Surfactant Protein C Gene (SFPTC) Mutation-Associated Lung Disease: High-Resolution Computed Tomography (HRCT) Findings and its Relation to Histological Analysis - Archive ouverte HAL Accéder directement au contenu
Article Dans Une Revue Pediatric Pulmonology Année : 2010

Surfactant Protein C Gene (SFPTC) Mutation-Associated Lung Disease: High-Resolution Computed Tomography (HRCT) Findings and its Relation to Histological Analysis

Meriem Mechri
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Ralph Epaud
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Aurore Coulomb
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Francis Jaubert
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Delphine Feldmann
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Ailbhe Tarrant
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Résumé

Aim of the study Determine high-resolution tomography (HRCT) scan characteristics in children with SFTPC mutation and correlate them to histological findings. Patients and Methods This retrospective multicenter study included 15 children (7 females and 8 males) with SFTPC mutations. HRCT scans have been performed in all the children and lung biopsies in 8 children. Results From all signs assessed on initial HRCT scans, ground-glass opacities (n=14, 93 %) and lung cysts (n=6, 40 %) were predominant. Interlobular septal thickening (n=1, 7 %), air space consolidation (n=1, 7 %), paraseptal emphysema (n=2, 13 %), and pulmonary nodules (n=1, 7 %) were also found. Histological analysis revealed accumulation of macrophages in the alveolar lumen, type II pneumocyte hyperplasia, and alveolar septal thickening. Dilatation of the respiratory bronchiole and alveolar duct associated with muscular hyperplasia were also described. Interestingly, lung cysts on HRCT scans were associated with dilatation of terminal bronchioli and alveolar duct in lung biopsies. Conclusion In children with SFTPC mutations, HRCT scan finding were highly correlated to the histological findings and, as such, represent a useful tool to identify patients that may require SFTPC gene sequencing.
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Dates et versions

hal-00558627 , version 1 (23-01-2011)

Identifiants

Citer

Meriem Mechri, Ralph Epaud, Sophie Emond, Aurore Coulomb, Francis Jaubert, et al.. Surfactant Protein C Gene (SFPTC) Mutation-Associated Lung Disease: High-Resolution Computed Tomography (HRCT) Findings and its Relation to Histological Analysis: HRCT and histological finding of SFTPC mutations. Pediatric Pulmonology, 2010, 45 (10), pp.1021. ⟨10.1002/ppul.21289⟩. ⟨hal-00558627⟩
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