Review of Prader-Willi syndrome: the endocrine approach

Prader-Willi syndrome (PWS) is a complex genetic disorder with implications on the endocrine and neurologic systems, metabolism, and behavior. Early in life, PWS is characterized by hypotonia and failure to thrive, followed by obesity and hyperphagia. Patients with PWS develop hypothalamic dysfuncti...

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Bibliographic Details
Published in:	Translational pediatrics Vol. 6; no. 4; pp. 274 - 285
Main Authors:	Heksch, Ryan, Kamboj, Manmohan, Anglin, Kathryn, Obrynba, Kathryn
Format:	Journal Article
Language:	English
Published:	China AME Publishing Company 01-10-2017
Subjects:	Review review Endocrinology Prader-Willi syndrome (PWS)
Online Access:	Get full text
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Summary:	Prader-Willi syndrome (PWS) is a complex genetic disorder with implications on the endocrine and neurologic systems, metabolism, and behavior. Early in life, PWS is characterized by hypotonia and failure to thrive, followed by obesity and hyperphagia. Patients with PWS develop hypothalamic dysfunction which may lead growth hormone deficiency (GHD), hypogonadism, hypothyroidism, adrenal insufficiency, and poor bone mineral density (BMD). In addition to hypothalamic dysfunction, individuals with PWS have increased risk for obesity which may be complicated by metabolic syndrome and type 2 diabetes mellitus (T2DM). In this paper, we will review the current literature pertaining to the endocrine concerns of PWS and current recommendations for screening and management of these conditions.
Bibliography:	ObjectType-Article-2 SourceType-Scholarly Journals-1 ObjectType-Feature-3 content type line 23 ObjectType-Review-1 Contributions: (I) Conception and design: All authors; (II) Administrative support: K Anglin; (III) Provision of study materials or patients: None; (IV) Collection and assembly of data: R Heksch, M Kamboj, K Obrynba; (V) Data analysis and interpretation: None; (VI) Manuscript writing: All authors; (VII) Final approval of manuscript: All authors.
ISSN:	2224-4344 2224-4336 2224-4344
DOI:	10.21037/tp.2017.09.04