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- Title
Endocrine management of children with Prader-Willi syndrome.
- Authors
Borschiver Medeiros, Clarice; Bordallo, Ana Paula; Moutinho Souza, Flavio; Ferrez Collett-Solberg, Paulo
- Abstract
Prader-Willi syndrome is a rare genetic condition afflicting nearly 1/15,000 live births. Clinical features include neonatal hypotonia, poor weight gain in early infancy followed by binge eating from childhood to adulthood, severe obesity, developmental delay, short stature, and hypogonadism of both central and peripheral etiology. Central hypothyroidism and adrenal insufficiency may occur. Sleep disordered breathing, by obstruction of upper airways associated with central hypoventilation, is a common feature. Most of these characteristics are assumed to be the result of a hypothalamic dysfunction. The most important complication and the most difficult to manage is the obesity. This review aims at discussing the most recent strategies to manage the endocrine complications of Prader-Willi syndrome patients, with a special approach on the treatment of obesity, hypogonadism, and short stature. We summarize the indication and effects of recombinant human growth hormone therapy on growth, cognitive development, and body composition, and discuss the effects of recombinant human growth hormone therapy on the resulting sleep disorders.
- Subjects
PRADER-Willi syndrome; HUMAN chromosome 15 abnormalities; INTELLECTUAL disabilities; HYPOGONADISM; KLINEFELTER'S syndrome; THERAPEUTICS
- Publication
Pediatric Health, Medicine & Therapeutics, 2013, Vol 4, p117
- ISSN
1179-9927
- Publication type
Article
- DOI
10.2147/PHMT.S32459