Acometimento hepático na paracoccidioidomicose em pacientes pediátricos : um estudo clínico, laboratorial e histológico = Hepatic involvement in pediatric patients with paràcoccidioidomycosis : a clinical, laboratory and hisptological study / Hepatic involvement in pediatric patients with paràcoccidioidomycosis : a clinical, laboratory and hisptological study

Giselle de Melo Braga

Paracoccidiodomycosis is a systemic mycosis, endemic in some countries of Latin America. The study aimed to describe the frequency, the clinical, laboratory and histological characteristics of liver involvement in children. The study has included 102 patients less than 16 years, treated between 1980 and 2010, with diagnosis confirmed by demonstration of the fungus in a pathologic examination. The clinical and laboratory data were obtained from patient charts. The liver tissue was obtained from one necropsy and 12 biopsies. Until September 2007, the biopsies were taken from patients with persistent jaundice or portal hypertension. Between October 2007 and April 2010, the indication of biopsy was elevation of aminotransferases and low level of albumin. The hematoxylin-eosin, Masson s trichrome and immunohistochemical (CK7 and CK 19) staining were used. This is the largest pediatric series that we have read about. Among 102 patients, forty one had liver involvement. The clinical findings were generalized lymphadenopathy (n=39), weight loss (n=34) and fever (n=32). Approximately one third of patients had jaundice. There was a higher proportion of boys, of undernourished and lower mean age in patients with liver involvement. The clinical presentation in these patients was more exuberant, with higher frequency of weight loss, adynamia, anorexia and pallor. There was a predominant increase of canalicular enzymes. There were differences in albumin (p<0,001) and hemoglobin levels (p=0,002) between patients with and without liver involvement, and the lowest values were found in the liver involvement group. A cutoff of albumin (<3,05g/dL) and hemoglobin (<9,2g/dL) can be used to infer liver involvement. Hypoalbuminemia is more intense in patients with liver involvement (median=2,4g/dL), witch may indicate worsening of liver function or disease complication (such as intestinal lymphangiectasia). Deaths (6) occurred only among patients with liver involvement. In liver histology, we found intense fibrosis in patients that were sick for about a month and a half and whose treatment was started few days before the biopsy. There was association between amount of fungus and severity of fibrosis. The study of ductal proliferation showed that immunochemistry is more sensitive to detect such reaction and it could be demonstrated earlier than the hematoxylin-eosin staining. There was bile duct cells lesion by inflammatory cells, which contributes in the pathogenesis of liver involvement. Liver paracoccidioidomycosis in pediatric patients has specific clinical and laboratorial presentation. Younger patients and those with severe hypoalbuminemia are more likely to present liver involvement by Paracoccidioides brasiliensis. Liver involvement is associated with more deaths. The bile duct cells lesion by inflammatory cells may explain the predominant increase of canalicular enzymes.

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