{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T16:33:17Z","timestamp":1790094797004,"version":"4.0.1"},"reference-count":21,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":11931,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[1983,1]]},"abstract":"<jats:sec>\n            <jats:title\/>\n            <jats:p>Five children were noted to have arteriohepatic dysplasia (Alagille's syndrome) between 3 and 7 months of age. Prior to diagnosis, four underwent Kasai procedures after intraoperative cholangio\u2013grams failed to demonstrate patency of the extrahepatic bile ducts. In three patients, a focal proximal hypoplasia of the common hepatic duct was demonstrated with fibrosis and increased vascularity. Hypoplasia of the gallbladder occurred in two patients.<\/jats:p>\n            <jats:p>Changes were observed in the porta hepatis. Eighty to 208 \/tm bile ducts were associated with peripherally located gland\u2013like structures. These changes are indistinguishable from those in fibrous remnants of extrahepatic biliary atresia.<\/jats:p>\n            <jats:p>Hepatic features of sequential liver biopsies obtained in the five patients were divided into early and late changes. From birth to 3 months of age, the pathology consisted of cholestasis and bile duct destruction. After 3 months of age, there was persistent cholestasis, paucity of interlobular bile ducts, and portal fibrosis. Ductular proliferation was not an intrinsic change. When present, it was related to a recent episode of cholangitis.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1002\/hep.1840030113","type":"journal-article","created":{"date-parts":[[2010,7,5]],"date-time":"2010-07-05T13:02:20Z","timestamp":1278334940000},"page":"77-83","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":39,"title":["Arteriohepatic Dysplasia. Ii. Hepatobiliary Morphology"],"prefix":"10.1097","volume":"3","author":[{"given":"Ellen I.","family":"Kahn","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fredric","family":"Daum","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Markowitz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harvey W.","family":"Aiges","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keith M.","family":"Schneider","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henry B.","family":"So","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Altman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roma S.","family":"Chandra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mervin","family":"Silverberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"10.1002\/hep.1840030113-BIB1|cit1","first-page":"471","article-title":"Cholestasis in the first three months of life","volume":"6","author":"Alagille","year":"1979","journal-title":"Prog Liver Dis"},{"key":"10.1002\/hep.1840030113-BIB2|cit2","doi-asserted-by":"crossref","first-page":"815","DOI":"10.1016\/0022-3468(76)90108-1","article-title":"The surgery of biliary hypoplasia","volume":"11","author":"Lilly","year":"1976","journal-title":"J Pediatr Surg"},{"key":"10.1002\/hep.1840030113-BIB3|cit3","first-page":"43","article-title":"A clinical experience with familial paucity of intrahepatic bile ducts associated with defective metabolism of trihydroxycoprostanic acid to cholic acid","volume":"49","author":"Isenberg","year":"1975","journal-title":"Liver Disease in Children: Hepatologie Infantile"},{"key":"10.1002\/hep.1840030113-BIB4|cit4","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1007\/BF01308095","article-title":"Paucity of interlobular bile ducts. Getting to know it better","volume":"26","author":"Perrault","year":"1981","journal-title":"Dig Dis Sci"},{"key":"10.1002\/hep.1840030113-BIB5|cit5","doi-asserted-by":"crossref","first-page":"690","DOI":"10.1136\/adc.45.243.690","article-title":"Lymphoedema in hereditary recurrent cholestasis from birth","volume":"45","author":"Aagenaes","year":"1970","journal-title":"Arch Dis Child"},{"key":"10.1002\/hep.1840030113-BIB6|cit6","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1016\/S0022-3476(75)80706-2","article-title":"Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental and sexual development and cardiac murmur","volume":"86","author":"Alagille","year":"1975","journal-title":"J Pediatr"},{"key":"10.1002\/hep.1840030113-BIB7|cit7","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1002\/hep.1840030112","article-title":"Arteriohepatic dysplasia. I. Pitfalls in diagnosis and management","volume":"3","author":"Markowitz","year":"1983","journal-title":"Hepatology"},{"key":"10.1002\/hep.1840030113-BIB8|cit8","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1002\/hep.1840020311","article-title":"Arteriohepatic dysplasia in infancy and childhood: a longitudinal study of six patients","volume":"2","author":"Dahms","year":"1982","journal-title":"Hepatology"},{"key":"10.1002\/hep.1840030113-BIB9|cit9","unstructured":"Witzleben CL Neonatal hepatitis and biliary atresia. 1977 339 350"},{"key":"10.1002\/hep.1840030113-BIB10|cit10","first-page":"177","volume-title":"Neonatal hepatitis and biliary atresia","author":"Alagille","year":"1977"},{"key":"10.1002\/hep.1840030113-BIB11|cit11","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1016\/S0022-3476(78)80495-8","article-title":"Ductal remnants in extrahepatic biliary atresia. A histopathological study with clinical correlations","volume":"93","author":"Chandra","year":"1978","journal-title":"J Pediatr"},{"key":"10.1002\/hep.1840030113-BIB12|cit12","first-page":"397","article-title":"Extrahepatic biliary atresia. Morphological study of 98 biliary remnants","volume":"105","author":"Gautier","year":"1981","journal-title":"Arch Pathol Lab Med"},{"key":"10.1002\/hep.1840030113-BIB13|cit13","first-page":"391","volume-title":"Neonatal hepatitis and biliary atresia. Bethesda","author":"Altman","year":"1977"},{"key":"10.1002\/hep.1840030113-BIB14|cit14","doi-asserted-by":"crossref","first-page":"460","DOI":"10.1136\/adc.56.6.460","article-title":"Hepatic portoenter-ostomy for biliary atresia","volume":"56","author":"Lawrence","year":"1981","journal-title":"Arch Dis Child"},{"key":"10.1002\/hep.1840030113-BIB15|cit15","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1007\/BF01308096","article-title":"Syndromatic hepatic ductular hypoplasia (arteriohepatic dysplasia). A clinical and he-patohistological study of three patients","volume":"26","author":"Berman","year":"1981","journal-title":"Dig Dis Sci"},{"key":"10.1002\/hep.1840030113-BIB16|cit16","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1111\/j.1651-2227.1977.tb07801.x","article-title":"Hereditary cholestasis combined with peripheral pulmonary stenosis and other anomalies","volume":"66","author":"Henriksen","year":"1977","journal-title":"Acta Paediatr Scand"},{"key":"10.1002\/hep.1840030113-BIB17|cit17","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1016\/S0022-3476(78)80428-4","article-title":"A father and son with cholestasis and peripheral pulmonic stenosis. 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