Familial Cirrhosis

Article1 October 1964Familial CirrhosisA Clinical and Pathological StudyWILLIS C. MADDREY, M.D., FRANK L. IBER, M.D.WILLIS C. MADDREY, M.D.Search for more papers by this author, FRANK L. IBER, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-61-4-667 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThe familial occurrence of cirrhosis is unusual except in hepatolenticular degeneration, hemochromatosis, and a few other rare conditions. Eight families showing multiple patients with liver disease are presented, and the evidence suggesting a genetic predisposition is reviewed.The criterion for acceptance into this study was histologically proved disease of the liver in two or more members of a sibship. All material was reviewed by the authors. Figure 1 indicates the time course of each patient.CASE STUDIESFAMILY 1Three of the ten children of W. E. (Figure 2) died of severe liver disease at early ages. A fourth died...References1. BAGGENSTOSS AH: Postnecrotic cirrhosis: morphology, etiology and pathogenesis. Progress in Liver Diseases, edited by POPPER, H., and SCHAFFNER, F., Grune and Stratton, New York, 1961, p. 14. Google Scholar2. BEARN AG: Wilson's disease: an inborn error of metabolism with multiple manifestations. Amer. J. Med. 22: 747, 1957. CrossrefMedlineGoogle Scholar3. FINCHFINCH SCCA: Idiopathic hemochromatosis, an iron storage disease. Medicine 34: 381, 1955. CrossrefMedlineGoogle Scholar4. ISSELBACHER KJ: Galactose metabolism and galactosemia. Amer. J. Med. 26: 715, 1959. CrossrefMedlineGoogle Scholar5. DI SANT 'AGNESEBLANC PAWA: A distinctive type of biliary cirrhosis of the liver associated with cystic fibrosis of the pancreas. Pediatrics 18: 387, 1956. MedlineGoogle Scholar6. 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Author, Article, and Disclosure InformationAffiliations: Baltimore, MarylandFrom the Department of Medicine, Johns Hopkins University, School of Medicine, Baltimore, Md.Dr. Maddrey is a Henry Strong Denison Scholar in Medical Sciences, and Dr. Iber is a John and Mary Markle Scholar in Medical Sciences.Requests for reprints should be addressed to Frank L. Iber, M.D., The Johns Hopkins Hospital, Baltimore, Md. 21205. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byDevelopmental and Inherited Liver DiseaseGenetic and metabolic liver diseaseFamilial clustering of (mostly) HCV-related cirrhosis. 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