Evaluation of the Nutritional Status of Gaucher Disease Type I Patients under Enzyme Replacement Treatment.

ABSTRACT: (1) Background: Gaucher disease (GD) is a rare lysosomal storage disease. The few studies analyzing Resting Energy Expenditure (REE) in GD involved mainly untreated patients and supported a hypermetabolic condition possibly due to the associated inflammatory state. Definitive conclusions could not be drawn also because of the heterogeneity and the small size of the samples investigated. In order to expand current knowledge concerning, in particular the condition of patients under Enzyme Replacement Therapy (ERT), we evaluated the nutritional status of a relatively large sample of GD patients followed at Federico II University Hospital in Naples, Italy. (2) Methods: The study, having a cross-sectional design and involving 26 patients on ERT, included routine biochemical analyses, bioelectri

SUBMITTER: Iaccarino Idelson P 

PROVIDER: S-EPMC9370155 | biostudies-literature | 2022 Aug

REPOSITORIES: biostudies-literature