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Intestinal transplantation in children with multiple intestinal atresias and immunodeficiency
Author(s) -
Fischer Ryan T.,
Friend Brian,
Talmon Geoffrey A.,
Grant Wendy J.,
QuirosTejeira Ruben E.,
Langnas Alan N.,
Coccia Peter F.
Publication year - 2014
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/petr.12211
Subject(s) - medicine , incidence (geometry) , immunodeficiency , transplantation , complication , biliary atresia , primary immunodeficiency , atresia , surgery , pediatrics , gastroenterology , immunology , disease , immune system , physics , optics , liver transplantation
GVHD has been reported in 8–10% of children after small bowel transplant ( SBT x). Immunodeficient children may be predisposed to aggressive, steroid‐resistant GVHD . There exists a unique association of immunodeficiency in children with MIA ( MIAI ). We report on our SBT x experience in patients with the diagnosis of MIAI , their high incidence of GVHD , and the possible role of stem cell transplantation in these patients. We performed a review of records from children that underwent SBT x or that we evaluated for SBT x at our institution. We focused on the diagnoses of atresia, multiple intestinal atresia, immunodeficiency, and GVHD in our patient population. Children with MIAI are likely to experience severe GVHD following SBT x. MIAI correlated with a 100% incidence of GVHD in these patients. Of the five patients with MIAI that underwent SBT x, three succumbed to severe GVHD within 1–6 months after SBT x. One patient received stem cell transplant prior to SBT x and did not develop severe GVHD , but died from influenza nine months after SBT x. Our unique patient survives long‐term, with engraftment of donor γ δ T cells. He has mild, persistent chronic GVHD . Atresia is a common referral diagnosis for SBT x. Patients with multiple atresias, especially MIAI , are at significant risk for the complication of GVHD following SBT x. We recommend careful immunologic assessment and antecedent stem cell transplant in children with MIAI prior to SBT x.