Pulmonary Alveolar Microlithiasis in Children Less than 5 Years of Age.

Autor: Sigur E; Children Hospital, Pediatric Pulmonology and Allergology Unit CHU Toulouse, Toulouse, France. Electronic address: elodiesigur@yahoo.fr., Roditis L; Children Hospital, Pediatric Pulmonology and Allergology Unit CHU Toulouse, Toulouse, France., Labouret G; Children Hospital, Pediatric Pulmonology and Allergology Unit CHU Toulouse, Toulouse, France., Bieth E; Genetic Unit CHU Toulouse, Toulouse, France., Simon S; Children Hospital, Pediatric Radiology Unit CHU Toulouse, Toulouse, France., Martin-Blondel A; Children Hospital, Pediatric Pulmonology and Allergology Unit CHU Toulouse, Toulouse, France., Michelet M; Children Hospital, Pediatric Pulmonology and Allergology Unit CHU Toulouse, Toulouse, France., Mittaine M; Children Hospital, Pediatric Pulmonology and Allergology Unit CHU Toulouse, Toulouse, France., Blanchon S; Children Hospital, Pediatric Pulmonology and Allergology Unit CHU Toulouse, Toulouse, France; Woman-Mother-Child, Service of Pediatrics, Pediatric Pulmonology Unit, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.
Jazyk: angličtina
Zdroj: The Journal of pediatrics [J Pediatr] 2020 Feb; Vol. 217, pp. 158-164.e1. Date of Electronic Publication: 2019 Nov 21.
DOI: 10.1016/j.jpeds.2019.10.014
Abstrakt: Objective: To collect all published cases up to January 2019 of pulmonary alveolar microlithiasis (PAM) in patients age 5 years and under and to compare their characteristics with those of the 1022 cases in the most recent all-age cohort published in 2015.
Study Design: We identified 28 cases of PAM worldwide in children age 5 years and under, accounting for only 2%-3% of all cases.
Results: Children seem more frequently symptomatic, notably with more cough and severe acute respiratory failure, but had no reported extrapulmonary manifestation. Children with PAM evidenced less typical radiologic findings, with frequent ground glass opacities not reported in adult cases and milder calcifications as less frequent, smaller, and mainly restricted to the lower lobes.
Conclusions: PAM remains an uncommon diagnosis in young children, as symptoms and radiologic findings are less specific. Physicians should be aware to look for calcifications in chest computed tomography at mediastinal window and avoid elution of the bronchoalveolar lavage to find microliths. Collecting longitudinal data through an international registry would help in characterizing PAM to predict disease progression and plan lung transplantation.
(Copyright © 2019 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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