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Biomedical subjects
Publications and source records attributed to C Milanesi.
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Two pairs of female siblings of French and Italian origin presented with the histological picture of glomerulocystic kidneys. The cases differ from the patients previously described with glomerulocystic kidneys by the absence of major extrarenal malformations, the reduction of kidney size with absence of renal papillae and by the presence of stable chronic renal failure, starting during the first months of life. Both mothers of the patients also had chronic renal failure with similar urographic abnormalities.
A 14-month-old child with congenital hypoplastic anemia, in temporary remission after steroid therapy, developed an acute megakarioblastic leukemia. After complete remission due to immunosuppressive treatment, the leukemia rapidly relapsed, and the child died at 23 months of age. The rapid development of leukemia puts some doubt on the initial diagnosis.
In the intestine of the filtering zooids of B. schlosseri three segments can be distinguished. In the intermediate segment the epithelium, which is encrusted by the ampullae of the pyloric gland, shows marked aspects of alteration. In the proximal and distal segments, ciliated mucous, vacuolated and endocrine-like cells are recognizable. Ciliated mucous cells, widely distributed along the intestine, possess the apical region filled with numerous mucous granules, which are extruded with merocrine modality. Variations in morphology of the granules are visible especially between cells of different regions. Vacuolated cells appear involved in absorptive function. They are characterized by developed microvilli, numerous apical small vesicles and great supranuclear vacuoles containing heterogeneous material. The vacuolated cells of the proximal segment resemble the gastric vacuolated cells of B. schlosseri. The vacuolated cells of the distal segment show many morphological similarities with protein absorbing cells of various animals for the presence of a giant vacuole and an apical network of vesicles and tubules with fuzzy coating on the luminal face. The intestinal endocrine-like cells are rare and characterized by strongly electron dense granules distributed in all the cytoplasm, but predominantly in the basal region.
The following five cell types have been recognized and defined on the basis of their fine structure in the gastric epithelium of B. schlosseri: vacuolated and zymogenic cells (described in a previous paper); ciliated mucous, endocrine and plicated cells. The ciliated mucous cells are distributed at the apex and the bottom of the gastric folds and along the dorsal groove. The mucus droplets appear to form from the Golgi complex as secretory granules of variable density and texture, which are released from the cell after fusion of their membranes with the apical plasma membrane. Holocrine or apocrine secretion has not been observed. The endocrine cells are scattered and are characterized by electron dense granules, especially numerous in the basal region of the cell. Finally, the plicated cells, present in the pyloric caecum, show rod-like microvilli, a well developed Golgi complex and abundant, deep infoldings of the basal plasma membrane, which are associated with numerous mitochondria. The possible role of the gastric cell types is discussed taking into account information concerning morphologically similar cells in other animals, as well as previously reported data on the biochemistry and physiology of digestion and excretion in ascidians.
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Comparative kinetics of porphyrin uptake and release by HeLa cells, incubated with equivalent concentrations of either hematoporphyrin (Hp) in aqueous solution or Hp and its dimethylester (HpDME) bound to unilamellar liposomes, show that liposomal porphyrins are bound at a higher rate and in considerably larger amounts. Moreover, the release of cell-bound porphyrins into the medium is remarkably reduced and slowered after cell loading with liposome-bound porphyrins. The presence of 1% bovine or human serum albumin (but not serum globulins) in the medium has no effect on uptake and release of liposome-bound porphyrins by HeLa cells, whereas it remarkably decreases the uptake of aqueous Hp. Parallel studies of cell photodamages under known concentrations of cell-bound porphyrin unequivocally demonstrate that the photodynamic effect is strictly related to the porphyrin load. As a consequence a dramatic increase of cell-photosensitizing efficiency is obtained by binding Hp (and even more HpDME) with liposomes. Electron microscopy investigations on cell damages caused by loading with liposome-bound porphyrins and subsequent illumination show that the plasmatic membrane is one important cell site of porphyrin interaction and photodynamic effect.
BACKGROUND: Bone marrow infiltration occurs rarely at presentation of rhabdomyosarcoma (RMS) or other childhood solid tumors. This possibility leads to misdiagnosis of leukemia and incorrect therapies might be administered. METHODS: We report two patients presenting with diffuse bone marrows involvement by neoplastic cells. Initial studies were not consistent with a diagnosis of leukemia and the cases were further studied extensively by indirect immunofluorescence, immunocytochemistry, electron microscopy and cytogenetics. RESULTS: In both cases blast cells were large, poorly differentiated, with immunological reactivity to the anti-desmin antibody. Ultrastructural findings of muscular features and chromosomal translocation t(2;13) (q37;q14) further confirmed the diagnosis of rhabdomyosarcoma of the alveolar subtype. This was then confirmed histologically in one patient. CONCLUSION: This study stresses the utility of analyzing cases of morphologically undifferentiated marrow blast cells by various techniques, as well as investigating for different types of both hematological and solid neoplasms.
We report 6 cases with membranous glomerulonephritis associated with chronic hepatitis B virus infection, who came under our observation for microscopic haematuria in 2 cases, haematuria and proteinuria in 1 case, and nephrotic syndrome in the others. At the examination all 6 children were found to have hepatomegaly with raised serum transaminase activity. All the patients were positive in the serum for HBsAg and anti-HBc, 3 were HBeAg and anti-HBe negative. Liver biopsy showed features of chronic hepatitis with moderate signs of activity. Renal biopsy was consistent with membranous glomerulonephritis in all patients. With a fluorescent antibody technic HBeAg was found to be deposited in diffuse granular fashion, along glomerular capillary walls, together with IgG, in 2 out of 3 cases stained, but no deposition of HBsAg was detected in all the patients. Steroid therapy was started from 18 to 33 months. Urine analysis became negative in 4 cases and persisted normal in the follow-up. One patient with haematuria developed nephrotic syndrome and one died from miliary tuberculosis. Our findings suggest that the clinical outcome is favourable in children with membranous glomerulonephritis and chronic active hepatitis and that the pathogenesis probably is not unique.
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