{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T14:00:09Z","timestamp":1753884009187,"version":"3.30.0"},"reference-count":23,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":11811,"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":[[1983,5]]},"abstract":"<jats:sec>\n            <jats:title\/>\n            <jats:p>Three of four adult siblings in a family which was studied for three generations had clinical and\/ or laboratory signs of slowly progressive intrahepatic cholestasis. Slight hyper pigmentation, facial hypertrichosis, and hypothyroidism were seen in affected individuals who also had prolonged increase in serum transaminase, \u03b3-glutamyltranspeptidase, and alkaline phosphatase activities. Asymptomatic intervals were characterized by abnormal bromosulfophthalein retention, reduced <jats:italic toggle=\"yes\">N<\/jats:italic>-demethylation capacity, elevated fasting total serum bile acid levels, and normal light microscopic findings. A high serum \u03b1-lipoprotein level was found in individuals affected with this hitherto unknown entity which appears to have an autosomal recessive mode of inheritance.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1002\/hep.1840030318","type":"journal-article","created":{"date-parts":[[2010,7,5]],"date-time":"2010-07-05T13:06:30Z","timestamp":1278335190000},"page":"391-398","update-policy":"http:\/\/dx.doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":5,"title":["Familial Benign Chronic Intrahepatic Cholestasis"],"prefix":"10.1097","volume":"3","author":[{"given":"Sten","family":"Eriksson","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christer","family":"Larsson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"276","reference":[{"key":"10.1002\/hep.1840030318-BIB1|cit1","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1172\/JCI108127","article-title":"The metabolism of 3\u03b1,7\u03b112\u03b1-trihydroxy-5\u03b2-cholestan-26-oic acid in two siblings with cholestasis due to intrahepatic bile duct anomalies","volume":"56","author":"Hanson","year":"1975","journal-title":"J Clin Invest"},{"key":"10.1002\/hep.1840030318-BIB2|cit2","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1136\/adc.43.232.646","article-title":"Hereditary recurrent intrahepatic cholestasis from birth","volume":"43","author":"Aagenaes","year":"1968","journal-title":"Arch Dis Child"},{"key":"10.1002\/hep.1840030318-BIB3|cit3","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1542\/peds.8.5.628","article-title":"Atresia of the intrahepatic bile ducts","volume":"8","author":"Ahrens","year":"1951","journal-title":"Pediatrics"},{"key":"10.1002\/hep.1840030318-BIB4|cit4","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1016\/0016-5085(81)90487-X","article-title":"Severe familial cholestasis in North American Indian children: a clinical model of microfilament dysfunction?","volume":"81","author":"Weber","year":"1981","journal-title":"Gastroenterology"},{"key":"10.1002\/hep.1840030318-BIB5|cit5","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1542\/peds.62.1.22","article-title":"\u03b11-Antitrypsin deficiency in early childhood","volume":"62","author":"Sveger","year":"1978","journal-title":"Pediatrics"},{"key":"10.1002\/hep.1840030318-BIB6|cit6","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1056\/NEJM198103053041001","article-title":"Heterozygous MZ alphai-antitrypsin deficiency in adults with chronic active hepatitis and cryptogenic cirrhosis","volume":"304","author":"Hodges","year":"1981","journal-title":"N Engl J Med"},{"key":"10.1002\/hep.1840030318-BIB7|cit7","doi-asserted-by":"crossref","first-page":"686","DOI":"10.1016\/S0140-6736(59)92128-2","article-title":"Benign recurrent intrahepatic obstructive jaundice","volume":"2","author":"Summerskill","year":"1959","journal-title":"Lancet"},{"key":"10.1002\/hep.1840030318-BIB8|cit8","first-page":"301","volume-title":"Diseases of the liver","author":"Schaffner","year":"1979"},{"key":"10.1002\/hep.1840030318-BIB9|cit9","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1016\/S0016-5085(76)80187-4","article-title":"Familial benign recurrent intrahepatic cholestasis. 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