Abstract

BackgroundParacoccidioidomycosis (PCM) is a systemic mycosis caused by pathogenic dimorphic fungi of the genus Paracoccidioides. It is the most important systemic mycosis in Latin America and the leading cause of hospitalizations and death among them in Brazil. Acute PCM is less frequent but relevant because vulnerable young patients are affected and the severity is usually higher than that of the chronic type.MethodsThe authors performed a retrospective cohort study from 2001 to 2009 including acute juvenile PCM patients from a reference center in Rio de Janeiro, Brazil. Clinical, epidemiological, diagnostic, therapeutic, and prognostic data were reported.ResultsTwenty-nine patients were included. The average age was 23 years old and the male to female ratio was 1:1.07. All cases were referred from 3 of 9 existing health areas in the state of Rio de Janeiro, predominantly from urban areas (96.5%). Lymph nodes were the most affected organs (100%), followed by the skin and the spleen (31% each). Twenty-eight patients completed treatment (median 25 months) and progressed to clinical and serological cure; 1 death occurred. Twenty-four patients completed 48-month median follow-up. Four patients abandoned follow-up after the end of treatment. The most frequent sequela was low adrenal reserve. Paracoccidioides brasiliensis S1 was identified by partial sequencing of the arf and gp43 genes from 4 patients who presented a viable fungal culture.ConclusionAcute juvenile PCM is a severe disease with a high rate of complications. There are few cohort clinical studies of acute PCM in the literature. More studies should be developed to promote improvement in patients’ healthcare.

Highlights

  • Paracoccidioidomycosis (PCM) is a severe systemic mycosis endemic to Latin America [1]

  • Paracoccidioides brasiliensis S1 was identified by partial sequencing of the arf and gp43 genes from 4 patients who presented a viable fungal culture

  • The present study aims to perform a descriptive analysis of epidemiological, clinical, therapeutic, and prognostic data in a cohort of patients with acute PCM, evaluated in a reference center for this mycosis in Rio de Janeiro state, Brazil, an important endemic area

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Summary

Introduction

Paracoccidioidomycosis (PCM) is a severe systemic mycosis endemic to Latin America [1]. Primary pathogenic dimorphic fungi of the genus Paracoccidioides are the etiological agents of this disease and, according to the literature; the remarkable genetic diversity between phylogenetic species seems to cause variations in clinical presentation, therapeutic response, diagnosis, and prognosis [5,6,7] These hypotheses are based on observations of a few case reports with molecular identification of the fungus [8,9,10,11]. Acute PCM, known as juvenile-type PCM, corresponds to 3% to 5% of all PCM cases but is considered the most severe clinical form of this mycosis because it affects vulnerable young or, less frequently, immunocompromised people, usually presenting as a disseminated disease involving the mononuclear phagocyte system including the lymph nodes, liver, spleen, and bone marrow [12]. Acute PCM is less frequent but relevant because vulnerable young patients are affected and the severity is usually higher than that of the chronic type

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