Body composition measurements using bioelectrical impedance analysis (BIA) in pediatric patients with hepatic glycogen storage disease – Preliminary data

Summary Background Patients with hepatic glycogen storage diseases (GSDs) are prone to accumulation of an excessive body fat due to dietary therapy with frequent meals and addition of uncooked cornstarch. The aim of this study was to estimate body composition, in particular body fat in Polish pediatric patients with GSDs using BIA. Patients and methods Twenty four patients with hepatic GSDs: I, III/VI/IX,0: 9,14,1, respectively, aged 8.5 years (SD = 2.8) were included into the study. The average fasting time preceding BIA was 2.7 h (SD = 0.9). Patient was weighed, then height, age, gender, weight, and round the waist and hips were entered into computer. Results Median weight was (median [interquartile range]) 25.3 kg [13.7; 60.5] and median height was 119 cm [89; 157]. An average BMI was 18.8 (SD = 3.4) and 81.7 (SD = 3.4) percentile. An average round the waist and hips were 65.4 (SD = 13.3) and 67.2 (SD = 15.0), respectively, and average waist/hips ratio was 0.99 (SD = 0,2). An average body fat percentage was 36.7% (SD = 5.7) and 11.6 kg (SD = 5.7). An average lean body mass (bm) was 17,0 kg (SD = 8.8) and dry lean bm 4.7 kg (SD = 2.2). An average body water percentage was 48.6% (SD = 4.2) and 11.9 kg (SD = 6.5). Conclusion Polish patients with GSD have excessive body fat percentage which may be due to a very high calorie diet/excessive cornstarch intake. It may also be affected by their low hydration levels.