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 Pediatric Endocrinology & Metabolism 제26권 제4호
- 발행연도
- 2021.12
- 수록면
- 227 - 236 (10page)
- DOI
- 10.6065/apem.2142164.082
이용수
초록· 키워드
오류제보하기
Prader-Willi syndrome (PWS) is a rare complex genetic disorder that results from a lack of expression of the paternally inherited chromosome 15q11-q13. PWS is characterized by hypotonia and feeding difficulty in early infancy and development of morbid obesity aggravated by uncontrolled hyperphagia after childhood and adolescent. Dysmorphic facial features, delayed motor and language development, various degrees of cognitive impairment, and behavioral problems are common in PWS. Without early, intensive nutritional therapy along with behavioral modification, PWS patients develop severe obesity associated with type 2 diabetes, obstructive sleep apnea, right-side heart failure, and other obesity-related metabolic complications. Hypothalamic dysfunction in PWS can lead to several endocrine disorders, including short stature with growth hormone deficiency, hypothyroidism, central adrenal insufficiency, and hypogonadism. In this review, we discuss the natural history of PWS and the mechanisms of hyperphagia and obesity. We also provide an update on obesity treatments and recommendations for screening and monitoring of various endocrine problems that can occur in PWS.
목차
등록된 정보가 없습니다.
참고문헌 (96)
참고문헌 신청
Cassidy SB / 2012 / Prader-Willi syndrome / Genet Med 14:10~26
전종근 / 2016 / Genetics of Prader-Willi syndrome and Prader-Will-Like syndrome / Annals of Pediatric Endocrinology & Metabolism 21(3):126~135
Butler MG / 2019 / Prader-Willi syndrome-clinical genetics, diagnosis and treatment approaches : an update / Curr Pediatr Rev 15:207~244
Irizarry KA / 2016 / Prader Willi syndrome : genetics, metabolomics, hormonal function, and new approaches to therapy / Adv Pediatr 63:47~77
Rocha CF / 2014 / Prader-Willi-like phenotypes : a systematic review of their chromosomal abnormalities / Genet Mol Res 13:2290~2298
전종근 / 2016 / Genetics of Prader-Willi syndrome and Prader-Will-Like syndrome / Annals of Pediatric Endocrinology & Metabolism 21(3):126~135
Butler MG / 2019 / Prader-Willi syndrome-clinical genetics, diagnosis and treatment approaches : an update / Curr Pediatr Rev 15:207~244
Irizarry KA / 2016 / Prader Willi syndrome : genetics, metabolomics, hormonal function, and new approaches to therapy / Adv Pediatr 63:47~77
Rocha CF / 2014 / Prader-Willi-like phenotypes : a systematic review of their chromosomal abnormalities / Genet Mol Res 13:2290~2298
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