{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T16:09:25Z","timestamp":1786118965329,"version":"build-2736575974"},"reference-count":19,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"Supplement 1","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":11566,"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":[[1984,1]]},"abstract":"<jats:sec>\n                    <jats:title\/>\n                    <jats:p>The liver may be involved in metabolic disorders in two ways, (i) Due to its critical role in several metabolic pathways, the liver may be the major site of expression of a biochemical abnormality. For example, in Type I Crigler-Najjar syndrome, absence of hepatic bilirubin glucuronyl transferase activity causes severe unconjugated hyperbilirubinemia which invariably leads to death from kernicterus. (ii) The liver may also be structurally damaged by the metabolic disorder. Several patients with protoporphyria have died in hepatic failure due to liver damage caused by protoporphyrin deposition. Numerous other metabolic disorders can be sited in which significant liver involvement occurs. In many of these disorders, there is no effective medical therapy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/hep.1840040707","type":"journal-article","created":{"date-parts":[[2008,9,12]],"date-time":"2008-09-12T05:44:29Z","timestamp":1221198269000},"page":"18S-21S","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":9,"title":["The Liver in Crigler-Najjar Syndrome, Protoporphyria, and Other Metabolic Disorders"],"prefix":"10.1097","volume":"4","author":[{"given":"Joseph R.","family":"Bloomer","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harvey L.","family":"Sharp","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"10.1002\/hep.1840040707-BIB1","first-page":"169","article-title":"Congenital familial nonhemolytic jaundice with kernicterus","volume":"10","author":"Crigler","year":"1952","journal-title":"Pediatrics"},{"key":"10.1002\/hep.1840040707-BIB2","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1016\/0002-9343(69)90224-1","article-title":"Chronic nonhemolytic unconjugated hyperbilirubinemia with glucuronyl transferase deficiency: clinical, biochemical, pharmacologic, and genetic evidence for heterogeneity","volume":"47","author":"Arias","year":"1969","journal-title":"Am J Med"},{"key":"10.1002\/hep.1840040707-BIB3","doi-asserted-by":"crossref","first-page":"970","DOI":"10.1172\/JCI108877","article-title":"Unconjugated bilirubin and an increased proportion of bilirubin monoconjugate in the bile of patients with Gilbert's syndrome and Crigler-Najjar disease","volume":"60","author":"Fevery","year":"1977","journal-title":"J Clin Invest"},{"key":"10.1002\/hep.1840040707-BIB4","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1203\/00006450-197106000-00003","article-title":"Bilirubin metabolism in congenital nonhemolytic jaundice","volume":"5","author":"Bloomer","year":"1971","journal-title":"Pediatr Res"},{"key":"10.1002\/hep.1840040707-BIB5","doi-asserted-by":"crossref","first-page":"761","DOI":"10.1016\/S0016-5085(76)80271-5","article-title":"Bilirubin secretion and conjugation in the Crigler-Najjar syndrome type II","volume":"70","author":"Gordon","year":"1976","journal-title":"Gastroenterology"},{"key":"10.1002\/hep.1840040707-BIB6","doi-asserted-by":"crossref","first-page":"840","DOI":"10.1016\/S0016-5085(79)80188-2","article-title":"Crigler-Najjar syndrome (type I) in an adult male","volume":"76","author":"Wolkoff","year":"1979","journal-title":"Gastroenterology"},{"key":"10.1002\/hep.1840040707-BIB7","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1203\/00006450-197405000-00006","article-title":"Crigler-Najjar syndrome: an unusual course with development of neurologic damage at age eighteen","volume":"8","author":"Blaschke","year":"1974","journal-title":"Pediatr Res"},{"key":"10.1002\/hep.1840040707-BIB8","doi-asserted-by":"crossref","first-page":"786","DOI":"10.1172\/JCI107617","article-title":"Removing substances from blood by affinity chromatography. 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Removing bilirubin from the blood of jaundiced rats by hemoperfusion over albumin-conjugated agarose beads","volume":"53","author":"Scharschmidt","year":"1974","journal-title":"J Clin Invest"},{"key":"10.1002\/hep.1840040707-BIB9","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1126\/science.182.4107.68","article-title":"Induction of an enzyme in genetically deficient rats after grafting of normal liver","volume":"182","author":"Mukherjee","year":"1973","journal-title":"Science"},{"key":"10.1002\/hep.1840040707-BIB10","doi-asserted-by":"crossref","first-page":"892","DOI":"10.1126\/science.818706","article-title":"Hepatocellular transplantation for metabolic deficiencies: decrease of plasma bilirubin in Gunn rats","volume":"192","author":"Matas","year":"1976","journal-title":"Science"},{"key":"10.1002\/hep.1840040707-BIB11","doi-asserted-by":"crossref","first-page":"448","DOI":"10.1016\/S0140-6736(61)92427-8","article-title":"Erythropoietic protoporphyria: a new porphyria with solar urticaria due to protopor-phyrinaemia","volume":"2","author":"Magnus","year":"1961","journal-title":"Lancet"},{"key":"10.1002\/hep.1840040707-BIB12","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1055\/s-2008-1040704","article-title":"Protoporphyria","volume":"2","author":"Bloomer","year":"1982","journal-title":"Seminars in Liver Disease"},{"key":"10.1002\/hep.1840040707-BIB13","doi-asserted-by":"crossref","first-page":"1231","DOI":"10.1056\/NEJM197005282822204","article-title":"Beta-carotene as a photoprotective agent in erythropoietic protoporphyria","volume":"282","author":"Mathews-Roth","year":"1970","journal-title":"N Engl J Med"},{"key":"10.1002\/hep.1840040707-BIB14","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1136\/jcp.21.2.157","article-title":"Erythropoietic porphyrin hepatitis","volume":"21","author":"Barnes","year":"1968","journal-title":"J Clin Pathol"},{"key":"10.1002\/hep.1840040707-BIB15","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1016\/0002-9343(75)90644-0","article-title":"Hepatic disease in erythropoietic protoporphyria","volume":"58","author":"Bloomer","year":"1975","journal-title":"Am J Med"},{"key":"10.1002\/hep.1840040707-BIB16","doi-asserted-by":"crossref","first-page":"817","DOI":"10.1002\/hep.1840020613","article-title":"Effect of hematin administration to patients with protoporphyria and liver disease","volume":"2","author":"Bloomer","year":"1982","journal-title":"Hepatology"},{"key":"10.1002\/hep.1840040707-BIB17","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1016\/0016-5085(80)90084-0","article-title":"Hepatic protoporphyrin production in human protoporphyria. Effects of intravenous hematin and analysis of erythrocyte protoporphyrin distribution","volume":"79","author":"Lamon","year":"1980","journal-title":"Gastroenterology"},{"key":"10.1002\/hep.1840040707-BIB18","first-page":"6347","article-title":"Effect of choleretics on canalicular transport of protoporphyrin in the rat liver","volume":"242","author":"Avner","year":"1982","journal-title":"Am J Physiol"},{"key":"10.1002\/hep.1840040707-BIB19","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1002\/hep.1840030319","article-title":"Cholic acid amelioration of light and electron microscopic hepatic lesions in experimental protoporphyria","volume":"3","author":"Lefkowitch","year":"1983","journal-title":"Hepatology"}],"container-title":["Hepatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fhep.1840040707","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.ovid.com\/01515467-198401001-00006","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,6]],"date-time":"2026-08-06T17:11:06Z","timestamp":1786036266000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/01515467-198401001-00006"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1984,1]]},"references-count":19,"journal-issue":{"issue":"Supplement 1","published-print":{"date-parts":[[1984]]}},"URL":"https:\/\/doi.org\/10.1002\/hep.1840040707","relation":{},"ISSN":["0270-9139"],"issn-type":[{"value":"0270-9139","type":"print"}],"subject":[],"published":{"date-parts":[[1984,1]]}}}