Original articleGhrelin levels in young children with Prader-Willi syndrome
Section snippets
Subjects
This study was approved by the Human Subjects Research Division at the University of Washington. A recruitment letter was published in the Northwest PWS Association Newsletter, and interested families contacted the principal investigator to review the protocol.
After written parental consent and assent (for verbal children) were obtained, age, height, weight, and time of the last meal were determined. The diagnosis of PWS was confirmed by history and chart review. There were nine children with
Results
We studied nine patients with PWS and eight healthy control subjects, who were either related to or close friends of the children with PWS, and who were of equivalent age, BMI, and sex (Table). In the PWS group, all patients had positive studies for methylation of genes in the PWS locus, confirming their diagnosis. One child had a deletion of 15q documented by fluorescence in situ hybridization (FISH), and eight had negative FISH studies and presumed uniparental disomy. There were three sibling
Discussion
Children <5 years of age with PWS did not display significant elevation of total or acylated plasma ghrelin levels, when compared with controls of equivalent age, BMI, sex, and levels of insulin, glucose, and insulin sensitivity. These results differ from those reported previously in obese adults with PWS (mean BMI ± SD of 33.9 ± 9.0)25 and in obese children with PWS who were 5 to 16 years of age.30, 32, 33 In these prior studies, people with PWS had elevated plasma ghrelin levels. One
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2016, Advances in PediatricsCitation Excerpt :Total ghrelin in young children with PWS and control children was significantly higher than in older counterparts and the decline in ghrelin with age was more exaggerated in the control subjects than in the patients with PWS. Other researchers published similar findings to ours that there were no significant differences in plasma insulin or ghrelin levels between young patients with PWS and controls [9,10]. Similar to our study, Goldstone and colleagues [9] also reported significantly higher plasma leptin levels in young patients with PWS versus controls, suggesting a relative excess of fat to lean body mass.
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2015, Seminars in Pediatric NeurologyCitation Excerpt :The patients are profoundly hypotonic in infancy, but manifest hyperphagia, hypersomnolence, and behavioral problems during childhood. Obesity develops in middle to late half of the first decade, and is likely related to elevated levels of ghrelin, an appetite-stimulating peptide that is secreted by the stomach.3 Daytime sleepiness in Prader-Willi syndrome (PWS) may be associated with sleep-onset rapid eye moment (REM) periods (SOREMP), though not to the degree seen in narcolepsy.
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Supported by NIH grants RO1 DK61516 and PO1 DK68384 (to D.E.C.).