Mari Tsukahara, Hideaki Yagasaki, Koichi Makino, Tomoaki Sano, Takeshi Inuka
University of Yamanashi.
Japan
Cureus
Cureus 2026; 18:
DOI: 10.7759/cureus.103890
Abstract
Prader-Willi syndrome (PWS) is a multisystem genetic disorder frequently complicated by sleep-disordered breathing, including obstructive sleep apnea. Respiratory infections may precipitate acute decompensation in vulnerable patients with PWS. A four-year-old boy with genetically confirmed PWS receiving growth hormone therapy developed respiratory distress and cyanosis. He was found to be respiratory syncytial virus-positive and presented with acute respiratory acidosis and markedly elevated aminotransferases with coagulopathy. Echocardiography showed right-sided cardiac dilation consistent with pulmonary hypertension. He was managed with noninvasive ventilation, oxygen supplementation, and diuretics, followed by step-down to nasal continuous positive airway pressure. Liver enzymes normalized as his respiratory status improved. Polysomnography after stabilization showed obstructive sleep apnea with considerable nocturnal desaturation, and home continuous positive airway pressure was initiated. Respiratory syncytial virus (RSV) infection can trigger acute-on-chronic respiratory failure and cardiopulmonary decompensation in children with PWS and unrecognized severe nocturnal hypoxemia. Early screening and ongoing surveillance for sleep-disordered breathing, cardiopulmonary complications, and careful management during intercurrent infections are essential. RSV immunoprophylaxis, such as palivizumab, may warrant individualized consideration in high-risk young children with PWS who have impaired airway clearance or considerable respiratory comorbidity.
Category
Class I. Pulmonary Hypertension Associated with Infection
Class III. Pulmonary Hypertension Associated with Airway Disease, Apnea or Hypoventilation
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes