Artikel ; Online: Liver Transplantation in Patients With Niemann-Pick Disease--Single-Center Experience.
2015 Band 47, Heft 10, Seite(n) 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. |
---|---|
Mesh-Begriff(e) | 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 |
Sprache | Englisch |
Erscheinungsdatum | 2015-12 |
Erscheinungsland | United States |
Dokumenttyp | 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 |
Datenquelle | MEDical Literature Analysis and Retrieval System OnLINE |
Volltext online
Zusatzmaterialien
Kategorien
Verfügbar in ZB MED Köln/Königswinter
Zs.A 835: Hefte anzeigen | Standort: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG) |
Über subito bestellen
Dieser Service ist kostenpflichtig (siehe Lieferbedingungen von subito). Bestellungen, die einen Artikel nebst Supplementary Material umfassen, werden grundsätzlich wie mehrfache Bestellungen bearbeitet. Gebühren fallen in diesen Fällen für jede einzelne Bestellung an.