Fabry disease is an X-linked glycosphingolipidosis caused by deficiency of alpha-galactosidase. Progressive chronic kidney disease (CKD) is a major cause of morbidity and mortality in males. Although 40% of heterozygous females may develop renal involvement, pathologic data on Fabry nephropathy in heterozygotes are scarce. We reviewed the kidney biopsies of four affected females who had normal to slightly sub-normal renal function, two of them with overt proteinuria. Chronic non-specific degenerative lesions and glycosphingolipid accumulation per cell type were semi-quantitatively assessed by light and electron microscopy. Cellular distribution of glycosphingolipid deposits was best assessed on semithin sections. Podocyte effacement was seen only in proteinuric patients. Combined analysis of our data with those of two earlier series showed that glomerular sclerosis and tubulointerstitial fibrosis are predictors of proteinuria and CKD stage. There was no histopathological evidence supporting a major role of vascular damage in the early pathogenesis of Fabry nephropathy in females.

There was an error in Table 6. The error was limited to the data shown in the table and did not affect any of the statistical analyses presented in the Results and Discussion, which were based on a SPSS file that the authors have double-checked for accuracy. The corrected table is reproduced here. The authors very much regret their error.

Um leitão oriundo de um criatório do norte do estado do Rio de Janeiro, mantido no biotério do Hospital Veterinário da Universidade Estadual do Norte Fluminense Darcy Ribeiro e utilizado como caso piloto de projeto de pesquisa para investigações sobre o acesso videolaparoscópico retal (NOTES - Natural Orifice Translumenal Endoscopic Surgery), foi eutanasiado e necropsiado. Amostras do ponto de acesso do tubo de inserção na região mucocutânea anal foram colhidas, fixadas em formalina neutra tamponada a 10%, processadas por inclusão em parafina e coradas pela hematoxilina e eosina para histologia. Observou-se o comprometimento de folículos que, dilatados, exibiam formações que lembravam Demodex spp. cortados em vários sentidos e, por se tratar de ácaros espécie-específicos, concluiu-se como D. phylloides. Na citopatologia, o ácaro foi recuperado e identificado a fresco, mostrando-se alongado, com quatro pares de patas ventrais e no terço anterior do corpo. Este se refere ao primeiro registro de demodicose suína no estado do Rio de Janeiro.

We have studied the subcellular distribution of ferritin in inflammatory macrophages present in regional lymph nodes from dogs subjected to a pulmonary inflammatory reaction. The inflammatory reaction was induced by intrabronchial instillation of calcium tungstate (CaWO4), a water-insoluble powder. Ferritin was identified by electron microscopy, and its electron density was enhanced by the use of a modified Perls method. From day 14 on after the CaWO4 deposition, tungsten-positive lymph node macrophages showed a massive accumulation of ferritin. Most of the ferritin was stored in membrane-bounded vesicles that showed heterogeneous concentrations of the protein. A significant complement of ferritin was also detected in the cytoplasmic ground substance of phagocytes. The cell surface of the ferritin-rich, tungsten-positive macrophages showed deep infoldings that encompassed small pockets of connective tissue fibers. These features were not observed in control samples or in lymph nodes from dogs subjected to CaWO4-induced inflammation for periods shorter than 1 week. Our data indicate that inflammatory macrophages greatly increase their content of ferritin macrophages greatly increased their content of ferritin and that ferritin is stored predominantly by a membrane-bounded vesicular compartment. This is in contrast with suggestions that the inflammation-induced increase in macrophage iron is restricted to the labile pool of iron and it does not involve the iron bound to ferritin molecules. Our observation of nodules of connective-tissue fibers in intimate topographical association with ferritin-rich macrophages may indicate that the increase in intracellular ferritin in the macrophage is in some way related to the secretion of factors by the phagocyte that will stimulate fibrillogenesis by neighboring fibroblats.

The authors studied the anatomical structures of the pulmonary lymphatic vessels in the dog and in man using injection techniques and macro- and microangiographic and histological methods. The results show that injection in the basal lobes is easier than in the apical lobes. The dog, an animal with a thin pleura, has a very dense superficial lymphatic network, like man. The superficial and the deep lymphatic vessels are connected through the pleural septa.