Clinical Characteristics and Growth Responses to GH Therapy in Children with Noonan Syndrome.

Hwang,  Il Tae; Shin,  Choong Ho; Yang,  Sei Won

Ann Pediatr Endocrinol Metab > Volume 8(2); 2003 > Article

Original Article

Journal of Korean Society of Pediatric Endocrinology 2003;8(2): 149-155.

Clinical Characteristics and Growth Responses to GH Therapy in Children with Noonan Syndrome.

Il Tae Hwang, Choong Ho Shin, Sei Won Yang

Abstract

PURPOSE
Noonan syndrome(NS) is characterized by short stature, congenital heart disease, and typical facies. Recombinant human growth hormone(GH) has been reported to improve growth rate in a similar fashion to that seen in Turner syndrome. We investigated the clinical characteristics and growth reponses to GH therapy in children with NS. METHODS:The cases of sixty seven patients with NS were reviewed retrospectively. Ten of the 65 patients were assessed height, weight and pubertal stage every 3 months during GH therapy.
RESULTS
Webbed neck(70%), delayed development(59.7%), low set posterior hairline(56.7%), eye abnormalities(56.7%) and mental retardation(55.2%) were the leading clinical characteristics. Short stature below the 3rd percentile was presented in 73.8 %. Growth patterns in NS children were variable and the evaluation of their growth must be individualized. The increments of height SDS were significant in children with GH therapy(height SDS:from -2.8+/-.6 to -2.3+/-.9, growth velocity:from 4.4+/-.8 cm to 9.2+/-.9 cm during first year, and 6.1+/-.1 cm during second year) (P<0.05).
CONCLUSION
This study characterized the clinical profiles in Korean children with NS, which should be further extended with more children with NS. Additionally, the significant increase in final adult height after GH therapy in children with NS should be observed.

Keywords: Noonan syndrome;Growth hormone therapy