잠시만 기다려 주세요. 로딩중입니다.

Effectiveness of growth hormone therapy in children with Noonan syndrome

Annals of Pediatric Endocrinology & Metabolism 2020년 25권 3호 p.182 ~ 186
석은미, 박홍규, 노정기, 금창대, 이해상, 황진순,
소속 상세정보
석은미 ( Seok Eun-Mi ) - Ajou University School of Medicine Ajou University Hospital Department of Pediatrics
박홍규 ( Park Hong-Kyu ) - Gyeongsang National University Changwon Hospital Department of Pediatrics
노정기 ( Rho Jung-Gi ) - Ajou University School of Medicine Ajou University Hospital Department of Pediatrics
금창대 ( Kum Change-Dae ) - Ajou University School of Medicine Ajou University Hospital Department of Pediatrics
이해상 ( Lee Hae-Sang ) - Ajou University School of Medicine Ajou University Hospital Department of Pediatrics
황진순 ( Hwang Jin-Soon ) - Ajou University School of Medicine Ajou University Hospital Department of Pediatrics

Abstract


Purpose: Recombinant human growth hormone (rhGH) has been used to improve growth in children with Noonan syndrome (NS). This study aimed to investigate the efficacy of rhGH therapy in Korean children with NS.

Methods: Seventeen prepubertal children (10 boys, 7 girls) with NS who received rhGH therapy for at least 3 years between 2008 and 2017 were included. To compare the response, age- and sex-matched children with GH deficiency (GHD; n=31) were included. Height and growth velocity before and during treatment were analyzed.

Results: The mean age of NS patients was 6.34±2.32 years. After treatment, the height standard deviation score (SDS) increased from -2.93±0.81 to -1.51±1.00 in patients with NS and from -2.45±0.42 to -1.09±0.47 in patients with GHD. There were no significant differences in growth velocity or change in height SDS between patients with NS and GHD. Growth velocity in the first year of treatment was higher in patients with PTPN11 mutations than those without PTPN11 mutations, but the change in height SDS was not significantly different between those 2 groups.

Conclusions: rhGH therapy can increase linear growth in prepubertal children with NS. The growth response between patients with NS and patients with GHD was not significantly different. Furthermore, we observed that lower doses of growth hormone have a similar effect on height compared to previous studies in patients with NS. Our study indicates that rhGH treatment is useful for growth promotion.

키워드

Noonan syndrome; Growth hormone; Efficacy

원문 및 링크아웃 정보

등재저널 정보

KCI
KoreaMed
KAMS