The Indian Journal of Pediatrics

, Volume 84, Issue 9, pp 721–723 | Cite as

Congenital Pulmonary Alveolar Proteinosis: From Birth to Ten-years of Age

Clinical Brief


Pulmonary alveolar proteinosis is a rare lung disease in which lipoproteinaceous material accumulates within the alveoli, interfering with gas exchange. The disease is classified into congenital, secondary, and acquired. The congenital form includes inborn errors of surfactant metabolism, lysinuric protein intolerance and mutations in the components of granulocyte-macrophage colony-stimulating factor receptor. The main symptoms are non-specific. The radiologic appearance of pulmonary alveolar proteinosis is bilateral, symmetric and perihilar airspace consolidation. Bronchoalveolar lavage is crucial for diagnosis of the disease. There is only one ten-year-old patient with diagnosed congenital form in Croatia. What makes him different from other children in the world is that since the ninth month of his life he has been mechanically ventilated. Diagnosis of postnatal alveolar proteinosis should be considered in every infant with respiratory distress with diffuse alveolar and interstitial infiltrate.


Congenital alveolar proteinosis Treatment Pulmonary lavage Surfactant 



Congenital alveolar proteinosis


Pulmonary alveolar proteinosis


Respiratory distress syndrome


Intensive care unit


Ethylene-diamine tetraacetic acid (edetic acid)


Synchronized intermittent mandatory ventilation


Continuous positive airway pressure


Positive end-expiratory pressure


Granulocyte-macrophage colony-stimulating factor


Periodic acid–Schiff



SAK: Conception and design of the case report and drafted the manuscript. LP: Critical revision of the manuscript, final approval of the version to be published and agreement to be accountable for all aspects of the work. Both the authors have participated sufficiently in the work to take public responsibility for the contents. LP will act as guarantor for the paper.

Compliance with Ethical Standards

Conflict of Interest


Source of Funding



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Copyright information

© Dr. K C Chaudhuri Foundation 2017

Authors and Affiliations

  1. 1.Department of Anesthesiology, Reanimatology and Intensive CareChildren’s Hospital ZagrebZagrebCroatia

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