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Long-term effects of growth hormone therapy on patients with Prader-Willi syndrome.
MedLine Citation:
PMID:  20545932     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
AIM: To assess the effects of recombinant human growth hormone (rhGH) treatment in children with Prader-Willi syndrome.
DESIGN: A 1-year study and an observational follow-up visit 10 years later.
METHODS: In 20 patients with Prader-Willi syndrome (PWS): clinical assessment, laboratory tests, body composition analysis by dual energy X-ray absorptiometry, sleep polygraphy, health-related quality of life assessed by 16D.
RESULTS: Only two patients had normal growth hormone secretion at baseline. All patients were significantly shorter than their expected heights, but experienced catch-up growth during growth hormone treatment. At follow-up, 13 patients had reached adult heights and were markedly taller than historical controls. The cumulative dose of rhGH over 10 years correlated inversely with the total body fat percentage (p = 0.033). However, patients remained severely obese at 10 years. Sleep polygraphy was abnormal in more than half of the patients. Health-related quality of life of the patients remained substantially below that of normal population.
CONCLUSION: Growth hormone markedly improved adult height in subjects with PWS when compared to historical data. The cumulative dose of growth hormone correlated with reduction in body fat; nevertheless, patients remained severely obese.
Authors:
Ilkka Sipilä; Harri Sintonen; Heikki Hietanen; Marjo Apajasalo; Soili Alanne; Anna-Mari Viita; Eeva Leinonen
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-07-05
Journal Detail:
Title:  Acta paediatrica (Oslo, Norway : 1992)     Volume:  99     ISSN:  1651-2227     ISO Abbreviation:  Acta Paediatr.     Publication Date:  2010 Nov 
Date Detail:
Created Date:  2010-11-02     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9205968     Medline TA:  Acta Paediatr     Country:  Norway    
Other Details:
Languages:  eng     Pagination:  1712-8     Citation Subset:  IM    
Copyright Information:
© 2010 Eli Lilly and Company/Journal Compilation © 2010 Foundation Acta Paediatrica.
Affiliation:
Hospital for Children and Adolescents, Paediatrics, University of Helsinki, Helsinki, Finland. ilkka.sipila@fimnet.fi
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