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[Immunologic disorders in children with developmental thoracic defects]
Insights
Suppurative complications after thoracoplasty in children are linked to immune system disorders. Specific syndromes like Marfan syndrome show significant immune deviations, increasing complication risks.
Area of Science:
- Immunology
- Pediatric Surgery
- Genetics
Context:
- Thoracoplasty is a surgical procedure for chest wall deformities.
- Connective tissue dysplasias are a group of genetic disorders affecting connective tissues.
- Suppurative complications are a known risk following thoracoplasty.
Purpose:
- To investigate the relationship between immune status and suppurative complications in children undergoing thoracoplasty.
- To compare immunological profiles in children with isolated chest defects versus those with hereditary connective tissue syndromes.
Summary:
- Immunological examination of 21 children with chest defects and postoperative analysis of 136 children (36 with connective tissue syndromes) revealed that immune status disorders significantly contribute to suppurative complications post-thoracoplasty.
- Children with Marfan syndrome exhibited the most severe immune deviations, including impaired neutrophil and monocyte phagocytic activity, reduced lymphocyte counts (T, T active, B), and diminished T helper function.
- Similar, though less profound, immunological issues were noted in unclassified developmental defects with Marfanoid phenotypes. Ehlers-Danlos syndrome showed decreased immunocompetent cells, T helper function, and neutrophils. Isolated funnel chest cases presented with reduced monocyte function and immunoglobulin levels.
Impact:
- Findings highlight the critical role of immune status in predicting and potentially preventing postoperative complications in pediatric thoracoplasty patients.
- Identifies specific immune deficits associated with different connective tissue syndromes, informing targeted perioperative management strategies.
- Suggests that preoperative immunological assessment could be beneficial for high-risk pediatric patients undergoing chest wall reconstruction.
Abstract:
As the result of immunological examination of 21 children with developmental defects of the chest and analysis of the course of the postoperative period in 136 children, among which 36 had hereditary syndromes of systemic connective-tissue dyshistogenesis, it was found that suppurative complications of thoracoplasty, which are encountered in 15% of children with isolated developmental chest defects and in 33.3% of those with the above mentioned syndromes, were caused to a great measure by disorders of the immune status. The most serious immunological deviations were encountered in the Marfan syndrome due to impaired phagocytic activity of neutrophils and monocytes, decreased number of T, T active, and B lymphocytes, and diminished function of T helpers. In unclassified complexes of developmental defects with Marfaneic ++ phenotypes, the immunological disorders were similar, but less deep. In the Ehlers-Danlos syndrome, a decrease of the number of immunocompetent cells, function of T helpers, and neutrophils was mainly revealed. In isolated forms of funnel chest the function of monocytes and the number of immunoglobulins are mainly decreased.