Issue |
J Extra Corpor Technol
Volume 26, Number 1, March 1994
|
|
---|---|---|
Page(s) | 40 - 43 | |
DOI | https://doi.org/10.1051/ject/199426140 | |
Published online | 21 August 2023 |
Case Report
Pulmonary Surfactant Protein B Deficiency: A Case Report
St. Louis University School of Allied Health Professions, St. Louis Children's Hospital, St. Louis, Missouri
* Address correspondence to: Verna E. Nelson, BS, St. Louis University Health Sciences Center, Surgery Department, Perfusion Division, 3635 Vista Avenue at Grand Blvd., St. Louis, Missouri 63110-0250
The first reported case of pulmonary alveolar proteinosis (PAP) was documented in 1958. Since then, more cases have become evident of this unusual disease. PAP is a disease characterized by positive periodic acid-Schiff, diastase-resistant granular eosinophilic material within the alveoli. Individuals with PAP have impaired gas exchange and progressive hypoxemia. In congenital PAP, newborn infants show severe and progressive pulmonary failure at birth which leads to death. In one family, a male sibling was born with severe respiratory distress syndrome and diagnosed with congenital alveolar proteinosis (CAP) by open lung biopsy. His female sibling died at one month of age nineteen years earlier with severe respiratory disease. Her autopsy slides were consistent with CAP. This case sibling followed the same clinical course as his brother and sister diagnosed with CAP by family history. Surfactant protein 'B' deficiency was identified as the cause of the congenital alveolar proteinosis. Aggressive therapy was initiated in the case sibling with exogenous surfactant replacements, extracorporeal membrane oxygenation (ECMO) as a bridge to transplant, lung lavage, and maximum ventilator settings via conventional mechanical ventilation. The child died on ECMO day 29 after 696 hours of support.
Key words: congenital alveolar proteinosis / surfactant protein B deficiency / extracorporeal membrane oxygenation / exogenous surfactant replacement
© 1994 AMSECT
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