Article ; Online: Liver Transplantation in Patients With Niemann-Pick Disease--Single-Center Experience.
2015 Volume 47, Issue 10, Page(s) 2929–2931
Abstract: Niemann-Pick disease (NPD) is a rare syndrome caused by abnormal intracellular sphingomyelin lipid storage in cells known as "Pick cells." NPD can start in childhood or develop insidiously, with a predilection for reticuloendothelial cells and the ... ...
Abstract | Niemann-Pick disease (NPD) is a rare syndrome caused by abnormal intracellular sphingomyelin lipid storage in cells known as "Pick cells." NPD can start in childhood or develop insidiously, with a predilection for reticuloendothelial cells and the nervous system. NPD is a heterogeneous clinical, and biomolecular disorder which has 6 variants. There is no curable treatment for NPD. Generally, the treatment for all types of Niemann-Pick disease is to support. Type B NPD (NPD-B) is mostly characterized by hepatosplenomegaly, which can evolve to hepatic cirrhosis. In patients who progress to liver failure, liver transplantation may be improve liver function. The Transplant Service of Hospital Universitário Walter Cantídio performed its first liver transplants in patients with NPD-B with good results, demonstrating the efficacy of this procedure in selected cases. |
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MeSH term(s) | Adult ; Female ; Hepatomegaly/etiology ; Hepatomegaly/surgery ; Humans ; Liver Failure/etiology ; Liver Failure/surgery ; Liver Transplantation ; Male ; Niemann-Pick Disease, Type B/complications ; Niemann-Pick Disease, Type B/surgery ; Splenomegaly/etiology ; Splenomegaly/surgery ; Treatment Outcome |
Language | English |
Publishing date | 2015-12 |
Publishing country | United States |
Document type | Case Reports ; Journal Article |
ZDB-ID | 82046-5 |
ISSN | 1873-2623 ; 0041-1345 |
ISSN (online) | 1873-2623 |
ISSN | 0041-1345 |
DOI | 10.1016/j.transproceed.2015.10.051 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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