Effect of Parenteral Hyperalimentation on Growth in Glycogen Storage Disease Type IA
スポンサーリンク
概要
- 論文の詳細を見る
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.
- 日本小児内分泌学会の論文
日本小児内分泌学会 | 論文
- 43 Common origin of the Q258X mutation in StAR gene responsible for congenital aderenal lipoid hyperplasia in Japanese
- Recent Change in the Annual Incidence of Childhood Type 2 Diabetes in the Tokyo Metropolitan Area
- A Case of Acute Lymphoblastic Leukemia with Prader-Willi Syndrome during Growth Hormone Therapy
- Characteristics of a Fulminant Onset Form of Idiopathic Type 1 Diabetes Mellitus in Japanese Children
- Different Skeletal Phenotypes in a Mother and Two Daughters with Short Stature Homeobox-Containing Haploinsufficiency