RESPIRATORY CONTROL IN CHILDREN WITH PRADER-WILLI-SYNDROME

Physiological parameters of infants and children with Prader-Willi syn drome were examined in order to clarify whether there were indicators of disturbed respiratory control mechanisms in the pre-obesity stage o f the syndrome. From January 1993 to March 1995 in eight patients with Prader-Willi syndrome (five boys, three girls, aged 6 weeks - 12.5 ye ars), polysomnography was performed and compared with 28 children matc hed for gestational age, sex, birth weight and age at sleep study. The recordings included thoracic and abdominal breathing movements, nasal airflow, tcPO(2), tcPCO(2), oxygen saturation, EEG, EOG and EGG. Resp iratory responses to hypercapnia during quiet sleep were obtained from five Prader-Willi patients and ten peers. The Prader-Willi group show ed an increased number of apnoeas per hour of sleep, a decreased nadir of oxygen saturation, increased maximum of the instantaneous heart ra te and decreased respiratory responses to hypercapnia during quiet sle ep. Conclusion These findings indicate a primary disturbance of centra l respiratory control inpatients with Prader-Willi syndrome which may be worsened by the development of obesity.