title: Severe acute paracoccidioidomycosis in children.
abstract: This report describes clinical and immunologic features of five illustrative cases of paracoccidioidomycosis in previously healthy children. All had disseminated disease and two of them died despite treatment. The major clinical presentation in four patients was fever and diffuse superficial and intraabdominal adenopathy, with or without hepatosplenomegaly. Other sites were also affected: three patients had multiple osteoarticular lesions, occasionally with intense tissue destruction; two had cutaneous eruptions; two had pericardial effusions; and two had pulmonary involvement, once considered an organ spared in the young. We detected variable lymphocyte responses to mitogens and to Candida albicans antigen and non-responsiveness to Paracoccidioides brasiliensis cell wall antigen. High concentrations of serum immunoglobulins and anti-P. brasiliensis antibodies were present. These immune alterations tended to resolve with treatment, suggesting a reversible nature of the immune defect. We conclude that this mycosis has a high morbidity and mortality in children, which is probably related to an antigen-specific immunodeficiency. Further studies are needed to increase knowledge of this mycosis in children.
authors.affiliation: Immunogenetics and Experimental Transplantation Laboratory, Faculty of Medicine of São Paulo University, Brazil.
authors.forename: G
authors.initials: G
authors.lastname: Benard
authors.forename: N M
authors.initials: NM
authors.lastname: Orii
authors.forename: H H
authors.initials: HH
authors.lastname: Marques
authors.forename: M
authors.initials: M
authors.lastname: Mendonça
authors.forename: M Z
authors.initials: MZ
authors.lastname: Aquino
authors.forename: A E
authors.initials: AE
authors.lastname: Campeas
authors.forename: G B
authors.initials: GB
authors.lastname: del Negro
authors.forename: A
authors.initials: A
authors.lastname: Durandy
authors.forename: A J
authors.initials: AJ
authors.lastname: Duarte
mesh_terms: D000208:Acute Disease; D002176:Candida albicans / Q000276:immunology; D002648:Child; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007111:Immunity, Cellular; D016867:Immunocompromised Host; D008213:Lymphocyte Activation; D008297:Male; D010228:Paracoccidioides / Q000276:immunology; D010229:Paracoccidioidomycosis / Q000175:diagnosis / Q000276:immunology* / Q000503:physiopathology* / Q000628:therapy