204:
44:
314:
causes a non-anion gap metabolic acidosis with hypokalemia and a positive urinary anion gap that looks a lot like distal RTA but there is no hydrogen secretion defect and the acidosis is due to acid production during the metabolism of
567:
195:. Aldosterone causes increased resorption of sodium and loss of potassium in the collecting duct of the kidney, so these increased aldosterone levels cause the hypokalemia which is a common symptom of dRTA.
332:
which is characterized by an inability to acidify urine, without affecting blood pH or plasma bicarbonate levels. The diagnosis of dRTA can be made by the observation of a relatively alkaline urinary
344:
or less). In the case of an incomplete dRTA, failure to acidify the urine following an oral acid loading challenge is often used as a test. The test usually performed is
739:
Karet FE, Finberg KE, Nelson RD, et al. (1999). "Mutations in the gene encoding B1 subunit of H+-ATPase cause renal tubular acidosis with sensorineural deafness".
224:
fashion in South East Asian cases. The South East Asian cases are associated with more severe hypokalemia. Other hereditary causes include mutations of subunits of the
90:. This failure of acid secretion may be due to a number of causes. It leads to relatively alkaline urine, due to the kidney's inability to acidify the urine to a
324:
The pH of patient's blood is highly variable, and acidemia is not necessarily characteristic of people with dRTA at any given time. One may have dRTA caused by
661:"Band 3 mutations, renal tubular acidosis and South-East Asian ovalocytosis in Malaysia and Papua New Guinea: loss of up to 95% band 3 transport in red cells"
395:
tablets which not only replace potassium but also inhibit calcium excretion and thus do not exacerbate stone disease as sodium bicarbonate or citrate may do.
391:. This will correct the acidemia and reverse bone demineralisation. Hypokalemia and urinary stone formation and nephrocalcinosis can be treated with
596:
412:
405:
1226:
Text was copied from this source which is copyright
European Medicines Agency. Reproduction is authorized provided the source is acknowledged.
864:
Boton R, Gaviria M, Batlle DC (1987). "Prevalence, pathogenesis, and treatment of renal dysfunction associated with chronic lithium therapy".
1653:
1127:
1570:
1340:
491:
Laing CM, Toye AM, Capasso G, Unwin RJ (2005). "Renal tubular acidosis: developments in our understanding of the molecular basis".
477:
84:
1053:"Urinary acidification assessed by simultaneous furosemide and fludrocortisone treatment: an alternative to ammonium chloride"
71:) is the classical form of RTA, being the first described. Distal RTA is characterized by a failure of acid secretion by the
580:
1794:
1255:
102:
Because renal excretion is the primary means of eliminating acid from the body, there is consequently a tendency towards
612:"Familial distal renal tubular acidosis is associated with mutations in the red cell anion exchanger (Band 3, AE1) gene"
1687:
1646:
704:
Wagner, CA; Finberg, KE; Breton, S; Marshansky, V; Brown, D; Geibel, JP (October 2004). "Renal
Vacuolar H-ATPase".
191:
dRTA commonly leads to sodium loss and volume contraction, which causes a compensatory increase in blood levels of
1391:
821:
Skinner R, Pearson AD, English MW, et al. (1996). "Risk factors for ifosfamide nephrotoxicity in children".
444:
429:
Potassium citrate/potassium hydrogen carbonate was approved for medical use in the
European Union in April 2021.
1580:
934:
Carlisle, E. J.; Donnelly, S. M.; Vasuvattakul, S.; Kamel, K. S.; Tobe, S.; Halperin, M. L. (February 1991).
231:
vH-ATPase, which are transmitted in an autosomal recessive fashion, and may be associated with sensorineural
1639:
1333:
424:
364:
246:
352:, in which ammonium chloride capsules are used as the acid load. More recently, an alternative test using
1367:
784:"Immune-related potassium-losing interstitial nephritis: a comparison with distal renal tubular acidosis"
1456:
1306:
270:. While it is a consequence of dRTA, it can also be a cause; related to calcium-induced damage of the
336:
of greater than 5.3 in the face of a systemic acidemia (usually taken to be a serum bicarbonate of 20
207:
Diagram depicting an alpha intercalated cell with the apical proton pump and basolateral band 3 (kAE1)
1799:
1714:
1491:
1211:
261:
1820:
1592:
1486:
1266:
271:
80:
76:
1135:
242:
1719:
1496:
1326:
325:
216:
the basolateral bicarbonate transporter of the intercalated cell, which may be transmitted in an
72:
783:
1724:
1702:
1662:
1532:
1446:
1438:
1403:
1386:
1201:
1199:
1197:
528:
439:
416:
254:
34:
1295:
1778:
1771:
1697:
1555:
1476:
1206:
277:
1194:
899:
McCurdy DK, Frederic M, Elkinton JR (1968). "Renal tubular acidosis due to amphotericin B".
1744:
1736:
1560:
1426:
250:
8:
1766:
1527:
1105:
221:
1158:
1679:
1618:
1575:
1178:
846:
764:
681:
660:
590:
469:
380:
283:
238:
217:
114:
877:
834:
636:
611:
541:
1421:
1183:
1109:
1074:
1033:
1006:
965:
957:
916:
881:
838:
803:
756:
721:
686:
641:
576:
545:
508:
420:
392:
388:
384:
379:
This is relatively straightforward. It involves correction of the acidemia with oral
368:
347:
301:
177:
850:
768:
419:
medication intended for the treatment of distal renal tubular acidosis. It contains
1468:
1416:
1411:
1173:
1101:
1064:
996:
947:
908:
873:
830:
795:
748:
713:
676:
668:
631:
623:
537:
500:
311:
267:
135:
203:
1707:
1271:
504:
357:
225:
912:
672:
1608:
1481:
1451:
1357:
1349:
717:
363:
dRTA has been proposed as a possible diagnosis for the unknown malady plaguing
305:
160:
The symptoms and sequelae of dRTA are variable and range from being completely
129:
1814:
1587:
1522:
961:
799:
185:
173:
1692:
1613:
1603:
1565:
1187:
1078:
1037:
1010:
725:
690:
512:
161:
153:
125:
1113:
1069:
1052:
969:
920:
885:
842:
807:
760:
645:
549:
1599:
1378:
1290:
1001:
984:
228:
192:
120:
1247:
952:
936:"Glue-sniffing and distal renal tubular acidosis: sticking to the facts"
935:
1024:
Wrong, O; Davies HEF (1959). "The
Excretion of Acid in Renal Disease".
449:
353:
297:
51:
43:
1631:
627:
1758:
1517:
1512:
290:
169:
111:
526:
Buckalew VM Jr (1989). "Nephrolithiasis in renal tubular acidosis".
1670:
1301:
232:
103:
575:(10th ed.). Philadelphia, PA: W B Saunders Co. p. 1045.
1318:
933:
752:
398:
181:
149:
139:
87:
55:
1260:
985:"Genetic causes and mechanisms of distal renal tubular acidosis"
1537:
213:
703:
341:
337:
165:
145:
1159:"Alkali therapy in renal tubular acidosis: who needs it?"
898:
333:
91:
1050:
820:
490:
1237:
328:failure without necessarily being acidemic; termed
863:
738:
561:
559:
1092:Lewis D (1992). "What was wrong with Tiny Tim?".
1051:Walsh SB, Shirley DG, Wrong OM, Unwin RJ (2007).
781:
610:Bruce LJ, Cope DL, Jones GK, et al. (1997).
58:, a complication of both distal and proximal RTA.
1812:
659:Bruce LJ, Wrong O, Toye AM, et al. (2000).
1156:
1023:
556:
106:. This leads to the clinical features of dRTA:
658:
609:
525:
413:Potassium citrate/potassium hydrogen carbonate
406:Potassium citrate/potassium hydrogen carbonate
399:Potassium citrate/potassium hydrogen carbonate
257:. Hypokalaemia is often severe in these cases.
1647:
1334:
940:Journal of the American Society of Nephrology
595:: CS1 maint: multiple names: authors list (
220:fashion in western European cases, or in an
982:
415:, sold under the brand name Sibnayal, is a
1654:
1640:
1341:
1327:
42:
1177:
1068:
1000:
951:
680:
635:
300:(more commonly causing pRTA than dRTA),
202:
1661:
1091:
782:Wrong OM, Feest TG, MacIver AG (1993).
519:
212:Hereditary causes include mutations of
184:in childhood forms as well as possible
1813:
128:formation (related to alkaline urine,
1635:
1322:
569:Campbell-walsh Urology Expert Consult
480:from the original on 20 October 2020.
97:
1571:Abderhalden–Kaufmann–Lignac syndrome
1106:10.1001/archpedi.1992.02160240013002
565:
484:
1795:Mixed disorder of acid-base balance
989:Nephrology Dialysis Transplantation
13:
1348:
1179:10.1097/01.ASN.0000027973.07189.00
1134:. 28 December 1992. Archived from
14:
1832:
1233:
983:Batlle, D.; Haque, S. K. (2012).
470:"Osmosis: Renal tubular acidosis"
245:, but it is also associated with
404:This section is an excerpt from
1157:Morris RC, Sebastian A (2002).
1150:
1120:
1085:
1044:
1017:
976:
927:
892:
857:
814:
445:Proximal renal tubular acidosis
142:in the substance of the kidney)
775:
732:
697:
652:
603:
462:
1:
878:10.1016/s0272-6386(87)80098-7
835:10.1016/S0140-6736(96)03480-0
542:10.1016/S0022-5347(17)40997-9
455:
65:Distal renal tubular acidosis
24:Distal renal tubular acidosis
505:10.1016/j.biocel.2005.01.002
425:potassium hydrogen carbonate
374:
319:
247:systemic lupus erythematosus
7:
1368:Template:Glomerular disease
913:10.1056/NEJM196801182780302
433:
132:, and low urinary citrate).
10:
1837:
1457:Balkan endemic nephropathy
718:10.1152/physrev.00045.2003
667:. 350 Pt 1 (Pt 1): 41–51.
493:Int. J. Biochem. Cell Biol
403:
148:demineralisation (causing
1787:
1757:
1735:
1678:
1669:
1548:
1505:
1492:Renovascular hypertension
1467:
1437:
1377:
1356:
1281:
1241:
1212:European Medicines Agency
673:10.1042/0264-6021:3500041
262:urinary tract obstruction
198:
50:
41:
28:
23:
1593:Renal papillary necrosis
1487:Hypertensive nephropathy
272:cortical collecting duct
81:cortical collecting duct
73:alpha intercalated cells
1497:Renal cortical necrosis
536:(3 (part 2)): 731–737.
326:alpha intercalated cell
1703:Alcoholic ketoacidosis
1533:Chronic kidney disease
1447:Interstitial nephritis
1404:Acute tubular necrosis
1387:Renal tubular acidosis
800:10.1093/qjmed/86.8.513
566:Wein, Alan, J (2011).
529:The Journal of Urology
440:Renal tubular acidosis
417:fixed-dose combination
255:hypergammaglobulinemia
208:
35:renal tubular acidosis
1800:Acid–base homeostasis
1772:Contraction alkalosis
1698:Diabetic ketoacidosis
1556:Analgesic nephropathy
1477:Renal artery stenosis
1128:"What Ailed Tiny Tim"
1070:10.1038/sj.ki.5002220
706:Physiological Reviews
278:Renal transplantation
206:
1561:Renal osteodystrophy
1138:on 30 September 2007
367:in Charles Dickens'
360:has been described.
251:rheumatoid arthritis
1663:Acid–base disorders
1528:Acute renal failure
1166:J. Am. Soc. Nephrol
953:10.1681/ASN.V181019
222:autosomal recessive
1619:Reflux nephropathy
1576:Diabetes insipidus
1358:Glomerular disease
1282:External resources
1002:10.1093/ndt/gfs442
381:sodium bicarbonate
296:Toxins, including
284:Sickle cell anemia
243:Sjögren's syndrome
239:Autoimmune disease
218:autosomal dominant
209:
115:metabolic acidosis
98:Symptoms and signs
94:of less than 5.3.
1808:
1807:
1753:
1752:
1629:
1628:
1506:General syndromes
1427:Liddle's syndrome
1422:Gitelman syndrome
1316:
1315:
1216:. 9 December 2020
995:(10): 3691–3704.
866:Am. J. Kidney Dis
628:10.1172/JCI119694
582:978-1-4160-6911-9
421:potassium citrate
393:potassium citrate
389:potassium citrate
369:A Christmas Carol
348:ammonium chloride
302:lithium carbonate
178:failure to thrive
62:
61:
54:of a person with
18:Medical condition
1828:
1715:Normal anion gap
1676:
1675:
1656:
1649:
1642:
1633:
1632:
1417:Bartter syndrome
1412:Fanconi syndrome
1343:
1336:
1329:
1320:
1319:
1239:
1238:
1227:
1225:
1223:
1221:
1203:
1192:
1191:
1181:
1163:
1154:
1148:
1147:
1145:
1143:
1124:
1118:
1117:
1089:
1083:
1082:
1072:
1048:
1042:
1041:
1032:(110): 259–313.
1021:
1015:
1014:
1004:
980:
974:
973:
955:
946:(8): 1019–1027.
931:
925:
924:
896:
890:
889:
861:
855:
854:
829:(9027): 578–80.
818:
812:
811:
779:
773:
772:
736:
730:
729:
701:
695:
694:
684:
656:
650:
649:
639:
607:
601:
600:
594:
586:
574:
563:
554:
553:
523:
517:
516:
488:
482:
481:
466:
330:incomplete dRTA,
268:Nephrocalcinosis
188:and even death.
152:in children and
136:Nephrocalcinosis
46:
21:
20:
1836:
1835:
1831:
1830:
1829:
1827:
1826:
1825:
1821:Kidney diseases
1811:
1810:
1809:
1804:
1783:
1749:
1731:
1665:
1660:
1630:
1625:
1544:
1501:
1463:
1433:
1373:
1352:
1347:
1317:
1312:
1311:
1277:
1276:
1250:
1236:
1231:
1230:
1219:
1217:
1207:"Sibnayal EPAR"
1205:
1204:
1195:
1161:
1155:
1151:
1141:
1139:
1126:
1125:
1121:
1090:
1086:
1049:
1045:
1022:
1018:
981:
977:
932:
928:
901:N. Engl. J. Med
897:
893:
862:
858:
819:
815:
780:
776:
737:
733:
712:(4): 1263–314.
702:
698:
657:
653:
622:(7): 1693–707.
616:J. Clin. Invest
608:
604:
588:
587:
583:
572:
564:
557:
524:
520:
489:
485:
468:
467:
463:
458:
436:
431:
430:
409:
401:
377:
358:fludrocortisone
322:
241:. Classically
201:
138:(deposition of
100:
19:
12:
11:
5:
1834:
1824:
1823:
1806:
1805:
1803:
1802:
1797:
1791:
1789:
1785:
1784:
1782:
1781:
1776:
1775:
1774:
1763:
1761:
1755:
1754:
1751:
1750:
1748:
1747:
1741:
1739:
1733:
1732:
1730:
1729:
1728:
1727:
1722:
1720:Hyperchloremic
1712:
1711:
1710:
1705:
1700:
1695:
1688:High anion gap
1684:
1682:
1673:
1667:
1666:
1659:
1658:
1651:
1644:
1636:
1627:
1626:
1624:
1623:
1622:
1621:
1616:
1611:
1609:Hydronephrosis
1597:
1596:
1595:
1585:
1584:
1583:
1573:
1568:
1563:
1558:
1552:
1550:
1546:
1545:
1543:
1542:
1541:
1540:
1535:
1530:
1520:
1515:
1509:
1507:
1503:
1502:
1500:
1499:
1494:
1489:
1484:
1482:Renal ischemia
1479:
1473:
1471:
1465:
1464:
1462:
1461:
1460:
1459:
1454:
1452:Pyelonephritis
1443:
1441:
1435:
1434:
1432:
1431:
1430:
1429:
1424:
1419:
1414:
1406:
1401:
1400:
1399:
1394:
1383:
1381:
1375:
1374:
1372:
1371:
1362:
1360:
1354:
1353:
1350:Kidney disease
1346:
1345:
1338:
1331:
1323:
1314:
1313:
1310:
1309:
1298:
1286:
1285:
1283:
1279:
1278:
1275:
1274:
1263:
1251:
1246:
1245:
1243:
1242:Classification
1235:
1234:External links
1232:
1229:
1228:
1193:
1149:
1119:
1100:(12): 1403–7.
1094:Am J Dis Child
1084:
1063:(12): 1310–6.
1043:
1016:
975:
926:
891:
856:
813:
774:
731:
696:
651:
602:
581:
555:
518:
499:(6): 1151–61.
483:
460:
459:
457:
454:
453:
452:
447:
442:
435:
432:
410:
402:
400:
397:
385:sodium citrate
376:
373:
321:
318:
317:
316:
309:
306:amphotericin B
294:
287:
281:
275:
265:
258:
236:
200:
197:
158:
157:
143:
133:
130:hypercalciuria
123:
118:
99:
96:
60:
59:
48:
47:
39:
38:
30:
26:
25:
17:
9:
6:
4:
3:
2:
1833:
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1819:
1818:
1816:
1801:
1798:
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1793:
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1790:
1786:
1780:
1777:
1773:
1770:
1769:
1768:
1765:
1764:
1762:
1760:
1756:
1746:
1743:
1742:
1740:
1738:
1734:
1726:
1725:Renal tubular
1723:
1721:
1718:
1717:
1716:
1713:
1709:
1706:
1704:
1701:
1699:
1696:
1694:
1691:
1690:
1689:
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1683:
1681:
1677:
1674:
1672:
1668:
1664:
1657:
1652:
1650:
1645:
1643:
1638:
1637:
1634:
1620:
1617:
1615:
1612:
1610:
1607:
1606:
1605:
1601:
1598:
1594:
1591:
1590:
1589:
1588:Renal papilla
1586:
1582:
1579:
1578:
1577:
1574:
1572:
1569:
1567:
1564:
1562:
1559:
1557:
1554:
1553:
1551:
1547:
1539:
1536:
1534:
1531:
1529:
1526:
1525:
1524:
1523:Renal failure
1521:
1519:
1516:
1514:
1511:
1510:
1508:
1504:
1498:
1495:
1493:
1490:
1488:
1485:
1483:
1480:
1478:
1475:
1474:
1472:
1470:
1466:
1458:
1455:
1453:
1450:
1449:
1448:
1445:
1444:
1442:
1440:
1436:
1428:
1425:
1423:
1420:
1418:
1415:
1413:
1410:
1409:
1407:
1405:
1402:
1398:
1395:
1393:
1390:
1389:
1388:
1385:
1384:
1382:
1380:
1376:
1370:
1369:
1364:
1363:
1361:
1359:
1355:
1351:
1344:
1339:
1337:
1332:
1330:
1325:
1324:
1321:
1308:
1304:
1303:
1299:
1297:
1293:
1292:
1288:
1287:
1284:
1280:
1273:
1269:
1268:
1264:
1262:
1258:
1257:
1253:
1252:
1249:
1244:
1240:
1215:
1213:
1208:
1202:
1200:
1198:
1189:
1185:
1180:
1175:
1172:(8): 2186–8.
1171:
1167:
1160:
1153:
1137:
1133:
1129:
1123:
1115:
1111:
1107:
1103:
1099:
1095:
1088:
1080:
1076:
1071:
1066:
1062:
1058:
1054:
1047:
1039:
1035:
1031:
1027:
1020:
1012:
1008:
1003:
998:
994:
990:
986:
979:
971:
967:
963:
959:
954:
949:
945:
941:
937:
930:
922:
918:
914:
910:
907:(3): 124–30.
906:
902:
895:
887:
883:
879:
875:
872:(5): 329–45.
871:
867:
860:
852:
848:
844:
840:
836:
832:
828:
824:
817:
809:
805:
801:
797:
794:(8): 513–34.
793:
789:
785:
778:
770:
766:
762:
758:
754:
750:
746:
742:
735:
727:
723:
719:
715:
711:
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1693:Ketoacidosis
1614:Pyonephrosis
1566:Nephroptosis
1439:Interstitium
1396:
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1218:. Retrieved
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1136:the original
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154:osteomalacia
101:
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1779:Respiratory
1745:Respiratory
1737:Respiratory
1600:Major calyx
1581:Nephrogenic
1291:MedlinePlus
229:proton pump
193:aldosterone
180:and severe
121:Hypokalemia
29:Other names
1057:Kidney Int
741:Nat. Genet
665:Biochem. J
456:References
450:Ifosfamide
354:furosemide
346:the short
298:ifosfamide
156:in adults)
52:Radiograph
1767:Metabolic
1759:Alkalosis
1680:Metabolic
1518:Nephrosis
1513:Nephritis
962:1046-6673
788:Q. J. Med
591:cite book
375:Treatment
320:Diagnosis
291:cirrhosis
253:and even
170:hematuria
168:pain and
117:/acidemia
112:anion gap
1815:Category
1671:Acidosis
1469:Vascular
1408:Genetic
1392:proximal
1302:Orphanet
1188:12138154
1079:17410104
1038:13658353
1011:23114896
851:12624019
769:34262548
726:15383652
691:10926824
513:15778079
478:Archived
434:See also
365:Tiny Tim
315:toluene.
260:Chronic
233:deafness
104:acidemia
1379:Tubules
1272:D000141
1114:1340779
970:1912400
921:5634966
886:3314489
843:8774570
808:8210309
761:9916796
682:1221222
646:9312167
550:2645431
474:Osmosis
312:Toluene
182:rickets
150:rickets
140:calcium
110:Normal
88:nephron
83:of the
75:of the
56:rickets
33:Type 1
1708:Lactic
1604:pelvis
1538:Uremia
1397:distal
1296:000493
1261:179800
1220:22 May
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1142:22 May
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289:Liver
226:apical
214:Band 3
199:Causes
85:distal
1788:Other
1549:Other
1214:(EMA)
1162:(PDF)
847:S2CID
765:S2CID
573:(PDF)
176:, to
172:from
164:, to
1366:See
1267:MeSH
1256:OMIM
1222:2021
1184:PMID
1144:2010
1132:Time
1110:PMID
1075:PMID
1034:PMID
1007:PMID
966:PMID
958:ISSN
917:PMID
882:PMID
839:PMID
804:PMID
757:PMID
722:PMID
687:PMID
642:PMID
597:link
577:ISBN
546:PMID
509:PMID
423:and
356:and
350:test
338:mmol
304:and
166:loin
146:Bone
79:and
69:dRTA
1174:doi
1102:doi
1098:146
1065:doi
1026:QJM
997:doi
948:doi
909:doi
905:278
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831:doi
827:348
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749:doi
714:doi
677:PMC
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632:PMC
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501:doi
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