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Renal involvement in the tuberous sclerosis complex includes angiomyolipomas, cysts, and carcinomas. Angiomyolipomas, despite a sometimes frightening histopathologic appearance, are benign. Cystic disease, apparently resulting from tubular epithelial hyperplasia, causes hypertension and renal insufficiency, progressing to end-stage renal disease. The same(More)
The ability to predict the course in children with newly diagnosed minimal change nephrotic syndrome (MCNS) may have significant therapeutic implications. Previous attempts based on data available at disease onset have not been successful. Therefore, it was investigated whether characterization of the initial response to adrenocortical steroids and the(More)
The fluorescent dye thioflavin T stains intensely the deposits in dense deposit disease and can be used for identification purposes. Comparison of thioflavin T staining with electron microscopy was carried out in 25 patients and showed excellent congruity. Thioflavin T is much easier to use, but is less specific than electron microscopy. Cases of light(More)
Nephritis in Henoch-Schönlein purpura (HSP) is the primary cause of morbidity and mortality. Although many therapeutic regimens have been reported to be effective, no therapy has been shown in a controlled trial to be beneficial. Fifty-six patients with histopathologically severe HSP nephritis were randomized to receive supportive therapy with or without(More)
Significant occupational exposure to polychlorinated biphenyls (PCBs) may occur in the manufacturing of capacitors and transformers, in which PCBs are used as electrical insulators. Reports of adverse health effects resulting from PCB exposure have come primarily from an incident resulting from the accidental ingestion of contaminated rice oil in Japan in(More)
Two cases of Wilms' tumor in adolescent males are presented. The clinical and radiographic findings were unusual and both presented atypical gross and microscopic features that could be correlated with the radiographic findings. Histologic examination of both tumors showed evidence of tubular and glomerular maturation, a feature usually associated with(More)
Severe segmental renal atrophy with loss of parenchymal elements in small kidneys is commonly known as segmental hypoplasia. The scars are seen as cortical depressions overlying shrunken medullary pyramids and their dilated calyces, and are characterized histologically by colloid-filled tubular microcysts and a paucity or absence of glomeruli. This lesion(More)
In utero exposure to non-steroidal anti-inflammatory agents (NSAIAs) can produce combinations of oligohydramnios, a bleeding diathesis, ileal perforation, premature closure of the ductus, and acute or chronic renal injury. NSAIAs induce renal dysgenesis in fetal monkeys and renal structural abnormalities in the developing human fetus. We report(More)
BACKGROUND The tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by seizures, mental retardation, and benign tumors of the brain, heart, skin, and kidney. Malignant tumors also can occur in patients with tuberous sclerosis, particularly in the kidney, although they occur less frequently than benign tumors. The types of(More)