|Author (year)||Study population||Main findings|
|Barton et al., 1989 ||25 untreated GD1 patients, both children and adults||REEa 44% higher than estimated REE|
|Corssmit et al., 1995 ||7 untreated, adult GD1 patients||REEa 24% greater than in age-, sex- and weight-matched healthy subjects|
|Doneda et al., 2011 ||14 GD1 patients (12 on ERT, 2 untreated), both children and adults||BMR of patients 6.3% higher than expected REE.|
Observed BMR of patients on ERT 27.1% higher than in age-, sex- and BMI-matched controls.
No difference between the BMR of subjects with or without ERT
|Giraldo et al., 2016 ||108 GD1 patients, 99 adults, 9 children 88% were receiving ERT or SRT||Prevalence of underweight (BMI < 18.5) - 3% in all GD1 patients - 2% in treated with ERT/SRT, 9% in untreated.|
Prevalence of overweight and obesity - 29% in all GD1 patients - 27% in treated with ERT/SRT, 46% in untreated.
Higher incidence of overweight and obesity in splenectomised vs. non-splenectomised patients - 36 vs. 27%
|Grigorescu Sido et al., 2007 ||24 GD1 patients, both children and adult, evaluated before and during ERT for at least 18 months.||A mean weight gain of 4.3 kg during 18 months of ERT|
|Heitner et al., 2004 ||9 paediatric GD1 patients, followed up for 2–10 years during ERT||An increase in weight during ERT was observed with an average weight gain of 3.9 kg per year|
|Hollak et al., 1997 ||12 adult GD1 patients, evaluated before and after 6 months of ERT||Pre-ERT REEa 25% greater than in age-, and weight-matched healthy subjects.|
Decrease of REEa after 6 months of ERT from 129 to 120% of expected values.
Appearance of increase in weight (mean 1.7 +/− 0.8 kg) after 6 months of ERT; mainly a significant increase in fat mass (mean 1.6 +/− 1.5 kg)
|Langeveld et al., 2008 ||42 adult GD1 patients (7 untreated, 35|
|Initial overweight incidence||14%||16%|
|Weight gain||3.8 kg or 2.2 kg/m2 during 8.2 year-follow up||6 kg or 2.4 kg/m2 during 11 years of ERT|
|Final overweight incidence||57%||56%|
|Data presented as median values|
|Weight changes in untreated GD1 subjects similar to the controls and to treated GD1 patients.|
|Oliveira et al., 2002 ||13 paediatric GD1 patients, 77% treated with ERT||Pre-ERT malnutrition rate - 26% .|
Post-ERT (after a median therapy of 5.3 years) malnutrition rate increased to 48%