Effect of Parenteral Hyperalimentation on Growth in Glycogen Storage Disease Type IA

Aki Asanuma, Daiki Abukawa, Yutaka Igarashi, Jun-ichirou Aikawa, Minako Ohba, Ikuma Fujiwara
1994 Clinical Pediatric Endocrinology  
A boy who was diagnosed at 8 months of age as suffering from glycogen storage disease (GSD) type Ia, was treated with parenteral hyperalimentation (PHA). He had previously been treated with frequent meals and uncooked cornstarch, but compliance was poor and physical development was delayed. Growth hormone secretion was normal, but somatomedin-C (Sm-C) levels were low. Multiple focal fatty changes were found in the liver at age 12 years, when home PHA (960kcal/day) was started. Soon thereafter
more » ... . Soon thereafter growth velocity increased markedly, and Sm-C levels became normal. The liver lesions also disappeared. Thus PHA was effective in the treatment of this patient with GSD Ia.
doi:10.1297/cpe.3.supple4_149 fatcat:xp2pkcpxinc3dmvquzyg3nrb4m