期刊
PEDIATRIC PULMONOLOGY
卷 34, 期 1, 页码 23-29出版社
WILEY-LISS
DOI: 10.1002/ppul.10125
关键词
desquamative interstitial pneumonitis; familial interstitial pneumonitis; fibrosing alveolitis; pulmonary fibrosis; hydroxychloroquine; prednisolone; methylprednisolone; lung biopsy; pathology
Interstitial lung disease (ILD) of unknown etiology in immunocompetent patients is rare in children. A national survey was carried out in the United Kingdom and Ireland over a 3-year period in order to identify prevalence, age distribution, histopathology, natural history of the illness, and response to current treatment. Forty-six cases were identified, including 29 males and 17 females. Seventy-six percent presented in the first year of life. Nine (16%) occurred within four families. Conventional treatment with pulsed methylprednisolone, prednisolone, or hydroxychloroquine, singly or in combination, resulted in an excellent response in 65% of cases. Seven children died (15%). The recurrence risk for further children within the same family to develop ILD is estimated to be approximately 10%. The prevalence rate of this condition in the United Kingdom and Ireland during the period of study for children aged 0-16 years is estimated to be 3.6 cases/million. (C) 2002 Wiley-Liss, Inc.
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