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

被引：0

作者：

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

Isabelle Meyts

机构：

[1] KU Leuven,Laboratory of Inborn Errors of Immunity, Department of Microbiology and Immunology

[2] University of Padova,Pediatric Oncology and Hematology, Department of Child’s and Woman’s Health

[3] University Hospitals Leuven and KU Leuven,Gastroenterology and Hepatology

[4] University Hospitals Leuven and KU Leuven,Anatomic Pathology

[5] University Hospitals Leuven,Department of Pediatrics

[6] University Hospitals Leuven and KU Leuven,Laboratory of Clinical Immunology, Microbiology and Immunology Department

[7] The Rockefeller University,St. Giles Laboratory of Human Genetics of Infectious Disease, Rockefeller Branch

[8] University Hospitals Leuven and KU Leuven,Nuclear Medicine

[9] Department of Cardiovascular Sciences,Department of Pediatric Cardiology

[10] KU Leuven,Department of Development and Regeneration

[11] University Hospitals Leuven,Department of Pediatric Nephrology

[12] KU Leuven,Abdominal Transplantation Surgery, Laboratory of Abdominal Transplantation

[13] University Hospitals Leuven,undefined

[14] University Hospitals Leuven and KU Leuven,undefined

来源：

Orphanet Journal of Rare Diseases | / 13卷

关键词：

Vitamin A; Liver transplantation; Shwachman-Diamond syndrome; Hepatopulmonary syndrome;

D O I：

暂无

中图分类号：

学科分类号：

摘要：

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.

引用

共 50 条

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Hoffman, Ilse
Witters, Peter
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Schaballie, Heidi
Bosch, Barbara
Putti, Maria Caterina
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