Idiopathic desquamative interstitial pneumonia in a child: a case report.

Details

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State: Public
Version: Final published version
Serval ID
serval:BIB_5760D867EA43
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Idiopathic desquamative interstitial pneumonia in a child: a case report.
Journal
BMC research notes
Author(s)
Bressieux-Degueldre S., Rotman S., Hafen G., Aubert J.D., Rochat I.
ISSN
1756-0500 (Electronic)
ISSN-L
1756-0500
Publication state
Published
Issued date
22/06/2014
Peer-reviewed
Oui
Volume
7
Pages
383
Language
english
Notes
Publication types: Case Reports ; Journal Article
Publication Status: epublish
Abstract
Desquamative interstitial pneumonia is a rare form of interstitial lung disease in children. Respiratory symptoms appear progressively, are often subtle, and diagnosis is often delayed by a mean of 6 months after onset. High resolution chest computed tomography is the most sensitive imaging technique for demonstrating and identifying interstitial pneumonia. The typical histologic pattern of desquamative interstitial pneumonia, with prominent clustered alveolar macrophages, diffuse reactive alveolar epithelial hyperplasia and globular proteinaceous material, is diagnostic. Desquamative interstitial pneumonia in children can be idiopathic, though it is mostly related to an inborn error of surfactant metabolism.
We present the complex clinical course and pathologic findings of a 30-months-old Mauritian and Senegalese girl with idiopathic desquamative interstitial pneumonia and multiple extrapulmonary manifestations. To our knowledge, this is the first case report of desquamative interstitial pneumonia to occur as part of a syndrome with multiple organ involvement.
We believe that desquamative interstitial pneumonia is not always associated with mutations of the surfactant proteins, and can still be idiopathic, especially when occurring as part of a syndrome with multiple organ involvement, as described in other interstitial lung diseases.

Keywords
Child, Preschool, Developmental Disabilities/complications, Female, Humans, Kidney Diseases/complications, Lung/pathology, Lung Diseases, Interstitial/diagnosis, Lung Diseases, Interstitial/etiology, Skin Diseases/complications
Pubmed
Open Access
Yes
Create date
07/07/2014 15:33
Last modification date
20/08/2019 14:11
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