Nocturnal hypoventilation in children with nonprogressive neuromuscular disease.
Eight patients between 4 and 24 years of age with nonprogressive neuromuscular disease sought medical attention because of severe nocturnal hypoventilation. There were two types of findings: subacute with progressive early morning headaches and daytime drowsiness and acute with ventilatory failure and cor pulmonale. Seven patients were ambulant. Seven were successfully treated with either a cuirass negative pressure ventilator or a positive pressure ventilator via a tracheostomy. The ventilatory assistance was only used at night and resulted in rapid resolution of early morning symptoms and a return to full daytime activity. One patient died as a result of an intercurrent respiratory infection before respiratory support could be given. It is important to be aware of this potentially life-threatening complication in patients with an otherwise good prognosis and of the benefit to be derived from active treatment.
Heckmatt
J Z
JZ
Department of Paediatrics, Royal Postgraduate Medical School, Hammersmith Hospital, London, England.
Loh
L
L
Dubowitz
V
V
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D000860:Hypoxia / Q000209:etiology; D008297:Male; D009468:Neuromuscular Diseases / Q000150:complications*; D010092:Oximetry; D012121:Respiration, Artificial; D012891:Sleep Apnea Syndromes / Q000209:etiology* / Q000601:surgery / Q000628:therapy; D014139:Tracheostomy; D014797:Vital Capacity