Cases reported "Calciphylaxis"

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1/9. 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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keywords = kidney
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2/9. Calcific uremic arteriolopathy in a transplanted kidney.

    Calcific uremic arteriolopathy is a severe and life-threatening condition that develops in patients with kidney disease. Most commonly, it presents with skin rash and painful subcutaneous nodules. However, other organs may be involved. We report a case of a young man who developed renal failure in the transplanted kidney. Renal biopsy demonstrated myointimal calcification in the renal arteries consistent with this diagnosis.
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ranking = 84.144129736645
keywords = kidney disease, kidney
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3/9. association between calciphylaxis and inflammation in two patients on chronic dialysis.

    The pathogenesis of calciphylaxis, which has a rising incidence in the chronic dialysis population and a high mortality rate, is poorly understood. Abnormalities in the calcium-phosphorus-parathyroid axis are clinically related to calciphylaxis, but alone, they cannot explain this condition. Here, we present two patients who had chronic inflammatory conditions and hyperparathyroidism and who developed calciphylaxis. A 41-year-old white woman on hemodialysis following scleroderma, hepatitis c, liver transplant, and failed kidney transplant, developed progressive ulcerative lower extremity calciphylaxis lasting more than 3 years. She had evidence of severe hyperparathyroidism and elevated serum c-reactive protein (CRP). A 39-year-old white woman on continuous ambulatory peritoneal dialysis for 6 years for renal failure secondary to lupus nephritis, with sustained lupus activity during the dialysis period, developed rapidly progressing ulcerative calciphylaxis of the lower and upper extremities not responding to adequate treatment of hyperphosphatemia and hyperparathyroidism. Her condition culminated in death within 2 months of the appearance of the skin lesions. Her serum CRP was elevated on a sustained basis before the development of the calciphylaxis and rose to a very high level after appearance of the skin lesions. inflammation may assist in the development of calciphylaxis through depression of serum levels of fetuin-A, an endogenous inhibitor of calcification that is also a negative acute-phase reactant. The interactions between inflammation-mediated changes in the levels of endogenous inhibitors of calcification and abnormalities in calcium-phosphorus metabolism merit intensive study in the future as potential mechanisms of calciphylaxis.
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keywords = kidney
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4/9. A case report comparing various radiological tests in the diagnosis of calcific uremic arteriolopathy.

    BACKGROUND: Calcific uremic arteriolopathy (CUA) is a rare necrotizing skin condition characterized by calcification in arterioles, leading to ischemia and skin ulcerations. This disease affects 1% to 4% of patients with chronic kidney disease and has a reported mortality rate up to 80%. The diagnosis of CUA is based on clinical judgment suggested by the characteristic skin lesions. Although skin biopsy is the gold standard for establishing the diagnosis, it is performed infrequently because of poor healing and risk for secondary infections. methods: In this case report, we compare the ability of various radiological tests to show arteriolar calcifications of patients with CUA. Our patient had biopsy-proven CUA manifesting as chronic nonhealing ulcers of the calves. She underwent soft-tissue x-ray of the affected extremities and high-resolution (0.5-mm slice) computed tomographic (CT) scanning with 3-dimensional image reconstruction. We also used a dedicated mammography machine to obtain images of the patient's calves. Images were compared based on the ability to show small-vessel calcification. RESULTS: Plain soft-tissue x-ray showed mildly increased soft-tissue density and very few calcified vessels, whereas CT showed few calcified small- and medium-sized arterioles. Diffuse calcification of small arterioles in a mesh-like pattern was shown by means of the mammography technique. CONCLUSION: Simple, safe, and inexpensive x-ray imaging using the mammography technique was superior to plain soft-tissue x-ray and 3-dimensional CT in showing the hallmark arteriolar calcifications of patients with CUA. Thus, we propose a possible role for this technique in diagnosing CUA.
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ranking = 79.144129736645
keywords = kidney disease, kidney
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5/9. nephrogenic fibrosing dermopathy and calciphylaxis with pseudoxanthoma elasticum-like changes.

    nephrogenic fibrosing dermopathy (NFD) and calciphylaxis are rare conditions that are associated with chronic kidney disease. Histopathologic changes, including dystrophic dermal calcification, often in association with elastic fibers have been observed in NFD and calciphylaxis. A pattern of dermal elastic fiber calcification that mimics pseudoxanthoma elasticum (PXE) has been previously reported as an incidental finding in the setting of calciphylaxis. Despite a shared association with renal disease and abnormal calcium deposits, however, NFD and calciphylaxis are discrete pathologic processes with distinct clinical and histopathologic features. Criteria for each are reviewed through case presentation of a patient meeting the clinical and histopathologic criteria for both NFD and calciphylaxis with histologic features mimicking PXE.
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ranking = 79.144129736645
keywords = kidney disease, kidney
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6/9. Fulminant calcinosis in two patients after kidney transplantation.

    The first patient had total parathyroidectomy (PTX) with autografting 2 years before. She developed disabling soft-tissue calcifications following kidney transplantation. After reduction of the autograft--a complete removal was not possible--the subcutaneous calcifications regressed. In the second patient, fulminant necrotizing vascular calcinosis developed after successful renal transplantation. Total PTX without autotransplantation was carried out, and progression of the vascular disease was ceased. In both patients, the parathyroid hormone was elevated but not the calcium-phosphate product. We suggest that fulminant calcinosis in patients with kidney transplants requires PTX.
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keywords = kidney
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7/9. A case of calciphylaxis. Case report.

    A young woman on hemodialysis with hyperparathyroidism suddenly developed areas of skin necrosis caused by arterial calcification. parathyroidectomy apparently arrested this process. Seven months after cadaver kidney transplantation, following reparation of a graft artery stenosis, necrosis spread anew from the wound gradually extending to large areas. The patient died and autopsy revealed calcification in many internal organs. The case fits the description of systemic calciphylaxis a syndrome now described in humans but originally experimentally induced in rats.
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ranking = 1
keywords = kidney
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8/9. Uremic small-artery disease with medial calcification and intimal hyperplasia (so-called calciphylaxis): a complication of chronic renal failure and benefit from parathyroidectomy.

    BACKGROUND: Uremic small-artery disease with medial calcification and intimal hyperplasia can lead to life-threatening skin necrosis or acral gangrene. It is a distinct complication of chronic renal failure that must be differentiated from soft-tissue calcification. An increased calcium-phosphate product and secondary hyperparathyroidism are the main underlying conditions. The benefit of parathyroidectomy is controversial. OBJECTIVE: This article is based on a literature search to determine prognostic factors and, in particular, the benefit of parathyroidectomy. methods: The literature on uremic small-artery disease (so-called calciphylaxis) was reviewed (full data set: 104 cases, including five of our own). The therapeutic benefit of parathyroidectomy and the relation between prognostic predictors (localization, dialysis, and transplant) and outcome were analyzed. The relation between diabetes and acral gangrene was also examined. Further epidemiologic data on the reviewed group of patients were established. RESULTS: Thirty-eight of 58 patients who underwent parathyroidectomy survived compared with 13 of 37 patients who did not undergo parathyroidectomy (p = 0.007, n = 95). Forty of 53 patients with distal localization of necrosis survived compared with 11 of 42 patients with proximal pattern (p < 0.00001; n = 95). Dialysis and kidney transplantation followed by immunosuppression showed no relation to disease outcome. No association was found between diabetes and acral gangrene (p = 0.50). CONCLUSION: Uremic small-artery disease is a distinct complication of chronic renal failure. Its recognition and early diagnosis should allow more effective treatment. In our retrospective study parathyroidectomy was significantly related to survival. Only a randomized, controlled, prospective trial (parathyroidectomy vs conservative treatment of secondary hyperparathyroidism) can establish the value of parathyroidectomy in uremic small-artery disease.
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keywords = kidney
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9/9. Severe calciphylaxis in a renal patient on long-term oral anticoagulant therapy.

    The pathogenesis of calciphylaxis, a potentially life-threatening condition, is not well understood. Several factors such as end-stage renal disease (azotemia), hyperparathyroidism, hyperphosphatemia, hypercalcemia, a high calcium-phosphate product, and the use of steroids and cytotoxic drugs after kidney transplantation are believed to interact in calciphylaxis. Recently, hypercoagulability due to functional protein c deficiency has been suggested to play a pathogenic role in this condition. Here, we present a renal transplant patient, with secondary hyperparathyroidism and on long-term oral anticoagulant therapy, who developed calciphylaxis with severe skin necrosis of her legs. The patient's condition improved dramatically after total parathyroidectomy. Hypercoagulability, therefore, does not appear to have played a significant role in this case of calciphylaxis.
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keywords = kidney
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