1/6. 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.- - - - - - - - - - ranking = 1keywords = calciphylaxis (Clic here for more details about this article) |
2/6. calciphylaxis: a rare complication of patients who required parathyroidectomy for advanced renal hyperparathyroidism.calciphylaxis is a relatively rare but life-threatening complication in uremic patients. Clinical findings and prognosis were evaluated in six patients who developed calciphylaxis from a group of 1499 patients who underwent parathyroidectomy (PTx) for advanced renal hyperparathyroidism (HPT) in our department from July 1972 to July 2003. The frequency of calciphylaxis was 0.40% (6/1499). Two patients were women and four were men. The mean age was 50.5 years, and the mean duration of hemodialysis (HD) treatment was 14.0 years. In five of six patients, calciphylaxis was classified as distal type; in one case, as proximal type. In three patients, calciphylaxis was diagnosed at the time for PTx. In two patients, calciphylaxis was identified after PTx, although the serum parathyroid hormone (PTH) level was within the appropriate range for dialysis patients. In two patients, calciphylaxis improved after PTx, but two patients required leg and toe amputations after PTx. In one patient with the proximal type of calciphylaxis, the condition occurred when a high PTH level recurred after the initial PTx. The patient died as a result of a serious infection due to uncontrollable skin ulcers. calciphylaxis is a rare complication in patients who require PTx for renal HPT. Especially the proximal type has a poor prognosis. High levels of the Ca x P product and/or PTH are risk factors. Therefore, this syndrome should be kept in mind and attention should be paid to reduce risk factors. It is important that PTx being performed at the right time in patients with advanced renal HPT refractory to medical treatment.- - - - - - - - - - ranking = 0.875keywords = calciphylaxis (Clic here for more details about this article) |
3/6. Fulminant pulmonary calciphylaxis and metastatic calcification causing acute respiratory failure in a uremic patient.calciphylaxis is a rare and life-threatening disorder characterized by small-vessel mural calcification with intimal proliferation, fibrosis, and thrombosis, resulting in tissue ischemic necrosis. Although it has been viewed as a systemic disease involving mainly the dermis, subcutaneous fat, or muscle, calciphylaxis of other organs rarely is reported. We describe the case of a 25-year-old uremic woman who rapidly developed massive pulmonary calcification that led to acute respiratory failure after the initiation of hemodialysis therapy. Chest radiography and high-resolution computed tomography showed typical pulmonary calcification. Pulmonary calciphylaxis and metastatic calcification were confirmed further by lung tissue biopsy. No skin or muscle calciphylaxis was discovered. Despite multiple factors precipitating pulmonary calciphylaxis in this patient, we speculate that hemodialysis was the main culprit in accelerating the development of fulminant pulmonary calciphylaxis and metastatic calcification. Alteration in the local environment from an acid to an alkaline condition and a relatively high dialysate calcium level in the presence of systemic hyperphosphatemia are believed to have facilitated the deposition of calcification. This case highlights the importance of "visceral calciphylaxis" and early identification of its causes.- - - - - - - - - - ranking = 1.25keywords = calciphylaxis (Clic here for more details about this article) |
4/6. 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.- - - - - - - - - - ranking = 0.625keywords = calciphylaxis (Clic here for more details about this article) |
5/6. The uremic gangrene syndrome: improved healing in spontaneously forming wounds following subtotal parathyroidectomy.patients with end-stage renal disease often demonstrate retarded healing of surgical wounds, but the basis for spontaneous wound formation in these patients is less well understood. We report our experience with four patients with a unique clinical entity previously described as the uremic gangrene syndrome (also known as calciphylaxis) that involves spontaneously forming and insidiously progressive wounds of the skin and soft tissue in uremic patients with hyperparathyroidism. The importance of recognizing this phenomenon relates to the potential benefit to wound-healing efforts resulting from subtotal parathyroidectomy and adjustment of serum calcium and phosphate levels when severe hyperparathyroidism is present. Disrupted parathyroid homeostasis as a mechanism for soft-tissue ischemia and subsequent infarction is supported by wound biopsies demonstrating microarterial calcification. As experts in factors resulting in refractory wounds, plastic surgeons need be aware of this peculiar vulnerability for spontaneously forming wounds in uremic patients. Clinical and laboratory findings, success with wound treatment in four patients, and currently popular pathophysiologic mechanisms are discussed.- - - - - - - - - - ranking = 0.125keywords = calciphylaxis (Clic here for more details about this article) |
6/6. Uremic small artery disease: calciphylaxis with penis involvement.Two male patients with chronic renal failure maintained on hemodialysis developed progressive clinical features of ischemic necrosis (so called calciphylaxis) of their extremities and penis. Both patients died. In one patient, penectomy provided tissue for histopathologic examination and there were changes of small artery calcification. A role of iron overload on the production of calciphylaxis is reviewed. This report is perhaps the first in nephrology literature on the occurrence of calciphylaxis involving penis and prepuce.- - - - - - - - - - ranking = 0.875keywords = calciphylaxis (Clic here for more details about this article) |