Cases reported "Nephrosclerosis"

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1/18. The Ask-Upmark kidney: a form of ascending pyelonephritis?

    The case is presented of a young girl with recurrent urinary tract infection and vesico-ureteric reflux who developed a small scarred kidney and subsequently, hypertension. Pathologically, the renal changes were compatible with those of an Ask-Upmark kidney. The pathogenesis of the Ask-Upmark kidney is discussed. It is postulated that the lesion is not necessarily of congenital origin but may well be related to infection and intrarenal reflux, it is concluded that long-term follow-up of a young patient with a scarred kidney is indicated.
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keywords = hypertension
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2/18. Severe hypertensive sequelae in a child with Seckel syndrome (bird-like dwarfism).

    We report a 19-year-old male with Seckel syndrome (bird-like dwarfism) who presents with malignant hypertension associated with hypertensive nephrosclerosis, dilated cardiomyopathy, and a ruptured cerebral artery aneurysm. Although end-organ injury due to chronic hypertension occurs frequently in adults, no previous reports of renal insufficiency due to hypertension exist in children or adolescents. We speculate that this patient may have been particularly prone to hypertensive end-organ injury due to his extreme short stature.
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ranking = 3
keywords = hypertension
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3/18. Disposition of [G-(3)H]paclitaxel and cremophor EL in a patient with severely impaired renal function.

    In the present work, we studied the pharmacokinetics and metabolic disposition of [G-(3)H]paclitaxel in a female patient with recurrent ovarian cancer and severe renal impairment (creatinine clearance: approximately 20 ml/min) due to chronic hypertension and prior cisplatin treatment. During six 3-weekly courses of paclitaxel at a dose level of 157.5 mg/m(2) (viz. a 10% dose reduction), the renal function remained stable. Pharmacokinetic evaluation revealed a reproducible and surprisingly high paclitaxel area under the plasma concentration-time curve of 26.0 /- 1.11 microM.h (mean /- S.D.; n = 6; c.v. = 4.29%), and a terminal disposition half-life of approximately 29 h. Both parameters are substantially increased ( approximately 1.5-fold) when compared with kinetic data obtained from patients with normal renal function. The cumulative urinary excretion of the parent drug was consistently low and averaged 1.58 /- 0.417% ( /- S.D.) of the dose. Total fecal excretion (measured in one course) was 52.9% of the delivered radioactivity, and mainly comprised known mono- and dihydroxylated metabolites, with unchanged paclitaxel accounting for only 6.18%. The plasma area under the plasma concentration-time curve of the paclitaxel vehicle Cremophor EL, which can profoundly alter the kinetics of paclitaxel, was 114.9 /- 5.39 microl.h/ml, and not different from historic data in patients with normal or mild renal dysfunction. Urinary excretion of Cremophor EL was less than 0.1% of the total amount administered. These data indicate that the substantial increase in systemic exposure of the patient to paclitaxel relates to decreased renal metabolism and/or urinary elimination of polar radioactive species, most likely lacking an intact taxane ring fragment.
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keywords = hypertension
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4/18. chloroquine-induced phospholipidosis of the kidney mimicking Fabry's disease: case report and review of the literature.

    A 46-year-old female patient with sjogren's syndrome, hypertension, and stable chronic renal insufficiency (creatinine [CR], 1.9 to 2.1 mg/dL) had a progressive worsening of renal function (CR, 5.0 mg/dL) after 11 months of chloroquine therapy (155 mg/day; cumulative dose of approximately 51 g). light microscopy revealed nonspecific angionephrosclerosis. Electron microscopy showed accumulations of lamellated myelinoid material and occasionally also of curvilinear bodies, especially in the glomerular podocytes and to a lesser extent in vascular myothelial and endothelial cells. In the tubular system, mainly protein droplets were stored. Activity of alpha-galactosidase A was normal in isolated leukocytes (56 nmol/mg; range, 33.2 to 109 nmol/mg), ruling out Fabry's disease. Clinical, morphological, and biochemical findings were consistent with chloroquine-associated deterioration of renal function that improved considerably after discontinuation of chloroquine treatment. Adverse effects of chloroquine may aggravate preexisting renal disease. Electron microscopy is a worthwhile tool for establishing the correct diagnosis.
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keywords = hypertension
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5/18. Renovascular hypertension: a unique cause of unilateral focal segmental glomerulosclerosis.

    A 48-year-old man presented with malignant hypertension and massive proteinuria. Renal angiography showed complete obstruction of the left renal artery and 99mTc-mercaptoacetylglycine (MAG3) renography showed a nonfunctioning left kidney. Percutaneous transluminal renal angioplasty of the left renal artery was unsuccessful; hence, the patient underwent left nephrectomy because of uncontrolled hypertension and proteinuria. Histological examination of a right kidney specimen revealed lesions of focal segmental glomerulosclerosis with benign nephrosclerosis. In contrast, histology of the left kidney showed typical ischemic kidney with hypertrophy of arteriolar smooth muscle cells. The patient responded favorably to the nephrectomy, as his blood pressure and urinary protein dramatically decreased with no antihypertensive medication. This case illustrates the heterogeneous effect of the renin-angiotensin system on either kidney in patients with renovascular hypertension due to unilateral renal artery stenosis.
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ranking = 7
keywords = hypertension
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6/18. Scleroderma renal crisis in progressive systemic sclerosis: a case report.

    We report a case of progressive systemic sclerosis with scleroderma renal crisis 10 years after onset. The patient (female) had progressive renal dysfunction, hypertension which was difficult to control, and massive gastrointestinal bleeding. An angiotensin converting enzyme inhibitor (enalapril) could not control her hypertension. Only intravenous nicardipine had a slight effect on her hypertension. Hemodialysis and plasma exchange, did not reverse the renal crisis, and the patient died. Microscopic examination of her kidney showed thickening of the capillary walls and mild nephrosclerosis.
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ranking = 3
keywords = hypertension
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7/18. Hemodialysis-resistant hypertension: control with an orally active inhibitor of angiotensin-converting enzyme.

    In two patients with end stage renal disease and dialysis-resistant hypertension, the orally active inhibitor of angiotensin-converting enzyme, captopril (SQ14,225; 2-D-methyl-3-mercaptopropranoyl-L-proline, dramatically lowered blood pressure both before and during dialysis. This agent holds promise as an alternate to bilateral nephrectomy in such patients.
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ranking = 5
keywords = hypertension
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8/18. Necrotizing arteriolitis of ileum, as the initial manifestation of malignant hypertension in childhood.

    Intestine is seldom a site of clinical manifestation of malignant hypertension, particularly in childhood. This report deals with a case of malignant nephrosclerosis superimposed on benign nephrosclerosis which probably resulted from a unilateral obstructive uropathy and chronic pyelonephritis. Clinical features included severe hypertension, neuroretinopathy with retinal exudate and hypertensive encephalopathy. An acute abdomen due to transmural infarction of the ileum caused by multiple thrombotic occlusion of necrotizing arteritis involving bowel wall and the mesentery was noted.
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ranking = 6
keywords = hypertension
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9/18. Recovery of renal function in patients with accelerated malignant nephrosclerosis on maintenance dialysis with management of blood pressure by captopril.

    Recovery of renal function to a self-sustaining level was observed in 4 patients with accelerated malignant hypertension who required chronic hemodialysis therapy. Excellent blood pressure control was achieved in all the patients on captopril therapy. Hemodialysis could be discontinued after 2-9 months of captopril therapy; on recovery of renal function levels of creatinine clearance became stable ranging from 28 to 56 ml/min within 5-15 months of captopril treatment, and remained at this level during 21-64 months of observation. The management of hypertension and the inhibition of the renin-angiotensin system afforded by chronic angiotensin-converting enzyme inhibition is very promising as a means of reversing the process of malignant nephrosclerosis.
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ranking = 2
keywords = hypertension
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10/18. Spontaneous hemorrhage of the kidney.

    A young hypertensive man with hypertensive nephrosclerosis presented with an acute abdominal emergency due to a spontaneous renal hemorrhage. Investigations demonstrated a large left perinephric hematoma. This was managed conservatively. With control of his hypertension, renal function has remained stable.
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ranking = 1
keywords = hypertension
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