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

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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Bronchiectasis / diagnosis*
  • Bronchiectasis / etiology
  • Bronchiectasis / therapy
  • Budesonide / adverse effects
  • Budesonide / therapeutic use*
  • Child
  • Chimerism / chemically induced
  • Cushing Syndrome / diagnosis*
  • Cushing Syndrome / immunology
  • Drug Interactions
  • Fluticasone-Salmeterol Drug Combination / adverse effects
  • Fluticasone-Salmeterol Drug Combination / therapeutic use*
  • Hematopoietic Stem Cell Transplantation* / adverse effects
  • Histoplasma / drug effects
  • Histoplasma / immunology*
  • Histoplasmosis / diagnosis*
  • Histoplasmosis / etiology
  • Histoplasmosis / therapy
  • Humans
  • Iatrogenic Disease
  • Immunosuppression Therapy
  • Infant, Newborn
  • Itraconazole / adverse effects
  • Itraconazole / therapeutic use*
  • Male
  • Pedigree
  • Protein-Losing Enteropathies / diagnosis*
  • Protein-Losing Enteropathies / etiology
  • Protein-Losing Enteropathies / therapy
  • Severe Combined Immunodeficiency / complications
  • Severe Combined Immunodeficiency / diagnosis*
  • Severe Combined Immunodeficiency / therapy
  • Weight Gain / immunology

Substances

  • Fluticasone-Salmeterol Drug Combination
  • Itraconazole
  • Budesonide