Prader-Willi syndrome and growth hormone therapy: effect on sleep

Abstract

Because of reports that growth hormone therapy causes increased severity of obstructive sleep apnea in Prader-Willi syndrome, a retrospective review was performed of polysomnograms performed in children with Prader-Willi syndrome before onset of therapy and 6 weeks, 6 months, 1 year, and 2 years after starting growth hormone therapy. The results from Al-Saleh et al are published in this issue of The Journal, which show that 13 of 15 children did well with no adverse sleep outcomes. However, 2 of the 15 had growth hormone therapy discontinued because of the development of severe obstructive sleep apnea after 6 weeks. The authors recommend that growth hormone therapy appears to be a safe treatment after an initial period of increased risk with regards to sleep-related breathing, but they recommend that children selected for treatment should be evaluated very carefully. This issue of growth hormone related-obstructive sleep apnea in Prader-Willi syndrome is discussed in the accompanying Editorial by Whitman and Myers. They propose recommendations for how to manage children with Prader-Willi syndrome with regards to starting growth hormone therapy. Article page 263▶ Editorial page 224▶ Longitudinal Evaluation of Sleep-Disordered Breathing in Children with Prader-Willi Syndrome during 2 Years of Growth Hormone TherapyThe Journal of PediatricsVol. 162Issue 2PreviewTo review longitudinal polysomnography data to assess sleep-related disordered breathing (SRDB) before and up to 2 years after initiation of growth hormone (GH) therapy in children with Prader-Willi syndrome (PWS). Full-Text PDF Prader-Willi Syndrome and Growth Hormone Therapy: Take a Deep Breath and Weigh the DataThe Journal of PediatricsVol. 162Issue 2PreviewPrader-Willi syndrome (PWS), a complex neurogenetic disorder originally described in 1956,1 was relatively unknown outside genetics and developmental clinics until the late 1990s when growth hormone replacement therapy (GHRT) emerged as an effective intervention. The cardinal feature is the early onset, unrelenting, treatment-refractory hyperphagia leading to morbid obesity and attendant comorbidities. In addition, however, PWS is characterized by infantile hypotonia and failure to thrive, followed by short stature, hypogonadism, abnormal body composition, sleep and respiratory abnormalities (including impaired ventilatory control, central and obstructive apnea, and excessive daytime sleepiness), and behavioral difficulties. Full-Text PDF