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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 populationMain findings
Corssmit et al., 1995 [18]7 untreated, adult GD1 patientsHepatil 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 patientsElevated 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 ERTInitial 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)parameteruntreatedtreated
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 ERTIR 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
  1. BMI body mass index; DM II type 2 diabetes mellitus; GD1 Gaucher disease type 1; HOMA IR homeostasis model assessment of insulin resistance; IMGU insulin-mediated glucose uptake; IR insulin resistance; NIMGU non-insulin-mediated glucose uptake
  2. a hepatic glucose production was measured by infusing radio-labelled 3-3H glucose
  3. b assessed when HOMA-IR > 4.65