Introduction:Rapid-onset obesity with hypothalamic dysfunction,hypoventilation,and autonomic dysregulation(ROHHAD)syndrome is an exceptionally rare clinical entity with significant morbidity and high mortality with ch...Introduction:Rapid-onset obesity with hypothalamic dysfunction,hypoventilation,and autonomic dysregulation(ROHHAD)syndrome is an exceptionally rare clinical entity with significant morbidity and high mortality with challenging-to-treat hypoventilation.Case presentation:An 11-year-old morbidly obese Chinese female presented with a putative diagnosis of ROHHAD associated with a left psoas ganglioneuroma.Initial polysomnography showed severe obstructive sleep apnea and hypoventilation.She was not adherent to prescribed non-invasive positive pressure ventilation(NIPPV).Echocardiography demonstrated evidence of pulmonary hypertension,likely secondary to chronic hypoventilation.With behavioral modification and trial of average volume-assured pressure support(AVAPS),adherence improved with eventual improvement of her pulmonary hypertension.Conclusion:AVAPS may improve ventilation and NIPPV adherence in central hypoventilation disorders such as ROHHAD,reducing risk of morbidity and mortality.展开更多
Obstructive sleep apnea(OSA)affects about 1%-5%of the pediatric population.The consequences of untreated OSA in children include neurocognitive deficits,behavioral problems,poor school performance as well as systemic ...Obstructive sleep apnea(OSA)affects about 1%-5%of the pediatric population.The consequences of untreated OSA in children include neurocognitive deficits,behavioral problems,poor school performance as well as systemic and pulmonary hypertension.The treatment options for pediatric OSA are numerous with a variety of surgical and non-surgical interventions.As our understanding of the complexities of OSA grows,the options for management have continued to expand as well.The objectives of this review are to describe the commonly prescribed treatments for pediatric OSA including adenotonsillectomy as well as use of positive airway pressure.We also highlight other surgical and non-surgical interventions available.In addition,we provide updates on current research focusing on newer diagnostic and experimental treatment modalities.展开更多
文摘Introduction:Rapid-onset obesity with hypothalamic dysfunction,hypoventilation,and autonomic dysregulation(ROHHAD)syndrome is an exceptionally rare clinical entity with significant morbidity and high mortality with challenging-to-treat hypoventilation.Case presentation:An 11-year-old morbidly obese Chinese female presented with a putative diagnosis of ROHHAD associated with a left psoas ganglioneuroma.Initial polysomnography showed severe obstructive sleep apnea and hypoventilation.She was not adherent to prescribed non-invasive positive pressure ventilation(NIPPV).Echocardiography demonstrated evidence of pulmonary hypertension,likely secondary to chronic hypoventilation.With behavioral modification and trial of average volume-assured pressure support(AVAPS),adherence improved with eventual improvement of her pulmonary hypertension.Conclusion:AVAPS may improve ventilation and NIPPV adherence in central hypoventilation disorders such as ROHHAD,reducing risk of morbidity and mortality.
文摘Obstructive sleep apnea(OSA)affects about 1%-5%of the pediatric population.The consequences of untreated OSA in children include neurocognitive deficits,behavioral problems,poor school performance as well as systemic and pulmonary hypertension.The treatment options for pediatric OSA are numerous with a variety of surgical and non-surgical interventions.As our understanding of the complexities of OSA grows,the options for management have continued to expand as well.The objectives of this review are to describe the commonly prescribed treatments for pediatric OSA including adenotonsillectomy as well as use of positive airway pressure.We also highlight other surgical and non-surgical interventions available.In addition,we provide updates on current research focusing on newer diagnostic and experimental treatment modalities.
