Sleep Disordered Breathing in Prader‐Willi patients treated with rh GH

Abstract

Background: Previous reports showed that Growth Hormone (GH) therapy in Prader‐Willi Syndrome (PWS) was associated with an increased risk of sudden death by Obstructive Sleep Apnea (OSA). The aim of this study was to evaluate the effects of GH therapy on OSA in PLWS patients. Method: We selected 12 prepubescent patients with genotype‐confirmed PWS without OSA: 6 boys, aged (mean 3.8 years; SD 1.9 years). ENT video endoscopy of upper airways was performed contemporary to one night 12 channels polysomnography (PSG) before starting GH therapy and after 6 weeks of treatment. The Katz et al. criteria were used for PSG evaluation. The main parameter considered was total apnea hypopnea index (AHI), resulting from central apnea hypopnea index and obstructive apnea hypopnea index (OAI). Results: Before treatment none of the patients showed symptoms of OSA or was receiving systemic steroids. However, PSG revealed that all patients showed signs of central and/or obstructive apnea. After 6 weeks of therapy OAI decreased in 3/12 (25%) patients was unchanged in 4/12 (33%) and increased in 5/12 (42%). In four of these patients OAI exceeded the cut‐off of 1. In these four patients treatment was temporary discontinued. On average during rhGH treatment we did not find any significant change of the polysomnographic index: before rhGH therapy mean OAI was 0.7, RDI (Respiratory Disturbance Index) 5.5, and minSpO2 87.4% while after 6 weeks of therapy mean OAI was 0.8, RDI 6.5, and min SpO2 83.9%. Conclusion: The increased death rate could be an adverse effect of GH therapy. In our patients, GH treatment caused an increase of OAI in one out of three of our patients. It is recommended that a PSG and ENT evaluations are undertaken before and during the therapy for monitoring respiratory function. In those patients with increased OAI, temporary discontinuation of GH treatment is recommended until respiratory function is normal.