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

doi:10.1297/cpe.3.Supple4_149

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

Medicine

Research output: Contribution to journal › Article

Abstract

A boy who was diagnosed at 8 months of age as suffering from glycogen storage disease (GSD) type la, 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 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 la.

Original language	English
Pages (from-to)	149-152
Number of pages	4
Journal	Clinical Pediatric Endocrinology
Volume	3
DOIs	https://doi.org/10.1297/cpe.3.Supple4_149
Publication status	Published - 1994 Jan 1

Keywords

focal fatty liver
glycogen storage disease type la
parenteral hyperalimentation
short stature

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Endocrinology, Diabetes and Metabolism
Endocrinology

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Asanuma, A., Abukawa, D., Igarashi, Y., Aikawa, J. I., Ohba, M., & Fujiwara, I. (1994). Effect of Parenteral Hyperalimentation on Growth in Glycogen Storage Disease Type IA. Clinical Pediatric Endocrinology, 3, 149-152. https://doi.org/10.1297/cpe.3.Supple4_149

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