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Published on: September 7, 2016
Therapeutic lung lavages in children and adults
Christian Paschen1, Karl Reiter, Franz Stanzel
1Dr. von Haunersches Kinderspital, University of Munich, Lindwurmstr. 4a, D-80337 Munich, Germany. christian-paschen@t-online.de
Insights
Therapeutic whole lung lavages effectively remove protein in Pulmonary Alveolar Proteinosis (PAP). Monitoring protein concentration during lavage helps optimize treatment, particularly for pediatric patients.
Area of Science:
- Pulmonology
- Biochemistry
- Pediatric Medicine
Background:
- Pulmonary alveolar proteinosis (PAP) is a rare condition with excessive surfactant accumulation in alveoli.
- Whole lung lavage is the primary treatment for adult PAP, but its kinetics, especially in children, are poorly understood.
Purpose of the Study:
- To investigate the kinetics of protein removal during therapeutic whole lung lavages in pediatric and adult PAP patients.
- To assess the utility of biochemical monitoring for optimizing lavage efficiency.
Main Methods:
- Retrospective analysis of 45 half lung lavages in 4 pediatric and 6 adult PAP patients.
- Measurement of total protein, protein concentration, and aberrant pro-SP-C protein in lavage fluid.
- Monitoring optical density of lavage fluid for real-time assessment.
Main Results:
- Protein removal followed an exponential decline, with varying amounts removed in adults and children.
- Pediatric patients showed elevated protein concentration per body weight.
- Optical density measurements correlated with protein concentration, enabling bedside monitoring and lavage termination.
Conclusions:
- Biochemical monitoring of therapeutic half lung lavages aids in estimating alveolar protein load.
- This approach can improve the efficiency of wash-out procedures, with particular benefit for pediatric PAP patients.
Background:
Pulmonary alveolar proteinosis (PAP) is a rare disease, characterized by excessive intra-alveolar accumulation of surfactant lipids and proteins. Therapeutic whole lung lavages are currently the principle therapeutic option in adults. Not much is known on the kinetics of the wash out process, especially in children.
Methods:
In 4 pediatric and 6 adult PAP patients 45 therapeutic half lung lavages were investigated retrospectively. Total protein, protein concentration and, in one child with a surfactant protein C mutation, aberrant pro-SP-C protein, were determined during wash out.
Results:
The removal of protein from the lungs followed an exponential decline and averaged for adult patients 2-20 g and <0.5 to 6 g for pediatric patients. The average protein concentration of consecutive portions was the same in all patient groups, however was elevated in pediatric patients when expressed per body weight. The amount of an aberrant pro-SP-C protein, which was present in one patient with a SP-C mutation, constantly decreased with ongoing lavage. Measuring the optical density of the lavage fluid obtained allowed to monitor the wash out process during the lavages at the bedside and to determine the termination of the lavage procedure at normal protein concentration.
Conclusion:
Following therapeutic half lung lavages by biochemical variables may help to estimate the degree of alveolar filling with proteinaceous material and to improve the efficiency of the wash out, especially in children.
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