Renal involvement in Fabry's disease.
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Biomedical subjects
Publications and source records attributed to K Joshi.
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We report a rare case of carcinosarcoma of parotid gland in a pleomorphic adenoma. The tumor showed two distinct heterologous components, a ductal carcinoma and an osteosarcoma. The latter has not been described so far in such tumors arising in pleomorphic adenoma of parotid gland.
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We report a child who developed juvenile rheumatoid arthritis at the age of 7 years and nephrotic syndrome due to renal amyloidosis within 2 years of the onset of arthropathy. Literature on the management and prognosis has also been reviewed.
Amongst 310 recipients of live related renal transplants, systemic fungal infections were observed in 19 patients (6.1%). These included cryptococcosis in eight (42%), candidiasis in seven (37%), mucormycosis in two (11%), aspergillosis in one (5.5%), and a mixed cryptococcal and Aspergillus infection in one patient (5.5%). Infections occurred within 12 months of transplantation in seven patients and after 13-37 months in the remaining patients. Prolonged fever not responding to antibiotics was the most common presentation. Central nervous system was involved in all patients with cryptococcosis, while Candida infection primarily involved the urinary tract. Both patients with mucormycosis exhibited signs and symptoms of florid rhinocerebral disease and a rapid downhill course. Pulmonary manifestations were the most prominent features of patients with aspergillosis. Parenteral amphotericin B with or without 5-fluorocytosine was started in all, but three patients could receive the therapy only for 1-3 weeks. Three patients with cryptococcosis developed amphotericin-related complications and were changed to oral fluconazole. Seven patients recovered and 12 (63%) died. None of the patients had cytomegalovirus infection preceding the onset of fungal disease. The high frequency of fungal infections amongst our allograft recipients could not be ascribed to over immunosuppression, since 16 patients (84%) were only on maintenance doses of immunosuppressive drugs and in 13 (68.4%) graft function was normal at the time of diagnosis. The high frequency of fungal infections in our patients was most probably related to the poor hygienic and sanitary conditions which continue to be prevalent in the tropical environment of third-world countries. Delays in the diagnosis and late institution of therapy result in a high mortality.(ABSTRACT TRUNCATED AT 250 WORDS)
We report a rare case of primary cutaneous myxoid melanoma. Histologically, the tumour was composed of spindle and stellate-shaped cells, embedded in a myxoid stroma. Positivity for S-100 protein and the presence of melanosomes were demonstrated in the tumour. Primary cutaneous myxoid melanoma is rare. This is the second report of such a case.
The binding of hematoporphyrin derivative (HpD) to brain tumor cells and their photosensitivity was studied as a function of HpD concentration, time of incubation and growth phase of cells. Upon binding to cells, HpD showed three fluorescence bands at 616, 636 and 678 nm. In plateau phase cells a fluorescence band at 636 nm was predominant, which was further enhanced by increasing HpD concentration and/or increasing incubation time. In exponential phase cells the maximum fluorescence was exhibited at 616 nm. After 1 h incubation of exponential phase cells with increasing HpD concentration an overall intensity enhancement occurred with no change in the distribution of bands, whereas longer incubation time caused an increase in relative intensity of the 636 nm band similar to that observed in plateau phase cells. After 1 h incubation with HpD plateau phase cells were more photosensitive than exponential phase cells, although cell bound HpD was much less in the former case. Incubation of cells for 24 h drastically enhanced the photosensitivity irrespective of the growth phase. Our results suggest a relationship between the fluorescence emission band of HpD at 636 nm and photosensitivity of cells.
We report the characteristic CT scan findings of renal cortical necrosis in a patient of acute renal failure, which was confirmed on renal biopsy. CT scan is a useful, noninvasive investigative modality for an early diagnosis of renal cortical necrosis.
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A case of retinitis following disseminated cryptococcus neoformans infection in a renal allograft recipient is reported. Therapy with oral fluconazole showed a remarkable improvement at the end of 8 weeks. This is the first report of the use of fluconazole in the treatment of cryptococcal retinitis.
A rare case of an intramuscular hemangioma of the temporalis muscle is reported. The clinical examination, carotid arteriogram, computed tomographic scan, and aspiration cytology suggested the vascular nature of the tumor, but an exact diagnosis could only be made after histopathologic examination. Temporary occlusion of the ipsilateral external carotid artery and subperiosteal dissection permitted complete, wide excision without much bleeding.
Acute renal failure developed in a young female having puerperal sepsis due to beta-haemolytic streptococci. The patient succumbed to her illness and an autopsy revealed extensive crescentic glomerulonephritis. The association between chronic visceral sepsis and glomerulonephritis is discussed.
In a young adult male with chronic renal failure from focal segmental glomerulosclerosis, massive proteinuria appeared within two weeks after transplantation. Although allograft biopsy at eight weeks was normal, a second biopsy eight months after transplant showed focal segmental glomerulosclerosis again. Sixteen months after transplantation maintenance hemodialysis had to be restarted.
The oral inoculation of Giardia lamblia trophozoites (Portland 1 strain) resulted in the establishment of infection by day 3-5 in NMRI mice. By 9-11 days postinfection, the trophozoites load reached maximum (acute phase) and later declined by day 17-21. The tissue sections from infected animals during the establishment phase of infection indicated limited changes in surface epithelium with normal villous length. Although trophozoites of G. lamblia were seen in all sections lying free in the lumen as discrete parasite, the active invasion by the parasite could not be demonstrated. During the acute phase of infection, fuzzy appearance of brush border, marked reduction in villous height and infiltration of intraepithelial lymphocytes were commonly seen in all tissues. Electron microscopic observation demonstrated large numbers of trophozoites of G. lamblia preferentially aggregated at the base of the villi. At some sites, adhesive marks indicating attachment of Giardia trophozoites were also seen by scanning electron microscopy. In addition, severe flattening and blunting of microvilli and occasional loss of basic morphology of intracellular organelles of columnar cells were noticed at the site of parasite colonization under electron microscopy. The brush border microvilli were noted to be damaged in areas where parasites were attached to surface epithelium with the help of suction discs. The morphological changes associated with Giardia infection tended to reverse as the parasite load declined by day 17-21. Thus we feel that malabsorption in giardiasis with total or varying degrees of morphological alterations of surface mucosa can be explained on the basis of reduced absorptive surface area.
In a 9-month-old girl a subperiosteal metastasis of the maxillary bone developed in the contralateral orbit about one year after enucleation of a poorly differentiated retinoblastoma.
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