Cases reported "Kidney Diseases"

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1/9. cyclosporine nephrotoxicity: associated allograft dysfunction at low trough concentration.

    A case report on the nephrotoxic effect of cyclosporine in a 9-year-old boy with a kidney transplant is presented. Nephrotoxicity was present even at low trough cyclosporine concentration. The literature on the range of cyclosporine nephrotoxicity is reviewed. cyclosporine (CsA), a fungal decapeptide first introduced in 1983, has significantly improved the outcome of renal transplantation, and remains the first line immunosupressant for pediatric recipients. CsA has a narrow therapeutic range because of the fine line between adequate immunosuppression and the risk of drug-induced side effects. Furthermore, considerable inter- and intrapatient variability does exist [Filler et al. 1999]. The pre-dose trough concentration is routinely used for therapeutic drug monitoring [Bunchman et al. 1998]. The most significant side effect is nephrotoxicity, which may present differently at different times after transplantation. Renal vasoconstriction, especially involving the afferent renal arterioles, has been strongly implicated as a primary factor in acute reversible CsA nephrotoxicity. Alpha-adrenergic and calcium channel blockade with either verapamil or nifedipine ameliorates vasospasm and impairment of renal function that accompany CsA toxicity. Because of this vasoconstrictive effect, CsA may increase ischemic graft damage in the early posttransplant period. CsA side effects can be eliminated by reducing the dosage of the drug. We present an unusual case of nephrotoxicity and impaired renal function with a very low CsA blood trough concentration (50 ng/ml) on posttransplant treatment. The side effects subsided only after the discontinuation of CsA.
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2/9. Renal cholesterol embolic disease effectively treated with steroid pulse therapy.

    A 65-year-old man developed acute renal failure with eosinophilia two weeks after a coronary bypass operation and angiography. Renal biopsy revealed cholesterol crystal embolism (CCE) in glomeruli and arterioles. Low-dose corticosteroid therapy failed to recover the renal function; further deterioration of renal function and peripheral ischemic symptoms such as livedo reticularis and blue toes occurred. However, steroid pulse therapy successfully attenuated CCE-induced renal failure and eosinophilia. It is suggested that steroid pulse therapy might be effective to treat CCE-induced renal failure and eosinophilia could be a useful marker for activity of CCE.
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3/9. Unilateral renal angiodysplasia in a girl with hypertension.

    We report a case of very unusual renal vascular anomaly: angiodysplasia. The patient suffered from acute pyelonephritis immediately after birth. Renal ultrasonography, performed at age 1 day, revealed an enlarged left kidney with heterogeneously increased echogenicity, which involuted rapidly in 3 months. At age 10 years, she presented with severe hypertension during a course of acute pyelonephritis. Peripheral plasma renin activity was high. Computed tomographic angiogram revealed a very small but functioning left kidney. A single, narrow, left renal artery did not have focal stenosis. pathology examination revealed dysplastic arterioles at the subcapsular area. After left nephrectomy, the blood pressure and the plasma renin activity were normalized. In conclusion, this is an unusual case of renal angiodysplasia, which induced renin-dependent hypertension.
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4/9. Anti-cardiolipin antibody and renal disease: a report three cases.

    Anti-cardiolipin antibodies have been linked to recurrent arterial and venous thrombosis in multiple organs. We present a biopsy-documented report of thrombotic renal disease apparently attributable to circulating anti-cardiolipin antibodies. One patient had primary anti-cardiolipin syndrome, one had mild SLE, and the third had a mild lupus-like syndrome. All three patients had a clinical course dominated by repeated multi-organ system thrombosis. Renal biopsy disclosed thrombosis at the level of the glomerular capillaries, arterioles, and interlobular arteries--similar to that described in other thrombotic microangiopathies. Renal thrombosis was not associated with active endocapillary proliferative lupus nephritis, suggesting a mechanism independent of subendothelial immune deposit injury. Renal presentation was variable, ranging from asymptomatic mild proteinuria to nephrotic-range proteinuria, renal insufficiency, and hypertension.
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5/9. Digestive tract and renal small vessel hyalinosis, idiopathic nonarteriosclerotic intracerebral calcifications, retinal ischemic syndrome, and phenotypic abnormalities. A new familial syndrome.

    A new familial syndrome that affected 3 of 7 siblings is described. All 3 patients were young women with a very peculiar phenotype, poikilodermia and hair greying, and idiopathic nonarteriosclerotic cerebral calcifications. Pathological studies demonstrated a marked and progressive hyalinosis involving capillaries and often arterioles and small veins of the digestive tract, kidneys, and calcified areas of the brain. Using electron microscopy, we found that the hyalin substance in the intestinal capillaries consisted of several concentric layers of basal membrane-like deposits within a finely granular fluffy material. Huge deposits of this material were present in the subepithelial and mesangial spaces of the kidneys. endothelial cells and, in the kidneys, mesangial cells were markedly abnormal, and a true mesangiolysis pattern was present in 2 patients. The clinical and biologic expression of these vascular changes was variable. diarrhea, rectal bleeding, malabsorption, and protein-losing enteropathy were the main and lethal clinical problems in the proband. hypertension appeared in the early stage of a second pregnancy in 1 sister, and mild proteinuria was found in all 3 affected patients. Peripheral retinal ischemic syndrome and chorioretinal scars were found in the ocular fundi of both affected sisters of the proband. A subarachnoid hemorrhage, due to a right sylvian aneurism, also occurred in both sisters and was lethal in 1 sister. None of the known causes of distal vessel hyalinosis could be ascertained.
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6/9. Renal vascular lesions after chemotherapy with vinblastine, bleomycin, and cisplatin.

    A 30 year old man with metastatic embryonal carcinoma became hypertensive during vinblastine, bleomycin, and cisplatin therapy. Three months after completion of therapy, accelerated hypertension occurred (blood pressure 210/140 mm Hg). nitroprusside failed to control the hypertension, but captopril resulted in a prompt and sustained normalization of the blood pressure. The plasma renin activity was markedly elevated before therapy. Renal biopsy disclosed "onionskin" narrowing of the interlobular arteries and fibrin thrombosis of a majority of the afferent arterioles. A form of drug-induced renovascular hypertension is suggested.
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7/9. Xeroradiographic observations in four patients with chronic renal disease and cutaneous gangrene.

    Cutaneous gangrene developed in four patients who had chronic renal disease that resulted in secondary hyperparathyroidism and systemic arterial calcification. Three of the four patients showed a unique pattern, by xeroradiography, of extensive medical calcification in subcutaneous arterioles. This noninvasive procedure demonstrated calcium in minute subcutaneous arterioles. parathyroidectomy, conservative local care, and skin grafting resulted in cure of the cutaneous ulcers in two of the four patients.
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keywords = arteriole
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8/9. Glomerulopathy and arteriolopathy in congenital nephrotic syndrome: light, electron, and fluorescence microscopy studies.

    This report describes the light, electron, and fluorescence microscopy studies of the kidney from a one month old, prematurely born infant with the nephrotic syndrome. light microscopy revealed enlargement of Bowman's space, an excessive number of glomerular epithelial cell nuclei, and cystic dilatation of cortical tubules. By electron microscopy two disparate types of glomerular lesions were found: a thin basement membrane with fusion of foot processes suggestive of lipoid nephrosis and linear electron-density in the basement membrane with excessive mesangial matrix and capillary collapse suggesting an antibasement membrane type of glomerulonephritis or mesangial sclerosis. Conspicuous cystic spaces were found in the proximal tubules only. In the arterioles there were cystic spaces in the endothelial cells and smooth muscle cells, thickening of the basement membranes of endothelial cells and smooth muscle cells, and excessive amounts of nonstriated fibrils in the subendothelial basement membrane. Masses of fibrin were noted in the lumina of veins, peritubular capillaries, and interstitium between veins and peritubular capillaries. Such multiple glomerular and arteriolar lesions have not been reported previously in congenital nephrotic syndrome. These lesions suggest an antigen-antibody reaction in the mother that might have been directed toward the glomerular basement membrane in the fetus, producing these lesions.
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9/9. Addison's disease with renal microangiopathy and renal failure (a new syndrome).

    A syndrome comprising Addison's disease, renal microangiopathy and renal failure is described in two patients. The renal lesions manifested despite corticosteroid replacement therapy and were characterized by glomerular damage and thrombo-microangiopathic changes in afferent arterioles and intralobular arteries. Both patients died as a result of their renal disease.
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