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Behavioural management of Prader-Willi syndrome

Published online by Cambridge University Press:  13 June 2014

Pat Gibbons  and
Kathryn O'Donoghue
Kathryn O'Donoghue
Affiliation:
Warrenstown house, Blanchardstown, Dublin 15, Ireland.
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Abstract

Prader-Willi syndrome is a genetic illness associated with a defect on chromosome 15. It presents with hypotonicity in infancy followed by voracious appetite and gross obesity from early childhood and with a range of other clinical and behavioural sequelae. This case report is of an adolescent boy who was admitted to a psychiatric unit for an intensive behavioural programme to control his weight gain and deal with associated behavioural problems, and demonstrates that an inpatient programme can lead to sustainable improvements in weight control and other indicators of well-being in PWS patients.

Keywords

Prader-Willi syndromeHyperphagiaObesityBehavioural management.
Type
Case Reports
Information
Irish Journal of Psychological Medicine , Volume 15 , Issue 1 , March 1998 , pp. 32 - 34
Copyright
Copyright © Cambridge University Press 1998

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References

1.Donaldson, MD, Chu, CE, Cooke, A, Wilson, A, Greene, SA, Stephenson, JB. The Prader-Willi syndrome. Arch Dis Child 1994; 70: 58–63.CrossRefGoogle ScholarPubMed
2.Laurance, BM, Brito, A, Wilkinson, J. Prader-Willi syndrome after 15 years. Arch Dis Child 1981, 56: 181–6.CrossRefGoogle Scholar
3.Holm, VA, Cassidy, SB, Butler, MGet al.Prader-Willi syndrome: consensus diagnostic criteria. Paediatrics 1993; 70: 58–63.Google Scholar
4.O'Brien, G, Yule, W. Behavioural Phenotypes. Cambridge: Mac Keith Press, 1995: 155–8.Google Scholar
5.Young, ID. Diagnosing Prader-Willi syndrome. Lancet 1995; 345: 1590.CrossRefGoogle ScholarPubMed
6.Oscelik, T, Leff, S, Robinson, WLet al.Small nuclear ribonucleoprotein N (SNRPN), an expressed gene in the Prader-Willi syndrome critical region. Nature Genetics 2: 265–9.CrossRefGoogle Scholar
7.Hoffman, C, Aultman, D, Pipes, P. A nutrition survey of and recommendations for individuals with Prader-Willi syndrome who live in group homes. J Am Diet Assoc 1992; 92: 823–33.CrossRefGoogle ScholarPubMed
8.Lee, P, Wilson, D, Rountree, L, Hintz, R, Rosenfeld, R. Linear growth response to exogenous growth hormone in Prader-Willi syndrome. Am J Med Genet 1987; 28: 865–71.CrossRefGoogle ScholarPubMed
9.Holland, AJ, Treasure, J, Coskeran, Pet al.Characteristics of the eating disorder in Prader-Willi syndrome: implications for treatment. JIDR 1996; 39(5): 373–81.Google Scholar
10.Zlotkin, S, Fettes, I, Stallings, V. The effects of naltrexone, an oral b-endorphin antagonist, in children with Prader-Willi syndrome. J Clin Endocrin Metab 1986; 63: 1229–32.CrossRefGoogle Scholar
11.Holm, VA, Pipes, PL. Food and children with Prader-Willi syndrome. Am J Dis Child 1976; 130: 1063–7.Google ScholarPubMed
12.Greenswag, LR. Adults with Prader-Willi syndrome: a survey of 232 cases. New York: Springer-Verlag, 1987.Google ScholarPubMed