Paracoccidioidomycosis in Childhood and Adolescence: Clinical-Epidemiological Review of South American Cases (1970-2023).

Abstract

Paracoccidioidomycosis (PCM) is a severe systemic mycosis classified as a neglected disease, predominantly found in South America, with Brazil reporting the highest number of cases. In children and adolescents, PCM primarily manifests as an acute/subacute (juvenile) form. This systematic review focused on PCM cases in individuals under 15 years of age, analyzing 163 reports published between 1970 and 2023. The literature search was conducted across LILACS, SciELO, and PubMed databases using DeCS and MeSH descriptors in Portuguese, English, and Spanish.Most cases (70%) occurred in Brazil, followed by Argentina, Peru, Venezuela, and Colombia. The mean age was 8.4 years, with a male predominance (63%) and a median diagnostic delay of six months, reflecting underreporting and late disease recognition. The predominant clinical presentation was the disseminated form (90%), with generalized lymphadenopathy as the most frequent manifestation. Common symptoms included fever (66%), weight loss (54%), hepatomegaly (30%), and splenomegaly (25%). Severe complications such as lymph node fistulization (27%) and bone involvement (16%) were associated with poorer prognosis.Among the cases, 53% were classified as severe, frequently exhibiting marked eosinophilia (> 25,000 cells/mm³), ascites, and visceral involvement. Seven fatalities (5%) were recorded, strongly associated with hepatosplenomegaly (86% of fatalities vs. 22% of survivors), significant eosinophilia (71% vs. 15%), and rapid diagnosis (< 3 months). A predictive model combining these factors achieved 85.7% sensitivity and 92.3% specificity for mortality. Regarding therapy, combined antifungal regimens (e.g., amphotericin B plus itraconazole) achieved a 25% cure rate, while monotherapy with trimethoprim-sulfamethoxazole (TMP-SMX) was associated with 49% of fatalities.Pediatric PCM remains a neglected and highly lethal disease in severe forms. Early diagnosis, appropriate therapeutic combinations, and monitoring of clinical-laboratory markers with accurate classification are crucial for improved outcomes. The absence of detailed data in 34% of cases highlights gaps in clinical records, underscoring the need for prospective studies and standardization of severity criteria.