Long-term effects of growth hormone therapy on patients with Prader-Willi syndrome.

作者: Ilkka Sipilä , Harri Sintonen , Heikki Hietanen , Marjo Apajasalo , Soili Alanne

DOI: 10.1111/J.1651-2227.2010.01904.X

关键词: Hormone therapyGrowth hormoneYoung adultGrowth hormone treatmentAdult heightMedicineQuality of lifeCumulative doseEndocrinologyHormoneInternal medicine

摘要: 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 syndrome (PWS): clinical assessment, laboratory tests, body composition analysis by dual energy X-ray absorptiometry, sleep polygraphy, health-related quality life assessed 16D. Results:  Only two had normal secretion at baseline. All were significantly shorter than their expected heights, but experienced catch-up during treatment. At follow-up, 13 reached adult heights markedly taller historical controls. The cumulative dose rhGH over correlated inversely total fat percentage (p = 0.033). However, remained severely obese 10 years. Sleep polygraphy was abnormal more half patients. Health-related substantially below that population. Conclusion:  Growth improved height subjects PWS when compared to data. reduction fat; nevertheless, obese.

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