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Noonan 증후군과 성장호르몬결핍증 환아에서 성장호르몬 치료 효과

Other Titles
 Efficacy of Growth Hormone Treatment in Patients with Noonan syndrome and Growth Hormone Deficiency 
 이 슬  ;  권아름  ;  채현욱  ;  김호성 
 Journal of Korean Society of Pediatric Endocrinology (대한소아내분비학회지), Vol.16(2) : 100-105, 2011 
Journal Title
Journal of Korean Society of Pediatric Endocrinology(대한소아내분비학회지)
Issue Date
Noonan syndrome ; Growth hormone ; Therapy ; Treatment outcome ; Safety
Purpose: Noonan syndrome (NS) is characterized by short stature, congenital heart defects, mild mental retardation,
and characteristic faces. We investigated the efficacy of growth hormone (GH) treatment and the adverse effect
compared to sex and age-matched patients with growth hormone deficiency (GHD).
Methods: We included patients whose Noonan scores were over 60, treated with GH in Severance Children’s
Hospital. We analyzed height and height velocity before and during GH treatment in 14 NS patients (0.81 ± 0.13
U/kg/wk) and also in 42 patients with sex- and age-matched GHD as a control group (0.78 ± 0.17 U/kg/wk) at
intervals of 3 months.
Results: At the start of GH treatment, mean age was 10.0 ± 2.4 years, and mean height was 123.3 ± 13.5 cm,
and the height SDS was -2.79 ± 0.85 in NS, while the mean age was 10.3 ± 2.6 years, mean height was 119.6
± 13.5 cm, and the height SDS was -3.43 ± 1.56 in GHD. Mean duration was 3.8 ± 2.1 years in NS and 4.9
± 2.4 years in GHD. Mean height SDS increased from -2.79 SDS to -1.94 SDS in NS (P = 0.007) and from -3.43
SDS to -1.82 SDS in GHD (P ˂ 0.0001). Growth velocity increased from 3.7 ± 1.2 cm/yr to 8.5 ± 2.5 cm/yr (P ˂ 0.0001) and 6.5 ± 2.9 cm/yr (P = 0.016) during the first and second years of GH treatment, respectively, in NS
and from 3.4 ± 1.5 cm/yr to 8.8 ± 2.3 cm/yr (P ˂ 0.0001) and 8.1 ± 3.2 cm/yr (P ˂ 0.0001) in GHD. No severe
adverse effects were observed during treatment.
Conclusion: GH treatment in the NS patients increased growth velocity significantly, especially during the 1st year
of treatment. GH treatment in NS is thought to be effective and relatively safe.
Files in This Item:
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Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아과학교실) > 1. Journal Papers
Yonsei Authors
Kwon, Ah Reum(권아름) ORCID logo https://orcid.org/0000-0002-9692-2135
Kim, Ho Seong(김호성) ORCID logo https://orcid.org/0000-0003-1135-099X
Chae, Hyun Wook(채현욱) ORCID logo https://orcid.org/0000-0001-5016-8539
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