title: Growth hormone therapy and respiratory disorders: long-term follow-up in PWS children.
abstract: BACKGROUND
  Adenotonsillar tissue hypertrophy and obstructive sleep apnea have been reported during short-term GH treatment in children with Prader-Willi syndrome (PWS).
  
  
  OBJECTIVE
  We conducted an observational study to evaluate the effects of long-term GH therapy on sleep-disordered breathing and adenotonsillar hypertrophy in children with PWS.
  
  
  METHODS
  This was a longitudinal observational study.
  
  
  METHODS
  We evaluated 75 children with genetically confirmed PWS, of whom 50 fulfilled the criteria and were admitted to our study. The patients were evaluated before treatment (t0), after 6 weeks (t1), after 6 months (t2), after 12 months (t3), and yearly (t4-t6) thereafter, for up to 4 years of GH therapy. The central apnea index, obstructive apnea hypopnea index (OAHI), respiratory disturbance index, and minimal blood oxygen saturation were evaluated overnight using polysomnography. We evaluated the adenotonsillar size using a flexible fiberoptic endoscope.
  
  
  RESULTS
  The percentage of patients with an OAHI of >1 increased from 3 to 22, 36, and 38 at t1, t4, and t6, respectively (χ(2) = 12.2; P < .05). We observed a decrease in the respiratory disturbance index from 1.4 (t0) to 0.8 (t3) (P < .05) and the central apnea index from 1.2 (t0) to 0.1 (t4) (P < .0001). We had to temporarily suspend treatment for 3 patients at t1, t4, and t5 because of severe obstructive sleep apnea. The percentage of patients with severe adenotonsillar hypertrophy was significantly higher at t4 and t5 than at t0. The OAHI directly correlated with the adenoid size (adjusted for age) (P < .01) but not with the tonsil size and IGF-1 levels.
  
  
  CONCLUSIONS
  Long-term GH treatment in patients with PWS is safe; however, we recommend annual polysomnography and adenotonsillar evaluation.
authors.affiliation: Pediatric Unit, Ospedale Del Ponte, Insubria University, 21100 Varese, Italy.
authors.forename: Jenny
authors.initials: J
authors.lastname: Berini
authors.forename: Valeria
authors.initials: V
authors.lastname: Spica Russotto
authors.forename: Paolo
authors.initials: P
authors.lastname: Castelnuovo
authors.forename: Stefania
authors.initials: S
authors.lastname: Di Candia
authors.forename: Luigi
authors.initials: L
authors.lastname: Gargantini
authors.forename: Graziano
authors.initials: G
authors.lastname: Grugni
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Iughetti
authors.forename: Luigi
authors.initials: L
authors.lastname: Nespoli
authors.forename: Luana
authors.initials: L
authors.lastname: Nosetti
authors.forename: Giovanni
authors.initials: G
authors.lastname: Padoan
authors.forename: Alba
authors.initials: A
authors.lastname: Pilotta
authors.forename: Giuliana
authors.initials: G
authors.lastname: Trifirò
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Chiumello
authors.forename: Alessandro
authors.initials: A
authors.lastname: Salvatoni
mesh_terms: D000234:Adenoids / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D020249:Hormone Replacement Therapy / Q000009:adverse effects*; D019382:Human Growth Hormone / Q000009:adverse effects* / Q000627:therapeutic use; D006801:Humans; D006984:Hypertrophy / Q000139:chemically induced; D007223:Infant; D008297:Male; D014066:Palatine Tonsil / Q000473:pathology*; D017286:Polysomnography; D011218:Prader-Willi Syndrome / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D020181:Sleep Apnea, Obstructive / Q000209:etiology* / Q000473:pathology
chemical_list: D019382:Human Growth Hormone