435:, an immotile, hair-like cellular organelle present on the surface of most cells in the body, anchored in the cell body by the basal body. In the kidney, primary cilia have been found to be present on most cells of the nephron, projecting from the apical surface of the renal epithelium into the tubule lumen. The cilia were believed to bend in the urine flow, leading to changes in signalling, however this has since been shown to be an experimental error (the bending of cilia was an artifact of focal plane compensation, and also the actual effect on micturition by severe hypertension and cardiac arrest) and that bending of cilia does not contribute to alterations in Ca flux. While it is not known how defects in the primary cilium lead to cyst development, it is thought to possibly be related to disruption of one of the many signaling pathways regulated by the primary cilium, including intracellular calcium, Wnt/β-catenin, cyclic adenosine monophosphate (cAMP), or planar cell polarity (PCP). Function of the primary cilium is impaired, resulting in disruption of a number of intracellular signaling cascades which produce differentiation of cystic epithelium, increased cell division, increased apoptosis, and loss of resorptive capacity.
298:
51:
290:
484:
2387:
504:(ADPKD) in mice. Patient communities have been combining both ketogenic diets and time-restricted feeding with a low-oxalate diet to prevent the formation of stones and early reports show an average of 17% increase in kidney function after approximately one year on a ketogenic, time-restricted dietary regimen. If and when the disease progresses enough in a given case, the
404:
523:, which can be done at least two different ways at varying frequencies and durations (whether it is done at home or in the clinic depends on the method used and the patient's stability and training) and eventually, if they are eligible because of the nature and severity of their condition and if a suitable match can be found, unilateral or bilateral
282:
499:
might be able to halt, or even reverse progression in mice, and the results of a first human case series study are showing potential benefit. The results of a 3-month randomized, prospective dietary intervention clinical trial are pending. In addition, recent research indicates that mild to moderate
427:
The disease is characterized by a 'second hit' phenomenon, in which a mutated dominant allele is inherited from a parent, with cyst formation occurring only after the normal, wild-type gene sustains a subsequent second genetic 'hit', resulting in renal tubular cyst formation and disease progression.
423:
proteins appear to be involved in both autosomal dominant and recessive polycystic kidney disease due to defects in both proteins. Both proteins have communication with calcium channel proteins, and cause reduction in resting (intracellular) calcium and endoplasmic reticulum storage of calcium.
139:
within the kidney. These cysts may begin to develop in utero, in infancy, in childhood, or in adulthood. Cysts are non-functioning tubules filled with fluid pumped into them, which range in size from microscopic to enormous, crushing adjacent normal tubules and eventually rendering them
565:
PKD is one of the most common hereditary diseases in the United States, affecting more than 600,000 people. It is the cause of nearly 10% of all end-stage renal disease. It equally affects men, women, and all races. PKD occurs in some animals as well as humans.
495:) was introduced as the first FDA-approved treatment for PKD. In a recent long-term study, patients using Tolvaptan had a 6.4% higher kidney function after 5 years compared to standard of care. In 2019, a team of researchers at UCSB found that a
2273:
2258:
143:
PKD is caused by abnormal genes that produce a specific abnormal protein; this protein has an adverse effect on tubule development. PKD is a general term for two types, each having their own pathology and genetic cause:
258:
PKD is caused by abnormal genes which produce a specific abnormal protein which has an adverse effect on tubule development. PKD is a general term for two types, each having their own pathology and genetic cause:
2433:
182:
Diagnosis may be suspected from one, some, or all of the following: new onset flank pain or red urine; a positive family history; palpation of enlarged kidneys on physical exam; an incidental finding on abdominal
387:(ARPKD) (OMIM #263200) is the less common of the two types of PKD, with an incidence of 1:20,000 live births and is typically identified in the first few weeks after birth. Unfortunately, the kidneys are often
832:
773:
2047:
1282:
Kimberling WJ, Kumar S, Gabow PA, Kenyon JB, Connolly CJ, Somlo S (December 1993). "Autosomal dominant polycystic kidney disease: localization of the second gene to chromosome 4q13-q23".
1190:
Kumar S, Kimberling WJ, Gabow PA, Kenyon JB (June 1991). "Genetic linkage studies of autosomal dominant polycystic kidney disease: search for the second gene in a large
Sicilian family".
2426:
1317:
Mochizuki T, Wu G, Hayashi T, Xenophontos SL, Veldhuisen B, Saris JJ, et al. (May 1996). "PKD2, a gene for polycystic kidney disease that encodes an integral membrane protein".
2419:
367:, although fewer than 5% of nephrons are thought to be involved. As the cysts accumulate fluid, they enlarge, separate from the nephron, compress the neighboring kidney
1608:"Ketogenic dietary interventions in autosomal dominant polycystic kidney disease-a retrospective case series study: first insights into feasibility, safety and effects"
334:
3319:
345:
and codes for a protein involved in cell cycle regulation and intracellular calcium transport in epithelial cells and is responsible for 85% of the cases of ADPKD.
308:
polycystic kidney disease (ADPKD) is the most common of all the inherited cystic kidney diseases with an incidence of 1:500 live births. Studies show that 10% of
575:
384:
264:
207:
149:
1427:
501:
276:
260:
145:
359:
PKD3 recently appeared in research papers as a postulated third gene. Fewer than 10% of cases of ADPKD appear in non-ADPKD families. Cyst formation begins
862:
840:
2073:
2739:
534:, to control infections of the cysts in the kidneys, and if affected, the liver, when needed for a certain duration to combat infection, by using, "
211:
2051:
650:
530:
A Cochrane Review study of autosomal dominant polycystic kidney disease made note of the fact that it is important at all times, while avoiding
628:
3276:
3066:
2031:
3144:
3061:
196:
778:
2288:
1818:
1437:
872:
672:
352:, with inward selectivity for K>Na>>Ca and outward selectivity for Ca2+ ≈ Ba2+ > Na+ ≈ K+, are coded for by PKD2 on
3180:
3073:
2834:
3271:
3106:
2802:
2764:
2732:
2511:
219:
3261:
2927:
1094:"Mutations in GANAB, Encoding the Glucosidase IIα Subunit, Cause Autosomal-Dominant Polycystic Kidney and Liver Disease"
483:
2992:
2838:
1770:"A mild reduction of food intake slows disease progression in an orthologous mouse model of polycystic kidney disease"
297:
289:
50:
3266:
3256:
3193:
3289:
3085:
2900:
2882:
2812:
2647:
509:
223:
3038:
3309:
3137:
2987:
2912:
2769:
2725:
2642:
2299:
1478:
309:
210:(RAAS), frequent cyst infections, urinary bleeding, and declining renal function. Hypertension is treated with
2411:
2402:
411:
Both autosomal dominant and autosomal recessive polycystic kidney disease cyst formation are tied to abnormal
1672:"Metabolic Reprogramming in Autosomal Dominant Polycystic Kidney Disease: Evidence and Therapeutic Potential"
2895:
2869:
2850:
2702:
2606:
2516:
2077:
234:. Management from the time of the suspected or definitive diagnosis is by an appropriately trained doctor.
176:
2370:
1891:
959:
Bisceglia M, Galliani CA, Senger C, Stallone C, Sessa A (January 2006). "Renal cystic diseases: a review".
550:
ADPKD individuals might have a normal life; conversely, ARPKD can cause kidney dysfunction and can lead to
2979:
2974:
2945:
524:
2854:
2495:
2487:
1844:"Crystal deposition triggers tubule dilation that accelerates cystogenesis in polycystic kidney disease"
1235:"Exclusion of autosomal dominant polycystic kidney disease type II (ADPKD2) from 160 cM of chromosome 1"
531:
231:
2807:
2784:
2667:
2540:
1326:
305:
301:
Cartoon of autosomal recessive polycystic kidney disease with normal kidney inset to right of diagram
1557:
Torres JA, Kruger SL, Broderick C, Amarlkhagva T, Agrawal S, Dodam JR, et al. (December 2019).
293:
Cartoon of autosomal dominant polycystic kidney disease with normal kidney inset to right of diagram
3314:
3251:
3130:
3020:
3005:
2442:
2277:
1092:
Porath B, Gainullin VG, Cornec-Le Gall E, Dillinger EK, Heyer CM, Hopp K, et al. (June 2016).
3111:
2756:
1936:
1350:
1215:
1030:
984:
348:
Gene PKD2 is identified, using genetic linkage study, on chromosome 4. A group of voltage-linked
75:
152:(ARPKD). The abnormal gene exists in all cells in the body; as a result, cysts may occur in the
3015:
2954:
2826:
2820:
2614:
2585:
2552:
2339:
2310:
2223:
2178:
2135:
2027:
2004:
1975:"Interventions for preventing the progression of autosomal dominant polycystic kidney disease"
1919:"Interventions for infected cysts in people with autosomal dominant polycystic kidney disease"
1873:
1799:
1750:
1701:
1637:
1588:
1539:
1490:
1433:
1408:
1342:
1299:
1264:
1207:
1172:
1123:
1074:
1022:
976:
930:
868:
814:
756:
725:
604:
172:
93:
63:
1005:
Torres VE, Harris PC, Pirson Y (April 2007). "Autosomal dominant polycystic kidney disease".
3235:
3219:
3078:
2997:
2969:
2959:
2535:
2525:
2477:
2445:
2213:
2203:
2168:
2160:
2125:
2117:
1994:
1986:
1926:
1863:
1855:
1789:
1781:
1740:
1732:
1691:
1683:
1627:
1619:
1578:
1570:
1529:
1521:
1398:
1388:
1334:
1291:
1254:
1246:
1199:
1162:
1154:
1113:
1105:
1064:
1014:
968:
920:
912:
804:
715:
707:
192:
169:
157:
128:
1955:
3206:
3201:
2964:
2798:
2697:
2557:
2506:
2482:
554:
by the age of 40–60. ADPKD1 and ADPKD2 are very different, in that ADPKD2 is much milder.
520:
372:
227:
222:(ARBs). Infections are treated with antibiotics. Declining renal function is treated with
2164:
1657:"Ketogenic Dietary Interventions in Autosomal Dominant Polycystic Kidney Disease (ADPKD)"
1606:
Strubl S, Oehm S, Torres JA, Grundmann F, Haratani J, Decker M, et al. (June 2022).
17:
2392:
1508:
Zhou X, Davenport E, Ouyang J, Hoke ME, Garbinsky D, Agarwal I, et al. (May 2022).
1330:
557:
Currently, there are no therapies proven effective to prevent the progression of ADPKD.
250:. Other symptoms include pain in the back, and cyst formation (renal and other organs).
3048:
3030:
3010:
2935:
2905:
2774:
2575:
2472:
2464:
2449:
2218:
2191:
2173:
2148:
2130:
2105:
1999:
1974:
1868:
1843:
1794:
1769:
1745:
1720:
1696:
1671:
1632:
1607:
1583:
1558:
1534:
1509:
1403:
1376:
1259:
1234:
1167:
1142:
1141:
Thivierge C, Kurbegovic A, Couillard M, Guillaume R, Coté O, Trudel M (February 2006).
1118:
1093:
972:
925:
900:
720:
695:
551:
535:
513:
496:
464:
432:
349:
2282:
1295:
1018:
711:
3303:
2545:
2530:
539:
460:
342:
184:
1940:
1354:
1219:
1158:
988:
3101:
2874:
2692:
2350:
2192:"Polycystic kidney disease: inheritance, pathophysiology, prognosis, and treatment"
1990:
1842:
Torres JA, Rezaei M, Broderick C, Lin L, Wang X, Hoppe B, et al. (July 2019).
1377:"Polycystic kidney disease: inheritance, pathophysiology, prognosis, and treatment"
1034:
505:
420:
416:
353:
313:
132:
1656:
1338:
135:
become structurally abnormal, resulting in the development and growth of multiple
2304:
1973:
Bolignano D, Palmer SC, Ruospo M, Zoccali C, Craig JC, Strippoli GF (July 2015).
1453:
916:
2917:
2637:
2334:
1785:
2359:
2356:
2353:
2315:
1719:
Warner G, Hein KZ, Nin V, Edwards M, Chini CC, Hopp K, et al. (May 2016).
1574:
1525:
1510:"Pooled Data Analysis of the Long-Term Treatment Effects of Tolvaptan in ADPKD"
1109:
809:
792:
3214:
3188:
3153:
2861:
2830:
2632:
2580:
2365:
1931:
1918:
452:
388:
368:
68:
1956:"Polycystic Kidney Disease: Practice Essentials, Background, Pathophysiology"
1494:
2345:
2267:
1233:
Kumar S, Kimberling WJ, Gabow PA, Shugart YY, Pieke-Dahl S (November 1990).
744:
492:
243:
187:; or an incidental finding of abnormal kidney function on routine lab work (
2227:
2182:
2139:
2008:
1877:
1803:
1754:
1736:
1721:"Food Restriction Ameliorates the Development of Polycystic Kidney Disease"
1705:
1641:
1592:
1543:
1412:
1176:
1127:
1078:
1026:
980:
934:
818:
729:
242:
Signs and symptoms include high blood pressure, headaches, abdominal pain,
2250:
2121:
1623:
1346:
1303:
1268:
1250:
1211:
1069:
1052:
760:
675:. National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
653:. National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
631:. National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
2887:
1687:
319:
247:
165:
161:
2208:
1393:
403:
2684:
2624:
1203:
508:
or other practitioner and the patient will have to decide what form of
500:
calorie restriction or time-restricted feeding slow the progression of
444:
364:
316:
in Europe and the U.S. were initially diagnosed and treated for ADPKD.
200:
58:
Severely affected polycystic kidneys removed at time of transplantation
1859:
2567:
2262:
360:
3122:
1559:"Ketosis Ameliorates Renal Cyst Growth in Polycystic Kidney Disease"
2717:
2048:"Polycystic kidney disease (PKD): Gene test and negative register"
456:
412:
402:
392:
323:
280:
153:
2748:
2293:
864:
330:
326:
281:
215:
206:
Complications include hypertension due to the activation of the
136:
3126:
2721:
2415:
448:
188:
2196:
International
Journal of Nephrology and Renovascular Disease
1917:
Montero N, Sans L, Webster AC, Pascual J (29 January 2014).
1381:
International
Journal of Nephrology and Renovascular Disease
1053:"Polycystic kidney disease: cell division without a c(l)ue?"
285:
CT scan showing autosomal dominant polycystic kidney disease
1768:
Kipp KR, Rezaei M, Lin L, Dewey EC, Weimbs T (April 2016).
455:
of the same area. A physical exam/test can reveal enlarged
1429:
Comprehensive
Clinical Nephrology: Expert Consult - Online
1143:"Overexpression of PKD1 causes polycystic kidney disease"
901:"MicroRNAs in the pathogenesis of cystic kidney disease"
391:, resulting in a 30% death rate in newborns with ARPKD.
1819:"6 Ways People with PKD Can Lower Their Blood Pressure"
743:
Kimberling WJ, Pieke-Dahl SA, Kumar S (November 1991).
475:
Most cases progress to bilateral disease in adulthood.
1676:
Clinical
Journal of the American Society of Nephrology
2074:"PKD - Polycystic Kidney Disease - British Shorthair"
2026:. Philadelphia, PA: F.A. Davis Company. p. 443.
1954:
Torra R (2018-07-20). Talavera F, Aronoff GR (eds.).
1892:"Breakthrough Results After 7 Years of Reversing Pkd"
516:, typically stage 4 or 5 of chronic kidney disease).
2240:
3244:
3228:
3160:
3094:
3047:
3029:
2944:
2926:
2783:
2755:
2683:
2676:
2660:
2623:
2605:
2598:
2566:
2463:
2456:
2325:
2244:
443:Polycystic kidney disease can be ascertained via a
102:
92:
84:
74:
62:
40:
35:
1375:Halvorson CR, Bremmer MS, Jacobs SC (2010-06-24).
1370:
1368:
1366:
1364:
164:. This genetic defect can also cause aortic root
1774:American Journal of Physiology. Renal Physiology
954:
952:
950:
948:
946:
944:
512:will be used to treat end-stage kidney disease (
2106:"The cell biology of polycystic kidney disease"
1912:
1910:
1908:
1426:Johnson RJ, Feehally J, Floege J (2014-09-05).
745:"The genetics of cystic diseases of the kidney"
651:"Autosomal Recessive Polycystic Kidney Disease"
629:"Autosomal Dominant Polycystic Kidney Disease"
3138:
2733:
2427:
1725:Journal of the American Society of Nephrology
1046:
1044:
1000:
998:
894:
892:
890:
888:
886:
884:
576:Autosomal recessive polycystic kidney disease
385:Autosomal recessive polycystic kidney disease
265:autosomal recessive polycystic kidney disease
199:). Definitive diagnosis is made by abdominal
150:autosomal recessive polycystic kidney disease
8:
2190:Halvorson CR, Bremmer MS, Jacobs SC (2010).
502:autosomal dominant polycystic kidney disease
277:Autosomal dominant polycystic kidney disease
261:autosomal dominant polycystic kidney disease
146:autosomal dominant polycystic kidney disease
1979:The Cochrane Database of Systematic Reviews
3145:
3131:
3123:
2740:
2726:
2718:
2680:
2602:
2460:
2434:
2420:
2412:
2241:
49:
32:
2217:
2207:
2172:
2129:
2024:Fifth Edition: Diseases of the Human Body
1998:
1930:
1867:
1793:
1744:
1695:
1631:
1582:
1533:
1402:
1392:
1258:
1166:
1117:
1068:
924:
808:
791:Cramer MT, Guay-Woodford LM (July 2015).
719:
337:have similar phenotypical presentations.
2050:. International Cat Care. Archived from
482:
296:
288:
212:angiotensin converting enzyme inhibitors
106:Antihypertensives, Life style management
1923:Cochrane Database of Systematic Reviews
587:
3320:Congenital disorders of urinary system
2104:Chapin HC, Caplan MJ (November 2010).
599:
597:
595:
593:
591:
3277:Autosomal recessive polycystic kidney
3067:Autosomal recessive polycystic kidney
1848:The Journal of Clinical Investigation
867:. Lippincott Williams & Wilkins.
175:, which if they rupture, can cause a
27:Congenital disorder of urinary system
7:
3062:Autosomal dominant polycystic kidney
2165:10.1146/annurev.med.60.101707.125712
673:"What Is Polycystic Kidney Disease?"
208:renin–angiotensin–aldosterone system
2147:Harris PC, Torres VE (2009-01-01).
1051:Simons M, Walz G (September 2006).
312:(ESKD) patients being treated with
1479:"FDA Approves Tolvaptan for ADPKD"
1098:American Journal of Human Genetics
973:10.1097/01.pap.0000201831.77472.d3
797:Advances in Chronic Kidney Disease
25:
793:"Cystic kidney disease: a primer"
519:That will either be some form of
487:Chr 11 FISH-mapped BACs from CGAP
2385:
609:MedlinePlus Medical Encyclopedia
431:PKD results from defects in the
3181:Asphyxiating thoracic dysplasia
3074:Medullary cystic kidney disease
2835:Proliferating trichilemmal cyst
1670:Nowak KL, Hopp K (April 2020).
1159:10.1128/MCB.26.4.1538-1548.2006
371:, and progressively compromise
2446:malformations and deformations
1991:10.1002/14651858.CD010294.pub2
1147:Molecular and Cellular Biology
961:Advances in Anatomic Pathology
322:mutations in any of the three
1:
2803:Proliferating epidermoid cyst
2765:Langerhans cell histiocytosis
2512:Multicystic dysplastic kidney
1339:10.1126/science.272.5266.1339
1296:10.1016/S0888-7543(11)80001-7
1019:10.1016/S0140-6736(07)60601-1
905:Current Opinion in Pediatrics
712:10.1016/S0140-6736(10)60236-X
220:angiotensin receptor blockers
2928:Human musculoskeletal system
1514:Kidney International Reports
1432:. Elsevier Health Sciences.
917:10.1097/mop.0000000000000168
899:Phua YL, Ho J (April 2015).
779:Dorland's Medical Dictionary
694:Hildebrandt F (April 2010).
3086:Congenital cystic dysplasia
2993:Congenital hepatic fibrosis
2839:Malignant trichilemmal cyst
2149:"Polycystic kidney disease"
2110:The Journal of Cell Biology
1786:10.1152/ajprenal.00551.2015
1454:"Polycystic Kidney Disease"
1239:Journal of Medical Genetics
833:"Polycystic Kidney Disease"
774:"polycystic kidney disease"
605:"Polycystic kidney disease"
3336:
3267:Orofaciodigital syndrome 1
3257:Primary ciliary dyskinesia
3039:Cystic leukoencephalopathy
2076:. Antagene. Archived from
1575:10.1016/j.cmet.2019.09.012
1526:10.1016/j.ekir.2022.02.009
1458:National Kidney Foundation
1110:10.1016/j.ajhg.2016.05.004
810:10.1053/j.ackd.2015.04.001
447:of abdomen, as well as an
274:
125:polycystic kidney syndrome
3285:
3272:McKusick–Kaufman syndrome
3171:Polycystic kidney disease
3107:Von Hippel–Lindau disease
3057:Polycystic kidney disease
2901:Keratin implantation cyst
2883:Pseudocyst of the auricle
2813:Eruptive vellus hair cyst
2648:Penoscrotal transposition
2643:Posterior urethral valves
2502:Polycystic kidney disease
2403:Polycystic kidney disease
2371:Polycystic kidney disease
2153:Annual Review of Medicine
1932:10.1002/14651858.cd010946
696:"Genetic kidney diseases"
510:renal replacement therapy
415:-mediated signaling. The
363:from any point along the
224:renal replacement therapy
113:Polycystic kidney disease
57:
48:
36:Polycystic kidney disease
18:Polycystic Kidney Disease
2988:Polycystic liver disease
2913:Adenoid cystic carcinoma
2868:nonstratified squamous:
2770:Lymphangioleiomyomatosis
1817:Spencer S (2021-12-25).
1659:. University of Cologne.
341:Gene PKD1 is located on
310:end-stage kidney disease
140:non-functional as well.
98:MRI, CT scan, Ultrasound
2896:Cutaneous columnar cyst
2870:Cutaneous ciliated cyst
2851:Steatocystoma multiplex
2794:follicular infundibulum
2517:Medullary sponge kidney
1655:Müller R (2022-08-23).
1612:Clinical Kidney Journal
177:subarachnoid hemorrhage
168:, and aneurysms in the
2980:Nasopalatine duct cyst
2975:Odontogenic keratocyst
2946:Human digestive system
1737:10.1681/ASN.2015020132
861:Porth C (2011-01-01).
749:Seminars in Nephrology
525:kidney transplantation
488:
408:
302:
294:
286:
3262:Senior–Løken syndrome
3194:Bardet–Biedl syndrome
2894:other and ungrouped:
2855:Steatocystoma simplex
2792:stratified squamous:
2775:Cystic bronchiectasis
2488:Papillorenal syndrome
2122:10.1083/jcb.201006173
1251:10.1136/jmg.27.11.697
1070:10.1038/sj.ki.5001534
532:antibiotic resistance
486:
406:
300:
292:
284:
3049:Genitourinary system
2668:Prune belly syndrome
2541:Supernumerary kidney
1688:10.2215/CJN.13291019
1057:Kidney International
3021:Bile duct hamartoma
2209:10.2147/IJNRD.S6939
2054:on 17 November 2016
1624:10.1093/ckj/sfab162
1569:(6): 1007–1023.e5.
1394:10.2147/ijnrd.s6939
1331:1996Sci...272.1339M
1325:(5266): 1339–1342.
1013:(9569): 1287–1301.
706:(9722): 1287–1295.
491:In 2018, Jynarque (
380:Autosomal recessive
248:excessive urination
44:Kidney - polycystic
3112:Tuberous sclerosis
3006:Biliary hamartomas
2757:Respiratory system
2326:External resources
2022:Tamparo C (2011).
1204:10.1007/BF00204167
489:
409:
306:Autosomal dominant
303:
295:
287:
271:Autosomal dominant
244:blood in the urine
238:Signs and symptoms
3297:
3296:
3154:Diseases of cilia
3120:
3119:
3016:Choledochal cysts
2955:Cysts of the jaws
2827:Trichilemmal cyst
2821:outer root sheath
2715:
2714:
2711:
2710:
2656:
2655:
2615:Bladder exstrophy
2594:
2593:
2586:Duplicated ureter
2380:
2379:
2080:on 17 August 2018
2033:978-0-8036-2505-1
1860:10.1172/JCI128503
1854:(10): 4506–4522.
1489:(6). 2018-06-01.
837:www.niddk.nih.gov
173:cerebral arteries
110:
109:
94:Diagnostic method
30:Medical condition
16:(Redirected from
3327:
3290:ciliary proteins
3252:Alström syndrome
3236:Nephronophthisis
3220:Joubert syndrome
3147:
3140:
3133:
3124:
3095:Other conditions
3079:Nephronophthisis
2998:Peliosis hepatis
2970:Dentigerous cyst
2960:Odontogenic cyst
2742:
2735:
2728:
2719:
2681:
2603:
2536:Nephronophthisis
2526:Horseshoe kidney
2478:Renal hypoplasia
2461:
2436:
2429:
2422:
2413:
2389:
2388:
2242:
2231:
2221:
2211:
2186:
2176:
2143:
2133:
2090:
2089:
2087:
2085:
2070:
2064:
2063:
2061:
2059:
2044:
2038:
2037:
2019:
2013:
2012:
2002:
1970:
1964:
1963:
1951:
1945:
1944:
1934:
1914:
1903:
1902:
1900:
1899:
1888:
1882:
1881:
1871:
1839:
1833:
1832:
1830:
1829:
1814:
1808:
1807:
1797:
1780:(8): F726–F731.
1765:
1759:
1758:
1748:
1731:(5): 1437–1447.
1716:
1710:
1709:
1699:
1667:
1661:
1660:
1652:
1646:
1645:
1635:
1618:(6): 1079–1092.
1603:
1597:
1596:
1586:
1554:
1548:
1547:
1537:
1520:(5): 1037–1048.
1505:
1499:
1498:
1475:
1469:
1468:
1466:
1465:
1450:
1444:
1443:
1423:
1417:
1416:
1406:
1396:
1372:
1359:
1358:
1314:
1308:
1307:
1279:
1273:
1272:
1262:
1230:
1224:
1223:
1187:
1181:
1180:
1170:
1153:(4): 1538–1548.
1138:
1132:
1131:
1121:
1104:(6): 1193–1207.
1089:
1083:
1082:
1072:
1048:
1039:
1038:
1002:
993:
992:
956:
939:
938:
928:
896:
879:
878:
858:
852:
851:
849:
848:
839:. Archived from
829:
823:
822:
812:
788:
782:
771:
765:
764:
740:
734:
733:
723:
691:
685:
684:
682:
680:
669:
663:
662:
660:
658:
647:
641:
640:
638:
636:
625:
619:
618:
616:
615:
601:
193:serum creatinine
170:circle of Willis
158:seminal vesicles
129:genetic disorder
123:, also known as
53:
33:
21:
3335:
3334:
3330:
3329:
3328:
3326:
3325:
3324:
3310:Kidney diseases
3300:
3299:
3298:
3293:
3281:
3245:Other/ungrouped
3240:
3224:
3207:Meckel syndrome
3202:mitotic spindle
3156:
3151:
3121:
3116:
3090:
3043:
3025:
2965:Periapical cyst
2940:
2922:
2881:no epithelium:
2799:Epidermoid cyst
2779:
2751:
2749:Cystic diseases
2746:
2716:
2707:
2698:Urachal fistula
2672:
2652:
2619:
2590:
2562:
2558:Alport syndrome
2507:Meckel syndrome
2483:Potter sequence
2452:
2440:
2410:
2409:
2408:
2390:
2386:
2381:
2376:
2375:
2321:
2320:
2253:
2239:
2234:
2189:
2146:
2103:
2099:
2097:Further reading
2094:
2093:
2083:
2081:
2072:
2071:
2067:
2057:
2055:
2046:
2045:
2041:
2034:
2021:
2020:
2016:
1985:(7): CD010294.
1972:
1971:
1967:
1953:
1952:
1948:
1916:
1915:
1906:
1897:
1895:
1890:
1889:
1885:
1841:
1840:
1836:
1827:
1825:
1816:
1815:
1811:
1767:
1766:
1762:
1718:
1717:
1713:
1669:
1668:
1664:
1654:
1653:
1649:
1605:
1604:
1600:
1563:Cell Metabolism
1556:
1555:
1551:
1507:
1506:
1502:
1477:
1476:
1472:
1463:
1461:
1452:
1451:
1447:
1440:
1425:
1424:
1420:
1374:
1373:
1362:
1316:
1315:
1311:
1281:
1280:
1276:
1245:(11): 697–700.
1232:
1231:
1227:
1189:
1188:
1184:
1140:
1139:
1135:
1091:
1090:
1086:
1050:
1049:
1042:
1004:
1003:
996:
958:
957:
942:
898:
897:
882:
875:
860:
859:
855:
846:
844:
831:
830:
826:
790:
789:
785:
772:
768:
742:
741:
737:
693:
692:
688:
678:
676:
671:
670:
666:
656:
654:
649:
648:
644:
634:
632:
627:
626:
622:
613:
611:
603:
602:
589:
584:
572:
563:
548:
481:
473:
471:Natural history
441:
401:
382:
373:kidney function
350:cation channels
279:
273:
256:
240:
232:transplantation
88:ADPKD and ARPKD
31:
28:
23:
22:
15:
12:
11:
5:
3333:
3331:
3323:
3322:
3317:
3312:
3302:
3301:
3295:
3294:
3286:
3283:
3282:
3280:
3279:
3274:
3269:
3264:
3259:
3254:
3248:
3246:
3242:
3241:
3239:
3238:
3232:
3230:
3226:
3225:
3223:
3222:
3210:
3209:
3197:
3196:
3184:
3183:
3174:
3173:
3164:
3162:
3158:
3157:
3152:
3150:
3149:
3142:
3135:
3127:
3118:
3117:
3115:
3114:
3109:
3104:
3098:
3096:
3092:
3091:
3089:
3088:
3083:
3082:
3081:
3071:
3070:
3069:
3064:
3053:
3051:
3045:
3044:
3042:
3041:
3035:
3033:
3031:Nervous system
3027:
3026:
3024:
3023:
3018:
3013:
3011:Caroli disease
3008:
3001:
3000:
2995:
2990:
2983:
2982:
2977:
2972:
2967:
2962:
2957:
2950:
2948:
2942:
2941:
2939:
2938:
2936:Cystic hygroma
2932:
2930:
2924:
2923:
2921:
2920:
2915:
2909:
2908:
2906:Verrucous cyst
2903:
2898:
2891:
2890:
2885:
2878:
2877:
2872:
2865:
2864:
2859:
2858:
2857:
2846:sebaceous duct
2843:
2842:
2841:
2817:
2816:
2815:
2810:
2805:
2789:
2787:
2781:
2780:
2778:
2777:
2772:
2767:
2761:
2759:
2753:
2752:
2747:
2745:
2744:
2737:
2730:
2722:
2713:
2712:
2709:
2708:
2706:
2705:
2700:
2695:
2689:
2687:
2678:
2674:
2673:
2671:
2670:
2664:
2662:
2658:
2657:
2654:
2653:
2651:
2650:
2645:
2640:
2635:
2629:
2627:
2621:
2620:
2618:
2617:
2611:
2609:
2600:
2596:
2595:
2592:
2591:
2589:
2588:
2583:
2578:
2576:Ectopic ureter
2572:
2570:
2564:
2563:
2561:
2560:
2555:
2553:Dent's disease
2549:
2548:
2543:
2538:
2533:
2528:
2522:
2521:
2520:
2519:
2514:
2509:
2504:
2491:
2490:
2485:
2480:
2475:
2473:Renal agenesis
2469:
2467:
2458:
2454:
2453:
2450:urinary system
2441:
2439:
2438:
2431:
2424:
2416:
2391:
2384:
2383:
2382:
2378:
2377:
2374:
2373:
2362:
2342:
2330:
2329:
2327:
2323:
2322:
2319:
2318:
2307:
2296:
2285:
2270:
2254:
2249:
2248:
2246:
2245:Classification
2238:
2237:External links
2235:
2233:
2232:
2187:
2144:
2116:(4): 701–710.
2100:
2098:
2095:
2092:
2091:
2065:
2039:
2032:
2014:
1965:
1946:
1904:
1883:
1834:
1809:
1760:
1711:
1682:(4): 577–584.
1662:
1647:
1598:
1549:
1500:
1470:
1445:
1438:
1418:
1360:
1309:
1290:(3): 467–472.
1274:
1225:
1198:(2): 129–133.
1192:Human Genetics
1182:
1133:
1084:
1063:(5): 854–864.
1040:
994:
940:
911:(2): 219–226.
880:
873:
853:
824:
803:(4): 297–305.
783:
766:
755:(6): 596–606.
735:
686:
664:
642:
620:
586:
585:
583:
580:
579:
578:
571:
568:
562:
559:
552:kidney failure
547:
544:
536:bacteriostatic
514:kidney failure
497:ketogenic diet
480:
477:
472:
469:
465:blood pressure
440:
437:
433:primary cilium
400:
397:
389:underdeveloped
381:
378:
377:
376:
357:
346:
275:Main article:
272:
269:
255:
252:
239:
236:
108:
107:
104:
100:
99:
96:
90:
89:
86:
82:
81:
80:Abdominal pain
78:
72:
71:
66:
60:
59:
55:
54:
46:
45:
42:
38:
37:
29:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
3332:
3321:
3318:
3316:
3313:
3311:
3308:
3307:
3305:
3292:
3291:
3284:
3278:
3275:
3273:
3270:
3268:
3265:
3263:
3260:
3258:
3255:
3253:
3250:
3249:
3247:
3243:
3237:
3234:
3233:
3231:
3227:
3221:
3217:
3216:
3212:
3211:
3208:
3205:
3203:
3199:
3198:
3195:
3191:
3190:
3186:
3185:
3182:
3179:
3176:
3175:
3172:
3169:
3166:
3165:
3163:
3159:
3155:
3148:
3143:
3141:
3136:
3134:
3129:
3128:
3125:
3113:
3110:
3108:
3105:
3103:
3100:
3099:
3097:
3093:
3087:
3084:
3080:
3077:
3076:
3075:
3072:
3068:
3065:
3063:
3060:
3059:
3058:
3055:
3054:
3052:
3050:
3046:
3040:
3037:
3036:
3034:
3032:
3028:
3022:
3019:
3017:
3014:
3012:
3009:
3007:
3003:
3002:
2999:
2996:
2994:
2991:
2989:
2985:
2984:
2981:
2978:
2976:
2973:
2971:
2968:
2966:
2963:
2961:
2958:
2956:
2953:oral cavity:
2952:
2951:
2949:
2947:
2943:
2937:
2934:
2933:
2931:
2929:
2925:
2919:
2916:
2914:
2911:
2910:
2907:
2904:
2902:
2899:
2897:
2893:
2892:
2889:
2886:
2884:
2880:
2879:
2876:
2873:
2871:
2867:
2866:
2863:
2860:
2856:
2852:
2849:
2848:
2847:
2844:
2840:
2836:
2832:
2828:
2825:
2824:
2823:
2822:
2818:
2814:
2811:
2809:
2806:
2804:
2800:
2797:
2796:
2795:
2791:
2790:
2788:
2786:
2782:
2776:
2773:
2771:
2768:
2766:
2763:
2762:
2760:
2758:
2754:
2750:
2743:
2738:
2736:
2731:
2729:
2724:
2723:
2720:
2704:
2703:Urachal sinus
2701:
2699:
2696:
2694:
2691:
2690:
2688:
2686:
2682:
2679:
2675:
2669:
2666:
2665:
2663:
2659:
2649:
2646:
2644:
2641:
2639:
2636:
2634:
2631:
2630:
2628:
2626:
2622:
2616:
2613:
2612:
2610:
2608:
2604:
2601:
2597:
2587:
2584:
2582:
2579:
2577:
2574:
2573:
2571:
2569:
2565:
2559:
2556:
2554:
2551:
2550:
2547:
2546:Pelvic kidney
2544:
2542:
2539:
2537:
2534:
2532:
2531:Renal ectopia
2529:
2527:
2524:
2523:
2518:
2515:
2513:
2510:
2508:
2505:
2503:
2500:
2499:
2498:
2497:
2493:
2492:
2489:
2486:
2484:
2481:
2479:
2476:
2474:
2471:
2470:
2468:
2466:
2462:
2459:
2455:
2451:
2447:
2444:
2437:
2432:
2430:
2425:
2423:
2418:
2417:
2414:
2406:
2405:
2404:
2398:
2394:
2372:
2368:
2367:
2363:
2361:
2358:
2355:
2352:
2348:
2347:
2343:
2341:
2337:
2336:
2332:
2331:
2328:
2324:
2317:
2313:
2312:
2308:
2306:
2302:
2301:
2297:
2295:
2291:
2290:
2286:
2284:
2280:
2279:
2275:
2271:
2269:
2265:
2264:
2260:
2256:
2255:
2252:
2247:
2243:
2236:
2229:
2225:
2220:
2215:
2210:
2205:
2201:
2197:
2193:
2188:
2184:
2180:
2175:
2170:
2166:
2162:
2158:
2154:
2150:
2145:
2141:
2137:
2132:
2127:
2123:
2119:
2115:
2111:
2107:
2102:
2101:
2096:
2079:
2075:
2069:
2066:
2053:
2049:
2043:
2040:
2035:
2029:
2025:
2018:
2015:
2010:
2006:
2001:
1996:
1992:
1988:
1984:
1980:
1976:
1969:
1966:
1961:
1957:
1950:
1947:
1942:
1938:
1933:
1928:
1924:
1920:
1913:
1911:
1909:
1905:
1893:
1887:
1884:
1879:
1875:
1870:
1865:
1861:
1857:
1853:
1849:
1845:
1838:
1835:
1824:
1820:
1813:
1810:
1805:
1801:
1796:
1791:
1787:
1783:
1779:
1775:
1771:
1764:
1761:
1756:
1752:
1747:
1742:
1738:
1734:
1730:
1726:
1722:
1715:
1712:
1707:
1703:
1698:
1693:
1689:
1685:
1681:
1677:
1673:
1666:
1663:
1658:
1651:
1648:
1643:
1639:
1634:
1629:
1625:
1621:
1617:
1613:
1609:
1602:
1599:
1594:
1590:
1585:
1580:
1576:
1572:
1568:
1564:
1560:
1553:
1550:
1545:
1541:
1536:
1531:
1527:
1523:
1519:
1515:
1511:
1504:
1501:
1496:
1492:
1488:
1484:
1480:
1474:
1471:
1459:
1455:
1449:
1446:
1441:
1439:9780323242875
1435:
1431:
1430:
1422:
1419:
1414:
1410:
1405:
1400:
1395:
1390:
1386:
1382:
1378:
1371:
1369:
1367:
1365:
1361:
1356:
1352:
1348:
1344:
1340:
1336:
1332:
1328:
1324:
1320:
1313:
1310:
1305:
1301:
1297:
1293:
1289:
1285:
1278:
1275:
1270:
1266:
1261:
1256:
1252:
1248:
1244:
1240:
1236:
1229:
1226:
1221:
1217:
1213:
1209:
1205:
1201:
1197:
1193:
1186:
1183:
1178:
1174:
1169:
1164:
1160:
1156:
1152:
1148:
1144:
1137:
1134:
1129:
1125:
1120:
1115:
1111:
1107:
1103:
1099:
1095:
1088:
1085:
1080:
1076:
1071:
1066:
1062:
1058:
1054:
1047:
1045:
1041:
1036:
1032:
1028:
1024:
1020:
1016:
1012:
1008:
1001:
999:
995:
990:
986:
982:
978:
974:
970:
966:
962:
955:
953:
951:
949:
947:
945:
941:
936:
932:
927:
922:
918:
914:
910:
906:
902:
895:
893:
891:
889:
887:
885:
881:
876:
874:9781582557243
870:
866:
865:
857:
854:
843:on 2017-01-04
842:
838:
834:
828:
825:
820:
816:
811:
806:
802:
798:
794:
787:
784:
781:
780:
775:
770:
767:
762:
758:
754:
750:
746:
739:
736:
731:
727:
722:
717:
713:
709:
705:
701:
697:
690:
687:
674:
668:
665:
652:
646:
643:
630:
624:
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561:Epidemiology
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417:polycystin-1
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3004:bile duct:
2918:Breast cyst
2638:Hypospadias
2335:MedlinePlus
2159:: 321–337.
1483:Kidney News
41:Other names
3315:Ciliopathy
3304:Categories
3288:See also:
3215:centrosome
3189:basal body
3161:Structural
2862:Keratocyst
2831:Pilar cyst
2633:Epispadias
2581:Megaureter
2443:Congenital
2366:Patient UK
2311:DiseasesDB
2084:2 November
2058:2 November
1898:2022-10-24
1828:2022-10-24
1464:2022-11-17
847:2015-07-31
614:2015-07-30
582:References
453:ultrasound
369:parenchyma
69:Nephrology
3229:Signaling
3168:receptor:
2677:Vestigial
2457:Abdominal
2346:eMedicine
2202:: 69–83.
1495:1555-905X
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679:3 January
657:3 January
635:3 January
546:Prognosis
493:Tolvaptan
479:Treatment
439:Diagnosis
399:Mechanism
267:(ARPKD).
166:aneurysms
103:Treatment
64:Specialty
2888:Mucocele
2360:radio/69
2357:radio/68
2354:ped/1846
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2183:18947299
2140:21079243
2009:26171904
1960:Medscape
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1755:26538633
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1593:31631001
1544:35570988
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1177:16449663
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570:See also
542:drugs".
521:dialysis
361:in utero
314:dialysis
228:dialysis
185:sonogram
162:pancreas
76:Symptoms
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2685:Urachus
2625:Urethra
2607:Bladder
2393:Scholia
2305:D007690
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1319:Science
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1269:1980516
1260:1017261
1212:1676697
1168:1367205
1119:4908191
1035:1700992
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445:CT scan
365:nephron
320:Genetic
230:and/or
226:(RRT):
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