Growth factor deficiency in patients with ataxia telangiectasia.
One prominent feature of patients with the autosomal recessive disease ataxia telangiectasia (AT) is somatic growth retardation. Due to their essential roles in development we examined levels of insulin-like growth factor-I (IGF-I) as well as its main binding protein (IGFBP-3) in a group of AT patients. Growth status of 19 patients was assessed by body mass index (BMI) and nutritional protocols. As suspected, BMI was low in AT patients despite adequate nutrition. Serum levels of IGF-I were found to be below the 3rd percentile in 9 (56%) out of 16 patients and of IGFBP-3 in 13 (81%) out of 16 patients. Our observations demonstrate that IGF-I and IGFBP-3 levels reflect the impaired growth status in patients with AT.
Schubert
R
R
Department of Paediatrics, Johann Wolfgang Goethe-University, Frankfurt am Main, Germany. ralf.schubert@kgu.de
Reichenbach
J
J
Zielen
S
S
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001260:Ataxia Telangiectasia / Q000097:blood* / Q000503:physiopathology; D015992:Body Mass Index; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D018972:Insulin-Like Growth Factor Binding Protein 3 / Q000097:blood; D007334:Insulin-Like Growth Factor I / Q000032:analysis / Q000172:deficiency*; D008297:Male; D009752:Nutritional Status; D012737:Sex Factors
D018972:Insulin-Like Growth Factor Binding Protein 3; D007334:Insulin-Like Growth Factor I