Short-Term Effects of Growth Hormone on Sleep Abnormalities in Prader-Willi Syndrome

Abstract

Most of our PWS patients had improvement after short-term GH treatment, but 32% had worsening of sleep disturbance. A subset of PWS patients are at risk during this window of vulnerability shortly after initiation of GH. Because it is difficult to predict who will worsen with GH, patients with PWS should have PSA before and after starting GH and should be monitored for sleep apnea with upper respiratory tract infections. Otorhinolaryngological evaluation is warranted if sleep apnea worsens on GH. IGF-I levels should be monitored, with the goal being physiological levels.

Prader–Willi syndrome

“…65 Because of concerns about unexpected death on account of respiratory obstruction early in growth hormone treatment, sleep polysomnography and assessment for enlarged tonsils and adenoids are recommended before initiating growth hormone treatment and 6 -8 weeks after starting. 68 However, there is controversy among experts about a direct role of growth hormone in these unexpected deaths. 31,63,68 In addition, thyroid function should be assessed before growth hormone therapy, as 15% of patients have hypothyroidism.…”

“…68 However, there is controversy among experts about a direct role of growth hormone in these unexpected deaths. 31,63,68 In addition, thyroid function should be assessed before growth hormone therapy, as 15% of patients have hypothyroidism. Serum IGF-1 levels, growth velocity and head circumference should be monitored during treatment and kept in the normal range.…”

“…Serum IGF-1 levels, growth velocity and head circumference should be monitored during treatment and kept in the normal range. 68 Hypogonadism Cryptorchidism in males is common and often requires surgical intervention. A trial of chorionic gonadotropin will enlarge the scrotum so it can better contain the testes post-surgically, and might circumvent the need for surgery.…”

Benefit of Early Commencement of Growth Hormone Therapy in Children with Prader-Willi Syndrome

“…In another study GH was continued for a total of 4 years in three cohorts receiving different doses of GH. The benefit on growth velocity, body composition (lean body mass) and resting energy expenditure was noted with higher doses of GH (7 17 . However, in a mortality review in patients with PWS, the majority of sudden death was related to respiratory pathology 18 ' 19 and this finding was also supported by a study of KIGS, the Pfizer International Growth Database °.…”

Growth Hormone Therapy in Adults with Prader-Willi Syndrome

“…It is possible that rhGH therapy could have beneficial effects in adults with PWS, similar to those in adults with GHD of non-syndromic cause. However, there is also concern about potential risks of this therapy, as rhGH has anti-insulin effects and might impair respiratory function in children with underlying sleep apnea, which is frequently present in patients with PWS 13,14) . However, there is a paucity of data on the use of GH in adults with PWS; existing studies have small in scope, suggesting that the findings of individual studies may be difficult to extrapolate to the population of adult patients with PWS at large.…”