Cases reported "Uremia"

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1/5. Prominent medial hypertrophy of renal arterioles in an infant with hyporeninemic hypoaldosteronism.

    We describe an 11-month-old boy who presented clinically with hyperkalemic renal tubular acidosis due to hyporeninemic hypoaldosteronism. Persistent hyperchloremic acidosis and mild azotemia were present. All abnormal laboratory values were corrected by the administration of fludrocortisone. Renal biopsy showed prominent medial hypertrophy of renal arterioles and interstitial fibrosis, which closely resemble those of the gene-targeted mice with disruption of the renin angiotensin system. This is the first case report raising the possibility that a defective renin angiotensin system in infancy may lead to tubulointerstitial damage with medial hypertrophy of intrarenal arterioles.
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ranking = 1
keywords = arteriole
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2/5. Healing of skin necrosis and regression of anticardiolipin antibodies achieved by parathyroidectomy in a dialyzed woman with calcific uremic arteriolopathy.

    AIM: To present the impact of parathyroidectomy on the spontaneous healing of necrotic lesions of the skin of the lower leg and on anticardiolipin antibodies regression in a 68-year-old female dialyzed patient with hyperparathyroidism and calcific-uremic arteriolopathy (CUA). methods: After the occurrence of initial lesions of the lower leg skin, the intact parathyroid (iPTH) level, calcium (Ca) and phosphorus (P) product were measured, and on two occasions at 6-week intervals, the titer of anticardiolipin antibodies was determined, followed by a clinical monitoring of the progress of necrotic skin lesions. Two months after the occurrence of the skin lesions, the patient's right leg was amputated below the knee due to gangrene, and a histopathological analysis of the skin tissue sample of the amputated lower leg was made. After parathyroidectomy, iPTH, Ca x P product were measured, and on two occasions at 6 weeks' intervals, anticardiolipin antibodies titer was determined, followed by a clinical monitoring of lesions of the left lower leg skin. RESULTS: Before parathyroidectomy, iPTH level and Ca x P product were increased, as well as IgG anticardiolipin antibody titer measured on two occasions 6 weeks apart. The histopathological analysis of the skin tissue sample of the amputated right lower leg showed mural calcification of artery walls and thrombotic occlusions of small arteries, arterioles, and dermal capillaries, in addition to epidermolysis. A week after parathyroidectomy, iPTH level and Ca x P product were within normal range. Two measurements 6 weeks apart revealed no anticardiolipin antibodies. Eight weeks after parathyroidectomy, spontaneous healing of necrotic skin lesions of the left lower leg was observed. CONCLUSION: Regression of anticardiolipin antibodies, normalization of Ca x P product, and healing of the skin lesions after parathyroidectomy all pointed to the elevated PTH level as a crucial factor in the pathogenesis of CUA.
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ranking = 0.16666666666667
keywords = arteriole
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3/5. Bone morphogenic protein-4 expression in vascular lesions of calciphylaxis.

    calciphylaxis is characterized by an extensive media-calcification of cutaneous and subcutaneous arterioles and capillaries. Recent studies have provided evidence that vascular calcification is a process with similarities to bone metabolism. Bone morphogenic protein-4 (BMP-4) is physiologically involved in bone development and repair. The presence of BMP-4 in atherosclerosis and in sclerotic heart valves led us to suggest that BMP-4 is also involved in calciphylaxis. A 47-year-old male patient developed end-stage renal failure due to chronic glomerulonephritis. He has had two kidney transplants with an immunosuppressive regimen consisting of cyclosporine A and steroids. He was admitted to our hospital because of an increase in serum creatinine (Cr) and he subsequently developed progressive dermal ulcerations. A skin biopsy led to the diagnosis of calciphylaxis. immunohistochemistry for BMP-4 of a skin specimen from our patient showed strong cytoplasmic immunoreactivity of intradermal cells with clear spatial association to arterioles and hair follicles. Whereas there are identified inhibitors and promoters of vascular calcification, the presence of BMP-4 has not been demonstrated in calcific uremic arteriolopathy. In contrast to atherosclerosis, BMP-4 in calciphylaxis cannot be found in vascular media, but in intradermal cells at the border of arterioles and hair follicles. Therefore, in calciphylaxis BMP-4 can play the role of a cytokine, a growth factor or a media-calcification promoter.
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ranking = 0.5
keywords = arteriole
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4/5. The vascular lesions associated with skin necrosis in renal disease.

    Three renal allograft recipients and one uraemic patient presented with skin necrosis. In all cases, the subcutaneous arteries and arterioles were narrowed or occluded by mural calcification with or without intimal fibrosis. A review of the literature shows that uraemic patients or allograft recipients with skin necrosis involving the trunk or thighs have a poor prognosis while recovery is common in patients with more peripheral lesions. The aetiology of the arterial lesions is unknown. Their relationship to renal disorders and/or hyperparathyroidism is uncertain and therapy remains unsatisfactory.
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ranking = 0.16666666666667
keywords = arteriole
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5/5. Reversible malignant hypertension and azotemia due to urethral stricture.

    A previously normotensive 24-year-old black man developed malignant hypertension and azotemia. The patient was found to have bladder outlet obstruction due to urethral stricture. Transurethral dilation resulted in brisk improvement in renal function and rapid lowering of blood pressure in association with minimal diuresis. On follow-up one year later, while he was not receiving medications, the blood pressure was 120/70 mm Hg and the creatinine clearance was 150 ml/min. A kidney biopsy specimen showed minimal residual pathologic abnormalities in the renal arteries and arterioles. The renin-angiotensin system was probably involved in the maintenance of the hypertension, in view of the elevated peripheral plasma renin activity on admission. This represents a rare case of hypertension due to urethral stricture. Despite rapid progression to azotemic malignant hypertension, urethral dilation restored the blood pressure and renal function to normal.
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ranking = 0.16666666666667
keywords = arteriole
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