Original ArticleGrowth hormone improves mobility and body composition in infants and toddlers with Prader-Willi syndrome
Section snippets
Patients and methods
Twenty-nine infants and toddlers with PWS 4 to 37 months of age, (mean age 15 ± 9 months; 13 females) were enrolled in the study after informed consent was obtained from a parent or legal guardian. All patients had a diagnosis of PWS confirmed by high-resolution cytogenetics, fluorescent in situ hybridization, and/or methylation studies. Seventeen subjects had deletion of chromosome 15q11-13, 11 had uniparental disomy, and 1 patient was diagnosed by an abnormal methylation test. Length/height
Results
Patient characteristics and study results are summarized in Table I, Table II, Table III and are presented as mean ± SD.
Discussion
Infants and toddlers with PWS commonly demonstrate hypotonia, associated with poor suck, poor feeding, compromised respiratory function, early failure to thrive, and delay in attainment of developmental motor skills. Body fat measurements are increased even in underweight infants with PWS.7., 8., 5., 33. Early abnormalities in body composition in PWS, therefore, are present before the onset of characteristic hyperphagia and progressive obesity, and they are qualitatively similar to those
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Supported by grants from NIH M01 RR33186 and Pharmacia Inc. (Pfizer).