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10.1186/s13023-018-0818-2

http://scihub22266oqcxt.onion/10.1186/s13023-018-0818-2
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C5930429!5930429!29716638
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suck abstract from ncbi


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pmid29716638      Orphanet+J+Rare+Dis 2018 ; 13 (ä): ä
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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[]; 13 (ä): ä PMID29716638show 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.
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