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Improved morbidity with the use of nasal continuous positive airway pressure in I‐cell disease
Author(s) -
Sheikh Shahid,
Madiraju Krishna,
Qazi Qutbuddin,
Rao Madu
Publication year - 1998
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/(sici)1099-0496(199802)25:2<128::aid-ppul9>3.0.co;2-j
Subject(s) - medicine , continuous positive airway pressure , failure to thrive , obstructive sleep apnea , respiratory failure , apnea , airway , respiratory tract , disease , respiratory disease , respiratory system , craniofacial , pediatrics , anesthesia , lung , psychiatry
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. Pediatr. Pulmonol. 1998; 25:128–129. © 1998 Wiley‐Liss, Inc.