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Idiopathic pulmonary fibrosis in infants
Author(s) -
Osika Eric,
Muller MarieHelene,
BocconGibod Liliane,
Fauroux Brigitte,
Sardet Anne,
Grosskopf Cecile,
Couvreur Jacques,
Tournier Guy,
Clement Annick
Publication year - 1997
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(199701)23:1<49::aid-ppul6>3.0.co;2-k
Subject(s) - medicine , idiopathic pulmonary fibrosis , pulmonary fibrosis , cystic fibrosis , intensive care medicine , fibrosis , pathology , lung
Idiopathic pulmonary fibrosis is a poorly characterized disease in infants. In the present report, we reviewed our experience with 10 infants during a 10‐year period. In 9 patients, onset of symptoms occurred before the age of 2 months and included tachypnea, cough, and inadequate weight gain. However, despite the presence of these symptoms, diagnosis was delayed for 3 months at which time the infants were referred to the pediatric pulmonary department, when the diagnosis was confirmed by open lung biopsy. At the time of admission, bronchoscopy with alveolar lavage was performed in 9 children and showed severe alveolitis with an increase in the neutrophil count. Nine infants were treated with prednisone alone or in combination with chloroquine, colchicine, or cyclophosphamide; all these patients died despite treatment. One infant was treated with pulses of methylprednisolone because of a failure in response to oral prednisone. This girl who displayed similar clinical, radiological, and histological abnormalities as the other children at the time of diagnosis is the only child still alive after 3 years of follow‐up. She is now free of respiratory symptoms and has a normal growth curve. The present report raised two important points: (1) a thorough evaluation of characteristic symptoms should lead to an early diagnosis of pulmonary fibrosis in infants; and (2) administration of pulse therapy using corticosteroids has been helpful and needs to be evaluated further. Pediatr Pulmonol. 1997; 23:49–54. © 1997 Wiley‐Liss, Inc.