Respiratory Depression in Young Prader Willi Syndrome Patients following Clonidine Provocation for Growth Hormone Secretion Testing

Objectives. To determine the sedative and respiratory effects of clonidine when used to evaluate growth hormone (GH) secretion in children with Prader Willi Syndrome (PWS). Methods. The study prospectively evaluated children with PWS who received clonidine (0.15 mg/m(2)) to assess GH responsiveness. Patients were studied up to four times over three years. Vital signs, oxygen saturation, and sedation level were recorded at baseline and every five minutes following clonidine. Changes between baseline and post-clonidine were evaluated using a repeated measurement analysis. Results. Sixty studies were performed on 17 patients, mean age 30.4 ± 15.0 months. The mean ± SD dose of clonidine was 0.074 ± 0.027 mg (5.3 ± 1.72 mcg/kg). All patients achieved a sedation score of 4 to 5 (drowsy to asleep). Mean declines in respiratory rate (7.5 ± 6.1 breaths/min; P < .001), and oxygen saturation (2.2 ± 2.0%; P < .001) occurred following clonidine. Five patients (29%) experienced oxygen saturations ≤94% on nine occasions. Three oxygen desaturations were accompanied by partial airway obstruction. Conclusions. Clonidine doses to assess GH secretion often exceed doses used for sedation and result in significant respiratory depression in some children with PWS. There was no association between oxygen desaturation and BMI.