1/131. calciphylaxis: one hospital's experience.The lives of the ESRD population have been enhanced and prolonged by improved conservative management, efficient dialysis and successful transplantation. However, practitioners are noting some significant morbidity in these long-term surviving nephrology patients. Clinical signs of renal osteodystrophy can be very subtle and insidious. Bone pain, muscle weakness, and pruritis can be vague symptoms. calciphylaxis, which is classified as a skin disorder is an extremely rare, painful, and progressive complication. Typically, the patient presents with vascular calcifications, cutaneous ulcerations and tissue necrosis. The treatment regimen for bone disease in the ESRD population will be reviewed. The symptoms of calciphylaxis, diagnostic tests and prognosis will be discussed. Four case studies describe our recent experience. Emphasis will be on the intense multidisciplinary care required for this devastating and often fatal syndrome.- - - - - - - - - - ranking = 1keywords = calciphylaxis (Clic here for more details about this article) |
2/131. Intensive tandem cryofiltration apheresis and hemodialysis to treat a patient with severe calciphylaxis, cryoglobulinemia, and end-stage renal disease.This is the first report on tandem cryofiltration apheresis (CFA) and hemodialysis (HD). A 44 year old white man with Type II mixed cryoglobulinemia, hepatitis c virus (HCV), severe skin lesions, and end-stage renal disease (ESRD) on maintenance hemodialysis was air-transferred for CFA, which is only available at our medical center. The patient failed to respond to high dose steroids, immunosuppression, intravenous immunoglobulin (IVIG), and plasma exchange for the treatment of his cryoglobulinemia, and he failed alpha-interferon therapy for his HCV. On arrival, he was also found to have severe calciphylaxis secondary to ESRD with generalized, painful skin ulceration, necrosis, and penile gangrene. To treat both conditions, intensive, tandem CFA/HD was initiated. He received extensive wound care and surgical debridement. To prevent pressure ulcers and worsening of skin lesions, he was placed on the FluidAir (Kinetic Concepts Inc., San Antonio, TX) controlled air bed. The patient received 18 tandem CFA/HD treatments, and four extra HD treatments in one month. sodium citrate was used as an anticoagulant for the CFA procedure. His plasma cryoglobulin (CG) level dropped from 6,157 to 420 microg/ml, and his calciphylaxis also improved. The CFA effectively removed 93% of CG, without significant removal of IgG, IgM, IgA, albumin, and fibrinogen. No albumin or fresh frozen plasma (FFP) was required as replacement fluid for CFA. No citrate toxicity or evidence of complement activation with the cryofilter was observed. The entire CFA procedure time (3(1/2) hours) was considered. Intensive, tandem CFA/HD was performed in a critically ill patient with no apparent adverse consequences.- - - - - - - - - - ranking = 6keywords = calciphylaxis (Clic here for more details about this article) |
3/131. Five cases of calciphylaxis and a review of the literature.calciphylaxis is a rare phenomenon of cutaneous necrosis that typically occurs in association with renal failure and has a poor prognosis. We report 5 new cases of calciphylaxis that illustrate the important clinical and histopathologic features of the disease. All patients had end-stage renal failure at the time that purpuric plaques and nodules were noted; these subsequently progressed to necrotic ulcers with eschars. All skin biopsy specimens showed varying degrees of calcification of the medial layer of blood vessel walls in the dermis and subcutaneous fat. Neither the product of serum calcium and phosphorus concentrations nor parathyroid hormone levels correlated temporally with the clinical observations in every case, emphasizing the importance of clinical-histopathologic correlation. Although certain features of calciphylaxis in humans resemble the animal model originally proposed, there are also some crucial differences. We review the pathogenesis, epidemiology, clinical and histopathologic features, and treatment of this disease.- - - - - - - - - - ranking = 6keywords = calciphylaxis (Clic here for more details about this article) |
4/131. calciphylaxis associated with metastatic breast carcinoma.calciphylaxis is a rare disorder associated with calcification of small- and medium-sized blood vessels, and progressive skin necrosis usually seen in the setting of end-stage renal disease (ESRD) and secondary hyperparathyroidism. It has also been observed in primary hyperparathyroidism, hypercalcemia of malignancy (extensive bony metastasis of breast cancer), and an isolated case reported with end-stage liver disease. We report an unusual case of calciphylaxis associated with metastatic breast carcinoma in the absence of renal or parathyroid disease. calciphylaxis has generally been associated with end-stage renal disease and hyperparathyroidism. One previous case report described calciphylaxis occurring in a patient with metastatic adenocarcinoma of the breast and hypercalcemia. Our case represents the second reported case of calciphylaxis associated with osteolytic, metastatic breast cancer. Although ESRD with secondary hyperparathyroidism is the most common presentation of calciphylaxis, this case demonstrates that other conditions that alter normal calcium metabolism must be considered in the differential diagnosis.- - - - - - - - - - ranking = 4keywords = calciphylaxis (Clic here for more details about this article) |
5/131. Calcific uremic arteriolopathy in association with low turnover uremic bone disease.We report seven cases (out of 100 dialysis patients) at the University of kentucky who were diagnosed with calciphylaxis syndrome between 1993 and 1998. Of note is the fact that five of these patients had bone biopsy-proven adynamic renal bone disease, rather low intact PTH, and relatively low calcium-phosphorus product. This is in contrast to the previous view that calciphylaxis is usually seen in patients with excessive parathyroid activity. The reemergence of calciphylaxis with renal bone disease is an intriguing finding and does not correspond to earlier reports associating calciphylaxis with hyperparathyroid bone disease. This report reviews the clinical, biochemical, and bone histology findings of these patients, and provides a review of the literature.- - - - - - - - - - ranking = 4keywords = calciphylaxis (Clic here for more details about this article) |
6/131. calciphylaxis precipitated by ultraviolet light in a patient with end-stage renal disease secondary to systemic lupus erythematosus.calciphylaxis is a rare and severe calcification syndrome described mainly in patients with end-stage renal disease (ESRD) undergoing dialysis or with a renal transplant. This life-threatening condition is characterized by the abrupt onset of painful ischemic skin ulcers and necrosis. Secondary local and systemic infection may supervene and, without timely and appropriate interventions, calciphylaxis may be fatal. A precipitant or challenging agent is believed to be necessary to initiate the process. We describe a case of a woman with ESRD receiving continuous ambulatory peritoneal dialysis who developed calciphylaxis in the setting of severe hyperparathyroidism after receiving UV photoradiation therapy. We suggest that the UV light served as the challenging agent in the precipitation of this devastating condition.- - - - - - - - - - ranking = 2keywords = calciphylaxis (Clic here for more details about this article) |
7/131. rhabdomyolysis as the presenting manifestation of calciphylaxis.A 43-year-old woman was admitted with progressive leg pains and weakness and was found to have rhabdomyolysis. Prior to this admission the patient had hypercalcemia, but this returned to normal following treatment with calcitonin. During the hospitalization, she developed the syndrome of calciphylaxis consisting of necrotic skin and muscle associated with vascular calcification. This is the first case report of rhabdomyolysis caused by calciphylaxis in a patient without chronic renal failure.- - - - - - - - - - ranking = 6keywords = calciphylaxis (Clic here for more details about this article) |
8/131. calciphylaxis treated with neurolytic lumbar sympathetic block: case report and review of the literature.BACKGROUND AND OBJECTIVE: calciphylaxis is a painful complication of end-stage renal disease and secondary hyperparathyroidism. Calcification most commonly affects skin and soft tissue of the lower extremities resulting in excruciatingly painful skin ulcers. Treatment involves correction of hypercalcemia and hyperphosphatemia, parathyroidectomy, and supportive measures. methods: The literature and the merits of neurolytic lumbar sympathetic blockade (LSB) for the treatment of pain associated with calciphylaxis are reviewed. CONCLUSIONS: The neurolytic LSB provided pain relief and is a treatment modality to be considered in managing the pain associated with calciphylaxis.- - - - - - - - - - ranking = 2keywords = calciphylaxis (Clic here for more details about this article) |
9/131. Typical features of calciphylaxis in a patient with end-stage renal failure, diabetes mellitus and oral anticoagulation.We report a multimorbid patient with end-stage renal failure showing a large necrosis and livedo racemosa on the right thigh. histology revealed medial calcification of the small arteries typical of calciphylaxis. We found the typical features of the disease with different risk factors like elevated calcium-phosphate product, diabetes mellitus and oral anticoagulation. On account of the location of the skin lesions, a bad prognosis was expected. In spite of therapeutical measures with lowering of the calcium and phosphate levels, the patient died 1 month after the diagnosis had been made.- - - - - - - - - - ranking = 5keywords = calciphylaxis (Clic here for more details about this article) |
10/131. calciphylaxis.calciphylaxis is a rare, life-threatening condition of widespread metastatic calcification most commonly seen in the setting of end-stage renal disease. The etiology of calciphylaxis is not well described, though there are several hypotheses. Cutaneous lesions are characteristically found on the abdomen, buttocks, or thighs as reticulated, painful, purple plaques that often undergo ulceration and may serve as a portal of entry for potentially life-threatening infectious agents. histology reveals medial calcification with intimal proliferation involving small vessels in the subcutaneous fat, associated with a lymphohistiocytic infiltrate of the affected lobules. Treatment, including phosphate binders and parathyroidectomy, is not universally effective. We present one case of calciphylaxis and discuss the clinical features, pathophysiology, histology, and treatment of the condition.- - - - - - - - - - ranking = 2keywords = calciphylaxis (Clic here for more details about this article) |
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