Journal of the Chinese Medical Association · 2008 · 15 citations · 27 references
MedicineEndocrinologyPediatricsEndocrine PharmacotherapyObesity PreventionPediatric EndocrinologyChildhood ObesityGrowth DisordersGrowth HormoneGrowth Hormone TherapyWeight ManagementGrowth Hormone Replacement TherapyGrowth Outcomes In ChildrenPrader-willi Syndrome TreatmentRetrospective Case Study
BACKGROUND: Information regarding the efficacy of growth hormone (GH) therapy in Asian Prader-Willi syndrome (PWS) patients is lacking. We report our experience with GH treatment in children with PWS in Taiwan. METHODS: Forty-six PWS patients (27 males, 19 females; age range, 1 year 4 months to 13 years 7 months) who received and/or who are currently receiving GH treatment (0.1 IU/kg/day subcutaneously) for a period from 1 year to 3 years were retrospectively analyzed. We evaluated height, weight, body mass index (BMI) and Rohrer index, before and after GH treatment. RESULTS: After patients had received GH for 1, 2 and 3 years, a significant improvement in mean height standard deviation score (SDS) was noted from -1.24 to -0.31 (p < 0.01), 0.00 (p < 0.001) and -0.26 (p < 0.001), respectively. Mean BMI SDS decreased significantly from 1.93 to 1.13 (p < 0.05) after 1 year of treatment; however, no significant changes were observed afterward. Mean Rohrer index decreased significantly, from 224.2 to 186.6 (p < 0.001), 178.9 (p < 0.001) and 169.3 (p < 0.001). No significant gender or genotype pattern differences were noted among the 4 parameters examined. CONCLUSION: This 3-year, retrospective study indicates that PWS patients benefit from GH therapy in height increase and improved body composition.
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The metabolic and molecular bases of inherited disease
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