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Cartilage-hair Hypoplasia Complicated with Liver Cirrhosis Due to Chronic Intrahepatic Cholestasis.
Kogami, Taro; Uojima, Haruki; Ebato, Takasuke; Bando, Yuki; Hoshino, Akiyoshi; Saegusa, Makoto; Ohbu, Makoto; Iwasaki, Shuichiro; Wada, Naohisa; Kubota, Kousuke; Tanaka, Yoshiaki; Hidaka, Hisashi; Nakazawa, Takahide; Shibuya, Akitaka; Koizumi, Wasaburo.
Afiliación
  • Kogami T; Department of Gastroenterology, Internal Medicine, Kitasato University School of Medicine, Japan.
  • Uojima H; Department of Gastroenterology, Internal Medicine, Kitasato University School of Medicine, Japan.
  • Ebato T; Department of Pediatrics, Kitasato University School of Medicine, Japan.
  • Bando Y; Department of Pediatrics, Kitasato University School of Medicine, Japan.
  • Hoshino A; Department of Pathology, Kitasato University School of Medicine, Japan.
  • Saegusa M; Department of Pathology, Kitasato University School of Medicine, Japan.
  • Ohbu M; Department of Pathology, Kitasato Institute Hospital, Japan.
  • Iwasaki S; Department of Gastroenterology, Internal Medicine, Kitasato University School of Medicine, Japan.
  • Wada N; Department of Gastroenterology, Internal Medicine, Kitasato University School of Medicine, Japan.
  • Kubota K; Department of Gastroenterology, Internal Medicine, Kitasato University School of Medicine, Japan.
  • Tanaka Y; Department of Gastroenterology, Internal Medicine, Kitasato University School of Medicine, Japan.
  • Hidaka H; Department of Gastroenterology, Internal Medicine, Kitasato University School of Medicine, Japan.
  • Nakazawa T; Department of Gastroenterology, Internal Medicine, Kitasato University School of Medicine, Japan.
  • Shibuya A; Department of Gastroenterology, Internal Medicine, Kitasato University School of Medicine, Japan.
  • Koizumi W; Department of Gastroenterology, Internal Medicine, Kitasato University School of Medicine, Japan.
Intern Med ; 60(21): 3427-3433, 2021 Nov 01.
Article en En | MEDLINE | ID: mdl-33967143
ABSTRACT
We herein report a rare case of cartilage-hair hypoplasia (CHH) complicated with liver cirrhosis. A 20-year-old Japanese man with CHH was found incidentally to have liver cirrhosis and an esophageal varix. This patient had been treated for infections due to immunodeficiency since early childhood. He ultimately died of liver failure at 31 years of age. An autopsy revealed an abnormality of the interlobular bile ducts and intrahepatic cholestasis. Liver cirrhosis was thought to have been caused by chronic intrahepatic cholestasis due to biliary duct hypoplasia and changes in the intestinal microbiome. Therefore, CHH may cause biliary cirrhosis due to multiple effects.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Colestasis Intrahepática / Enfermedades de Inmunodeficiencia Primaria / Enfermedad de Hirschsprung Tipo de estudio: Diagnostic_studies Límite: Adult / Humans / Male Idioma: En Revista: Intern Med Asunto de la revista: MEDICINA INTERNA Año: 2021 Tipo del documento: Article País de afiliación: Japón
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Colestasis Intrahepática / Enfermedades de Inmunodeficiencia Primaria / Enfermedad de Hirschsprung Tipo de estudio: Diagnostic_studies Límite: Adult / Humans / Male Idioma: En Revista: Intern Med Asunto de la revista: MEDICINA INTERNA Año: 2021 Tipo del documento: Article País de afiliación: Japón