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Idiopathic interstitial pneumonitis in children: A national survey in the United Kingdom and Ireland

Identifieur interne : 002219 ( Main/Exploration ); précédent : 002218; suivant : 002220

Idiopathic interstitial pneumonitis in children: A national survey in the United Kingdom and Ireland

Auteurs : R. Dinwiddie [Royaume-Uni] ; N. Sharief [Royaume-Uni] ; O. Crawford [Royaume-Uni]

Source :

RBID : ISTEX:2F2A42A20CB2BEF20778D650B9EE95D65958F42F

Descripteurs français

English descriptors

Abstract

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. Pediatr Pulmonol. 2002; 34:23–29. © 2002 Wiley‐Liss, Inc.

Url:
DOI: 10.1002/ppul.10125


Affiliations:


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Le document en format XML

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<div type="abstract" xml:lang="en">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. Pediatr Pulmonol. 2002; 34:23–29. © 2002 Wiley‐Liss, Inc.</div>
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