Long-term effect of growth hormone on sleep-disordered breathing in Malaysian children with Prader-Willi syndrome: a retrospective study

STUDY OBJECTIVES: The effect of recombinant human growth hormone (rhGH) on sleep-disordered breathing (SDB) in Malaysian children with Prader-Willi syndrome (PWS) is under-investigated. We determined (a) the short- and long-term effects of rhGH and (b) factors associated with worsening SDB, in children with PWS on rhGH.

METHODS: This retrospective study included children with PWS (with and without rhGH) who had at least one polysomnography (PSG). Outcomes measured were the presence of SDB: before and after starting rhGH and the progress of SDB with and without rhGH. Serial insulin-like growth factor-1 (IGF-1) measurements were recorded.

RESULTS: One-hundred and thirteen PSGs were analyzed. The majority (92.3%) of initial PSGs had SDB with AHI median (IQR) 5.0 (2.6,16.3) events/h. The age for receiving rhGH was median (IQR) 1.9 (0.7, 3.4) years old. A third (36.8%) had worsening SDB after initiating rhGH, which was associated with higher IGF-1 levels post-rhGH (p=0.007). After a median of 5 years of rhGH, 73.6% maintained or reduced their positive airway pressure (PAP) settings. Without rhGH, 80% had increased their PAP settings. Worsening SDB while on rhGH was associated with higher BMI, lower rhGH dose, higher IGF-1 levels and non-15q deletion.

CONCLUSIONS: Majority of Malaysian children with PWS had SDB. At initiation rhGH, one-third of patients had worsening SDB, associated with increased IGF-1 levels. Stabilization of SDB was more frequently seen in those on long-term rhGH. Worsening SDB while on rhGH was associated with a higher BMI, on a lower dose of rhGH, higher IGF-1 levels and non-15q deletion.