Pulmonary alveolar proteinosis in children on La Reunion Island: a new inherited disorder?

ABSTRACT:

Background

Pulmonary alveolar proteinosis (PAP) is very rare in children. Only a few small series have been published, with little information about long-term progression. The objective of our study was to describe the clinical, radiological and pathological features, and the long-term course of PAP in a cohort of 34 children from La Réunion Island.

Methods

Data were retrospectively collected from medical files. Radiological and pathological elements were reviewed by two pediatric radiologists and three pathologists, respectively.

Results

Thirteen cases were familial and 32/34 (94%) cases were family connected. Disease onset occurred in the first six months of life in 82% of the patients. Thoracic computed tomography scans showed the typical "crazy-paving" pattern in

SUBMITTER: Enaud L 

PROVIDER: S-EPMC4062771 | biostudies-literature | 2014 Jun

REPOSITORIES: biostudies-literature