The study continues to evaluate immediate and long-term effects of continuous glucose feedings on the metabolic and hormonal state, and physical growth and development of patients with type one glycogen storage disease. The broad goals of this study are to test the hypothesis that optimal continuous glucose therapy of type 1 glycogen storage disease (GSD-1) provided by an appropriate diet supplemented with a regimen of intermittent feedings of uncooked cornstarch can promote normal physical growth and development, and will prevent or delay renal and hepatic complications of the disease. The study has previously shown that continuous glucose treatment improves growth in previously untreated GSD-1 patients, but catch-up growth was incomplete and predicted final adult height was significantly less than target height. The study has shown that night feeds of uncooked cornstarch does not impair nocturnal GH secretion. Careful long-term follow-up of this cohort of GSD-1 subjects, who were diagnosed early in life and have been intensively treated from an early age, will give the investigators the opportunity to test the hypothesis that maintenance of near normal biochemical control throughout childhood and adolescence results in normal growth and attainment of target or mid-parental height. The study to date, has preliminary data which suggests that micro- albunimuria, a sign of early renal glomerular damage, is significantly associated with delays in starting glucose therapy and with chronic hyperlactatemia. There is reason to hope that optimal continuous glucose therapy from infancy with maintenance of near to normal concentrations of blood lactate may delay or prevent renal dysfunction in GSD-1. All subjects will continue to be closely monitored for early signs of renal dysfunction (microalbuminuria, hypertension).

Project Start
Project End
Budget Start
Budget End
Support Year
Fiscal Year
Total Cost
Indirect Cost
Children's Hospital Boston
United States
Zip Code
Cassidy, Adam R; Bernstein, Jane Holmes; Bellinger, David C et al. (2018) Visual-spatial processing style is associated with psychopathology in adolescents with critical congenital heart disease. Clin Neuropsychol :1-19
Bean Jaworski, Jessica L; White, Matthew T; DeMaso, David R et al. (2018) Visuospatial processing in adolescents with critical congenital heart disease: Organization, integration, and implications for academic achievement. Child Neuropsychol 24:451-468
Hron, Bridget M; Ebbeling, Cara B; Feldman, Henry A et al. (2017) Hepatic, adipocyte, enteric and pancreatic hormones: response to dietary macronutrient composition and relationship with metabolism. Nutr Metab (Lond) 14:44
Rollins, Caitlin K; Asaro, Lisa A; Akhondi-Asl, Alireza et al. (2017) White Matter Volume Predicts Language Development in Congenital Heart Disease. J Pediatr 181:42-48.e2
Sakai Bizmark, Rie; Chang, Ruey-Kang R; Tsugawa, Yusuke et al. (2017) Impact of AHA's 2007 guideline change on incidence of infective endocarditis in infants and children. Am Heart J 189:110-119
Selamet Tierney, Elif Seda; Hollenbeck-Pringle, Danielle; Lee, Caroline K et al. (2017) Reproducibility of Left Ventricular Dimension Versus Area Versus Volume Measurements in Pediatric Patients With Dilated Cardiomyopathy. Circ Cardiovasc Imaging 10:
Kim, So Hyun; Joseph, Robert M; Frazier, Jean A et al. (2016) Predictive Validity of the Modified Checklist for Autism in Toddlers (M-CHAT) Born Very Preterm. J Pediatr 178:101-107.e2
Leviton, Alan; Allred, Elizabeth N; Fichorova, Raina N et al. (2016) Antecedents of inflammation biomarkers in preterm newborns on days 21 and 28. Acta Paediatr 105:274-80
Cousminer, Diana L; Widén, Elisabeth; Palmert, Mark R (2016) The genetics of pubertal timing in the general population: recent advances and evidence for sex-specificity. Curr Opin Endocrinol Diabetes Obes 23:57-65
Keerthy, Divya; Youk, Ada; Srinath, Arvind I et al. (2016) Effect of Psychotherapy on Health Care Utilization in Children With Inflammatory Bowel Disease and Depression. J Pediatr Gastroenterol Nutr 63:658-664

Showing the most recent 10 out of 463 publications