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
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Abstract

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.

Keywords

Congenital alveolar proteinosis Treatment Pulmonary lavage Surfactant 

Abbrevations

CAP

Congenital alveolar proteinosis

PAP

Pulmonary alveolar proteinosis

RDS

Respiratory distress syndrome

ICU

Intensive care unit

EDTA

Ethylene-diamine tetraacetic acid (edetic acid)

SIMV

Synchronized intermittent mandatory ventilation

CPAP

Continuous positive airway pressure

PEEP

Positive end-expiratory pressure

GM-CSF

Granulocyte-macrophage colony-stimulating factor

PAS

Periodic acid–Schiff

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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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