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Clinical Characteristics and Growth Responses to GH Therapy in Children with Noonan Syndrome / 대한소아내분비학회지
Journal of Korean Society of Pediatric Endocrinology ; : 149-155, 2003.
Artigo em Coreano | WPRIM | ID: wpr-138147
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 SDSfrom -2.8+/-.6 to -2.3+/-.9, growth velocityfrom 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.
Assuntos

Texto completo: DisponíveL Índice: WPRIM (Pacífico Ocidental) Assunto principal: Síndrome de Turner / Estudos Retrospectivos / Fácies / Cardiopatias Congênitas / Síndrome de Noonan Tipo de estudo: Estudo observacional Limite: Adulto / Criança / Humanos Idioma: Coreano Revista: Journal of Korean Society of Pediatric Endocrinology Ano de publicação: 2003 Tipo de documento: Artigo

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Texto completo: DisponíveL Índice: WPRIM (Pacífico Ocidental) Assunto principal: Síndrome de Turner / Estudos Retrospectivos / Fácies / Cardiopatias Congênitas / Síndrome de Noonan Tipo de estudo: Estudo observacional Limite: Adulto / Criança / Humanos Idioma: Coreano Revista: Journal of Korean Society of Pediatric Endocrinology Ano de publicação: 2003 Tipo de documento: Artigo