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Lymphangiectasia

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The clinical presentation of intestinal lymphangiectasia can range from asymptomatic to severe, implying a broad clinical spectrum. Some patients may exhibit minimal or subtle clinical features, diverging from the "textbook" presentations often associated with severe cases in adults. This variability
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fluid into the intestines leads to the loss of lymphocytes, immunoglobulins, and proteins, causing lymphopenia, hypogammaglobulinemia, and hypoalbuminemia, respectively. The loss of proteins contributes to the development of protein-losing enteropathy, a major clinical manifestation of this disease.
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Patients with intestinal lymphangiectasia present with a range of symptoms, significantly influenced by the extent of protein loss. Chronic diarrhea and malabsorption are common symptoms. The loss of protein can lead to edema, particularly in the legs and abdomen, due to decreased oncotic pressure.
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The pathophysiology of intestinal lymphangiectasia is centered around the dilation of the lymphatic vessels in the intestinal mucosa, submucosa, and sometimes the mesentery. This dilation impedes the normal flow of lymph from the intestines back to the circulatory system. The overflow of lymphatic
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in cases where there is no secondary cause. The primary defect lies in the inability of the lymphatic system to adequately drain lymph, resulting in its subsequent accumulation and leakage into the intestinal lumen. This condition, first described by Waldmann in 1961, is typically diagnosed in
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has been shown to reduce symptom effects. This diet, however, is not a cure. If the diet is stopped, the symptoms will eventually reappear. Medication is also used to treat this disease, including Octreotide, Sirolimus, Anti-plasmin and, at least in one case, Trametinib.
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Nutritional deficiencies may develop due to malabsorption, leading to growth retardation in children and weight loss in adults. Immune abnormalities resulting from lymphocyte loss can predispose patients to recurrent infections.
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indicating dilated lacteals. Computerized tomography (CT) can show low attenuation material within the bowel walls which corresponds to lipid-containing chylous fluid within the dilated lymphatic vessels.
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Kull P, Hess R, Craig L, Saunders H, Washabau R (2001). "Clinical, clinicopathologic, radiographic, and ultrasonographic characteristics of intestinal lymphangiectasia in dogs: 17 cases (1996-1998)".
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Nishino, Koichi; Yoshimi, Kaku; Shibuya, Tomoyoshi; Hayashi, Takuo; Mitani, Keiko; Kobayashi, Etsuko; Ichikawa, Masako; Asao, Tetsuhiko; Suzuki, Yohei; Sato, Tadashi; Shiota, Satomi (2017-04-15).
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Sutherland-Smith J, Penninck D, Keating J, Webster C (2007). "Ultrasonographic intestinal hyperechoic mucosal striations in dogs are associated with lacteal dilation".
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underscores the importance of considering PIL in differential diagnoses, even when clinical manifestations are not severe or typical.
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can also lead to inflammation of the lymphatics and lymphangiectasia through migration of inflammatory cells through the lymphatics.
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infancy or early childhood. However, it can also manifest in adults, exhibiting a broad spectrum of clinical symptoms.
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Fogle, Jonathan E.; Bissett, Sally A. (May 2007). "Mucosal Immunity and Chronic Idiopathic Enteropathies in Dogs".
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Awiwi, Muhammad O.; Naik, Sagar (7 June 2022). "Tyrosine Kinase Inhibitor–related Intestinal Lymphangiectasia".
1038: 1120: 1012: 243: 169: 69:, also known as "lymphangiectasis", is a pathologic dilation of lymph vessels. When it occurs in the 56: 830: 201: 157: 1017: 847: 242:
Dog breeds commonly affected by lymphangiectasia and/or protein-losing enteropathy include the
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Proceedings of the 28th World Congress of the World Small Animal Veterinary Association
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Waldmann, T.A.; Steinfeld, J.L.; Dutcher, T.F.; Davidson, J.D.; Gordon, R.S. (2008).
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In the case of primary intestinal lymphangiectasia, a diet of low-fat and high-
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Diagnosis is through biopsy. The presence of hypoproteinemia, decreased blood
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malformation of the lymphatics. Secondary lymphangiectasia may be caused by
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it is known as intestinal lymphangiectasia, colloquially recognized as
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Compendium on Continuing Education for the Practicing Veterinarian
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McGavin/ Zachary (2007), Pathologic Basis of Veterinary Disease
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and calcium, and secondary to low protein binding of calcium.
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Pediatric Gastroenterology, Hepatology & Nutrition
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or cancer causing lymphatic obstruction, or increased
549: 797: 417:Vignes, StĂ©phane; Bellanger, JĂ©rĂ´me (2008-02-22). 771:Ettinger, Stephen J.; Feldman, Edward C. (1995). 1145: 416: 133:syndrome of the small intestine, especially of 863: 764: 593:(5). Veterinary Learning Systems: 290–302. 285: 870: 856: 372:World Journal of Gastrointestinal Oncology 55: 27: 730: 712: 646: 526: 452: 434: 401: 383: 196:) is also seen due to poor absorption of 773:Textbook of Veterinary Internal Medicine 775:(4th ed.). W.B. Saunders Company. 694: 467: 368:"Intestinal lymphangiectasia in adults" 365: 1146: 951:Asplenia with cardiovascular anomalies 470:"Protein-Losing Enteropathies in Dogs" 1023:Intranodal palisaded myofibroblastoma 851: 690: 688: 686: 84: 578: 361: 359: 357: 355: 353: 351: 349: 347: 345: 343: 303: 301: 299: 297: 695:Kwon, Yiyoung; Kim, Mi Jin (2021). 13: 683: 14: 1175: 793: 423:Orphanet Journal of Rare Diseases 340: 294: 1126:Postmastectomy lymphangiosarcoma 1094:Lymphedema–distichiasis syndrome 626:10.1111/j.1740-8261.2007.00204.x 511:10.2169/internalmedicine.56.7769 1154:Dermal and subcutaneous growths 745: 739: 640: 605: 1039:Template:Respiratory pathology 543: 486: 461: 410: 268: 1: 746:PIL, Unraveling Adult-Onset. 326:10.1016/s0016-5085(19)35130-3 261: 237: 748:"Unraveling Adult-Onset PIL" 366:Freeman, Hugh James (2011). 280:Dorland's Medical Dictionary 216: 175: 7: 1121:Postinflammatory lymphedema 1013:Generalized lymphadenopathy 881:: organ and vessel diseases 244:Soft-Coated Wheaten Terrier 10: 1180: 714:10.5223/pghn.2021.24.5.413 564:10.2460/javma.2001.219.197 170:Inflammatory bowel disease 36:Lymphangiectasia shown on 1164:Lymphatic vessel diseases 1049: 998: 936: 886: 801: 468:Steiner, Jörg M. (2003). 44: 35: 26: 21: 188:support the diagnosis. 101: 225:aliments, supplemental 202:Medical ultrasonography 158:central venous pressure 144:The most common cause 121:and distension of the 113:shows dilation of the 1079:Congenital lymphedema 661:10.1148/radiol.220137 614:Vet Radiol Ultrasound 436:10.1186/1750-1172-3-5 385:10.4251/wjgo.v3.i2.19 148:lymphangiectasia was 1116:Factitial lymphedema 1106:Secondary lymphedema 752:lymphangiectasia.com 1018:Castleman's disease 248:Norwegian Lundehund 1111:Bullous lymphedema 1084:Lymphedema praecox 1074:Primary lymphedema 985:Splenic infarction 552:J Am Vet Med Assoc 275:"lymphangiectasia" 85:Signs and symptoms 75:Waldmann's disease 1141: 1140: 1051:Lymphatic vessels 911:DiGeorge syndrome 879:Lymphatic disease 845: 844: 782:978-0-7216-6795-9 499:Internal Medicine 256:Yorkshire Terrier 210:intestinal mucosa 123:lymphatic vessels 64: 63: 16:Medical condition 1171: 1133:Waldmann disease 1099:Milroy's disease 1089:Lymphedema tarda 1064:Lymphangiectasia 978:Banti's syndrome 968:Wandering spleen 958:Accessory spleen 928:Thymic carcinoma 872: 865: 858: 849: 848: 799: 798: 787: 786: 768: 762: 761: 759: 758: 743: 737: 736: 734: 716: 692: 681: 680: 644: 638: 637: 609: 603: 602: 582: 576: 575: 547: 541: 540: 530: 490: 484: 483: 481: 480: 465: 459: 458: 456: 438: 414: 408: 407: 405: 387: 363: 338: 337: 314:Gastroenterology 305: 292: 289: 283: 272: 184:, and decreased 137:and fat-soluble 129:flow leads to a 67:Lymphangiectasia 60: 59: 31: 22:Lymphangiectasia 19: 18: 1179: 1178: 1174: 1173: 1172: 1170: 1169: 1168: 1144: 1143: 1142: 1137: 1045: 1028:Kikuchi disease 1008:Lymphadenopathy 994: 932: 882: 876: 846: 841: 840: 810: 796: 791: 790: 783: 769: 765: 756: 754: 744: 740: 693: 684: 645: 641: 610: 606: 583: 579: 548: 544: 491: 487: 478: 476: 466: 462: 415: 411: 364: 341: 306: 295: 290: 286: 273: 269: 264: 240: 219: 178: 164:or right-sided 111:small intestine 104: 87: 81: 54: 17: 12: 11: 5: 1177: 1167: 1166: 1161: 1156: 1139: 1138: 1136: 1135: 1130: 1129: 1128: 1123: 1118: 1113: 1103: 1102: 1101: 1096: 1091: 1086: 1081: 1071: 1066: 1061: 1055: 1053: 1047: 1046: 1044: 1043: 1042: 1041: 1030: 1025: 1020: 1015: 1010: 1004: 1002: 996: 995: 993: 992: 987: 982: 981: 980: 970: 965: 960: 955: 954: 953: 942: 940: 934: 933: 931: 930: 925: 920: 918:Ectopic thymus 915: 914: 913: 903: 898: 892: 890: 884: 883: 875: 874: 867: 860: 852: 843: 842: 839: 838: 827: 811: 806: 805: 803: 802:Classification 795: 794:External links 792: 789: 788: 781: 763: 738: 682: 639: 604: 577: 558:(2): 197–202. 542: 505:(8): 943–948. 485: 460: 409: 339: 320:(3): 197–207. 293: 284: 266: 265: 263: 260: 239: 236: 218: 215: 177: 174: 103: 100: 86: 83: 62: 61: 48: 42: 41: 33: 32: 24: 23: 15: 9: 6: 4: 3: 2: 1176: 1165: 1162: 1160: 1157: 1155: 1152: 1151: 1149: 1134: 1131: 1127: 1124: 1122: 1119: 1117: 1114: 1112: 1109: 1108: 1107: 1104: 1100: 1097: 1095: 1092: 1090: 1087: 1085: 1082: 1080: 1077: 1076: 1075: 1072: 1070: 1067: 1065: 1062: 1060: 1057: 1056: 1054: 1052: 1048: 1040: 1036: 1035: 1034: 1031: 1029: 1026: 1024: 1021: 1019: 1016: 1014: 1011: 1009: 1006: 1005: 1003: 1001: 997: 991: 990:Splenic tumor 988: 986: 983: 979: 976: 975: 974: 971: 969: 966: 964: 961: 959: 956: 952: 949: 948: 947: 944: 943: 941: 939: 935: 929: 926: 924: 921: 919: 916: 912: 909: 908: 907: 904: 902: 899: 897: 894: 893: 891: 889: 885: 880: 873: 868: 866: 861: 859: 854: 853: 850: 837: 833: 832: 828: 826: 822: 821: 817: 813: 812: 809: 804: 800: 784: 778: 774: 767: 753: 749: 742: 733: 728: 724: 720: 715: 710: 706: 702: 698: 691: 689: 687: 678: 674: 670: 666: 662: 658: 655:(2): 220137. 654: 650: 643: 635: 631: 627: 623: 619: 615: 608: 600: 596: 592: 588: 581: 573: 569: 565: 561: 557: 553: 546: 538: 534: 529: 524: 520: 516: 512: 508: 504: 500: 496: 489: 475: 471: 464: 455: 450: 446: 442: 437: 432: 428: 424: 420: 413: 404: 399: 395: 391: 386: 381: 377: 373: 369: 362: 360: 358: 356: 354: 352: 350: 348: 346: 344: 335: 331: 327: 323: 319: 315: 311: 304: 302: 300: 298: 288: 282: 281: 276: 271: 267: 259: 257: 253: 249: 245: 235: 232: 228: 224: 214: 211: 207: 203: 199: 195: 191: 187: 183: 173: 171: 167: 166:heart failure 163: 159: 155: 151: 147: 142: 140: 136: 132: 131:malabsorption 128: 124: 120: 116: 112: 108: 99: 95: 91: 82: 79: 76: 72: 68: 58: 52: 49: 47: 43: 39: 34: 30: 25: 20: 1159:Dog diseases 1063: 1059:Lymphangitis 973:Splenomegaly 829: 814: 772: 766: 755:. 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Reduced 38:enteroscopy 1148:Categories 1069:Lymphedema 1000:Lymph node 906:Hypoplasia 757:2023-11-30 707:(5): 413. 479:2007-03-20 262:References 238:In animals 206:striations 154:granulomas 150:congenital 71:intestines 723:2234-8646 677:249432951 649:Radiology 519:0918-2918 445:1750-1172 394:1948-5204 378:(2): 19. 334:0016-5085 217:Treatment 204:may show 198:vitamin D 176:Diagnosis 51:Angiology 46:Specialty 946:Asplenia 669:35670716 634:17236361 599:17724983 572:11469575 537:28420844 231:vitamins 139:vitamins 115:lacteals 1033:Tonsils 923:Thymoma 896:Abscess 836:D008200 732:8443852 528:5465412 454:2288596 403:3046182 252:Basenji 227:calcium 223:protein 208:in the 194:calcium 117:of the 109:of the 938:Spleen 888:Thymus 779:  729:  721:  675:  667:  632:  597:  570:  535:  525:  517:  451:  443:  400:  392:  332:  254:, and 107:Biopsy 53:  825:457.1 673:S2CID 429:(1). 192:(low 127:lymph 119:villi 102:Cause 1037:see 831:MeSH 820:9-CM 777:ISBN 719:ISSN 665:PMID 630:PMID 595:PMID 568:PMID 533:PMID 515:ISSN 441:ISSN 390:ISSN 330:ISSN 816:ICD 727:PMC 709:doi 657:doi 653:305 622:doi 560:doi 556:219 523:PMC 507:doi 449:PMC 431:doi 398:PMC 380:doi 322:doi 277:at 168:. 135:fat 1150:: 834:: 823:: 750:. 725:. 717:. 705:24 703:. 699:. 685:^ 671:. 663:. 651:. 628:. 618:48 616:. 591:29 589:. 566:. 554:. 531:. 521:. 513:. 503:56 501:. 497:. 472:. 447:. 439:. 425:. 421:. 396:. 388:. 374:. 370:. 342:^ 328:. 318:41 316:. 312:. 296:^ 258:. 250:, 246:, 146:of 871:e 864:t 857:v 818:- 808:D 785:. 760:. 735:. 711:: 679:. 659:: 636:. 624:: 601:. 574:. 562:: 539:. 509:: 482:. 457:. 433:: 427:3 406:. 382:: 376:3 336:. 324:: 40:.

Index


enteroscopy
Specialty
Angiology
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intestines
Waldmann's disease
Biopsy
small intestine
lacteals
villi
lymphatic vessels
lymph
malabsorption
fat
vitamins
of
congenital
granulomas
central venous pressure
pericarditis
heart failure
Inflammatory bowel disease
lymphocytes
cholesterol
Hypocalcemia
calcium
vitamin D
Medical ultrasonography
striations

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