Growth and factors affecting it in thalassemia major.
In our thalassemic (T) cohort, 45% of them had height standard deviation score (HtSDS) less than -2 and 56% of them had growth velocity standard deviation score (GVSDS) less than -1. Their mid-arm circumference and triceps skin-fold thickness were decreased versus normal controls. Their circulating insulin-like growth factor-I (IGF-I) concentrations were significantly lower than normal children. Growth hormone (GH) response to provocation with clonidine and glucagon was defective in half of the short T children (peak GH < 7 ng/dL). Some of the short T children, with normal GH response to provocation, had defective spontaneous nocturnal GH secretion. IGF-I generation after one GH injection was reduced in T children than those with GH deficiency (GHD) and constitutional delay of growth and puberty (CDGP). GH therapy for a year significantly increased IGF-I concentrations, GV, and HtSDS in T children but to a lower level compared to those with GHD or CDGP, suggesting partial GH insensitivity. Pubertal induction with human chorionic gonadotropin in T adolescents was associated with increased IGF-I concentrations, GV, and HtSDS.
Soliman
Ashraf T
AT
Department of Pediatrics, Hamad Medical Center, Doha, Qatar. atsoliman@yahoo.com
Khalafallah
Hany
H
Ashour
Rasha
R
D000293:Adolescent; D049628:Body Size; D002648:Child; D019382:Human Growth Hormone / Q000097:blood; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000032:analysis; D012885:Skinfold Thickness; D017086:beta-Thalassemia / Q000503:physiopathology*
D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I