Early Onset Granulomatous Colitis Associated with a Mutation in NCF4 Resolved with Hematopoietic Stem Cell Transplantation.

Wright, Mathew; Chandrakasan, Shanmuganathan; Okou, David T; Yin, Hong; Jurickova, Ingrid; Denson, Lee A; Kugathasan, Subra · J Pediatr · 2019

case_report · Level V

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Abstract

A 4-year-old boy presented with perianal abscess and granulomatous colitis, which led the diagnosis of Crohn's disease. He became refractory to all available therapies and required colectomy. Targeted sequencing revealed a deleterious variant in NCF4, causing severe neutrophil dysfunction. He underwent hematopoietic stem cell transplantation (HSCT) with an excellent outcome.

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