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A 15-year-old boy with severe combined immunodeficiency, fungal infection, and weight gain.

作者信息

Abul Mehtap Haktanir, Tuano Karen, Healy C Mary, Vece Timothy J, Quintanilla Norma M, Davis Carla M, Seeborg Filiz O, Hanson Imelda Celine

机构信息

Section of Immunology, Allergy and Rheumatology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA.

出版信息

Allergy Asthma Proc. 2015 Sep-Oct;36(5):407-11. doi: 10.2500/aap.2015.36.3876.

Abstract

Hematopoietic stem cell transplantation (HSCT) outcomes in X-linked severe combined immune deficiency are most effective when performed with patients <3 months of age and without coexisting morbidity, and with donor cells from a matched sibling. Even under such favorable circumstances, outcomes can be suboptimal, and full cellular engraftment may not be complete, which results in poor B or natural killer cell function. Protein losing enteropathies can accompany persistent immune deficiency disorders with resultant low serum globulins (immunoglobulin A [IgA], IgG, IgM) and lymphopenia. Patients with immune disorders acquire infections that can be predicted by their immune dysfunction. Fungal infections are typically noted in neutropenic (congenital or acquired) and T-cell deficient individuals. Coexisting fungal infections are rare, even in hosts who are immunocompromised, and they require careful evaluation. Antifungal treatment may result in drug-drug interactions with significant complications.

摘要

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