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 › peer-review

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

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

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1297/cpe.3.Supple4_149

Cite this

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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.",

keywords = "focal fatty liver, glycogen storage disease type la, parenteral hyperalimentation, short stature",

author = "Aki Asanuma and Daiki Abukawa and Yutaka Igarashi and Aikawa, {Jun Ichirou} and Minako Ohba and Ikuma Fujiwara",

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T1 - Effect of Parenteral Hyperalimentation on Growth in Glycogen Storage Disease Type IA

AU - Asanuma, Aki

AU - Abukawa, Daiki

AU - Igarashi, Yutaka

AU - Aikawa, Jun Ichirou

AU - Ohba, Minako

AU - Fujiwara, Ikuma

PY - 1994

Y1 - 1994

N2 - 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.

AB - 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.

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KW - glycogen storage disease type la

KW - parenteral hyperalimentation

KW - short stature

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

Abstract

Keywords

ASJC Scopus subject areas

UN SDGs

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