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.
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 ketogenic diet 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 calorie restriction or time-restricted feeding slow the progression of
298:
51:
290:
484:
2382:
499:(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:
518:, 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:
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 causes 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
560:
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.
2268:
2253:
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:
2428:
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
827:
768:
2042:
1277:
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".
1185:
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".
2421:
1312:
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".
2414:
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
1603:"Ketogenic dietary interventions in autosomal dominant polycystic kidney disease-a retrospective case series study: first insights into feasibility, safety and effects"
334:
3314:
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
570:
384:
264:
207:
149:
1422:
496:
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
857:
835:
2068:
2734:
529:, 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:
2046:
645:
525:
A Cochrane Review study of autosomal dominant polycystic kidney disease made note of the fact that it is important at all times, while avoiding
623:
3271:
3061:
2026:
3139:
3056:
196:
773:
2283:
1813:
1432:
867:
667:
352:, with inward selectivity for K>Na>>Ca and outward selectivity for Ca2+ ≈ Ba2+ > Na+ ≈ K+, are coded for by PKD2 on
3175:
3068:
2829:
3266:
3101:
2797:
2759:
2727:
2506:
219:
3256:
2922:
1089:"Mutations in GANAB, Encoding the Glucosidase IIα Subunit, Cause Autosomal-Dominant Polycystic Kidney and Liver Disease"
483:
2987:
2833:
1765:"A mild reduction of food intake slows disease progression in an orthologous mouse model of polycystic kidney disease"
297:
289:
50:
3261:
3251:
3188:
3284:
3080:
2895:
2877:
2807:
2642:
504:
223:
3033:
3304:
3132:
2982:
2907:
2764:
2720:
2637:
2294:
1473:
309:
210:(RAAS), frequent cyst infections, urinary bleeding, and declining renal function. Hypertension is treated with
2406:
2397:
411:
Both autosomal dominant and autosomal recessive polycystic kidney disease cyst formation are tied to abnormal
1667:"Metabolic Reprogramming in Autosomal Dominant Polycystic Kidney Disease: Evidence and Therapeutic Potential"
2890:
2864:
2845:
2697:
2601:
2511:
2072:
234:. Management from the time of the suspected or definitive diagnosis is by an appropriately trained doctor.
176:
2365:
1886:
954:
Bisceglia M, Galliani CA, Senger C, Stallone C, Sessa A (January 2006). "Renal cystic diseases: a review".
545:
ADPKD individuals might have a normal life; conversely, ARPKD can cause kidney dysfunction and can lead to
2974:
2969:
2940:
519:
2849:
2490:
2482:
1839:"Crystal deposition triggers tubule dilation that accelerates cystogenesis in polycystic kidney disease"
1230:"Exclusion of autosomal dominant polycystic kidney disease type II (ADPKD2) from 160 cM of chromosome 1"
526:
231:
2802:
2779:
2662:
2535:
1321:
305:
301:
Cartoon of autosomal recessive polycystic kidney disease with normal kidney inset to right of diagram
1552:
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
3309:
3246:
3125:
3015:
3000:
2437:
2272:
1087:
Porath B, Gainullin VG, Cornec-Le Gall E, Dillinger EK, Heyer CM, Hopp K, et al. (June 2016).
3106:
2751:
1931:
1345:
1210:
1025:
979:
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
3010:
2949:
2821:
2815:
2609:
2580:
2547:
2334:
2305:
2218:
2173:
2130:
2022:
1999:
1970:"Interventions for preventing the progression of autosomal dominant polycystic kidney disease"
1914:"Interventions for infected cysts in people with autosomal dominant polycystic kidney disease"
1868:
1794:
1745:
1696:
1632:
1583:
1534:
1485:
1428:
1403:
1337:
1294:
1259:
1202:
1167:
1118:
1069:
1017:
971:
925:
863:
809:
751:
720:
599:
172:
93:
63:
1000:
Torres VE, Harris PC, Pirson Y (April 2007). "Autosomal dominant polycystic kidney disease".
3230:
3214:
3073:
2992:
2964:
2954:
2530:
2520:
2472:
2440:
2208:
2198:
2163:
2155:
2120:
2112:
1989:
1981:
1921:
1858:
1850:
1784:
1776:
1735:
1727:
1686:
1678:
1622:
1614:
1573:
1565:
1524:
1516:
1393:
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1329:
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1194:
1157:
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1108:
1100:
1059:
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963:
915:
907:
799:
710:
702:
192:
169:
157:
128:
1950:
3201:
3196:
2959:
2793:
2692:
2552:
2501:
2477:
549:
by the age of 40–60. ADPKD1 and ADPKD2 are very different, in that ADPKD2 is much milder.
515:
372:
227:
222:(ARBs). Infections are treated with antibiotics. Declining renal function is treated with
2159:
1652:"Ketogenic Dietary Interventions in Autosomal Dominant Polycystic Kidney Disease (ADPKD)"
1601:
Strubl S, Oehm S, Torres JA, Grundmann F, Haratani J, Decker M, et al. (June 2022).
2387:
1503:
Zhou X, Davenport E, Ouyang J, Hoke ME, Garbinsky D, Agarwal I, et al. (May 2022).
1325:
552:
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).
3043:
3025:
3005:
2930:
2900:
2769:
2570:
2467:
2459:
2444:
2213:
2186:
2168:
2143:
2125:
2100:
1994:
1969:
1863:
1838:
1789:
1764:
1740:
1715:
1691:
1666:
1627:
1602:
1578:
1553:
1529:
1504:
1398:
1371:
1254:
1229:
1162:
1137:
1136:
Thivierge C, Kurbegovic A, Couillard M, Guillaume R, Coté O, Trudel M (February 2006).
1113:
1088:
967:
920:
895:
715:
690:
546:
530:
508:
464:
432:
349:
2277:
1290:
1013:
706:
3298:
2540:
2525:
534:
460:
342:
184:
1935:
1349:
1214:
1153:
983:
3096:
2869:
2687:
2345:
2187:"Polycystic kidney disease: inheritance, pathophysiology, prognosis, and treatment"
1985:
1837:
Torres JA, Rezaei M, Broderick C, Lin L, Wang X, Hoppe B, et al. (July 2019).
1372:"Polycystic kidney disease: inheritance, pathophysiology, prognosis, and treatment"
1029:
500:
420:
416:
353:
313:
132:
1651:
1333:
135:
become structurally abnormal, resulting in the development and growth of multiple
2299:
1968:
Bolignano D, Palmer SC, Ruospo M, Zoccali C, Craig JC, Strippoli GF (July 2015).
1448:
911:
17:
2912:
2632:
2329:
1780:
2354:
2351:
2348:
2310:
1714:
Warner G, Hein KZ, Nin V, Edwards M, Chini CC, Hopp K, et al. (May 2016).
1569:
1520:
1505:"Pooled Data Analysis of the Long-Term Treatment Effects of Tolvaptan in ADPKD"
1104:
804:
787:
3209:
3183:
3148:
2856:
2825:
2627:
2575:
2360:
1926:
1913:
452:
388:
368:
68:
1951:"Polycystic Kidney Disease: Practice Essentials, Background, Pathophysiology"
1489:
2340:
2262:
1228:
Kumar S, Kimberling WJ, Gabow PA, Shugart YY, Pieke-Dahl S (November 1990).
739:
492:
243:
187:; or an incidental finding of abnormal kidney function on routine lab work (
2222:
2177:
2134:
2003:
1872:
1798:
1749:
1731:
1716:"Food Restriction Ameliorates the Development of Polycystic Kidney Disease"
1700:
1636:
1587:
1538:
1407:
1171:
1122:
1073:
1021:
975:
929:
813:
724:
242:
Signs and symptoms include high blood pressure, headaches, abdominal pain,
2245:
2116:
1618:
1341:
1298:
1263:
1245:
1206:
1064:
1047:
755:
670:. National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
648:. National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
626:. National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
2882:
1682:
319:
247:
165:
161:
2203:
1388:
403:
2679:
2619:
1198:
503:
or other practitioner and the patient will have to decide what form 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
1854:
2562:
2257:
360:
3117:
1554:"Ketosis Ameliorates Renal Cyst Growth in Polycystic Kidney Disease"
2712:
2043:"Polycystic kidney disease (PKD): Gene test and negative register"
456:
412:
402:
392:
323:
280:
153:
2743:
2288:
859:
330:
326:
281:
215:
206:
Complications include hypertension due to the activation of the
136:
3121:
2716:
2410:
448:
188:
2191:
International
Journal of Nephrology and Renovascular Disease
1912:
Montero N, Sans L, Webster AC, Pascual J (29 January 2014).
1376:
International
Journal of Nephrology and Renovascular Disease
1048:"Polycystic kidney disease: cell division without a c(l)ue?"
285:
CT scan showing autosomal dominant polycystic kidney disease
1763:
Kipp KR, Rezaei M, Lin L, Dewey EC, Weimbs T (April 2016).
455:
of the same area. A physical exam/test can reveal enlarged
1424:
Comprehensive
Clinical Nephrology: Expert Consult - Online
1138:"Overexpression of PKD1 causes polycystic kidney disease"
896:"MicroRNAs in the pathogenesis of cystic kidney disease"
391:, resulting in a 30% death rate in newborns with ARPKD.
1814:"6 Ways People with PKD Can Lower Their Blood Pressure"
738:
Kimberling WJ, Pieke-Dahl SA, Kumar S (November 1991).
475:
Most cases progress to bilateral disease in adulthood.
1671:
Clinical
Journal of the American Society of Nephrology
2069:"PKD - Polycystic Kidney Disease - British Shorthair"
2021:. Philadelphia, PA: F.A. Davis Company. p. 443.
1949:
Torra R (2018-07-20). Talavera F, Aronoff GR (eds.).
1887:"Breakthrough Results After 7 Years of Reversing Pkd"
511:, typically stage 4 or 5 of chronic kidney disease).
2235:
3239:
3223:
3155:
3089:
3042:
3024:
2939:
2921:
2778:
2750:
2678:
2671:
2655:
2618:
2600:
2593:
2561:
2458:
2451:
2320:
2239:
443:Polycystic kidney disease can be ascertained via a
102:
92:
84:
74:
62:
40:
35:
1370:Halvorson CR, Bremmer MS, Jacobs SC (2010-06-24).
1365:
1363:
1361:
1359:
164:. This genetic defect can also cause aortic root
1769:American Journal of Physiology. Renal Physiology
949:
947:
945:
943:
941:
939:
507:will be used to treat end-stage kidney disease (
2101:"The cell biology of polycystic kidney disease"
1907:
1905:
1903:
1421:Johnson RJ, Feehally J, Floege J (2014-09-05).
740:"The genetics of cystic diseases of the kidney"
646:"Autosomal Recessive Polycystic Kidney Disease"
624:"Autosomal Dominant Polycystic Kidney Disease"
3133:
2728:
2422:
1720:Journal of the American Society of Nephrology
1041:
1039:
995:
993:
889:
887:
885:
883:
881:
879:
571: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:
2185:Halvorson CR, Bremmer MS, Jacobs SC (2010).
497:autosomal dominant polycystic kidney disease
277:Autosomal dominant polycystic kidney disease
261:autosomal dominant polycystic kidney disease
146:autosomal dominant polycystic kidney disease
1974:The Cochrane Database of Systematic Reviews
3140:
3126:
3118:
2735:
2721:
2713:
2675:
2597:
2455:
2429:
2415:
2407:
2236:
49:
32:
2212:
2202:
2167:
2124:
2019:Fifth Edition: Diseases of the Human Body
1993:
1925:
1862:
1788:
1739:
1690:
1626:
1577:
1528:
1397:
1387:
1253:
1161:
1112:
1063:
919:
803:
786:Cramer MT, Guay-Woodford LM (July 2015).
714:
337:have similar phenotypical presentations.
2045:. International Cat Care. Archived from
482:
296:
288:
212:angiotensin converting enzyme inhibitors
106:Antihypertensives, Life style management
1918:Cochrane Database of Systematic Reviews
582:
3315:Congenital disorders of urinary system
2099:Chapin HC, Caplan MJ (November 2010).
594:
592:
590:
588:
586:
3272:Autosomal recessive polycystic kidney
3062:Autosomal recessive polycystic kidney
1843:The Journal of Clinical Investigation
862:. Lippincott Williams & Wilkins.
175:, which if they rupture, can cause a
27:Congenital disorder of urinary system
7:
3057:Autosomal dominant polycystic kidney
2160:10.1146/annurev.med.60.101707.125712
668:"What Is Polycystic Kidney Disease?"
208:renin–angiotensin–aldosterone system
2142:Harris PC, Torres VE (2009-01-01).
1046:Simons M, Walz G (September 2006).
312:(ESKD) patients being treated with
1474:"FDA Approves Tolvaptan for ADPKD"
1093:American Journal of Human Genetics
968:10.1097/01.pap.0000201831.77472.d3
792:Advances in Chronic Kidney Disease
25:
788:"Cystic kidney disease: a primer"
514:That will either be some form of
487:Chr 11 FISH-mapped BACs from CGAP
2380:
604:MedlinePlus Medical Encyclopedia
431:PKD results from defects in the
3176:Asphyxiating thoracic dysplasia
3069:Medullary cystic kidney disease
2830:Proliferating trichilemmal cyst
1665:Nowak KL, Hopp K (April 2020).
1154:10.1128/MCB.26.4.1538-1548.2006
371:, and progressively compromise
2441:malformations and deformations
1986:10.1002/14651858.CD010294.pub2
1142:Molecular and Cellular Biology
956:Advances in Anatomic Pathology
322:mutations in any of the three
1:
2798:Proliferating epidermoid cyst
2760:Langerhans cell histiocytosis
2507:Multicystic dysplastic kidney
1334:10.1126/science.272.5266.1339
1291:10.1016/S0888-7543(11)80001-7
1014:10.1016/S0140-6736(07)60601-1
900:Current Opinion in Pediatrics
707:10.1016/S0140-6736(10)60236-X
220:angiotensin receptor blockers
2923:Human musculoskeletal system
1509:Kidney International Reports
1427:. Elsevier Health Sciences.
912:10.1097/mop.0000000000000168
894:Phua YL, Ho J (April 2015).
774:Dorland's Medical Dictionary
689:Hildebrandt F (April 2010).
3081:Congenital cystic dysplasia
2988:Congenital hepatic fibrosis
2834:Malignant trichilemmal cyst
2144:"Polycystic kidney disease"
2105:The Journal of Cell Biology
1781:10.1152/ajprenal.00551.2015
1449:"Polycystic Kidney Disease"
1234:Journal of Medical Genetics
828:"Polycystic Kidney Disease"
769:"polycystic kidney disease"
600:"Polycystic kidney disease"
3331:
3262:Orofaciodigital syndrome 1
3252:Primary ciliary dyskinesia
3034:Cystic leukoencephalopathy
2071:. Antagene. Archived from
1570:10.1016/j.cmet.2019.09.012
1521:10.1016/j.ekir.2022.02.009
1453:National Kidney Foundation
1105:10.1016/j.ajhg.2016.05.004
805:10.1053/j.ackd.2015.04.001
447:of abdomen, as well as an
274:
125:polycystic kidney syndrome
3280:
3267:McKusick–Kaufman syndrome
3166:Polycystic kidney disease
3102:Von Hippel–Lindau disease
3052:Polycystic kidney disease
2896:Keratin implantation cyst
2878:Pseudocyst of the auricle
2808:Eruptive vellus hair cyst
2643:Penoscrotal transposition
2638:Posterior urethral valves
2497:Polycystic kidney disease
2398:Polycystic kidney disease
2366:Polycystic kidney disease
2148:Annual Review of Medicine
1927:10.1002/14651858.cd010946
691:"Genetic kidney diseases"
505: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
2983:Polycystic liver disease
2908:Adenoid cystic carcinoma
2863:nonstratified squamous:
2765:Lymphangioleiomyomatosis
1812:Spencer S (2021-12-25).
1654:. University of Cologne.
341:Gene PKD1 is located on
310:end-stage kidney disease
140:non-functional as well.
98:MRI, CT scan, Ultrasound
2891:Cutaneous columnar cyst
2865:Cutaneous ciliated cyst
2846:Steatocystoma multiplex
2789:follicular infundibulum
2512:Medullary sponge kidney
1650:Müller R (2022-08-23).
1607:Clinical Kidney Journal
177:subarachnoid hemorrhage
168:, and aneurysms in the
2975:Nasopalatine duct cyst
2970:Odontogenic keratocyst
2941:Human digestive system
1732:10.1681/ASN.2015020132
856:Porth C (2011-01-01).
744:Seminars in Nephrology
520:kidney transplantation
488:
408:
302:
294:
286:
3257:Senior–Løken syndrome
3189:Bardet–Biedl syndrome
2889:other and ungrouped:
2850:Steatocystoma simplex
2787:stratified squamous:
2770:Cystic bronchiectasis
2483:Papillorenal syndrome
2117:10.1083/jcb.201006173
1246:10.1136/jmg.27.11.697
1065:10.1038/sj.ki.5001534
527:antibiotic resistance
486:
406:
300:
292:
284:
3044:Genitourinary system
2663:Prune belly syndrome
2536:Supernumerary kidney
1683:10.2215/CJN.13291019
1052:Kidney International
3016:Bile duct hamartoma
2204:10.2147/IJNRD.S6939
2049:on 17 November 2016
1619:10.1093/ckj/sfab162
1564:(6): 1007–1023.e5.
1389:10.2147/ijnrd.s6939
1326:1996Sci...272.1339M
1320:(5266): 1339–1342.
1008:(9569): 1287–1301.
701:(9722): 1287–1295.
491:In 2018, Jynarque (
380:Autosomal recessive
248:excessive urination
44:Kidney - polycystic
3107:Tuberous sclerosis
3001:Biliary hamartomas
2752:Respiratory system
2321:External resources
2017:Tamparo C (2011).
1199:10.1007/BF00204167
489:
409:
306:Autosomal dominant
303:
295:
287:
271:Autosomal dominant
244:blood in the urine
238:Signs and symptoms
3292:
3291:
3149:Diseases of cilia
3115:
3114:
3011:Choledochal cysts
2950:Cysts of the jaws
2822:Trichilemmal cyst
2816:outer root sheath
2710:
2709:
2706:
2705:
2651:
2650:
2610:Bladder exstrophy
2589:
2588:
2581:Duplicated ureter
2375:
2374:
2075:on 17 August 2018
2028:978-0-8036-2505-1
1855:10.1172/JCI128503
1849:(10): 4506–4522.
1484:(6). 2018-06-01.
832:www.niddk.nih.gov
173:cerebral arteries
110:
109:
94:Diagnostic method
30:Medical condition
18:Polycystic kidney
16:(Redirected from
3322:
3285:ciliary proteins
3247:Alström syndrome
3231:Nephronophthisis
3215:Joubert syndrome
3142:
3135:
3128:
3119:
3090:Other conditions
3074:Nephronophthisis
2993:Peliosis hepatis
2965:Dentigerous cyst
2955:Odontogenic cyst
2737:
2730:
2723:
2714:
2676:
2598:
2531:Nephronophthisis
2521:Horseshoe kidney
2473:Renal hypoplasia
2456:
2431:
2424:
2417:
2408:
2384:
2383:
2237:
2226:
2216:
2206:
2181:
2171:
2138:
2128:
2085:
2084:
2082:
2080:
2065:
2059:
2058:
2056:
2054:
2039:
2033:
2032:
2014:
2008:
2007:
1997:
1965:
1959:
1958:
1946:
1940:
1939:
1929:
1909:
1898:
1897:
1895:
1894:
1883:
1877:
1876:
1866:
1834:
1828:
1827:
1825:
1824:
1809:
1803:
1802:
1792:
1775:(8): F726–F731.
1760:
1754:
1753:
1743:
1726:(5): 1437–1447.
1711:
1705:
1704:
1694:
1662:
1656:
1655:
1647:
1641:
1640:
1630:
1613:(6): 1079–1092.
1598:
1592:
1591:
1581:
1549:
1543:
1542:
1532:
1515:(5): 1037–1048.
1500:
1494:
1493:
1470:
1464:
1463:
1461:
1460:
1445:
1439:
1438:
1418:
1412:
1411:
1401:
1391:
1367:
1354:
1353:
1309:
1303:
1302:
1274:
1268:
1267:
1257:
1225:
1219:
1218:
1182:
1176:
1175:
1165:
1148:(4): 1538–1548.
1133:
1127:
1126:
1116:
1099:(6): 1193–1207.
1084:
1078:
1077:
1067:
1043:
1034:
1033:
997:
988:
987:
951:
934:
933:
923:
891:
874:
873:
853:
847:
846:
844:
843:
834:. Archived from
824:
818:
817:
807:
783:
777:
766:
760:
759:
735:
729:
728:
718:
686:
680:
679:
677:
675:
664:
658:
657:
655:
653:
642:
636:
635:
633:
631:
620:
614:
613:
611:
610:
596:
193:serum creatinine
170:circle of Willis
158:seminal vesicles
129:genetic disorder
123:, also known as
53:
33:
21:
3330:
3329:
3325:
3324:
3323:
3321:
3320:
3319:
3305:Kidney diseases
3295:
3294:
3293:
3288:
3276:
3240:Other/ungrouped
3235:
3219:
3202:Meckel syndrome
3197:mitotic spindle
3151:
3146:
3116:
3111:
3085:
3038:
3020:
2960:Periapical cyst
2935:
2917:
2876:no epithelium:
2794:Epidermoid cyst
2774:
2746:
2744:Cystic diseases
2741:
2711:
2702:
2693:Urachal fistula
2667:
2647:
2614:
2585:
2557:
2553:Alport syndrome
2502:Meckel syndrome
2478:Potter sequence
2447:
2435:
2405:
2404:
2403:
2385:
2381:
2376:
2371:
2370:
2316:
2315:
2248:
2234:
2229:
2184:
2141:
2098:
2094:
2092:Further reading
2089:
2088:
2078:
2076:
2067:
2066:
2062:
2052:
2050:
2041:
2040:
2036:
2029:
2016:
2015:
2011:
1980:(7): CD010294.
1967:
1966:
1962:
1948:
1947:
1943:
1911:
1910:
1901:
1892:
1890:
1885:
1884:
1880:
1836:
1835:
1831:
1822:
1820:
1811:
1810:
1806:
1762:
1761:
1757:
1713:
1712:
1708:
1664:
1663:
1659:
1649:
1648:
1644:
1600:
1599:
1595:
1558:Cell Metabolism
1551:
1550:
1546:
1502:
1501:
1497:
1472:
1471:
1467:
1458:
1456:
1447:
1446:
1442:
1435:
1420:
1419:
1415:
1369:
1368:
1357:
1311:
1310:
1306:
1276:
1275:
1271:
1240:(11): 697–700.
1227:
1226:
1222:
1184:
1183:
1179:
1135:
1134:
1130:
1086:
1085:
1081:
1045:
1044:
1037:
999:
998:
991:
953:
952:
937:
893:
892:
877:
870:
855:
854:
850:
841:
839:
826:
825:
821:
785:
784:
780:
767:
763:
737:
736:
732:
688:
687:
683:
673:
671:
666:
665:
661:
651:
649:
644:
643:
639:
629:
627:
622:
621:
617:
608:
606:
598:
597:
584:
579:
567:
558:
543:
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:
3328:
3326:
3318:
3317:
3312:
3307:
3297:
3296:
3290:
3289:
3281:
3278:
3277:
3275:
3274:
3269:
3264:
3259:
3254:
3249:
3243:
3241:
3237:
3236:
3234:
3233:
3227:
3225:
3221:
3220:
3218:
3217:
3205:
3204:
3192:
3191:
3179:
3178:
3169:
3168:
3159:
3157:
3153:
3152:
3147:
3145:
3144:
3137:
3130:
3122:
3113:
3112:
3110:
3109:
3104:
3099:
3093:
3091:
3087:
3086:
3084:
3083:
3078:
3077:
3076:
3066:
3065:
3064:
3059:
3048:
3046:
3040:
3039:
3037:
3036:
3030:
3028:
3026:Nervous system
3022:
3021:
3019:
3018:
3013:
3008:
3006:Caroli disease
3003:
2996:
2995:
2990:
2985:
2978:
2977:
2972:
2967:
2962:
2957:
2952:
2945:
2943:
2937:
2936:
2934:
2933:
2931:Cystic hygroma
2927:
2925:
2919:
2918:
2916:
2915:
2910:
2904:
2903:
2901:Verrucous cyst
2898:
2893:
2886:
2885:
2880:
2873:
2872:
2867:
2860:
2859:
2854:
2853:
2852:
2841:sebaceous duct
2838:
2837:
2836:
2812:
2811:
2810:
2805:
2800:
2784:
2782:
2776:
2775:
2773:
2772:
2767:
2762:
2756:
2754:
2748:
2747:
2742:
2740:
2739:
2732:
2725:
2717:
2708:
2707:
2704:
2703:
2701:
2700:
2695:
2690:
2684:
2682:
2673:
2669:
2668:
2666:
2665:
2659:
2657:
2653:
2652:
2649:
2648:
2646:
2645:
2640:
2635:
2630:
2624:
2622:
2616:
2615:
2613:
2612:
2606:
2604:
2595:
2591:
2590:
2587:
2586:
2584:
2583:
2578:
2573:
2571:Ectopic ureter
2567:
2565:
2559:
2558:
2556:
2555:
2550:
2548:Dent's disease
2544:
2543:
2538:
2533:
2528:
2523:
2517:
2516:
2515:
2514:
2509:
2504:
2499:
2486:
2485:
2480:
2475:
2470:
2468:Renal agenesis
2464:
2462:
2453:
2449:
2448:
2445:urinary system
2436:
2434:
2433:
2426:
2419:
2411:
2386:
2379:
2378:
2377:
2373:
2372:
2369:
2368:
2357:
2337:
2325:
2324:
2322:
2318:
2317:
2314:
2313:
2302:
2291:
2280:
2265:
2249:
2244:
2243:
2241:
2240:Classification
2233:
2232:External links
2230:
2228:
2227:
2182:
2139:
2111:(4): 701–710.
2095:
2093:
2090:
2087:
2086:
2060:
2034:
2027:
2009:
1960:
1941:
1899:
1878:
1829:
1804:
1755:
1706:
1677:(4): 577–584.
1657:
1642:
1593:
1544:
1495:
1465:
1440:
1433:
1413:
1355:
1304:
1285:(3): 467–472.
1269:
1220:
1193:(2): 129–133.
1187:Human Genetics
1177:
1128:
1079:
1058:(5): 854–864.
1035:
989:
935:
906:(2): 219–226.
875:
868:
848:
819:
798:(4): 297–305.
778:
761:
750:(6): 596–606.
730:
681:
659:
637:
615:
581:
580:
578:
575:
574:
573:
566:
563:
557:
554:
547:kidney failure
542:
539:
531:bacteriostatic
509:kidney failure
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:
3327:
3316:
3313:
3311:
3308:
3306:
3303:
3302:
3300:
3287:
3286:
3279:
3273:
3270:
3268:
3265:
3263:
3260:
3258:
3255:
3253:
3250:
3248:
3245:
3244:
3242:
3238:
3232:
3229:
3228:
3226:
3222:
3216:
3212:
3211:
3207:
3206:
3203:
3200:
3198:
3194:
3193:
3190:
3186:
3185:
3181:
3180:
3177:
3174:
3171:
3170:
3167:
3164:
3161:
3160:
3158:
3154:
3150:
3143:
3138:
3136:
3131:
3129:
3124:
3123:
3120:
3108:
3105:
3103:
3100:
3098:
3095:
3094:
3092:
3088:
3082:
3079:
3075:
3072:
3071:
3070:
3067:
3063:
3060:
3058:
3055:
3054:
3053:
3050:
3049:
3047:
3045:
3041:
3035:
3032:
3031:
3029:
3027:
3023:
3017:
3014:
3012:
3009:
3007:
3004:
3002:
2998:
2997:
2994:
2991:
2989:
2986:
2984:
2980:
2979:
2976:
2973:
2971:
2968:
2966:
2963:
2961:
2958:
2956:
2953:
2951:
2948:oral cavity:
2947:
2946:
2944:
2942:
2938:
2932:
2929:
2928:
2926:
2924:
2920:
2914:
2911:
2909:
2906:
2905:
2902:
2899:
2897:
2894:
2892:
2888:
2887:
2884:
2881:
2879:
2875:
2874:
2871:
2868:
2866:
2862:
2861:
2858:
2855:
2851:
2847:
2844:
2843:
2842:
2839:
2835:
2831:
2827:
2823:
2820:
2819:
2818:
2817:
2813:
2809:
2806:
2804:
2801:
2799:
2795:
2792:
2791:
2790:
2786:
2785:
2783:
2781:
2777:
2771:
2768:
2766:
2763:
2761:
2758:
2757:
2755:
2753:
2749:
2745:
2738:
2733:
2731:
2726:
2724:
2719:
2718:
2715:
2699:
2698:Urachal sinus
2696:
2694:
2691:
2689:
2686:
2685:
2683:
2681:
2677:
2674:
2670:
2664:
2661:
2660:
2658:
2654:
2644:
2641:
2639:
2636:
2634:
2631:
2629:
2626:
2625:
2623:
2621:
2617:
2611:
2608:
2607:
2605:
2603:
2599:
2596:
2592:
2582:
2579:
2577:
2574:
2572:
2569:
2568:
2566:
2564:
2560:
2554:
2551:
2549:
2546:
2545:
2542:
2541:Pelvic kidney
2539:
2537:
2534:
2532:
2529:
2527:
2526:Renal ectopia
2524:
2522:
2519:
2518:
2513:
2510:
2508:
2505:
2503:
2500:
2498:
2495:
2494:
2493:
2492:
2488:
2487:
2484:
2481:
2479:
2476:
2474:
2471:
2469:
2466:
2465:
2463:
2461:
2457:
2454:
2450:
2446:
2442:
2439:
2432:
2427:
2425:
2420:
2418:
2413:
2412:
2409:
2401:
2400:
2399:
2393:
2389:
2367:
2363:
2362:
2358:
2356:
2353:
2350:
2347:
2343:
2342:
2338:
2336:
2332:
2331:
2327:
2326:
2323:
2319:
2312:
2308:
2307:
2303:
2301:
2297:
2296:
2292:
2290:
2286:
2285:
2281:
2279:
2275:
2274:
2270:
2266:
2264:
2260:
2259:
2255:
2251:
2250:
2247:
2242:
2238:
2231:
2224:
2220:
2215:
2210:
2205:
2200:
2196:
2192:
2188:
2183:
2179:
2175:
2170:
2165:
2161:
2157:
2153:
2149:
2145:
2140:
2136:
2132:
2127:
2122:
2118:
2114:
2110:
2106:
2102:
2097:
2096:
2091:
2074:
2070:
2064:
2061:
2048:
2044:
2038:
2035:
2030:
2024:
2020:
2013:
2010:
2005:
2001:
1996:
1991:
1987:
1983:
1979:
1975:
1971:
1964:
1961:
1956:
1952:
1945:
1942:
1937:
1933:
1928:
1923:
1919:
1915:
1908:
1906:
1904:
1900:
1888:
1882:
1879:
1874:
1870:
1865:
1860:
1856:
1852:
1848:
1844:
1840:
1833:
1830:
1819:
1815:
1808:
1805:
1800:
1796:
1791:
1786:
1782:
1778:
1774:
1770:
1766:
1759:
1756:
1751:
1747:
1742:
1737:
1733:
1729:
1725:
1721:
1717:
1710:
1707:
1702:
1698:
1693:
1688:
1684:
1680:
1676:
1672:
1668:
1661:
1658:
1653:
1646:
1643:
1638:
1634:
1629:
1624:
1620:
1616:
1612:
1608:
1604:
1597:
1594:
1589:
1585:
1580:
1575:
1571:
1567:
1563:
1559:
1555:
1548:
1545:
1540:
1536:
1531:
1526:
1522:
1518:
1514:
1510:
1506:
1499:
1496:
1491:
1487:
1483:
1479:
1475:
1469:
1466:
1454:
1450:
1444:
1441:
1436:
1434:9780323242875
1430:
1426:
1425:
1417:
1414:
1409:
1405:
1400:
1395:
1390:
1385:
1381:
1377:
1373:
1366:
1364:
1362:
1360:
1356:
1351:
1347:
1343:
1339:
1335:
1331:
1327:
1323:
1319:
1315:
1308:
1305:
1300:
1296:
1292:
1288:
1284:
1280:
1273:
1270:
1265:
1261:
1256:
1251:
1247:
1243:
1239:
1235:
1231:
1224:
1221:
1216:
1212:
1208:
1204:
1200:
1196:
1192:
1188:
1181:
1178:
1173:
1169:
1164:
1159:
1155:
1151:
1147:
1143:
1139:
1132:
1129:
1124:
1120:
1115:
1110:
1106:
1102:
1098:
1094:
1090:
1083:
1080:
1075:
1071:
1066:
1061:
1057:
1053:
1049:
1042:
1040:
1036:
1031:
1027:
1023:
1019:
1015:
1011:
1007:
1003:
996:
994:
990:
985:
981:
977:
973:
969:
965:
961:
957:
950:
948:
946:
944:
942:
940:
936:
931:
927:
922:
917:
913:
909:
905:
901:
897:
890:
888:
886:
884:
882:
880:
876:
871:
869:9781582557243
865:
861:
860:
852:
849:
838:on 2017-01-04
837:
833:
829:
823:
820:
815:
811:
806:
801:
797:
793:
789:
782:
779:
776:
775:
770:
765:
762:
757:
753:
749:
745:
741:
734:
731:
726:
722:
717:
712:
708:
704:
700:
696:
692:
685:
682:
669:
663:
660:
647:
641:
638:
625:
619:
616:
605:
601:
595:
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556:Epidemiology
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417:polycystin-1
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2999:bile duct:
2913:Breast cyst
2633:Hypospadias
2330:MedlinePlus
2154:: 321–337.
1478:Kidney News
41:Other names
3310:Ciliopathy
3299:Categories
3283:See also:
3210:centrosome
3184:basal body
3156:Structural
2857:Keratocyst
2826:Pilar cyst
2628:Epispadias
2576:Megaureter
2438:Congenital
2361:Patient UK
2306:DiseasesDB
2079:2 November
2053:2 November
1893:2022-10-24
1823:2022-10-24
1459:2022-11-17
842:2015-07-31
609:2015-07-30
577:References
453:ultrasound
369:parenchyma
69:Nephrology
3224:Signaling
3163:receptor:
2672:Vestigial
2452:Abdominal
2341:eMedicine
2197:: 69–83.
1490:1555-905X
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674:3 January
652:3 January
630:3 January
541:Prognosis
493:Tolvaptan
479:Treatment
439:Diagnosis
399:Mechanism
267:(ARPKD).
166:aneurysms
103:Treatment
64:Specialty
2883:Mucocele
2355:radio/69
2352:radio/68
2349:ped/1846
2346:med/1862
2223:21694932
2178:18947299
2135:21079243
2004:26171904
1955:Medscape
1936:70649130
1873:31361604
1799:26764208
1750:26538633
1701:32086281
1637:35664270
1588:31631001
1539:35570988
1408:21694932
1350:28192819
1279:Genomics
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1172:16449663
1123:27259053
1074:16816842
1022:17434405
984:12417947
976:16462154
930:25490692
814:26088074
725:20382325
565:See also
537:drugs".
516:dialysis
361:in utero
314:dialysis
228:dialysis
185:sonogram
162:pancreas
76:Symptoms
2981:liver:
2680:Urachus
2620:Urethra
2602:Bladder
2388:Scholia
2300:D007690
2214:3108786
2169:2834200
2126:2983067
1995:8406618
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1322:Bibcode
1314:Science
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1264:1980516
1255:1017261
1207:1676697
1163:1367205
1114:4908191
1030:1700992
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716:2898711
445:CT scan
365:nephron
320:Genetic
230:and/or
226:(RRT):
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2594:Pelvic
2563:Ureter
2491:cystic
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1026:S2CID
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393:PKHD1
324:genes
254:Cause
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