70:
654:
178:, the term RTA is reserved for individuals with poor urinary acidification in otherwise well-functioning kidneys. Several different types of RTA exist, which all have different syndromes and different causes. RTA is usually an incidental finding based on routine blood draws that show abnormal results. Clinically, patients may present with vague symptoms such as dehydration, mental status changes, or delayed growth in adolescents.
32:
535:
defects in the proteins that facilitate urine acidification at the distal tubule. Hereditary dRTA generally presents as failure to thrive during the first several months of life. Other common clinical manifestations in children include a variety of gastrointestinal and urinary symptoms, including polyuria, polydipsia, constipation, diarrhea, bouts of dehydration, and decreased appetite.
580:. The distal intercalated cells function normally, so the acidemia is less severe than dRTA and the alpha intercalated cells can produce H to acidify the urine to a pH of less than 5.3. pRTA also has several causes, and may occasionally be present as a solitary defect, but is usually associated with a more generalized dysfunction of the proximal tubular cells called
544:
430:
This leads to the clinical features of dRTA; In other words, the intercalated cells' apical H+/K+ antiporter is non-functional, resulting in proton retention and potassium excretion. Since calcium phosphate stones demonstrate a proclivity for deposition at higher pHs (alkaline), the substance of the
615:
In some patients, RTA shares features of both dRTA and pRTA. This rare pattern was observed in the 1960s and 1970s as a transient phenomenon in infants and children with dRTA (possibly in relation with some exogenous factor such as high salt intake) and is no longer observed. This form of RTA has
534:
dRTA is the most common form of RTA diagnosed in
Western countries, and can be classified as either hereditary (primary) or acquired (secondary). Primary RTA generally results from systemic and autoimmune diseases or drug and toxin exposure in adults, whereas pediatric RTA results from genetic
665:
Type 4 RTA is not actually a tubular disorder at all nor does it have a clinical syndrome similar to the other types of RTA described above. It was included in the classification of renal tubular acidoses as it is associated with a mild (normal anion gap) metabolic acidosis due to a
1401:
Sly WS, Whyte MP, Sundaram V, Tashian RE, Hewett-Emmett D, Guibaud P, et al. (July 1985). "Carbonic anhydrase II deficiency in 12 families with the autosomal recessive syndrome of osteopetrosis with renal tubular acidosis and cerebral calcification".
1439:"Carbonic anhydrase II deficiency syndrome (osteopetrosis with renal tubular acidosis and brain calcification): novel mutations in CA2 identified by direct sequencing expand the opportunity for genotype-phenotype correlation"
1527:
Fathallah DM, Bejaoui M, Lepaslier D, Chater K, Sly WS, Dellagi K (May 1997). "Carbonic anhydrase II (CA II) deficiency in
Maghrebian patients: evidence for founder effect and genomic recombination at the CA II locus".
1017:
Alonso-Varela M, Gil-Peña H, Coto E, GĂłmez J, RodrĂguez J, RodrĂguez-Rubio E, Santos F (September 2018). "Distal renal tubular acidosis. Clinical manifestations in patients with different underlying gene mutations".
1808:
1793:
1071:
Clericetti CM, Milani GP, Lava SA, Bianchetti MG, Simonetti GD, Giannini O (March 2018). "Hyperammonemia associated with distal renal tubular acidosis or urinary tract infection: a systematic review".
531:. In a large Asian series of Distal renal Tubular Acidosis in Sjogren's Syndrome, late diagnosis is a rule in spite of overt hypokalemic periodic paralysis in a vast majority of them
353:
Distal RTA (dRTA) is the classical form of RTA, being the first described. Distal RTA is characterized by a failure of H+ secretion into lumen of nephron by the alpha intercalated
511:
Distal RTA has also been linked to specific genetic mutations that will alter when the disease will present in the patient's life. Patient's with mutations in
745:
Renal tubular acidosis was first described in 1935 by
Lightwood and 1936 by Butler et al. in children. Baines et al. first described it in adults in 1945.
947:
2221:
576:. Reabsorption of bicarbonate is typically 80-90% in the proximal tubule and failure of this process leads to decreased systemic buffer and
2138:
821:
Laing CM, Toye AM, Capasso G, Unwin RJ (June 2005). "Renal tubular acidosis: developments in our understanding of the molecular basis".
961:
1908:
642:
there. The kidney problems are treated as described above. There is no treatment for the osteopetrosis or cerebral calcification.
523:
have delayed onset around 10 years of age. Electrolyte imbalances remain the same, while in severe cases symptoms can advance to
634:, and mental retardation. It is very rare and cases from all over the world have been reported, of which about 70% are from the
362:
898:
2362:
1823:
53:(radiopacities (white) in the right upper and left upper quadrant of the image), as seen in distal renal tubular acidosis
371:
This failure of acid secretion may be due to a number of causes, and it leads to an inability to acidify the urine to a
1661:
Baines AM, Barelay JA, Cooke WT (1945). "Nephrocalcinosis associated with hyperchloremia and low plasma-bicarbonate".
2255:
2214:
682:, and measured urinary acidification is normal, hence it is often called hyperkalemic RTA or tubular hyperkalemia.
279:
1959:
798:
559:
495:
2148:
195:. When the blood pH is below normal (7.35), this is called acidemia. The metabolic acidosis caused by RTA is a
207:
An overview of types 1, 2, and 4 is presented below (type 3 is usually excluded from modern classifications):
1964:
348:
2207:
1901:
749:
713:
623:
II deficiency. Mutations in the gene encoding this enzyme give rise to an autosomal recessive syndrome of
1935:
1346:
1306:"Proximal renal tubular acidosis. A defect in bicarbonate reabsorption with normal urinary acidification"
2024:
1632:
Butler AM, Wilson JL, Farber S (1936). "Dehydration and acidosis with calcification at renal tubules".
899:"Epidemiology of Distal Renal Tubular Acidosis: A Study Using Linked UK Primary Care and Hospital Data"
500:
645:
Type 3 is rarely discussed. Most comparisons of RTA are limited to a comparison of types 1, 2, and 4.
2367:
2282:
2059:
1634:
1479:
358:
196:
69:
2388:
2160:
2054:
1834:
2287:
2064:
1894:
788:
734:
320:
111:
2270:
2230:
2100:
2014:
2006:
1971:
896:
175:
2346:
2339:
2265:
2123:
2044:
941:
147:
2312:
2304:
2128:
1994:
1728:
793:
519:
will present with symptoms within the first year of life, while those with mutation of the
1478:
Pushkin A, Abuladze N, Gross E, Newman D, Tatishchev S, Lee I, et al. (August 2004).
8:
2334:
2095:
1812:
1693:
1574:
1480:"Molecular mechanism of kNBC1-carbonic anhydrase II interaction in proximal tubule cells"
782:
766:
1732:
2247:
2186:
2143:
1817:
1751:
1716:
1617:
1592:
1553:
1504:
1363:
1281:
1256:
1150:
1115:
1096:
1053:
929:
874:
620:
577:
439:
377:
143:
1647:
995:
1989:
1845:
1756:
1697:
1545:
1509:
1460:
1419:
1368:
1327:
1322:
1305:
1286:
1237:
1196:
1155:
1137:
1088:
1045:
999:
933:
921:
879:
838:
754:
690:
675:
316:
58:
1557:
1114:
Sandhya, Pulukool; Danda, Debashish; Rajaratnam, Simon; Thomas, Nihal (2014-12-19).
1057:
2036:
1984:
1979:
1874:
1746:
1736:
1689:
1643:
1612:
1604:
1537:
1499:
1491:
1450:
1411:
1358:
1317:
1276:
1268:
1227:
1186:
1145:
1127:
1100:
1080:
1035:
1027:
991:
913:
869:
830:
581:
565:
505:
468:
127:
103:
43:
648:
2275:
1839:
1741:
1495:
857:
834:
678:, and results in a decrease in urine buffering capacity. Its cardinal feature is
286:
171:
135:
1415:
1132:
431:
kidney develops stones bilaterally; this does not occur in the other RTA types.
2176:
2049:
2019:
1925:
1917:
1850:
1272:
1232:
1215:
1175:"Clinical and laboratory approaches in the diagnosis of renal tubular acidosis"
721:
709:
528:
462:
453:
354:
1802:
1191:
1174:
1084:
1031:
375:
of less than 5.3. Because renal excretion is the primary means of eliminating
119:
40:
2382:
2155:
2090:
1141:
1116:"Sjögren's, Renal Tubular Acidosis And Osteomalacia - An Asian Indian Series"
639:
631:
624:
524:
155:
151:
47:
115:
2260:
2181:
2171:
2133:
1760:
1513:
1464:
1372:
1290:
1241:
1200:
1159:
1092:
1049:
925:
883:
842:
725:
679:
600:
585:
486:
458:
449:
268:
260:
159:
1701:
1608:
1549:
1541:
1423:
1331:
1003:
653:
568:
cells to reabsorb filtered bicarbonate from the urine, leading to urinary
131:
95:
2167:
1946:
729:
696:
658:
593:
569:
445:
312:
163:
16:
Higher blood acidity due to failure of the kidneys to fully acidify urine
1785:
1173:
Santos F, Ordóñez FA, Claramunt-Taberner D, Gil-Peña H (December 2015).
897:
Bianic F, Guelfucci F, Robin L, Martre C, Game D, Bockenhauer D (2021).
1172:
1040:
982:
Buckalew VM (March 1989). "Nephrolithiasis in renal tubular acidosis".
717:
589:
547:
543:
63:
2199:
1717:"The role of inbreeding in the extinction of a European royal dynasty"
1455:
1438:
917:
2326:
2085:
2080:
1869:
1579:
1437:
Shah GN, Bonapace G, Hu PY, Strisciuglio P, Sly WS (September 2004).
1303:
436:
394:
255:
599:
The principal feature of
Fanconi syndrome is bone demineralization (
2238:
1304:
Rodriguez
Soriano J, Boichis H, Stark H, Edelmann CM (March 1967).
762:
671:
628:
619:
Combined dRTA and pRTA is also observed as the result of inherited
573:
389:
291:
183:
167:
31:
1886:
1216:"Pathophysiology of Renal Tubular Acidosis: Core Curriculum 2016"
635:
604:
554:, a complication of both proximal and, less commonly, distal RTA.
551:
482:
472:
365:
123:
1828:
154:(which are acidic) into the latter portions of the nephron (the
2105:
1797:
1526:
962:"Prevalence and incidence of rare diseases: Bibliographic data"
858:"Review of the Diagnostic Evaluation of Renal Tubular Acidosis"
649:
Type 4: absolute hypoaldosteronism or aldosterone insensitivity
638:
region of North Africa, possibly due to the high prevalence of
297:
294:
91:
1070:
1016:
770:
769:
may have suffered from renal tubular acidosis in tandem with
705:
693:): Primary vs. hyporeninemic (including diabetic nephropathy)
170:) from the filtrate in the early portion of the nephron (the
139:
107:
99:
50:
1255:
Pereira PC, Miranda DM, Oliveira EA, Silva AC (March 2009).
1113:
823:
1680:
Lewis DW (December 1992). "What was wrong with Tiny Tim?".
1254:
674:
excretion (impaired ammoniagenesis), which is secondary to
478:
188:
174:). Although a metabolic acidosis also occurs in those with
146:
that results from RTA may be caused either by insufficient
87:
1477:
1436:
86:) is a medical condition that involves an accumulation of
405:
cannot be reclaimed by the cell, leading to acidemia (as
1714:
388:
from the body, there is consequently a tendency towards
1400:
610:
372:
192:
1715:
Alvarez G, Ceballos FC, Quinteiro C (15 April 2009).
1385:
1257:"Molecular pathophysiology of renal tubular acidosis"
820:
187:
refers to the tendency for RTA to cause an excess of
1775:
1390:(3rd ed.). Maarssen: Elsevier. pp. 141–2.
1660:
1631:
564:Proximal RTA (pRTA) is caused by a failure of the
1344:
1213:
765:in 2009 speculated that the infamously afflicted
2380:
785:, who is speculated to have suffered with dRTA
2215:
1902:
1351:Journal of the American Society of Nephrology
1347:"Renal tubular acidosis: the clinical entity"
855:
758:, was suffering from renal tubular acidosis.
1590:
946:: CS1 maint: multiple names: authors list (
657:Type 4 RTA is due either to a deficiency of
1386:de Jong PE, Koomans HA, Weening JJ (2000).
890:
416:builds up in the body) and hypokalemia (as
392:. There is an inability to excrete H while
2222:
2208:
1909:
1895:
1214:Soleimani M, Rastegar A (September 2016).
68:
30:
1750:
1740:
1616:
1503:
1454:
1362:
1321:
1280:
1231:
1190:
1149:
1131:
1039:
873:
748:Donald L. Lewis postulated the character
592:, aminoaciduria, uricosuria, and tubular
427:cannot be reabsorbed by the alpha cell).
1682:American Journal of Diseases of Children
981:
652:
542:
2229:
1569:
1567:
975:
816:
814:
616:also been referred to as juvenile RTA.
334:Autosomal dominant: 1 family described
2381:
461:formation (related to alkaline urine,
331:Prevalence of 46 per 1 million people
2203:
1890:
1679:
771:combined pituitary hormone deficiency
2139:Abderhalden–Kaufmann–Lignac syndrome
1694:10.1001/archpedi.1992.02160240013002
1564:
811:
661:, or to a resistance to its effects.
611:Type 3: combined proximal and distal
90:in the body due to a failure of the
46:(calcification of the kidneys) on a
2363:Mixed disorder of acid-base balance
1404:The New England Journal of Medicine
1345:RodrĂguez Soriano J (August 2002).
1338:
1220:American Journal of Kidney Diseases
538:
315:, or a resistance to its effects, (
230:Collecting Tubules, distal tubules
13:
1916:
1364:10.1097/01.ASN.0000023430.92674.E5
856:Yaxley J, Pirrone C (2016-12-21).
14:
2400:
1771:
342:
1323:10.1203/00006450-196703000-00001
1708:
1673:
1654:
1625:
1584:
1575:Hyporeninemic Hypoaldosteronism
1520:
1471:
1430:
1394:
1379:
1297:
1248:
1207:
560:Proximal renal tubular acidosis
475:in the substance of the kidney)
1166:
1107:
1064:
1010:
954:
849:
670:reduction in proximal tubular
130:, acid equivalents, and other
1:
1648:10.1016/s0022-3476(36)80111-5
1120:The Open Rheumatology Journal
996:10.1016/S0022-5347(17)40997-9
804:
349:Distal renal tubular acidosis
1742:10.1371/journal.pone.0005174
1496:10.1113/jphysiol.2004.065110
835:10.1016/j.biocel.2005.01.002
607:) due to phosphate wasting.
7:
1936:Template:Glomerular disease
1416:10.1056/NEJM198507183130302
1133:10.2174/1874312901408010103
799:Lightwood–Albright syndrome
776:
506:Hereditary hemolytic anemia
465:, and low urinary citrate).
282:to secrete H and reclaim K
191:, which lowers the blood's
126:, allowing for exchange of
10:
2405:
2025:Balkan endemic nephropathy
1273:10.2174/138920209787581262
1233:10.1053/j.ajkd.2016.03.422
740:
627:, renal tubular acidosis,
557:
501:Sensorineural hearing loss
481:demineralisation (causing
346:
134:before it drains into the
2355:
2325:
2303:
2246:
2237:
2116:
2073:
2060:Renovascular hypertension
2035:
2005:
1945:
1924:
1860:
1779:
1635:The Journal of Pediatrics
1484:The Journal of Physiology
1192:10.1007/s00467-015-3083-9
1085:10.1007/s00467-017-3829-7
1032:10.1007/s00467-018-3965-8
761:Researchers published in
584:, in which there is also
359:medullary collecting duct
197:normal anion gap acidosis
57:
38:
29:
24:
2161:Renal papillary necrosis
2055:Hypertensive nephropathy
689:Aldosterone deficiency (
202:
2065:Renal cortical necrosis
789:Hyperchloremic acidosis
735:Pseudohypoaldosteronism
572:wasting and subsequent
321:pseudohypoaldosteronism
2271:Alcoholic ketoacidosis
2101:Chronic kidney disease
2015:Interstitial nephritis
1972:Acute tubular necrosis
1955:Renal tubular acidosis
984:The Journal of Urology
662:
555:
287:proximal tubular cells
176:chronic kidney disease
80:Renal tubular acidosis
25:Renal tubular acidosis
2368:Acid–base homeostasis
2340:Contraction alkalosis
2266:Diabetic ketoacidosis
2124:Analgesic nephropathy
2045:Renal artery stenosis
1609:10.1136/adc.10.57.205
1593:"Communication no. 1"
1591:Lightwood R. (1935).
1542:10.1007/s004390050419
656:
546:
328:Prevalence/Incidence
2129:Renal osteodystrophy
1388:Klinische Nefrologie
1179:Pediatric Nephrology
1073:Pediatric Nephrology
1020:Pediatric Nephrology
794:Hypokalemic acidosis
280:α intercalated cells
2231:Acid–base disorders
2096:Acute renal failure
1733:2009PLoSO...4.5174A
862:The Ochsner Journal
783:Charles II of Spain
767:Charles II of Spain
158:) or by failure to
118:passes through the
114:by the kidney, the
2187:Reflux nephropathy
2144:Diabetes insipidus
1926:Glomerular disease
1861:External resources
1310:Pediatric Research
663:
621:carbonic anhydrase
578:metabolic acidosis
556:
440:metabolic acidosis
250:Mild when present
244:Yes (very severe)
144:metabolic acidosis
2376:
2375:
2321:
2320:
2197:
2196:
2074:General syndromes
1995:Liddle's syndrome
1990:Gitelman syndrome
1884:
1883:
1456:10.1002/humu.9266
990:(3 Pt 2): 731–7.
918:10.1159/000516876
755:A Christmas Carol
691:hypoaldosteronism
676:hypoaldosteronism
492:Growth deficiency
340:
339:
317:hypoaldosteronism
233:Proximal tubules
94:to appropriately
77:
76:
19:Medical condition
2396:
2283:Normal anion gap
2244:
2243:
2224:
2217:
2210:
2201:
2200:
1985:Bartter syndrome
1980:Fanconi syndrome
1911:
1904:
1897:
1888:
1887:
1777:
1776:
1765:
1764:
1754:
1744:
1712:
1706:
1705:
1677:
1671:
1670:
1658:
1652:
1651:
1629:
1623:
1622:
1620:
1588:
1582:
1571:
1562:
1561:
1524:
1518:
1517:
1507:
1475:
1469:
1468:
1458:
1434:
1428:
1427:
1398:
1392:
1391:
1383:
1377:
1376:
1366:
1342:
1336:
1335:
1325:
1301:
1295:
1294:
1284:
1261:Current Genomics
1252:
1246:
1245:
1235:
1211:
1205:
1204:
1194:
1185:(12): 2099–107.
1170:
1164:
1163:
1153:
1135:
1111:
1105:
1104:
1068:
1062:
1061:
1043:
1026:(9): 1523–1529.
1014:
1008:
1007:
979:
973:
972:
966:
958:
952:
951:
945:
937:
903:
894:
888:
887:
877:
853:
847:
846:
818:
685:Causes include:
582:Fanconi syndrome
566:proximal tubular
550:of a child with
539:Type 2: proximal
485:in children and
469:Nephrocalcinosis
426:
425:
424:
415:
414:
413:
404:
402:
401:
387:
385:
384:
308:
307:
306:
275:Pathophysiology
210:
209:
166:ions (which are
104:renal physiology
73:
72:
44:nephrocalcinosis
34:
22:
21:
2404:
2403:
2399:
2398:
2397:
2395:
2394:
2393:
2389:Kidney diseases
2379:
2378:
2377:
2372:
2351:
2317:
2299:
2233:
2228:
2198:
2193:
2112:
2069:
2031:
2001:
1941:
1920:
1915:
1885:
1880:
1879:
1856:
1855:
1788:
1774:
1769:
1768:
1713:
1709:
1678:
1674:
1659:
1655:
1630:
1626:
1589:
1585:
1572:
1565:
1525:
1521:
1490:(Pt 1): 55–65.
1476:
1472:
1435:
1431:
1399:
1395:
1384:
1380:
1343:
1339:
1302:
1298:
1253:
1249:
1212:
1208:
1171:
1167:
1112:
1108:
1069:
1065:
1015:
1011:
980:
976:
964:
960:
959:
955:
939:
938:
901:
895:
891:
854:
850:
819:
812:
807:
779:
743:
651:
613:
562:
541:
496:Medullary cysts
471:(deposition of
423:
421:
420:
419:
417:
412:
410:
409:
408:
406:
400:
398:
397:
396:
393:
383:
381:
380:
379:
376:
351:
345:
305:
302:
301:
300:
290:
205:
172:proximal tubule
67:
20:
17:
12:
11:
5:
2402:
2392:
2391:
2374:
2373:
2371:
2370:
2365:
2359:
2357:
2353:
2352:
2350:
2349:
2344:
2343:
2342:
2331:
2329:
2323:
2322:
2319:
2318:
2316:
2315:
2309:
2307:
2301:
2300:
2298:
2297:
2296:
2295:
2290:
2288:Hyperchloremic
2280:
2279:
2278:
2273:
2268:
2263:
2256:High anion gap
2252:
2250:
2241:
2235:
2234:
2227:
2226:
2219:
2212:
2204:
2195:
2194:
2192:
2191:
2190:
2189:
2184:
2179:
2177:Hydronephrosis
2165:
2164:
2163:
2153:
2152:
2151:
2141:
2136:
2131:
2126:
2120:
2118:
2114:
2113:
2111:
2110:
2109:
2108:
2103:
2098:
2088:
2083:
2077:
2075:
2071:
2070:
2068:
2067:
2062:
2057:
2052:
2050:Renal ischemia
2047:
2041:
2039:
2033:
2032:
2030:
2029:
2028:
2027:
2022:
2020:Pyelonephritis
2011:
2009:
2003:
2002:
2000:
1999:
1998:
1997:
1992:
1987:
1982:
1974:
1969:
1968:
1967:
1962:
1951:
1949:
1943:
1942:
1940:
1939:
1930:
1928:
1922:
1921:
1918:Kidney disease
1914:
1913:
1906:
1899:
1891:
1882:
1881:
1878:
1877:
1865:
1864:
1862:
1858:
1857:
1854:
1853:
1842:
1831:
1820:
1805:
1789:
1784:
1783:
1781:
1780:Classification
1773:
1772:External links
1770:
1767:
1766:
1707:
1688:(12): 1403–7.
1672:
1653:
1624:
1597:Arch Dis Child
1583:
1563:
1530:Human Genetics
1519:
1470:
1443:Human Mutation
1429:
1393:
1378:
1357:(8): 2160–70.
1337:
1296:
1247:
1206:
1165:
1106:
1079:(3): 485–491.
1063:
1009:
974:
953:
912:(5): 486–495.
889:
868:(4): 525–530.
848:
829:(6): 1151–61.
809:
808:
806:
803:
802:
801:
796:
791:
786:
778:
775:
742:
739:
738:
737:
732:
722:Spironolactone
710:ACE inhibitors
701:
700:
694:
650:
647:
612:
609:
558:Main article:
540:
537:
529:hyperammonemia
525:amino aciduria
509:
508:
503:
498:
493:
490:
476:
466:
463:hypercalciuria
456:
454:Hyperchloremia
443:
422:
411:
399:
382:
347:Main article:
344:
343:Type 1: distal
341:
338:
337:
335:
332:
329:
325:
324:
311:Deficiency of
309:
303:
283:
276:
272:
271:
266:
263:
258:
252:
251:
248:
245:
242:
238:
237:
234:
231:
228:
224:
223:
220:
217:
214:
204:
201:
75:
74:
61:
55:
54:
36:
35:
27:
26:
18:
15:
9:
6:
4:
3:
2:
2401:
2390:
2387:
2386:
2384:
2369:
2366:
2364:
2361:
2360:
2358:
2354:
2348:
2345:
2341:
2338:
2337:
2336:
2333:
2332:
2330:
2328:
2324:
2314:
2311:
2310:
2308:
2306:
2302:
2294:
2293:Renal tubular
2291:
2289:
2286:
2285:
2284:
2281:
2277:
2274:
2272:
2269:
2267:
2264:
2262:
2259:
2258:
2257:
2254:
2253:
2251:
2249:
2245:
2242:
2240:
2236:
2232:
2225:
2220:
2218:
2213:
2211:
2206:
2205:
2202:
2188:
2185:
2183:
2180:
2178:
2175:
2174:
2173:
2169:
2166:
2162:
2159:
2158:
2157:
2156:Renal papilla
2154:
2150:
2147:
2146:
2145:
2142:
2140:
2137:
2135:
2132:
2130:
2127:
2125:
2122:
2121:
2119:
2115:
2107:
2104:
2102:
2099:
2097:
2094:
2093:
2092:
2091:Renal failure
2089:
2087:
2084:
2082:
2079:
2078:
2076:
2072:
2066:
2063:
2061:
2058:
2056:
2053:
2051:
2048:
2046:
2043:
2042:
2040:
2038:
2034:
2026:
2023:
2021:
2018:
2017:
2016:
2013:
2012:
2010:
2008:
2004:
1996:
1993:
1991:
1988:
1986:
1983:
1981:
1978:
1977:
1975:
1973:
1970:
1966:
1963:
1961:
1958:
1957:
1956:
1953:
1952:
1950:
1948:
1944:
1938:
1937:
1932:
1931:
1929:
1927:
1923:
1919:
1912:
1907:
1905:
1900:
1898:
1893:
1892:
1889:
1876:
1872:
1871:
1867:
1866:
1863:
1859:
1852:
1848:
1847:
1843:
1841:
1837:
1836:
1832:
1830:
1826:
1825:
1821:
1819:
1815:
1814:
1810:
1806:
1804:
1800:
1799:
1795:
1791:
1790:
1787:
1782:
1778:
1762:
1758:
1753:
1748:
1743:
1738:
1734:
1730:
1726:
1722:
1718:
1711:
1703:
1699:
1695:
1691:
1687:
1683:
1676:
1668:
1664:
1657:
1649:
1645:
1642:(4): 489–99.
1641:
1637:
1636:
1628:
1619:
1614:
1610:
1606:
1603:(57): 205–6.
1602:
1598:
1594:
1587:
1581:
1577:
1576:
1570:
1568:
1559:
1555:
1551:
1547:
1543:
1539:
1535:
1531:
1523:
1515:
1511:
1506:
1501:
1497:
1493:
1489:
1485:
1481:
1474:
1466:
1462:
1457:
1452:
1448:
1444:
1440:
1433:
1425:
1421:
1417:
1413:
1410:(3): 139–45.
1409:
1405:
1397:
1389:
1382:
1374:
1370:
1365:
1360:
1356:
1352:
1348:
1341:
1333:
1329:
1324:
1319:
1315:
1311:
1307:
1300:
1292:
1288:
1283:
1278:
1274:
1270:
1266:
1262:
1258:
1251:
1243:
1239:
1234:
1229:
1226:(3): 488–98.
1225:
1221:
1217:
1210:
1202:
1198:
1193:
1188:
1184:
1180:
1176:
1169:
1161:
1157:
1152:
1147:
1143:
1139:
1134:
1129:
1125:
1121:
1117:
1110:
1102:
1098:
1094:
1090:
1086:
1082:
1078:
1074:
1067:
1059:
1055:
1051:
1047:
1042:
1037:
1033:
1029:
1025:
1021:
1013:
1005:
1001:
997:
993:
989:
985:
978:
971:. 2022-01-01.
970:
963:
957:
949:
943:
935:
931:
927:
923:
919:
915:
911:
907:
900:
893:
885:
881:
876:
871:
867:
863:
859:
852:
844:
840:
836:
832:
828:
824:
817:
815:
810:
800:
797:
795:
792:
790:
787:
784:
781:
780:
774:
772:
768:
764:
759:
757:
756:
751:
746:
736:
733:
731:
727:
723:
719:
715:
711:
707:
703:
702:
698:
695:
692:
688:
687:
686:
683:
681:
677:
673:
669:
668:physiological
660:
655:
646:
643:
641:
640:consanguinity
637:
633:
632:calcification
630:
626:
625:osteopetrosis
622:
617:
608:
606:
602:
597:
595:
591:
587:
583:
579:
575:
571:
567:
561:
553:
549:
545:
536:
532:
530:
526:
522:
518:
514:
507:
504:
502:
499:
497:
494:
491:
488:
484:
480:
477:
474:
470:
467:
464:
460:
459:Urinary stone
457:
455:
451:
447:
444:
441:
438:
434:
433:
432:
428:
403:
391:
386:
374:
369:
367:
364:
360:
356:
350:
336:
333:
330:
327:
326:
322:
318:
314:
310:
299:
296:
293:
288:
284:
281:
277:
274:
273:
270:
267:
264:
262:
259:
257:
254:
253:
249:
246:
243:
240:
239:
235:
232:
229:
226:
225:
221:
218:
215:
212:
211:
208:
200:
198:
194:
190:
186:
185:
179:
177:
173:
169:
165:
161:
157:
156:distal tubule
153:
152:hydrogen ions
149:
145:
141:
137:
133:
129:
125:
121:
117:
113:
109:
105:
101:
97:
93:
89:
85:
81:
71:
65:
62:
60:
56:
52:
49:
45:
42:
37:
33:
28:
23:
2292:
2261:Ketoacidosis
2182:Pyonephrosis
2134:Nephroptosis
2007:Interstitium
1954:
1933:
1868:
1844:
1833:
1822:
1807:
1792:
1727:(4): e5174.
1724:
1720:
1710:
1685:
1681:
1675:
1666:
1662:
1656:
1639:
1633:
1627:
1600:
1596:
1586:
1573:
1536:(5): 634–7.
1533:
1529:
1522:
1487:
1483:
1473:
1446:
1442:
1432:
1407:
1403:
1396:
1387:
1381:
1354:
1350:
1340:
1316:(2): 81–98.
1313:
1309:
1299:
1264:
1260:
1250:
1223:
1219:
1209:
1182:
1178:
1168:
1123:
1119:
1109:
1076:
1072:
1066:
1023:
1019:
1012:
987:
983:
977:
968:
956:
942:cite journal
909:
905:
892:
865:
861:
851:
826:
822:
760:
753:
747:
744:
726:Trimethoprim
684:
680:hyperkalemia
667:
664:
644:
618:
614:
601:osteomalacia
598:
586:phosphaturia
563:
533:
520:
516:
512:
510:
487:osteomalacia
450:Hypocalcemia
429:
370:
352:
289:to reabsorb
269:Hyperkalemia
265:Hypokalemia
261:Hypokalaemia
206:
182:
180:
83:
79:
78:
39:Significant
2347:Respiratory
2313:Respiratory
2305:Respiratory
2168:Major calyx
2149:Nephrogenic
1267:(1): 51–9.
1126:: 103–109.
1041:10651/48680
730:Pentamidine
697:Aldosterone
659:Aldosterone
594:proteinuria
570:bicarbonate
446:Hypokalemia
313:aldosterone
285:Failure of
278:Failure of
164:bicarbonate
162:sufficient
1846:DiseasesDB
1449:(3): 272.
805:References
718:Eplerenone
699:resistance
590:glycosuria
548:Radiograph
489:in adults)
64:Nephrology
2335:Metabolic
2327:Alkalosis
2248:Metabolic
2086:Nephrosis
2081:Nephritis
1870:eMedicine
1669:: 113–23.
1580:eMedicine
1142:1874-3129
934:235713623
442:/acidemia
437:anion gap
256:Potassium
241:Acidemia
227:Location
181:The word
148:secretion
59:Specialty
41:bilateral
2383:Category
2239:Acidosis
2037:Vascular
1976:Genetic
1960:proximal
1875:med/1071
1761:19367331
1721:PLOS ONE
1558:24978813
1514:15218065
1465:15300855
1373:12138150
1291:19721811
1242:27188519
1201:25823989
1160:25584094
1093:29134448
1058:19096598
1050:29725771
969:Orphanet
926:34198293
884:27999512
843:15778079
777:See also
763:PLOS ONE
750:Tiny Tim
672:ammonium
629:cerebral
574:acidemia
517:ATP6V0A4
513:ATP6V1B1
390:acidemia
236:Adrenal
184:acidosis
168:alkaline
160:reabsorb
116:filtrate
112:filtered
1947:Tubules
1840:D000141
1752:2664480
1729:Bibcode
1702:1340779
1663:Q J Med
1618:1975385
1550:9150731
1505:1665076
1424:3925334
1332:6029811
1282:2699831
1151:4286932
1101:3291184
1004:2645431
906:Nephron
875:5158160
741:History
704:Drugs:
636:Maghreb
605:rickets
552:rickets
483:rickets
473:calcium
435:Normal
366:nephron
361:of the
357:of the
222:Type 4
219:Type 2
216:Type 1
136:bladder
132:solutes
124:nephron
122:of the
120:tubules
106:, when
96:acidify
92:kidneys
48:frontal
2276:Lactic
2172:pelvis
2106:Uremia
1965:distal
1829:179800
1818:588.89
1759:
1749:
1700:
1615:
1556:
1548:
1512:
1502:
1463:
1422:
1371:
1330:
1289:
1279:
1240:
1199:
1158:
1148:
1140:
1099:
1091:
1056:
1048:
1002:
932:
924:
882:
872:
841:
706:NSAIDs
521:SLC4A1
363:distal
142:. The
66:
2356:Other
2117:Other
1851:11687
1803:N25.8
1554:S2CID
1097:S2CID
1054:S2CID
965:(PDF)
930:S2CID
902:(PDF)
752:, of
355:cells
213:Type
203:Types
140:urine
128:salts
108:blood
102:. In
100:urine
51:X-ray
1934:See
1835:MeSH
1824:OMIM
1813:9-CM
1757:PMID
1698:PMID
1546:PMID
1510:PMID
1461:PMID
1420:PMID
1369:PMID
1328:PMID
1287:PMID
1238:PMID
1197:PMID
1156:PMID
1138:ISSN
1089:PMID
1046:PMID
1000:PMID
948:link
922:PMID
880:PMID
839:PMID
714:ARBs
712:and
527:and
515:and
479:Bone
247:Yes
189:acid
98:the
88:acid
1809:ICD
1794:ICD
1747:PMC
1737:doi
1690:doi
1686:146
1644:doi
1613:PMC
1605:doi
1578:at
1538:doi
1500:PMC
1492:doi
1488:559
1451:doi
1412:doi
1408:313
1359:doi
1318:doi
1277:PMC
1269:doi
1228:doi
1187:doi
1146:PMC
1128:doi
1081:doi
1036:hdl
1028:doi
992:doi
988:141
914:doi
910:145
870:PMC
831:doi
603:or
319:or
150:of
138:as
110:is
84:RTA
2385::
1873::
1849::
1838::
1827::
1816::
1801::
1798:10
1755:.
1745:.
1735:.
1723:.
1719:.
1696:.
1684:.
1667:14
1665:.
1638:.
1611:.
1601:10
1599:.
1595:.
1566:^
1552:.
1544:.
1534:99
1532:.
1508:.
1498:.
1486:.
1482:.
1459:.
1447:24
1445:.
1441:.
1418:.
1406:.
1367:.
1355:13
1353:.
1349:.
1326:.
1312:.
1308:.
1285:.
1275:.
1265:10
1263:.
1259:.
1236:.
1224:68
1222:.
1218:.
1195:.
1183:30
1181:.
1177:.
1154:.
1144:.
1136:.
1122:.
1118:.
1095:.
1087:.
1077:33
1075:.
1052:.
1044:.
1034:.
1024:33
1022:.
998:.
986:.
967:.
944:}}
940:{{
928:.
920:.
908:.
904:.
878:.
866:16
864:.
860:.
837:.
827:37
825:.
813:^
773:.
728:,
724:,
720:,
716:,
708:,
596:.
588:,
452:,
448:,
373:pH
368:.
323:)
199:.
193:pH
2223:e
2216:t
2209:v
2170:/
1910:e
1903:t
1896:v
1811:-
1796:-
1786:D
1763:.
1739::
1731::
1725:4
1704:.
1692::
1650:.
1646::
1640:8
1621:.
1607::
1560:.
1540::
1516:.
1494::
1467:.
1453::
1426:.
1414::
1375:.
1361::
1334:.
1320::
1314:1
1293:.
1271::
1244:.
1230::
1203:.
1189::
1162:.
1130::
1124:8
1103:.
1083::
1060:.
1038::
1030::
1006:.
994::
950:)
936:.
916::
886:.
845:.
833::
418:K
407:H
395:K
378:H
304:3
298:O
295:C
292:H
82:(
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