Caroli's disease: report of 10 cases in children and adolescents in southern Brazil.

PURPOSE: The aim of this study was to describe the authors' experience with Caroli's disease in children and adolescents. METHODS: The authors reviewed the hospital charts of 10 children and adolescents with Caroli's disease diagnosed between 1968 and 1996. RESULTS: The median age at the onset of symptoms was 5.5 months and the median age at diagnosis was 12 months, both much lower than those reported in the literature. Clinical symptoms were compatible with the classical findings of Caroli's disease, but jaundice and hepatosplenomegaly occurred more frequently. There was an association with congenital renal malformation in eight cases (80%), congenital hepatic fibrosis in five cases, and choledochal cyst in two cases. One case presented the pure form of the disease.

Postradiation pleomorphic malignant fibrous histiocytoma of the breast.

A case of primary malignant fibrous histiocytoma (MFH) of the breast occurring two years after surgical excision and radiation therapy for a carcinoma of the left breast is reported. Fine needle aspiration was positive for malignant cells, consistent with a pleomorphic sarcoma. Cytologic examination revealed giant cells with marked pleomorphism. Some cells showed single large nuclei with cytoplasmic vacuoles while others revealed multinucleation with foamy cytoplasm, phagocytosed erythrocytes and cellular debris. These findings are considered useful in the cytologic diagnosis of the pleomorphic variant of MFH.

Relationship between nutritional status and histologic findings in small bowel mucosa of children presenting with diarrhoea of more than 14 days' duration.

In order to study the eventual effects of malnutrition on small intestinal mucosa, we evaluated 85 children with diarrhoea of more than 14 days' duration, aged from 4 to 114 months (median 17 months). A proximal small intestinal biopsy was obtained and villus height, crypt depth, mucosal thickness, and total mucosal thickness were measured. Gomez, Waterlow, and Z score criteria were applied. Statistical analyses were performed with the Spearman correlation test and the non-parametrical tests of Wilcoxon, Mann-Whitney, and Kruskal-Wallis. A value of p < 0.05 was considered significant. Average villus height was 269.2 microns (+/- 87.5 microns); crypt depth 113.0 microns (+/- 33.8 microns); mucosal thickness 210.5 microns (+/- 73.2 microns); total mucosal thickness 485.9 microns (+/- 111.8 microns); and villus height/crypt depth ratio 2.5:1 (+/- 0.8:1). Five children had kwashiorkor and 13 had marasmus. Villus height for kwashiorkor children ranged from 151 microns to 353.3 microns (average 286.7 microns), crypt depth from 90.3 microns to 154 microns (average 111.11 microns). According to Gomez criteria, as malnutrition increased, mucosal thickness and the villus/crypt ratio decreased. Waterlow criteria had no relation to mucosal sizes. When distributed in sequential decrease according to their nutritional state, the Z score for weight for age and weight for height indices showed a positive correlation with villus height, total mucosal thickness, and villus/crypt ratio.

Estudo citologico em nodulos da tiroide por biopsia de aspiracao. / Cytological study in thyroid nodules using aspiration biopsy

Os autores fazem um estudo comparativo entre os resultados dos exames citopatologicos de material obtido por puncao aspirativa, em 105 casos de nodulos isolados da tireoide, com os resultados dos exames anatomopatologicos por inclusao em parafina