title: Chronic mucocutaneous candidiasis in childhood and complications of non-Candida infection: a report of the Pediatric Immunodeficiency Collaborative Study Group.
abstract: We reviewed the clinical course in 43 patients from eight medical centers who were given the diagnosis of chronic mucocutaneous candidiasis, a rare disorder of unknown cause that may occur in childhood. Recurrent or severe infections with organisms other than Candida were seen in 80% of the patients. There were nine cases of septicemia. Seven patients have died; six of these deaths were directly related to non-Candida infectious complications. Endocrine dysfunction, including Addison disease (11 patients) and hypothyroidism (9 patients), was seen in 19 of 43 patients. Immunologic studies failed to reveal a consistent abnormality, although two of five patients with reversed T4/T8 ratios are among those who have died. Ketoconazole was effective in controlling symptoms of candidiasis in most patients. The findings from this study indicate that non-Candida infections cause serious morbidity and may result in death in patients with chronic mucocutaneous candidiasis.
authors.affiliation: Department of Pediatrics, University of Tennessee, Memphis 38163.
authors.forename: H G
authors.initials: HG
authors.lastname: Herrod
mesh_terms: D000293:Adolescent; D000666:Amphotericin B / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000453:epidemiology; D002177:Candidiasis / Q000150:complications*; D002178:Candidiasis, Chronic Mucocutaneous / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D004700:Endocrine System Diseases / Q000150:complications; D005190:Family; D005260:Female; D005437:Flucytosine / Q000627:therapeutic use; D006801:Humans; D007070:Immunoglobulin A / Q000378:metabolism; D007074:Immunoglobulin G / Q000378:metabolism; D015994:Incidence; D007223:Infant; D007239:Infections / Q000150:complications* / Q000453:epidemiology / Q000401:mortality; D007654:Ketoconazole / Q000009:adverse effects / Q000627:therapeutic use; D008171:Lung Diseases / Q000150:complications; D008297:Male; D015996:Survival Rate
chemical_list: D007070:Immunoglobulin A; D007074:Immunoglobulin G; D000666:Amphotericin B; D005437:Flucytosine; D007654:Ketoconazole