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

Research output: Contribution to journalArticle

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 languageEnglish
Pages (from-to)149-152
Number of pages4
JournalClinical Pediatric Endocrinology
Volume3
DOIs
Publication statusPublished - 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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