|Author (year)||Study population||Main findings|
|Corssmit et al., 1995 ||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 ||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 ||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 ||42 adult GD1 patients (7 untreated, 35 on ERT)||parameter||untreated||treated|
DM II prevalence
|Data presented as median values|
|Langeveld et al., 2008 ||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 ||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 ||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