Investigation of the effect of nutritional status on sleep quality and quality of life in glycogen storage patients with hepatic involvement

This study was planned and conducted to evaluate the effect of nutritional status on the quality of life and sleep of patients followed up with glycogen storage disease (GSD) with hepatic involvement. A total of 40 patients, 24 men (60.0%) and 16 women (40.0%) aged 10-18 years, treated with the diagnosis of hepatic GDH in Çukurova University, Department of Pediatric Metabolism and Nutrition were included in the study. The general characteristics, health information, nutritional habits, anthropometric measurements, food consumption records, biochemical parameters, quality of life (Child Quality of Life Scale-PedsQl) and sleep quality (Sleep Quality Scale and Sleep Variables Questionnaire-SQS-SVQ) of the participants were evaluated. 2.5% of the patients are followed up with GSD Type 0, 35% with Type 1, 25% with Type III, 7.5% with Type VI, 25% with Type IX and 5% with Type XI. 20.0% of patients take Glycosade, 35.0% take raw cornstarch; Total daily consumption of raw corn starch and Glycosade and consumption per body weight were 73.8±78.6 g and 2.2±2.1 g/kg, respectively. There was no significant difference between sleep quality and efficiency and consumption of corn starch and Glycosade (p>0.05). However, patients who did not consume corn starch and Glycosade or who consumed them less frequently had higher quality of life scores (p<0.05). The scores of patients with normal body weight, height and body mass index were found to be higher in all sub-headings of the quality of life scale answered by parents and children. The total sleep duration during the night was 500.8±103.25 minutes, there was no statistically significant difference between GDH types in terms of sleep duration, quality and efficiency (p>0.05).In order to improve the quality of life of patients with hepatic GSD, their nutritional status should be evaluated regularly and individual nutrition therapy programs should be developed. Keywords: Anthropometric measurements, nutritional status, GDH, sleep quality, quality of life.