Criptococose disseminada fatal em criança com síndrome Hyper-IgM

• Ano de defesa: 2016
• Autor(a) principal: Suzuki, Simone Mancini Liduário
• Tipo de documento: Dissertação
• Tipo de acesso: Acesso aberto
• dARK ID: ark:/35916/0013000003kzw
• Idioma: por
• Instituição de defesa: Universidade Estadual de Maringá; Brasil; Programa de Pós-Graduação em Ciências da Saúde; UEM; Maringá, PR; Centro de Ciências da Saúde
• Palavras-chave em Português: Síndrome Hyper-IgM; Cryptococose; Cryptococcus; Micoses; Brasil.; Hyper-IgM syndrome; Cryptococcosis; Brazil.; Ciências da Saúde; Medicina
• Link de acesso: http://repositorio.uem.br:8080/jspui/handle/1/2030

Cryptococcosis is an opportunist mycosis common worldwide, the incidence has increased in recent years. It is more common in patients with HIV / AIDS and transplant, but has been linked there are other immunocompromised. The hyper-IgM syndrome, X-linked (XHIGM) is a primary immune deficiency disorder, rare caused by mutations in the gene encoding the production of CD40L. Characterized by normal or elevated levels of IgM and diminished levels or normal IgG and IgA. They were only recorded four cases of XHIGM associated with cryptococcosis. The objective of this study was to evaluate microbiological, pathogenic and its response to therapy in a patient with isolated fungus XHIGM.Trata syndrome is a retrospective descriptive study of an unusual case, in patients five years of age XHIGM carrier syndrome, definitive diagnosis was later evolving to death. We conducted a field diary, isolation and identification of patient samples fungus. The microbiological diagnosis was performed by examination with ink China spinal cerebrospinal fluid (CSF) and revealed typical encapsulated yeast Cryptococcus genus. The CSF chocolate agar culture were revealed rounded yeast unibrotantes, capsulated, phenol oxidase positive Agar Niger, urease positive, which were identified by phenotypic characterization as Cryptococcus neoformans by means of canavanine-glycine-bromothymol. Antifungal susceptibility testing the indicated minimum inhibitory concentrations (0.5 mg / L) for amphotericin B (4.0 mg / L) and fluconazole (0.12 mg / L) for voriconazole. The average size of the capsule clinical isolate corresponds to the average of C. neoformans ATCC 40283. The capsule pathogenicity evaluation made by experimental infection in Balb / C mice showed spread of fungus reached the spleen, lung and brain, causing significant macroscopic changes of texture and size thereof. Treatment with amphotericin B reduced the fungal load in the lungs and spleen but not in brain. We conclude that patient outcome can not be attributed solely to the weakness of the host, or the virulence of the agent or the strength of the antifungals, but probably the sum of all these factors.