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Liver transplantation for very severe hepatopulmonary syndrome due to vitamin A-induced chronic liver disease in a patient with Shwachman-Diamond syndrome.
Bucciol, Giorgia; Cassiman, David; Roskams, Tania; Renard, Marleen; Hoffman, Ilse; Witters, Peter; Schrijvers, Rik; Schaballie, Heidi; Bosch, Barbara; Putti, Maria Caterina; Gheysens, Olivier; Knops, Noel; Gewillig, Marc; Mekahli, Djalila; Pirenne, Jacques; Meyts, Isabelle.
Afiliación
  • Bucciol G; Laboratory of Inborn Errors of Immunity, Department of Microbiology and Immunology, KU Leuven, Herestraat 49, 3000, Leuven, Belgium.
  • Cassiman D; Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium.
  • Roskams T; Gastroenterology and Hepatology, University Hospitals Leuven and KU Leuven, Leuven, Belgium.
  • Renard M; Anatomic Pathology, University Hospitals Leuven and KU Leuven, Leuven, Belgium.
  • Hoffman I; Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium.
  • Witters P; Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium.
  • Schrijvers R; Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium.
  • Schaballie H; Laboratory of Clinical Immunology, Microbiology and Immunology Department, University Hospitals Leuven and KU Leuven, Leuven, Belgium.
  • Bosch B; Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium.
  • Putti MC; Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium.
  • Gheysens O; St. Giles Laboratory of Human Genetics of Infectious Disease, Rockefeller Branch, The Rockefeller University, New York, USA.
  • Knops N; Pediatric Oncology and Hematology, Department of Child's and Woman's Health, University of Padova, Padova, Italy.
  • Gewillig M; Nuclear Medicine, University Hospitals Leuven and KU Leuven, Leuven, Belgium.
  • Mekahli D; Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium.
  • Pirenne J; Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium.
  • Meyts I; Department of Pediatric Cardiology, University Hospitals Leuven, Leuven, Belgium.
Orphanet J Rare Dis ; 13(1): 69, 2018 05 02.
Article en En | MEDLINE | ID: mdl-29716638
Vitamin A intoxication is a rare cause of liver disease, but the risk increases in patients with underlying liver dysfunction. We present a patient with Shwachman-Diamond Syndrome who developed liver fibrosis, portal hypertension and very severe hepatopulmonary syndrome as a consequence of chronic vitamin A intoxication. She underwent successful liver transplantation with complete resolution of the pulmonary shunting.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Insuficiencia Pancreática Exocrina / Vitamina A / Enfermedades de la Médula Ósea / Trasplante de Hígado / Síndrome Hepatopulmonar / Lipomatosis / Hepatopatías Tipo de estudio: Etiology_studies Límite: Child / Child, preschool / Female / Humans Idioma: En Revista: Orphanet J Rare Dis Asunto de la revista: MEDICINA Año: 2018 Tipo del documento: Article País de afiliación: Bélgica Pais de publicación: Reino Unido
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Insuficiencia Pancreática Exocrina / Vitamina A / Enfermedades de la Médula Ósea / Trasplante de Hígado / Síndrome Hepatopulmonar / Lipomatosis / Hepatopatías Tipo de estudio: Etiology_studies Límite: Child / Child, preschool / Female / Humans Idioma: En Revista: Orphanet J Rare Dis Asunto de la revista: MEDICINA Año: 2018 Tipo del documento: Article País de afiliación: Bélgica Pais de publicación: Reino Unido