Year: 2009
Type: dissertation
Study design: Observational Study
Abstract: Objectives and backgroundGaucher disease (glucocerebrosidase deficiency) is characterized by massive accumulation of lipid-laden macrophages in various tissues.Gaucher patients show a hitherto unexplained increased hepatic glucose output.Since adiponectin is thought to influence hepatic glucose output, we studied its serum concentration in a cohort of Gaucher patients. ResultsSerum adiponectin was indeed found to be markedly reduced in patients (median value 3.1 µg/ml, range 1.4 -6.3) as compared to healthy subjects (median value 5.6 µg/ml range 1.9 -14.0).Successful therapy of Gaucher patients was accompanied by an increase in serum adiponectin, from 3.1 to 3.6 µg/ml (p=0.002).In healthy individuals low levels of circulating adiponectin, are generally associated with obesity.In Gaucher patients however adiponectin levels correlated not with body mass index. ConclusionsThe hypoadiponectinemia in Gaucher patients is most likely attributable to their lowgrade chronic inflammation.The characteristic storage macrophages produce inflammatory cytokines such as TNF-alpha that is known to suppress adiponectin production.It is of interest that the very low adiponectin levelsin Gaucher patients are not accompanied by hyperglycemia, contrary to their effect in obese individuals.It is hypothesized that the excessive hepatic glucose production on Gaucher patients balances the assumed increased glucose consumption by the massive amounts of storage macrophages.Hypoadiponectemia may play a regulatory role in preventing hypoglycaemia in this condition.
Author: Mirjam Langeveld
Language: English
Subfield: Genetics (clinical)
Field: Medicine
Domain: Health Sciences
Open Access status: green
OpenAlex ID: W1937022660