title: A 15-year-old boy with severe combined immunodeficiency, fungal infection, and weight gain.
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.
authors: Section of Immunology, Allergy and Rheumatology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA.
authors: Mehtap Haktanir
authors: MH
authors: Abul
authors: Karen
authors: K
authors: Tuano
authors: C Mary
authors: CM
authors: Healy
authors: Timothy J
authors: TJ
authors: Vece
authors: Norma M
authors: NM
authors: Quintanilla
authors: Carla M
authors: CM
authors: Davis
authors: Filiz O
authors: FO
authors: Seeborg
authors: Imelda Celine
authors: IC
authors: Hanson
mesh_terms: D000293:Adolescent; D001987:Bronchiectasis / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D019819:Budesonide / Q000009:adverse effects / Q000627:therapeutic use*; D002648:Child; D046528:Chimerism / Q000139:chemically induced; D003480:Cushing Syndrome / Q000175:diagnosis* / Q000276:immunology; D004347:Drug Interactions; D000068297:Fluticasone-Salmeterol Drug Combination / Q000009:adverse effects / Q000627:therapeutic use*; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects; D006658:Histoplasma / Q000187:drug effects / Q000276:immunology*; D006660:Histoplasmosis / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D006801:Humans; D007049:Iatrogenic Disease; D007165:Immunosuppression Therapy; D007231:Infant, Newborn; D017964:Itraconazole / Q000009:adverse effects / Q000627:therapeutic use*; D008297:Male; D010375:Pedigree; D011504:Protein-Losing Enteropathies / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D016511:Severe Combined Immunodeficiency / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D015430:Weight Gain / Q000276:immunology
chemical_list: D000068297:Fluticasone-Salmeterol Drug Combination; D017964:Itraconazole; D019819:Budesonide