Improved morbidity with the use of nasal continuous positive airway pressure in I-cell disease.

Abstract

Patients with I-cell disease (mucolipidosis II) present with progressive morbidity failure to thrive, cardiomegaly, and recurrent respiratory tract infections leading to progressive deterioration and early death. We evaluated use of nasal continuous positive airway pressure (NCPAP) for 6 months in a 2-year-old girl with I-cell disease, obstructive sleep apnea (OSA), and craniofacial anomalies. We observed a marked decrease in hospitalizations for respiratory problems and a marked improvement in arterial blood gases with the use of NCPAP. In patients with I-cell disease, anatomical defects with superimposed upper respiratory tract infections cause worsening of OSA, and OSA contributes significantly to morbidity. In such patients NCPAP can lessen morbidity and can improve the quality of life.

• Authors: S Sheikh, K Madiraju, Q Qazi, M Rao
• Journal: Pediatric pulmonology (Pediatr Pulmonol) Vol. 25 Issue 2 Pg. 128-9 (Feb 1998) ISSN: 8755-6863 [Print] United States
• PMID: 9516097 (Publication Type: Case Reports, Journal Article)

Chemical References

• Carbon Dioxide
• Oxygen

Topics

• Carbon Dioxide (blood)
• Child, Preschool
• Female
• Hospitalization
• Humans
• Mucolipidoses (complications)
• Oxygen (blood)
• Positive-Pressure Respiration
• Respiratory Tract Diseases (blood, complications, therapy)
• Sleep Apnea Syndromes (complications, therapy)