11/34. Distal calcific uremic arteriolopathy in a hemodialysis patient responds to lowering of Ca x P product and aggressive wound care.Calcific uremic arteriolopathy (CUA; calciphylaxis), is reported in approximately 4% of patients receiving hemodialysis, and is characterized by skin lesions that may include firm plaques or subcutaneous nodules. The syndrome has been associated with the use of calcium-containing phosphate binders, high serum phosphorus levels, and elevated calcium x phosphorus (Ca x P) product. This report describes a 73-year-old white male with chronic renal failure due to diabetes mellitus and hypertension, who had been on home hemodialysis for 3 years. He developed CUA after an acute elevation in serum phosphorus (8.1 mg/dl) and Ca x P product (84.2), with painful skin lesions that rapidly progressed to become circumferentially located around the entire lower left extremity. The patient declined amputation, opting for a treatment approach that included aggressive management of phosphorus and calcium, more frequent dialysis, and rigorous wound care. All calcium-containing phosphate binders were discontinued. The patient was switched from calcitriol to paricalcitol, a less calcemic form ofvitamin D replacement therapy, from which he was slowly weaned. dialysis dose and frequency was also increased to 4 hours, 6 times weekly. The patient was given sevelamer hydrochloride (Renagel)--a calcium-free phosphate binder--with meals at an initial dose of 6.4 g/day. After 5 months, the dose was increased to 8 g/day, with additional dietary counseling to restrict phosphorus intake. At this point, serum phosphorus decreased to 4.9 mg/dl and calcium levels had fallen to 8.5 mg/dl, compared to 9.5 - 10.4 mg/dl prior to diagnosis of CUA with an overall decline in the Ca x P product. Significant healing of the lesions was noted at 8 months following diagnosis, with near-total healing by 12 months. Our studies support that lowering of elevated serum phosphorus, calcium, and Ca x P product, together with aggressive wound care may contribute to the successful outcome of patients with CUA.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
12/34. Malignant melanoma of the soft parts showing calciphylaxis.calciphylaxis is a poorly understood and highly morbid syndrome of vascular calcification and skin necrosis. hypercalcemia, hyperphosphatemia, and secondary hyperparathyroidism are the factors implicated in the pathogenesis of calciphylaxis, which is generally identified in patients with hyperparathyroidism secondary to end-stage renal disease. It has also been observed in primary hyperparathyroidism, end-stage liver disease, and rheumatoid arthritis, in the absence of renal disease. There are few case reports of calciphylaxis occurring in hypercalcemia of malignancy. An unusual case is reported of calciphylaxis associated with malignant melanoma of the soft parts in the absence of renal or parathyroid disease. This is the first reported case of this soft tissue sarcoma showing calciphylactic changes.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
13/34. calciphylaxis in a patient with alcoholic liver disease in the absence of renal failure.calciphylaxis is a rare, potentially life-threatening syndrome characterized by progressive microvascular and superficial soft tissue calcification, usually seen in patients with chronic renal failure. We describe an unusual case of calciphylaxis in a patient with alcoholic liver disease and normal renal function who responded well to conservative wound care.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
14/34. weight loss-induced calciphylaxis: potential role of matrix metalloproteinases.calciphylaxis is an uncommon and often devastating syndrome of calcification of small vessels, leading to tissue infarction. The mechanism of how calcium deposits on small vessels is unknown. Recently, metalloproteinase digestion of elastin has been shown to enhance deposition of calcium, suggesting a possible mechanism of calciphylaxis. We describe a case of a patient who developed calciphylaxis after rapid weight loss, but had normocalcemia and normal renal function. She was found to have high levels of matrix metalloproteinases, which may have chemically altered elastin, allowing deposition of calcium on small vessels. Inhibitors of matrix metalloproteinases may be useful in the prevention and treatment of calciphylaxis.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
15/34. calciphylaxis associated with acute, reversible renal failure in the setting of alcoholic cirrhosis.We describe a case of calciphylaxis in a 47-year-old man with alcohol-induced end-stage liver disease and acute renal failure secondary to hepatorenal syndrome. Possible contributing factors included transiently impaired renal function, protein c and S deficiencies, elevated calcium-phosphate product, hyperphosphatemia, low serum albumin, repeated albumin infusions, and elevated alkaline phosphatase level.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
16/34. Bilateral extensive vascular calcification of the breast associated with coagulative necrosis: a calciphylaxis-like syndrome.A case of asynchronous bilateral breast necrosis in a 40-year-old woman with untreated chronic hypercholesteraemia is described. Mastectomies were performed, and histological examination revealed bilateral breast calciphylaxis characterized by extensive vascular calcification with coagulative necrosis. breast calciphylaxis is a rare disease clinically characterized by progressive tissular necrosis with secondary cutaneous ulceration and by vascular calcification and thrombosis. However, the nature of the vascular calcification has remained poorly understood up to now, owing to the absence of any precise mineralogical examination. In this case the mineral deposits were analysed for the first time: only hydroxyapatite was found. The etiology is discussed, and the high cholesterol rate of the patient is implicated.- - - - - - - - - - ranking = 4keywords = syndrome (Clic here for more details about this article) |
17/34. calciphylaxis in a patient with poems syndrome without renal failure and/or hyperparathyroidism. A case report.POEMS (Crow-Fukase) syndrome is a rare plasma cell lymphoproliferative disorder associated with polyneuropathy (P), organomegaly (O), endocrinopathy (E), monoclonal (M) gammopathy and skin (S) abnormalities. The latter are usually not specific and include hyperpigmentation, hypertrichosis, cutaneous angioma and skin-thickening. A 45-year-old Italian woman was admitted to hospital because of muscle weakness, marked fatigue and paresthesia of the upper and lower extremities. Two and a half years earlier, a poems syndrome had been diagnosed on the basis of a history of organomegaly and mild lymphadenopathy, IgA-lambda monoclonal gammopathy, hypothyroidism, severe lower and upper limb sensory-motor peripheral neuropathy and a single osteosclerotic lesion in the left humerus. Eight weeks later, she developed skin lesions bioptically shown to be due to calciphylaxis-induced cutaneous vasculitis. To our knowledge, this is the first case of poems syndrome with this peculiar type of vasculitis. The absence of predisposing conditions, namely renal failure, hyperparathyroidism or clotting disorders renders the pathogenetic mechanism(s) of this severe type of vasculitis more intriguing.- - - - - - - - - - ranking = 7keywords = syndrome (Clic here for more details about this article) |
18/34. 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 = 1keywords = syndrome (Clic here for more details about this article) |
19/34. Widespread cutaneous and systemic calcification (calciphylaxis) in patients with the acquired immunodeficiency syndrome and renal disease.BACKGROUND: calciphylaxis is a form of widespread calcification that may occur in patients with renal disease and hyperparathyroidism. The skin is often affected secondary to vascular compromise and ischemia. patients infected with the human immunodeficiency virus (hiv) are predisposed to renal failure and thus may develop this serious complication. OBJECTIVE: The purpose of this article is to describe two cases of fatal calciphylaxis in patients with the acquired immunodeficiency syndrome and renal disease. methods: Two patients were examined clinically and through the performance of serologic studies and skin biopsies. RESULTS: Both patients had a widespread livedo reticularis-like eruption, which rapidly progressed to cutaneous gangrene. Patient 1 had elevated serum calcium and phosphorus levels whereas patient 2 had normal values but had markedly elevated serum parathyroid hormone levels. biopsy specimens in both cases showed vascular thrombosis and soft tissue calcification. CONCLUSION: Widespread calcification may develop in patients with hiv infection and renal failure and, in some cases, calcium and phosphorus levels may be normal.- - - - - - - - - - ranking = 5keywords = syndrome (Clic here for more details about this article) |
20/34. Bone scintigraphy of calciphylaxis: a syndrome of vascular calcification and skin necrosis.calciphylaxis is a highly morbid syndrome of vascular calcification and skin necrosis, the pathophysiology of which remains largely elusive. We report a patient with end-stage renal disease and multiple painful skin lesions who underwent a bone scan for extremity pain. Increased tracer accumulation was seen in the subcutaneous tissues of the trunk and lower extremities. In this case, the bone scan aided in the diagnosis and treatment of calciphylaxis for a patient who experienced a relatively short hospital stay.- - - - - - - - - - ranking = 5keywords = syndrome (Clic here for more details about this article) |
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