RESUMO
Paracoccidioidomycosis (PCM) is a neglected systemic mycosis endemic to Latin America caused by dimorphic fungi of the genus Paracoccidioides. The acute juvenile PCM is a severe type of presentation that usually affects young vulnerable patients and rarely progresses to portal hypertension. Here, two cases of liver disease and portal hypertension as complications of acute juvenile PCM are reported. Diagnosis of PCM was performed by isolation of the fungus and molecular identification of the strains provided through partial sequencing of two protein encoding genes, arf and gp43. Genotypic analysis revealed that Paracoccidioides brasiliensis S1 was the phylogenic species involved in both cases. Patients presented a good clinical response to amphotericin B and sulfamethoxazole-trimethoprim. These results highlight the importance of the interdisciplinary approach in patients with severe forms of PCM to avoid and treat complications, and the necessity of further investigations focusing on host-pathogen interaction in order to explain the broad clinical spectrum in PCM as well as the severity and poor outcome in some clinical cases.
Assuntos
Hipertensão Portal/etiologia , Hipertensão Portal/patologia , Hepatopatias/etiologia , Hepatopatias/patologia , Paracoccidioides/isolamento & purificação , Paracoccidioidomicose/complicações , Paracoccidioidomicose/diagnóstico , Adolescente , Adulto , Anfotericina B/uso terapêutico , Feminino , Proteínas Fúngicas/genética , Humanos , América Latina , Masculino , Paracoccidioides/classificação , Paracoccidioides/genética , Paracoccidioidomicose/tratamento farmacológico , Filogenia , Análise de Sequência de DNA , Resultado do Tratamento , Combinação Trimetoprima e Sulfametoxazol/uso terapêuticoRESUMO
We present two cases of juvenile form of paracoccidioidomycosis (PCM), a systemic mycosis frequently found in rural areas, whose prognosis is poor in children and young adults. They are a 14-year-old boy and a 25-year-old woman, both residents in an urban area in São Paulo - Brazil, without any history of travelling to an endemic area. They have been admitted to the hospital due to fever, weight loss and lymphadenopathy. The diagnosis was confirmed by serologic and histopathologic study. Patients have recovered after therapy with oral itraconazole and were discharged from hospital, maintaining outpatient visits. In this article, the authors discuss the unusual presentation of PCM in an urban area.
