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Pneumatosis intestinale in children with primary combined immunodeficiency
1Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA.
Insights
Pneumatosis intestinale (PI) in children with primary combined immunodeficiency is usually benign and treatable with conservative therapy. Consider PI in immunocompromised children with unexplained gastrointestinal issues, especially post-transplant.
Area of Science:
- Pediatric Gastroenterology
- Immunology
- Neonatal Medicine
Background:
- Primary combined immunodeficiency (CVID) compromises the immune system, increasing susceptibility to infections.
- Gastrointestinal complications are common in immunocompromised patients.
- Pneumatosis intestinale (PI), or air in the bowel wall, can occur in various clinical settings.
Observation:
- Six cases of PI were observed in children diagnosed with primary combined immunodeficiency.
- The PI episodes were generally not severe and did not require invasive interventions.
- Patients typically responded well to conservative management strategies.
Findings:
- PI in this cohort was predominantly benign.
- Conservative therapy, such as supportive care and management of underlying conditions, was effective in resolving PI.
- Potential predisposing factors include increased susceptibility to infection and disruption of the gastrointestinal mucosa.
Implications:
- PI should be considered in the differential diagnosis of gastrointestinal symptoms in children with primary combined immunodeficiency.
- Early recognition and conservative management can lead to favorable outcomes.
- Further research into the specific mechanisms linking CVID and PI is warranted.
Abstract:
We report six episodes of pneumatosis intestinale (PI) in children with primary combined immunodeficiency. PI was generally benign and responded to conservative therapy. Increased risk of infection or disruption of gastrointestinal mucosa are probable predisposing factors. A diagnosis of PI should be considered in children with primary combined immunodeficiency who experience otherwise unexplained gastrointestinal symptoms, particularly in the early post-transplant period.