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TL;DR
She achieved dramatic clinical improvement and avoided lifelong parenteral nutrition dependence after treatment with therapeutic plasma exchange, rituximab, and cyclophosphamide.
JPGN Reports · 5 authors, 2 centres
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She achieved dramatic clinical improvement and avoided lifelong parenteral nutrition dependence after treatment with therapeutic plasma exchange, rituximab, and cyclophosphamide.
The patient was then treated with a combination of therapeutic plasma exchange (TPE), rituximab, and cyclophosphamide. This regimen led to rapid resolution of abdominal symptoms, restoration of bowel function, and prevention of chronic intestinal failure. The authors hypothesize an antibody-mediated mechanism for the dysmotility. Limitations include the inability to perform motility studies and the single-case nature of the report, which limits generalizability. The case highlights the importance of early autoimmune evaluation in pediatric intestinal pseudo-obstruction.