jueves, 3 de mayo de 2018

Liver transplantation for very severe hepatopulmonary syndrome due to vitamin A-induced chronic liver disease in a patient with Shwachman-Diamond syndrome | Orphanet Journal of Rare Diseases | Full Text

Liver transplantation for very severe hepatopulmonary syndrome due to vitamin A-induced chronic liver disease in a patient with Shwachman-Diamond syndrome | Orphanet Journal of Rare Diseases | Full Text

Orphanet Journal of Rare Diseases

Liver transplantation for very severe hepatopulmonary syndrome due to vitamin A-induced chronic liver disease in a patient with Shwachman-Diamond syndrome

  • Giorgia Bucciol,
  • David Cassiman,
  • Tania Roskams,
  • Marleen Renard,
  • Ilse Hoffman,
  • Peter Witters,
  • Rik Schrijvers,
  • Heidi Schaballie,
  • Barbara Bosch,
  • Maria Caterina Putti,
  • Olivier Gheysens,
  • Noel Knops,
  • Marc Gewillig,
  • Djalila Mekahli,
  • Jacques Pirenne and
  • Isabelle MeytsEmail author
Contributed equally
Orphanet Journal of Rare Diseases201813:69
Received: 10 August 2017
Accepted: 27 April 2018
Published: 2 May 2018


Abstract

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.

Keywords

Vitamin ALiver transplantationShwachman-Diamond syndromeHepatopulmonary syndrome

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