Brief report: deficiency of pulmonary surfactant protein B in congenital alveolar proteinosis

Article Abstract:

Deficiency of pulmonary surfactant protein B (SP-B) may cause congenital pulmonary alveolar proteinosis. Congenital pulmonary alveolar proteinosis is a rare form of respiratory failure among infants that causes death within one year of birth. Pulmonary surfactant is a mixture of lipids and proteins that reduces surface tension in the lungs, and SP-B is thought to be essential to surfactant function. Lung tissue samples were analysed from two siblings with congenital pulmonary alveolar proteinosis and from control patients undergoing lung transplantation. SP-B was detected in the samples from the controls but not in the samples from the two siblings. In an RNA analysis of samples from one of the affected siblings and all of the controls, messenger RNA for SP-B was detected in all of the controls but not in the affected sibling. These findings suggest congenital pulmonary alveolar proteinosis may be an inherited disorder of SP-B production.

Causes of, Respiratory insufficiency, Pulmonary alveolar proteinosis

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ABCA3 gene mutations in newborns with fatal surfactant deficiency

Article Abstract:

The ATP-binding cassette transporter A3 (ABCA3) is extremely important for the proper formation of lamellar bodies, surfactant function and also may be required for lung function in various pulmonary diseases. Infact, the mutation of the ABCA3 gene would lead to surfactant deficiency among newborns that may turn to be fatal.

Science & research, England, Care and treatment, Research, Risk factors, Neonatal diseases, Adenosine triphosphatase genes, ATP genes

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A mutation in the surfactant protein C gene associated with familial interstitial lung disease

Article Abstract:

Researchers discovered a mutation in the gene for surfactant protein C in a six-week-old baby who developed interstitial lung disease and her mother. This protein is a component of surfactant, a natural substance that coats the lungs and prevents them from collapsing.

Health aspects, Abnormalities, Lung diseases, Interstitial, Pulmonary surfactant, Interstitial lung diseases, Pulmonary surfactants

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