title: Defective growth hormone (GH) secretion and short-term treatment in Noonan syndrome.
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-I) 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.
authors.affiliation: Department of Pediatrics, Royal Hospital, Muscat, Oman. zalabani@cns.sisnet.net
authors.forename: A T
authors.initials: AT
authors.lastname: Soliman
authors.forename: A
authors.initials: A
authors.lastname: Rajab
authors.forename: M
authors.initials: M
authors.lastname: el Zalabany
authors.forename: I
authors.initials: I
authors.lastname: alSalmi
authors.forename: M A
authors.initials: MA
authors.lastname: Fattah
mesh_terms: D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004393:Dwarfism, Pituitary / Q000097:blood / Q000175:diagnosis; D005260:Female; D019382:Human Growth Hormone / Q000172:deficiency*; D006801:Humans; D008297:Male; D009634:Noonan Syndrome / Q000097:blood / Q000175:diagnosis*
chemical_list: D019382:Human Growth Hormone