Biomedical subjects
Publications and source records attributed to A A Ucci.
Testicular microlithiasis occurring in a postorchiopexy testis is described. The histologic characteristics of this uncommon entity are presented, and its etiology and clinical significance are briefly reviewed.
Multifocal pigmentation of the bladder and prostatic urothelium is described in 2 white men. The light microscopic, electron microscopic and histochemical characterization of these foci identified the contained pigment as melanin.
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Lectin histochemical studies were performed on frozen and paraffin-embedded brain tissue sections from six cases of galactosylceramide lipidosis (i.e., globoid cell leukodystrophy, or Krabbe's disease) in Twitcher mice and one case of canine infantile GM1-gangliosidosis. The globoid cells in Krabbe's disease stained with Ricinus communis agglutinin-I (RCA-I), peanut agglutinin (PNA), and Bandeirea simplicifolia agglutinin-I (BS-I) in frozen sections. However, paraffin sections and frozen sections pretreated with chloroform-methanol or xylene, from the same animals, stained with Concanavlia ensiformis agglutinin (ConA), wheat germ agglutinin (WGA), and succinylated-WGA (S-WGA), in addition to staining with RCA-I, PNA, and BS-I. The affected neurons of canine infantile GM1-gangliosidosis stained only with RCA-I in frozen sections. In paraffin sections, however, these cells were negative with RCA-I but positive with BS-I, ConA, Dolichos biflorus agglutinin (DBA), soybean agglutinin (SBA) and Ulex europaeus agglutinin (UEA-I) in paraffin sections. These results indicate that in paraffin processing of glycolipid storage disease tissue, some lectin receptors are lost and others are unmasked. The retained receptors can be stained with specific lectins and could serve as markers to characterize and differentiate among the various glycolipid storage diseases.
Beta-galactosidase-deficient siblings in two litters of English springer spaniel puppies showed a progressive neurological impairment, dwarfism, orbital hypertelorism, and dysostosis multiplex. An excess of GM1-ganglioside was found in the brain. Three abnormal oligosaccharides were present in samples of urine, brain, liver, and cartilage. Light microscopy of selected tissue specimens revealed cytoplasmic vacuoles in neurons, circulating blood cells, macrophages, and chondrocytes. Ultrastructural studies demonstrated that these membrane-bound vacuoles were of two types--one containing lamellated membranes and the other, finely granular material. These clinical and pathological findings are similar to those observed in human patients affected by the infantile form of GM1-gangliosidosis.
A patient was diagnosed as having angioendotheliomatosis proliferans systemisata (APS) based on characteristic clinical and histologic features. A few days later, malignant lymphoma involving the gut was discovered. Immunohistochemical and electronmicroscopic studies confirmed the nonendothelial and lymphoid nature of intravascular tumor cells. This is the sixth case in which malignant lymphoma has been shown to involve the vessels of the skin (and probably of other organs) in a pattern identical to that seen in APS.
Selected formalin-fixed, paraffin-embedded tissues of human and canine fucosidosis were stained with nine different lectins. Neurons, splenic sinusoidal cells, hepatic Kupffer cells, tissue macrophages, and capillary endothelium from human patients with fucosidosis stained intensely with Ulex europaeus agglutinin-I (UEA-I), but the same cells were unstained in tissues from canine fucosidosis. Since UEA-I specifically binds to terminal fucose residues, and fucose-rich undegraded metabolites are stored in affected cells of both human and canine fucosidosis, the variable lectin staining pattern demonstrates an unexpected species-specific histochemical variability. This finding highlights the fact that although both species have decreased fucosidase activity, the precursor substrates, undegraded stored metabolites, and particular cells affected by this enzyme deficiency are different.
The case of a biliary cystadenoma containing foci of cystadenocarcinoma is reported, along with the first electron microscopic description of this lesion. Both neoplastic and congenital origins have been implicated in the pathogenesis of biliary cystadenomas. In this case hamartomatous bile ducts were found, supporting a congenital origin for at least some biliary cystadenomas. Careful pathologic evaluation is emphasized since malignant degeneration can be detected only after thorough sectioning.
Excimer lasers are pulsed gas lasers that use a mixture of a rare gas and halogen as the active medium to generate pulses of short wavelength, high energy ultraviolet light. A krypton-fluoride gas mixture was used to achieve an excimer emission at a wavelength of 248 nm. A total of 30 atherosclerotic coronary artery segments were irradiated over a range of pulse energies (250 to 750 mJ), repetition rates (2 to 25 Hz), average powers (1.9 to 18.8 watts) and cumulative exposures (3 to 12 seconds). In no case was there gross, light microscopic or ultrastructural evidence of the pathologic injury typically associated with continuous wave laser irradiation of coronary artery segments. Similar results were achieved after excimer laser irradiation of 30 samples of myocardium. Excimer irradiation of calcified aortic valve leaflets accomplished focal debridement without pathologic tissue injury; when total debridement was attempted, however, gross charring was observed. The paucity of pathologic alterations observed after excimer irradiation of cardiovascular tissue may prove beneficial in precisely controlling laser ablation of pathologic tissue without injury to the surrounding normal tissue. Clinical application of excimer laser irradiation requires resolution of several issues, including the development of suitable fiber optics and laser coupling, evaluation of potential ultraviolet toxicity, and demonstration that ultraviolet light can be transmitted through a blood-filled system.
Cells affected by locoweed (Astragalus lentiginosus) and Swainsona galegifolia toxicosis or mannosidosis exhibit similarities in their catabolism of N-linked glycoproteins and accumulation of cytoplasmic vacuoles. We used nine different biotinylated lectins as histochemical markers for specific sugars and avidin-biotin-peroxidase complex as a visualant to study the cells affected with these conditions. Since locoweed and Swainsona spp block mannosidase activity, we expected a similar lectin staining pattern in cells under these conditions as that seen in mannosidosis. Concanavalia ensiformis agglutinin, wheat germ agglutinin and succinyl wheat germ agglutinin stained the undegraded glycoproteins and oligosaccharides stored in the lysosomes of affected cells in all three conditions. Bandeirea simplicifolia-I, Dolichos biflorus agglutinin, peanut agglutinin, Ricinus communis agglutinin-I, soybean agglutinin and Ulex europaeus agglutinin-I did not stain any of these cells. These results indicate that in all three conditions there is an accumulation of undegraded oligosaccharides that contain alpha-mannosyl and beta-N-acetyl glucosamine residues which are revealed by lectin staining in the vacuoles of all affected cells.
Accurate preoperative diagnosis of renal angiomyolipomas is essential if conservative resection or angiographic embolization is to be used. Computed tomographic (CT) scanning failed to diagnose angiomyolipoma preoperatively in 2 patients, and this led to a retrospective review of 6 patients to define the limits of CT scanning in preoperative diagnosis. The CT findings were correlated with the histology of the tumors. Two tumors with positive attenuation coefficients suggestive of renal adenocarcinoma had significant amounts of immature "fetal" fat, and one of these had a virtual absence of mature fat. The relative inability of CT scanning to identify immature "fetal" fat, especially in the presence of abundant vessel and muscle elements, is a limitation to its use in the preoperative diagnosis of angiomyolipoma. A negative attenuation coefficient is highly characteristic of renal angiomyolipoma with mature fat elements. A positive attenuation coefficient, although suggestive of renal cell carcinoma, may be found in angiomyolipomas with paucity of mature fat or high proportions of immature fat.
Nephrogenic adenoma a rare bladder, ureter, or urethral lesion, is of disputed pathogenesis, metaplastic and congenital etiologies both being implicated in its development. Since light and electron microscopy have been unable to fully resolve the lesion's pathogenesis, the authors used biotinylated lectins as probes and avidin-biotin peroxidase complex (ABC) as a visualant to study cases of nephrogenic adenomas and compared their lectin binding patterns with those of normal transitional epithelium, human embryonic kidneys, and cases of cystitis cystica and glandularis and squamous metaplasia of the bladder in an effort to clarify this issue. Only the epithelial lining of the luminal surface and tubuli in nephrogenic adenoma and tubules in embryonic kidney exhibited free PNA receptor sites. The striking staining similarities between the epithelial components of nephrogenic adenomas and mesonephric and metanephric tubules complement previous findings concerning the origin of nephrogenic adenoma.
The specific diagnosis of glycoprotein storage diseases is made by demonstrating a deficiency in enzyme activity or an elevation of undegraded oligosaccharides in cells or body fluids. Prospective sampling and expensive specialized biochemistry, which is also time consuming, are required for such studies. We used lectin reagents on paraffin-embedded tissue sections to identify the specific sugars in undegraded stored substances. We studied 22 cases of glycoprotein storage diseases and differentiated histochemically between alpha- and beta-mannosidosis, fucosidosis, and sialisidosis. Cells affected with alpha-mannosidosis stained with Concanavalia ensiformis (Con A), Triticum vulgaris (WGA), and succinyl-WGA (S-WGA), while beta-mannosidosis cells did not stain with any of the lectins used. In fucosidosis the affected cells stained with Ulex europeus-I (UEA-I), while sialisidosis-affected cells stained with WGA, and in three cases with Arachis hypogea (PNA). This study indicates that lectin histochemistry provides a reliable specific diagnostic pattern for some glycoprotein storage diseases using a simple and inexpensive method.
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We have studied the ultrastructure of glycocalyx at the luminal surface of normal and diseased urothelium from humans and rats with ruthenium red staining. A correlation between the thickness and staining intensity of the glycocalyx and the surface topography of the luminal surface was observed. An intensely stained thick glycocalyx was associated with prominent surface microvilli seen in the following conditions in humans: some control urothelium, inverted papilloma, well and moderately differentiated transitional cell carcinomas and mucin producing adenocarcinomas. These changes were also present in rats with FANFT-induced preneoplastic and neoplastic changes. A thin glycocalyx was associated with a scalloped luminal surface containing asymmetric unit membrane plaques and was found in some control humans urothelium and in normal rat urothelium. A thin glycocalyx was also associated with the relatively smooth surface seen in poorly differentiated transitional cell carcinomas as well as in some mucin producing adenocarcinomas. We suggest that urothelial glycocalyx, as demonstrated by ruthenium red staining, correlates with the luminal surface topography rather than specific pathological conditions of the bladder.
A renal tumor developing in a patient receiving cyclophosphamide (Cytoxan) therapy for Wegener granulomatosis is reported. The tumor was similar histologically to the "immunoblastic" sarcoma that develops in renal allograft recipients as a complication of immunosuppressive therapy. This case report strengthens the cause and effect relationship between immunosuppressive drug usage and the subsequent development of neoplasia.
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