PEDIATRICS Vol. 70 No. 5 November 1982, pp. 684-694
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Congenital Central Alveolar Hypoventilation Syndrome in Six Infants

Christian Guilleminault MD1, John McQuitty MD1, Ronald L. Ariagno MD1, Marie Joseph Challamel MD1, Rowena Korobkin MD1, and Richard E. McClead Jr MD1

1 Sleep Disorders Center, and Department of Pediatrics, Stanford University School of Medicine, Stanford, California; Department of Pulmonary Medicine, Children's Hospital Medical Center, Oakland, California; Hopital St Eugenie, Lyon School of Medicine, Lyon, France; and Columbus Children's Hospital, Columbus, Ohio

Six infants with congenital central alveolar hypoventilation syndrome (CCHS) were seen and observed over several years. Two had an association of CCHS with Hirchsprung's disease. All infants were treated by tracheostomy and mechanical ventilation. Three infants have survived (including one with CCHS and Hirchsprung's disease). However, all survivors have required frequent rehospitalization. The infant with the longest survival (now 4 years of age) has developed significant daytime problems involving the "behavioral control" of ventilation. One infant was considered as a "near miss for sudden infant death syndrome" and became significantly symptomatic after establishment of delta (stage 3-4 non-rapid eye movement) sleep, which normally develops between 2 and 4 months of age. CCHS involves autonomic nervous system dysfunction, and the question of a defect involving the integration of chemoreceptor information more than a direct defect of the central chemoreceptor is discussed.

Submitted on November 30, 1981
Accepted on January 15, 1982




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