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 제17권 제3호
- 발행연도
- 2012.1
- 수록면
- 145 - 152 (8page)
이용수
초록· 키워드
오류제보하기
Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder characterized by hypothalamic-pituitary dysfunction. The main clinical features consist of neonatal hypotonia, distinctive facial features, delayed overall development with mental deficiency, behavioral abnormalities, poor growth in infancy followed by overeating with severe obesity, short stature, and hypogonadism. Recently, patients with PWS have been diagnosed at an earlier age, especially in the neonatal period. In addition,early interventions such as commencement of growth hormone therapy and dietary programs, have received attention in PWS treatment. Since early diagnosis is now possible based on both clinical symptoms and signs and on molecular genetic criteria,early dietary intervention and early growth hormone therapy during the first two years may improve neurodevelopment, increase muscle mass, and reduce obesity. Our aim in this review is to document the characteristics of infants with PWS and to provide a recent update regarding early management.
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등록된 정보가 없습니다.
참고문헌 (23)
참고문헌 신청
Angulo MA / 2007 / Final adult height in children with Prader-Willi syndrome with and without human growth hormone treatment / Am J Med Genet A 143A : 1456 ~ 1461
Bachere N / 2008 / Early diagnosis and multidisciplinary care reduce the hospitalization time and duration of tube feeding and prevent early obesity in PWS infants / Horm Re
Burman P / 2001 / Endocrine dysfunction in Prader-Willi syndrome: a review with special reference to GH / Endocr Rev 22 : 787 ~ 799
Butler MG / 1991 / Standards for selected anthropometric measurements in Prader-Willi syndrome / Pediatrics 88 : 853 ~ 860
Carrel AL / 2002 / Benefits of long-term GH therapy in Prader-Willi syndrome: a 4-year study / J Clin Endocrinol Metab 87 : 1581 ~ 1585
Bachere N / 2008 / Early diagnosis and multidisciplinary care reduce the hospitalization time and duration of tube feeding and prevent early obesity in PWS infants / Horm Re
Burman P / 2001 / Endocrine dysfunction in Prader-Willi syndrome: a review with special reference to GH / Endocr Rev 22 : 787 ~ 799
Butler MG / 1991 / Standards for selected anthropometric measurements in Prader-Willi syndrome / Pediatrics 88 : 853 ~ 860
Carrel AL / 2002 / Benefits of long-term GH therapy in Prader-Willi syndrome: a 4-year study / J Clin Endocrinol Metab 87 : 1581 ~ 1585
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