title: Successful use of BiPAP in infants with congenital myotonic dystrophy.
abstract: Reported herein are two cases of severe phenotype of congenital myotonic dystrophy (CDM) with presentation of respiratory insufficiency at birth. The infants were successfully managed with bi-level positive airway pressure (BiPAP) via nasal mask. The use of BiPAP in infants with CDM has not been reported before. The rationale for using BiPAP is discussed. BiPAP may be more effective than continuous positive airway pressure in managing respiratory insufficiency, especially in infants with the more severe phenotype of CDM.
authors.affiliation: Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, LKS Faculty of Medicine, University of Hong Kong, Hong Kong.
authors.forename: Shuk-Kuen
authors.initials: SK
authors.lastname: Chau
authors.forename: So-Lun
authors.initials: SL
authors.lastname: Lee
mesh_terms: D045422:Continuous Positive Airway Pressure / Q000379:methods*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009223:Myotonic Dystrophy / Q000150:complications* / Q000628:therapy; D012131:Respiratory Insufficiency / Q000209:etiology / Q000628:therapy*