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10.1186/s13023-018-0818-2

http://scihub22266oqcxt.onion/10.1186/s13023-018-0818-2
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suck abstract from ncbi


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pmid29716638
      Orphanet+J+Rare+Dis 2018 ; 13 (1 ): 69
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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 #MMPMID29716638
  • Bucciol G ; Cassiman D ; Roskams T ; Renard M ; Hoffman I ; Witters P ; Schrijvers R ; Schaballie H ; Bosch B ; Putti MC ; Gheysens O ; Knops N ; Gewillig M ; Mekahli D ; Pirenne J ; Meyts I
  • Orphanet J Rare Dis 2018[May]; 13 (1 ): 69 PMID29716638 show ga
  • 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.
  • |Bone Marrow Diseases/*surgery [MESH]
  • |Child [MESH]
  • |Child, Preschool [MESH]
  • |Exocrine Pancreatic Insufficiency/*surgery [MESH]
  • |Female [MESH]
  • |Hepatopulmonary Syndrome/*etiology/*surgery [MESH]
  • |Humans [MESH]
  • |Lipomatosis/*surgery [MESH]
  • |Liver Diseases/*complications/etiology [MESH]
  • |Liver Transplantation/*methods [MESH]
  • |Shwachman-Diamond Syndrome [MESH]


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