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

• Asanuma Aki

  Department of Pediatrics, Tohoku University School of Medicine

• Abukawa Daiki

  Department of Pediatrics, Tohoku University School of Medicine

• Igarashi Yutaka

  Department of Pediatrics, Tohoku University School of Medicine

• Aikawa Jun-ichirou

  Department of Pediatrics, Tohoku University School of Medicine

• Ohba Minako

  Department of Pediatrics, Tohoku University School of Medicine

• Fujiwara Ikuma

  Department of Pediatrics, Tohoku University School of Medicine

抄録

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 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.

収録刊行物

• Ｃｌｉｎｉｃａｌ　Ｐｅｄｉａｔｒｉｃ　Ｅｎｄｏｃｒｉｎｏｌｏｇｙ

  Ｃｌｉｎｉｃａｌ　Ｐｅｄｉａｔｒｉｃ　Ｅｎｄｏｃｒｉｎｏｌｏｇｙ 3 (Supple4), 149-152, 1994

  日本小児内分泌学会

キーワード

• glycogen storage disease type Ia
• parenteral hyperalimentation
• short stature
• focal fatty liver

詳細情報

• CRID: 1390282679460114432
• NII論文ID: 130004237982
• DOI: 10.1297/cpe.3.supple4_149
• ISSN: 13477358
• ISSN: 09185739
• Web Site: http://www.jstage.jst.go.jp/article/cpe1992/3/Supple4/3_Supple4_149/_pdf
• 本文言語コード: en
• データソース種別:
  • JaLC
  • Crossref
  • CiNii Articles
• 抄録ライセンスフラグ: 使用不可