Table 2 Studies assessing carbohydrate metabolism disorders in adult GD1 patients
From: Endocrine and metabolic disorders in patients with Gaucher disease type 1: a review
Author (year) | Study population | Main findings | ||
|---|---|---|---|---|
Corssmit et al., 1995 [18] | 7 untreated, adult GD1 patients | Hepatil glucose productiona increased by 30% in GD patients vs. age-, sex- and weight-matched controls. Increased glucose production not related to increased plasma concentrations of glucose or glucoregulatory hormones. | ||
Ghauharali-van der Vlugt, 2008 [29] | 40 untreated, adult GD1 patients | Elevated plasma GM3 concentrations in most GD1 subjects. GM3 plasma levels correlated with plasma chitotriosidase activity overall severity of disease and hepatomegaly. | ||
Hollak et al., 1997 [17] | 7 GD1 adult patients, evaluated before and after 6 months of ERT | Initial hepatil glucose productiona increased by 23% in GD patients vs. age-, and weight-matched controls. No differences in plasma glucose concentrations in GD patients vs. controls. Persistent increase in glucose production after 6 months of ERT | ||
Langeveld et al., 2008 [16] | 42 adult GD1 patients (7 untreated, 35 on ERT) | parameter | untreated | treated |
Fasting glucose Fasting insulin IR prevalenceb DM II prevalence | 5.2 mmol/l 53 pmol/l 0% 0% | 4.9 mmol/l 50 pmol/l 6% 8.2% | ||
Data presented as median values | ||||
Langeveld et al., 2008 [35] | 6 adult GD1 patients (3 untreated, 3 treated with ERT) | Peripheral insulin resistance in GD1 subjects compared with age-, sex- and BMI-matched controls. Comparable NIMGU at euglycaemia and hyperglycaemia in patients and control subjects. Lower IMGU in patients vs. controls. Tendency toward less effective suppression of lipolysis by cinsulin in patients vs. controls | ||
Ucar et al., 2009 [38] | 14 non-overweight, adult GD1 patients on ERT | IR incidenceb 6.6%. IR in GD1 is not related to overweight. Higher basal insulin, glucose and insulin at 2 h after load and HOMA-IR in GD vs. healthy subjects | ||
Zimmermann et al., 2015 [39] | 12 treatment-naive, non-overweight, adult GD1 patients; assessed before and every 6 months up to 3 years under ERT | No differences in baseline glucose parameters between GD1 subjects and age-, sex- and BMI-matched controls. Significant increase in HOMA-IR under ERT after 18 months (+  61.2%). Higher levels of HOMA-IR, basal insulin and glucose after 3 years of ERT vs. controls | ||