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Gaucher disease and the clinical experience with substrate reduction therapy.

Gaucher disease is caused by an enzymatic defect with consequent accumulation of glucocerebroside. Type I, the non-neuronopathic form, is rather common and panethnic. Patients may present with hepatosplenomegaly, anaemia, thrombocytopenia and skeletal or lung involvement. Enzyme replacement therapy...

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Bibliografiske detaljer
Main Authors: Zimran, Ari, Elstein, Deborah
Format: Artigo
Sprog:Inglês
Udgivet: 2003
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC1693183/
https://ncbi.nlm.nih.gov/pubmed/12803930
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1098/rstb.2003.1272
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