Treatment outcomes on growth of children diagnosed with glycogen storage disease presenting to national institute of child health, Karachi, Pakistan.

Abstract

Objective: To determine the treatment outcomes on growth of children diagnosed with glycogen storage disease (GSD) presenting to National Institute of Child Health (NICH), Karachi, Pakistan. Study Design: Descriptive Longitudinal study. Setting: Department of Hepatology and Gastroenterology, NICH, Karachi, Pakistan. Period: March 2021 to September 2022. Material & Methods: Children of either gender aged above 1 year diagnosed with GSD were enrolled. Demographic details and disease details of all children were included and necessary biochemical and laboratory investigations were assessed. Children were treated with uncooked corn starch (corn flour) and dietary restrictions were also advised. Growth outcomes in terms of height, weight and liver size were measured for the period of 6-months and compared with the baseline data. Results: In a total of 36 children with GSD, 20 (55.6%) were male. The mean age was 2.81±1.80 years (ranging between 1 to 6 years). At the time of presentation, abdominal distension and hepatomegaly were observed in 36 (100%) children each. Significant improvements were found in terms of height gain among children aged 1-3 years (p<0.0001) and 4-6 years (p=0.0039) after 6-months of treatment. Although, children between 1-3 years and 4-6 years gained weight after 6 months of treatment but the difference was insignificant (p>0.05). Reduction in liver size was observed among children between 1-3 years (p=0.0073) and 4-6 years (p=0.0376) after 6-months of treatment. Conclusion: Treatment with uncooked corn starch (corn flour) and dietary restriction resulted in significant improvement on the growth of children diagnosed with GSD presenting to National Institute of Child Health, Karachi, Pakistan.