: 2067: 2064: 2061: 2058: 2055: 2052: 2051: 2047: 2044: 2041: 2038: 2035: 2032: 2031: 2027: 2024: 2021: 2018: 2015: 2012: 2011: 2007: 2004: 2001: 1998: 1995: 1992: 1991: 1987: 1984: 1981: 1978: 1975: 1973:Burkina Faso 1972: 1971: 1967: 1964: 1961: 1958: 1955: 1952: 1951: 1947: 1944: 1942:0.1286666667 1941: 1938: 1935: 1932: 1931: 1927: 1924: 1921: 1918: 1915: 1912: 1911: 1907: 1904: 1901: 1898: 1895: 1892: 1891: 1887: 1884: 1881: 1878: 1875: 1872: 1871: 1867: 1864: 1861: 1858: 1855: 1852: 1851: 1847: 1844: 1842:0.1286666667 1841: 1838: 1835: 1832: 1831: 1827: 1824: 1821: 1818: 1815: 1812: 1811: 1807: 1804: 1801: 1798: 1795: 1792: 1791: 1787: 1784: 1781: 1778: 1775: 1772: 1771: 1767: 1764: 1762:0.1163333333 1761: 1758: 1755: 1752: 1751: 1747: 1744: 1741: 1738: 1735: 1732: 1731: 1727: 1724: 1721: 1718: 1715: 1712: 1711: 1708: 1705: 1703: 1700: 1698: 1695: 1693: 1690: 1688: 1685: 1683: 1680: 1679: 1674: 1672: 1668: 1665: 1661: 1651: 1647: 1645: 1641: 1637: 1633: 1628: 1626: 1622: 1618: 1607: 1598: 1596: 1592: 1582: 1580: 1576: 1572: 1568: 1558: 1556: 1555:bone grafting 1552: 1548: 1544: 1540: 1535: 1531: 1530: 1526: 1524: 1519: 1510: 1508: 1505: 1501: 1496: 1487: 1485: 1481: 1479: 1475: 1469: 1467: 1463: 1454: 1451: 1447: 1442: 1440: 1429: 1426: 1422: 1416: 1407: 1405: 1401: 1400:Crizanlizumab 1396: 1392: 1390: 1386: 1382: 1378: 1367: 1365: 1355: 1346: 1344: 1340: 1336: 1326: 1324: 1320: 1313: 1303: 1299: 1297: 1293: 1289: 1284: 1280: 1275: 1273: 1268: 1266: 1262: 1258: 1257:haemoglobin C 1254: 1250: 1245: 1243: 1239: 1234: 1232: 1228: 1224: 1220: 1216: 1212: 1202: 1200: 1196: 1191: 1189: 1184: 1175: 1171: 1162: 1160: 1156: 1152: 1148: 1144: 1139: 1136: 1135: 1128: 1126: 1122: 1118: 1117:Mediterranean 1114: 1109: 1107: 1103: 1102:chromosome 11 1099: 1091: 1090:chromosome 11 1087: 1083: 1082: 1077: 1073: 1071: 1067: 1063: 1059: 1055: 1051: 1047: 1042: 1040: 1036: 1032: 1028: 1024: 1020: 1016: 1012: 1008: 1004: 1000: 997: 992: 988: 985: 980: 977: 973: 972:haemoglobin F 969: 965: 964:haemoglobin A 956: 948: 941: 936: 928: 921: 916: 904: 900: 896: 892: 888: 884: 880: 877: 873: 870: 866: 863: 860: 856: 855:heart failure 852: 848: 844: 841: 838: 836: 835:pre-eclampsia 832: 828: 824: 821: 818: 815: 812: 809: 808: 803: 799: 795: 794: 789: 788:Osteomyelitis 786: 784: 780: 776: 773: 770: 766: 762: 759: 755: 752: 748: 744: 743:cholecystitis 740: 737: 733: 732:Silent stroke 730: 727: 723: 720: 717: 716:S. pneumoniae 713: 709: 708: 703: 702: 697: 693: 692: 691: 686:Complications 683: 681: 677: 667: 664: 662: 658: 649: 647: 643: 638: 634: 630: 626: 622: 613: 610: 606: 597: 594: 588: 586: 582: 578: 577:spleen damage 574: 570: 560: 558: 554: 550: 546: 542: 538: 534: 530: 526: 522: 518: 514: 504: 502: 498: 494: 490: 486: 482: 471: 468: 463: 461: 450: 448: 447:complications 438: 430: 423: 419: 410: 408: 404: 400: 396: 392: 388: 384: 380: 375: 373: 369: 365: 361: 357: 353: 349: 345: 340: 338: 334: 330: 326: 322: 318: 317:chromosome 11 314: 310: 309:β-globin gene 305: 302: 298: 294: 290: 287:) in joints, 286: 282: 278: 274: 270: 266: 262: 259: 255: 251: 247: 238: 234: 230: 226: 222: 219: 217: 213: 210: 206: 202: 198: 194: 191: 187: 184: 181: 179: 175: 171: 167: 163: 159: 155: 151: 148: 144: 140: 136: 132: 128: 124: 120: 117: 115: 114:Complications 111: 108: 104: 100: 96: 92: 90: 86: 83: 79: 76: 74: 70: 66: 61: 57: 52: 49: 45: 41: 36: 30: 19: 12551:Rural health 12536:Nanomedicine 12087:Rheumatology 12018: / 11937:Hand surgery 11922:Neurosurgery 11766:normochromic 11758: 11734: 11616: 11524: 11510: 11482: 11456: 11445: 11338:Polycythemia 11269: 11258: 11235: 11224: 11200: 11189: 11178: 11163: 11148: 11117: 11075: 11053: 11032: 11011: 10983: 10935: 10931: 10921: 10909:. 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Retrieved 3372: 3330: 3317: 3301: 3297: 3281: 3274: 3270:gene therapy 3267: 3259: 3256:Gene therapy 3243: 3215: 3185: 3177: 3169: 3148: 3139: 3118: 3100: 3089: 3076: 3069: 3062: 3042:Lemuel Diggs 3039: 3025: 2998: 2986: 2977:Tharu people 2973:Chhattisgarh 2962: 2946: 2938:Saudi Arabia 2935: 2926: 2909: 2869: 2840: 2827: 2815: 2810: 2803: 2798: 2793: 2693:South Sudan 2236:114,963,588 2156:102,334,404 1836:206,139,589 1706: 1701: 1696: 1691: 1686: 1681: 1669: 1657: 1648: 1629: 1613: 1610:Epidemiology 1604: 1588: 1585:Gene therapy 1564: 1536: 1532: 1528: 1527: 1522: 1516: 1493: 1482: 1478:chemotherapy 1470: 1460: 1443: 1435: 1425:generic drug 1420: 1417: 1413: 1397: 1393: 1373: 1360: 1332: 1315: 1300: 1276: 1269: 1251:, a form of 1246: 1235: 1227:target cells 1223:hyposplenism 1215:reticulocyte 1209:In HbS, the 1208: 1192: 1179: 1140: 1132: 1129: 1110: 1095: 1079: 1046:heterozygous 1043: 1005:– SNP) (GAG 993: 989: 981: 961: 891:hypertension 805: 801: 797: 791: 769:hyposplenism 715: 705: 699: 689: 673: 665: 655: 642:Reticulocyte 619: 603: 589: 566: 510: 477: 464: 456: 453:First events 443: 395:North Africa 376: 341: 312: 306: 284: 268: 253: 249: 245: 244: 170:hemoglobin S 119:Chronic pain 47: 29: 12621:Wikiproject 12410:Venereology 12355:Neonatology 12252:Dermatology 12107:Gynaecology 12099:gynaecology 12082:Pulmonology 11900:Eye surgery 11862:Specialties 11771:hypochromic 11727:Blood tests 11489:Thalassemia 11447:enzymopathy 11376:Nutritional 11260:GeneReviews 11226:MedlinePlus 10633:NCT00012545 10615:NCT02247843 10598:17 December 10571:17 December 10000:: 145–152. 9738:25 February 8865:: 1215605. 8764:12 November 8644:13 December 8592:13 December 8566:27 November 8401:27 November 7927:17 November 7296:(1): 5–11. 7266:12 December 7051:16 November 7016:www.fda.gov 6843:24 February 6637:23 February 6588:23 February 6558:24 February 6533:27 November 6374:27 November 6348:27 November 4838:(1): 3–13. 4768:www.cdc.gov 4302:27 November 3252:may occur. 3173:self-esteem 3034:Verne Mason 2942:thalassemia 2883:, ahead of 2776:14,862,924 2756:11,818,619 2716:43,849,260 2696:11,193,725 2676:59,308,690 2656:15,893,222 2613:Seychelles 2596:16,743,927 2556:12,952,218 2516:31,255,435 2513:Mozambique 2496:36,910,560 2453:Mauritania 2436:20,250,833 2416:19,129,952 2396:27,691,018 2393:Madagascar 2316:53,771,296 2116:26,378,274 2056:16,425,864 2013:Cabo Verde 1996:11,890,784 1976:20,903,273 1936:12,123,200 1916:43,851,044 1896:18,383,955 1876:45,741,007 1856:59,734,218 1845:26,523,294 1816:24,206,644 1796:13,132,795 1776:31,072,940 1765:10,418,977 1756:89,561,403 1736:26,545,863 1716:32,866,272 1636:subtropical 1621:haemophilia 1462:Hydroxyurea 1457:Hydroxyurea 1323:L-glutamine 1199:bone marrow 1143:West Africa 1125:Middle East 1066:capillaries 1039:polymerizes 876:arrhythmias 593:hypovolemic 557:atelectasis 348:antibiotics 344:vaccination 325:dehydration 273:haemoglobin 254:sickle cell 197:antibiotics 193:Vaccination 153:Usual onset 65:haemoglobin 43:Other names 18:Sickle cell 12678:Categories 12383:Psychiatry 12369:(PM&R) 12362:Phlebology 12350:Pediatrics 12177:Anatomical 12142:Diagnostic 12122:Obstetrics 12072:Nephrology 12057:Hematology 12052:Geriatrics 12045:Hepatology 12030:Cardiology 12020:Immunology 11752:macrocytic 11747:microcytic 11742:normocytic 11705:Acquired: 11691:Hereditary 11458:glycolysis 11438:Hereditary 11202:DiseasesDB 10711:28 January 10054:(1): 561. 9851:15 January 9826:15 January 9547:28 January 9279:(1): 1–4. 9018:Give Blood 8974:www.nhs.uk 8952:www.nhs.uk 8670:11 October 8618:6 February 8474:(1): 561. 8059:8 December 7873:7 December 7663:9 December 7525:Charache S 6393:, InTech, 5996:12 October 5102:Pediatrics 5043:StatPearls 4159:22 October 3839:8 December 3802:23 January 3346:References 3336:methionine 3156:witchcraft 3066:Bill Cosby 2811:91,868,664 2736:8,278,724 2725:1,702,813 2685:1,719,952 2645:1,288,283 2636:7,976,983 2605:1,176,261 2536:2,540,905 2525:1,093,940 2505:1,070,406 2476:1,271,768 2473:Mauritius 2456:4,649,658 2445:1,660,568 2405:1,294,555 2376:6,871,292 2356:5,057,681 2336:2,142,249 2325:2,513,808 2296:1,968,001 2276:2,416,668 2256:2,225,734 2245:3,333,944 2216:1,160,164 2196:3,546,421 2176:1,402,985 2165:2,967,698 2125:1,853,074 2096:5,518,087 2036:4,829,767 1985:1,468,455 1956:2,351,627 1945:1,559,852 1925:1,271,680 1905:1,507,484 1885:3,213,306 1865:3,255,515 1825:1,700,517 1805:1,830,383 1785:2,913,088 1745:3,105,866 1725:3,081,213 1702:Prevalence 1404:p-selectin 1385:diclofenac 1339:folic acid 1335:penicillin 1306:Management 1219:blood film 1195:haemolysis 1134:Plasmodium 1123:, and the 1070:dehydrated 1058:homozygous 1044:In people 996:nucleotide 819:Leg ulcers 793:Salmonella 779:infarction 763:Decreased 751:haemolysis 712:penicillin 676:dactylitis 541:diclofenac 525:analgesics 460:dactylitis 422:Dactylitis 397:and among 352:folic acid 337:blood test 263:typically 201:folic acid 183:Blood test 166:Homozygous 135:leg ulcers 131:gallstones 78:Hematology 12571:Physician 12455:education 12313:Neurology 12308:Narcology 12172:Pathology 12150:Radiology 12025:Angiology 11989:Andrology 11423:Hemolytic 11254:emerg/406 11237:eMedicine 10838:213060203 10369:7 October 10362:The Voice 10289:(1): 78. 9960:192050177 8805:0098-7484 7968:1536-3678 7869:. 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Daily 545:naproxen 539:such as 521:necrosis 501:acidosis 333:carriers 321:mutation 139:priapism 89:Symptoms 12641:Outline 12611:Commons 12556:Therapy 12453:Medical 12016:Allergy 11984:Urology 11877:Surgery 11245:oph/490 11196:D000755 10953:7390490 10878:6606394 10793:31 July 10761:5898607 10740:Bibcode 10685:5128871 10519:Bibcode 10511:Science 10487:8275984 10306:4394397 10262:6 April 10237:6 April 10208:3534410 10116:7 April 10071:5924488 10017:6778728 9918:6186193 9767:7460122 9710:4167855 9682:Bibcode 9631:Bibcode 9623:Science 9608:3900438 9495:2588723 9369:3200316 9346:Bibcode 9217:2931779 9137:3119961 9086:3119971 8758:NPR.org 8740:7644129 8610:. 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