The obesity hypoventilation syndrome and the Prader‐Willi syndrome

Abstract

ABSTRACT. Fourteen children with the Prader‐Wllli syndrome have been managed at the Royal Alexandra Hospital for Children between the years 1964–1980 — twelve male, two female. Six male children developed features of the obesity hypoventilation syndrome. The age of onset of this complication ranged from 4.0 to 12.6 years. With one exception those children with the obesity hypoventilation syndrome were more obese than those without it. At the time of onset of the syndrome, five of six patients had weights greater than or equal to 6.5 standard deviations above ideal body weight. Those children without the obesity hypoventilation syndrome had a range of standard deviations 1.0 to 4.2 above the ideal body weight. In four of six cases weight reduction and a cardiac failure regimen resulted in reversal of the obesity hypoventilation syndrome. With two of the six children there had been cardiomegaly and increased pulmonary venous vascularity on x‐ray at a chronological age of three months. Two of the six children died.

Central sleep-disordered breathing and the effects of oxygen therapy in infants with Prader-Willi syndrome

“…In our case series, we report the presence of central apnoeas in REM sleep, which may share similarities with the REM hypoventilation reported in older children with PWS who are obese 20 21. Thus, a dysrhythmic breathing pattern seen in PWS may be inherent to the condition (due to the chemoreceptor insensitivity that arises from the NDN gene deletion) and which can expresses itself in the presence of appropriate factors, namely hypotonia and immature respiratory control in infancy, or obesity in the older child.…”

Prader–Willi syndrome: an update

“…Findings of cardiomegaly and increased pulmonary venous vascularity are poor prognostic signs. OHS carries a very high risk of morbidity and mortality in PWS, which exceeds that of the general obese population [48]. This may be because of baseline nocturnal hypoventilation [41].…”