지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 저널정보
- 대한소아내분비학회 Annals of Pediatirc Endocrinology & Metabolism Annals of Pediatirc Endocrinology & Metabolism 제21권 제1호
- 발행연도
- 2016.1
- 수록면
- 26 - 30 (5page)
이용수
초록· 키워드
오류제보하기
Purpose: Noonan syndrome (NS) is characterized by short stature, heart anomalies, developmental delays, dysmorphic features, cryptorchidism, and coagulation defects. Several studies reported the short-term effects of recombinant human growth hormone (rhGH) treatment on the improvement of height. This study was performed to evaluate the long-term efficacy of rhGH in children with NS in Korea.
Methods: This study included 15 prepubertal NS children who received rhGH subcutaneously at a dose of 50–75 μg/kg/day for 6 days a week for at least >3 years. Preand posttreatment data, such as height, weight, bone age, insulin-like growth factor 1 (IGF-1), and IGF binding protein 3 (IGFBP-3) levels, were collected every 6 months.
Results: Chronologic age and bone age at the start of treatment were 7.97±1.81 and 5.09±2.12 years, respectively. Height standard deviation score (SDS) was increased from –2.64±0.64 to –1.54±1.24 years after 3 years (P<0.001). Serum IGF-1 SDS levels were elevated from –1.28±1.03 to –0.10±0.94 (P<0.001). Height SDS was more increased in subjects without PTPN11 mutations compared to those with mutations after 3 years (P=0.012). However, the other parameters, including bone age, IGF-1 SDS, and IGFBP-3 SDS, were not significantly different between patients with and without PTPN11 mutations.
Conclusion: Although this study included a relatively small number of patients, long-term rhGH therapy in NS patients was safe and effective at improving height, growth velocity, and serum IGF-1 levels, in accordance with previous studies.
However, the meticulous monitoring of potential adverse events is still needed because of high dose of rhGH and preexisting hyperactivity of RAS-MAPK pathway.
Patients with PTPN11 mutations demonstrated a decreased response to rhGH therapy compared to those without mutations.
목차
등록된 정보가 없습니다.
참고문헌 (30)
참고문헌 신청
Mendez HM / 1985 / Noonan syndrome: a review / Am J Med Genet 21:493~506
Sharland M / 1992 / A clinical study of Noonan syndrome / Arch Dis Child 67:178~183
Tartaglia M / 2001 / Mutations in PTPN11, encoding the protein tyrosine phosphatase SHP-2, cause Noonan syndrome / Nat Genet 29:465~468
Roberts AE / 2007 / Germline gain-of-function mutations in SOS1 cause Noonan syndrome / Nat Genet 39:70~74
Tartaglia M / 2007 / Gain-of-function SOS1 mutations cause a distinctive form of Noonan syndrome / Nat Genet 39:75~79
Sharland M / 1992 / A clinical study of Noonan syndrome / Arch Dis Child 67:178~183
Tartaglia M / 2001 / Mutations in PTPN11, encoding the protein tyrosine phosphatase SHP-2, cause Noonan syndrome / Nat Genet 29:465~468
Roberts AE / 2007 / Germline gain-of-function mutations in SOS1 cause Noonan syndrome / Nat Genet 39:70~74
Tartaglia M / 2007 / Gain-of-function SOS1 mutations cause a distinctive form of Noonan syndrome / Nat Genet 39:75~79
함께 읽어보면 좋을 논문
논문 유사도에 따라 DBpia 가 추천하는 논문입니다. 함께 보면 좋을 연관 논문을 확인해보세요!
-
Effectiveness of growth hormone therapy in children with Noonan syndrome
Annals of Pediatirc Endocrinology & Metabolism
2020 .01
-
Comparison of effectiveness of growth hormone therapy according to disease-causing genes in children with Noonan syndrome
Clinical and Experimental Pediatrics
2019 .01
-
Effect of -202 A/C IGFBP-3 polymorphisms on growth responses in children with idiopathic short stature
Annals of Pediatirc Endocrinology & Metabolism
2020 .01
-
Factors affecting height velocity in normal prepubertal children
Annals of Pediatirc Endocrinology & Metabolism
2018 .01
-
Relationship between growth velocity and change of levels of insulin-like growth factor-1, insulin-like growth factor binding protein-3 and, IGFBP-3 promoter polymorphism during GnRH agonist treatment
Annals of Pediatirc Endocrinology & Metabolism
2020 .01
-
Discriminatory performance of insulin-like growth factor 1 and insulin-like growth factor binding protein-3 by correlating values to chronological age, bone age, and pubertal status for diagnosis of isolated growth hormone deficiency
Annals of Pediatirc Endocrinology & Metabolism
2020 .01
-
Correlation of tri-ponderal mass index with insulin-like growth factor-I and insulin-like growth factor binding protein-3 in children and adolescents
Annals of Pediatirc Endocrinology & Metabolism
2024 .08
-
Factors influencing growth hormone therapy effect during the prepubertal period in small for gestational age children without catch-up growth
Annals of Pediatirc Endocrinology & Metabolism
2021 .01
-
Reference Ranges of Serum Insulin-Like Growth Factor-I and Insulin-Like Growth Factor Binding Protein-3: Results from a Multicenter Study in Healthy Korean Adults
Endocrinology and Metabolism
2020 .01
-
A case of Noonan syndrome diagnosed using the facial recognition software (FACE2GENE)
Journal of genetic medicine
2019 .01
-
An overview of growth hormone therapy in pediatric cases documented in the Kabi International Growth Study (Pfizer International Growth Database)
Annals of Pediatirc Endocrinology & Metabolism
2024 .02
-
Insulin-like growth factor (IGF)-I and IGF binding proteins axis in diabetes mellitus
Annals of Pediatirc Endocrinology & Metabolism
2015 .01
-
소아의 키 성장에 대한 성장호르몬의 치료효과
약학회지
2016 .01
-
Influences of Socioeconomic Status on Short Stature in Childhood
고신대학교 의과대학 학술지
2020 .01
-
Growth Hormone Therapy in Adults with Prader-Willi Syndrome
Journal of mucopolysaccharidosis and rare disease
2015 .01
-
The Serum Level of Insulin Growth Factor-1 and Insulin Growth Factor Binding Protein-3 in Children with Henoch-Schönlein Purpura
Childhood Kidney Diseases
2016 .01
-
Effects of long-term growth hormone therapy in a girl with Floating-Harbor syndrome
Annals of Pediatirc Endocrinology & Metabolism
2020 .01
-
Changes in Insulin-like Growth Factor-1 Level in Patients with Sepsis and Septic Shock
Acute and Critical Care
2016 .01
-
The effects of growth hormone treatment on height in short children
Annals of Pediatirc Endocrinology & Metabolism
2022 .03
이 논문의 저자 정보
최근 본 자료
댓글(0)
0