{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T16:54:26Z","timestamp":1791824066698,"version":"4.3.4"},"reference-count":20,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2005,6,3]],"date-time":"2005-06-03T00:00:00Z","timestamp":1117756800000},"content-version":"vor","delay-in-days":7703,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Am. J. Med. Genet."],"published-print":{"date-parts":[[1984,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Few reports are available on the age\u2010related risk of relatives of affected persons to manifest adult polycystic kidney disease (APKD). For 371 persons in 17 kindreds, at risk for APKD by virtue of having an affected first degree relative, we calculated the estimated probability of clinical diagnosis of APKD to be 0.011 by age 20, 0.041 by age 30, 0.115 by age 40, 0.299 by age 50, and 0.404 by age 60 years (expected = 0.50). Ultrasonographic examination of 172 asymptomatic persons at risk showed definite APKD is 60. The probability of ultrasonographic detection of asymptomatic APKD is estimated as 0.222, 0.657, and 0.855 at age 5, 15, and 25 years, respectively. The probability of having APKD following normal ultrasonogram results (conservatively assuming 90% specificity) is estimated as 0.46, 0.28, and 0.14 for persons at 50% risk in their first, second, or third decade. The marginal benefit of ultrasound as a diagnostic test for APKD for persons in the second or third decade is estimated as 0.37 and 0.41, respectively for a \u201cpositive\u201d test and 0.22 and 0.37 for a \u201cnegative\u201d test.<\/jats:p>","DOI":"10.1002\/ajmg.1320180108","type":"journal-article","created":{"date-parts":[[2005,6,11]],"date-time":"2005-06-11T19:41:50Z","timestamp":1118518910000},"page":"45-53","source":"Crossref","is-referenced-by-count":126,"title":["Age at clinical onset and at ultrasonographic detection of adult polycystic kidney disease: Data for genetic counselling"],"prefix":"10.1002","volume":"18","author":[{"given":"J. C.","family":"Bear","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"McManamon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Morgan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. H.","family":"Payne","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"Lewis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. H.","family":"Gault","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. N.","family":"Churchill","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John M.","family":"Opitz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2005,6,3]]},"reference":[{"key":"e_1_2_1_2_1","unstructured":"Canadian Renal Failure Register(1981):1981 Report. Kidney Foundation of Canada."},{"key":"e_1_2_1_3_1","first-page":"383","article-title":"Adult onset polycystic kidney disease\u2010a reappraisal of prognosis","volume":"16","author":"Churchill DN","year":"1983","journal-title":"Ann Roy Col Phys Surg Canada"},{"key":"e_1_2_1_4_1","first-page":"1","article-title":"Bilateral polycystic disease of the kidneys. A follow\u2010up of 284 patients and their families","volume":"328","author":"Dalgaard OZ","year":"1957","journal-title":"Acta Med Scand"},{"key":"e_1_2_1_5_1","first-page":"1097","article-title":"Adult polycystics disease of the kidneys","volume":"1","year":"1981","journal-title":"Br Med J"},{"key":"e_1_2_1_6_1","first-page":"1","article-title":"Genetic counselling renal disease","volume":"6","author":"Fialkow PJ","year":"1973","journal-title":"Kidney"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.222.12.1527"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1980.tb00865.x"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1080\/01621459.1958.10501452"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcu.1870060503"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198108203050805"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/0090-4295(74)90099-5"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/ki.1972.78"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/73.6.740"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(80)91674-8"},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.1159\/000181715"},{"key":"e_1_2_1_17_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-92-2-243"},{"key":"e_1_2_1_18_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiology.135.2.7367638"},{"key":"e_1_2_1_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/0021-9681(79)90012-2"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320110412"},{"key":"e_1_2_1_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1978.tb02053.x"}],"container-title":["American Journal of Medical Genetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajmg.1320180108","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajmg.1320180108","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,19]],"date-time":"2023-10-19T22:35:16Z","timestamp":1697754916000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajmg.1320180108"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1984,5]]},"references-count":20,"journal-issue":{"issue":"1","published-print":{"date-parts":[[1984,5]]}},"alternative-id":["10.1002\/ajmg.1320180108"],"URL":"https:\/\/doi.org\/10.1002\/ajmg.1320180108","archive":["Portico"],"relation":{},"ISSN":["0148-7299","1096-8628"],"issn-type":[{"value":"0148-7299","type":"print"},{"value":"1096-8628","type":"electronic"}],"subject":[],"published":{"date-parts":[[1984,5]]}}}