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01.07.2014 | Original Article

Primary pulmonary alveolar proteinosis: computed tomography features at diagnosis

verfasst von: Laureline Berteloot, Rola Abou Taam, Sophie Emond-Gonsard, Tania Mamou-Mani, Karen Lambot, David Grévent, Caroline Elie, Muriel Le Bourgeois, Christophe Delacourt, Francis Brunelle, Jacques de Blic

Erschienen in: Pediatric Radiology | Ausgabe 7/2014

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Abstract

Background

Pulmonary alveolar proteinosis (PAP) is characterized by an abnormal accumulation of periodic acid-schiff-positive lipoproteinaceous material in the alveoli. Early diagnosis allows setting up of therapeutic lung lavages, which reduces the need for oxygen supplementation and weight gain.

Objective

To provide a description of radiological features by CT at the onset of primary PAP in children.

Materials and methods

The clinical and radiological data of 24 patients, including 16 boys and 8 girls (median age: 12 months), diagnosed with a primary form of PAP between April 1992 and May 2012 in a tertiary referral hospital, were retrospectively reviewed. CT images were examined for the presence of alveolar and interstitial elementary lesions. Correlation between clinical and radiological findings was assessed.

Results

The types of elementary lesions detected were: ground-glass opacities (n = 24), intralobular lines (n = 24), thickened interlobular septa (n = 22), thickened fissures (n = 21), airspace consolidation (n = 16), hyperinflation (n = 16), cystic lesions (n = 2) and micronodules (n = 1). A crazy-paving pattern was found in 92% of cases. Consolidation and hyperinflation were especially detected in younger children (median age, 8 months, P < 0.01). A density dependent gradient was found. The distribution of the lesions was symmetrical. There was no correlation between radiological and clinical data of severity of the disease.

Conclusion

CT findings are suggestive of diagnosis of PAP in immunocompetent children with chronic respiratory failure.

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Titel: Primary pulmonary alveolar proteinosis: computed tomography features at diagnosis
verfasst von: Laureline Berteloot
Rola Abou Taam
Sophie Emond-Gonsard
Tania Mamou-Mani
Karen Lambot
David Grévent
Caroline Elie
Muriel Le Bourgeois
Christophe Delacourt
Francis Brunelle
Jacques de Blic
Publikationsdatum: 01.07.2014
Verlag: Springer Berlin Heidelberg
Erschienen in: Pediatric Radiology / Ausgabe 7/2014
Print ISSN: 0301-0449
Elektronische ISSN: 1432-1998
DOI: https://doi.org/10.1007/s00247-014-2888-1

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Primary pulmonary alveolar proteinosis: computed tomography features at diagnosis