IMPROVED MORBIDITY WITH THE USE OF NASAL CONTINUOUS POSITIVE AIRWAY PRESSURE IN I-CELL DISEASE

Patients with I-cell disease (mucolipidosis II) present with progressi ve morbidity failure to thrive, cardiomegaly, and recurrent respirator y tract infections leading to progressive deterioration and early deat h. We evaluated use of nasal continuous positive airway pressure (NCPA P) for 6 months in a 2-year-old girl with I-cell disease, obstructive sleep apnea (OSA), and craniofacial anomalies. We observed a marked de crease in hospitalizations for respiratory problems and a marked impro vement in arterial blood gases with the use of NCPAP. In patients with I-cell disease, anatomical defects with superimposed upper respirator y tract infections cause worsening of OSA, and OSA contributes signifi cantly to morbidity. In such patients NCPAP can lessen morbidity and c an improve the quality of life. (C) 1998 Wiley-Liss, Inc.