Abstract
Auxological and endocrine data from 12 prepubertal children (3 males, 9 females) with Noonan syndrome (NS) were compared with those of 15 children with constitutional short stature (CSS), 20 children with partial GH deficiency (GHD), and 6 children with Turner syndrome (TS). Four children With NS were treated with human growth hormone (hGH) (n=4) (25 units/m2 week, divided on daily s.c. doses). In children with NS, the peak serum GH response to clonidine (5.4 ± 2.7 ug/L) and glucagon (7.4 ± 3.4 ug/L) were significantly lower than those for children with CSS (14.8 ±3.4 and 12.8 ± 2.8 ug/L respectively). Nine out of the 12 (75%) children with NS did not mount normal GH peak (10 ug/L or more) after provocation. The 12-h integrated GH secretion in the 3 children With NS who had normal GH response to provocation (2.7 ± 0.7 ug/L) was markedly lower compared to that for children with CSS (6.7 ±1.2 ug/L). The serum insulin-like growth factor-1 (IGF-l) concentrations were lower in children with NS (67 ± 32 ng/ml) vs CSS (165 ±35 ng/ml), but not different from those for GHD children (59 ± 33 ng/ml). In 4 children with NS, hGH therapy for a year increased height growth velocity from 4.1 ± 0.3 cm/yr to 7.4 ±0.6 cm/yr and height standard deviation score (Ht SDS) from -2.2 ± 0.6 to -1.45 ±0.3. This growth acceleration was accompanied by an increase in IGF-I concentration (from 52 ±21 ng/ml to 89 ± 25 ng/ml). In summary, these results prove a defect of the GH secretion in children with NS and suggest that GH therapy has an important role in the management of their short stature.
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Soliman, A.T., Rajab, A., El Zalabany, M. et al. Defective Growth Hormone (GH) secretion and short-term treatment in Noonan syndrome. Indian J Pediatr 65, 741–749 (1998). https://doi.org/10.1007/BF02731057
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DOI: https://doi.org/10.1007/BF02731057