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Calciphylaxis is an uncommon phenomenon usually observed in patients with chronic renal failure who are on dialysis and who have secondary hyperparathyroidism. Treatment is multifocal and may include wound debridement, wound excision, and various methods of achieving closure. Removal of the parathyroid glands often is advocated, but this option remains controversial. Despite treatment, there is a fatal outcome in a majority of cases due to septicemia. Prompt recognition and treatment are imperative to help prevent septicemia. A review of the literature and two case studies are presented.
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PMID:Calciphylaxis and its relation to end-stage renal disease: a literature review and case presentation. 1188 32

Calciphylaxis is a rate complication of unknown pathogenesis in patients with end stage renal disease. It is characterized by calcification of tunica media of small arteries associated with intimal fibrosis and thrombus formation which leads to the development of skin and subcutaneous tissue necrosis. Superinfection of skin lesions is a common consequence of this syndrome which may lead to the sepsis. The prognosis of this condition in serious. We performed a retrospective study of 6 subjects (4 men and 2 women) in the age of 35 to 59 years. We followed the parameters of calcium-phosphate metabolism, presence of calciphylaxis risk factors and the effect of parathyreoidectomy. Five patients were on hemodialysis, one had a kidney transplant. Skin and subcutaneous tissue necrosis were present in all subjects. The serum levels of parathormone were either high, normal or low, levels of calcium were normal or slightly elevated and phosphate levels were high or normal. Calcium was substituted before calciphylaxis development in 5 patients, calcitriole in 3 of therm. Five patients underwent parathyroidectomy. Three patients died (all of sepsis), one patient had the lower into amputation for infected lesions and the remaining two achieved regression. Our findings do support the hypothesis that calcium and calcitriole administration participates in development of calciphylaxis. Fatal prognosis of the once infections skin lesions was also proved.
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PMID:[The calciphylaxis syndrome as a complication of chronic kidney failure. 6 case reports and literature review]. 1209 60

Calciphylaxis is an uncommon condition usually seen in patients with end-stage renal disease. The typical features include violaceous skin lesions overlying painful, indurated, subcutaneous nodules. Necrosis and nonhealing ulcers, with secondary gangrene, sepsis, and death frequently follow. The outpatient hemodialysis population has a reported calciphylaxis prevalence of 1% to 4.1%; however, published studies contain only a few case reports of penile calciphylaxis. The urologic presentation consists primarily of penile gangrene. A description of our patient, the underlying pathologic features, a review of the relevant published studies, and the possible predisposing conditions are included.
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PMID:Penile calciphylaxis. 1213 44

Calciphylaxis is a rare and potentially fatal complication of end stage renal disease (ESRD) and secondary hyperparathyroidism associated with abnormal calcium metabolism. Painful purple skin lesions are often the presenting sign with digital ischemia, ulcerations, and necrosis being the characteristic features of the disease. Skin or incisional biopsy showing widespread calcifications and fibrinous thrombi without inflammation will confirm the diagnosis. Early recognition and prompt treatment of calciphylaxis is vital for symptom relief, ulcer healing, and prevention of sepsis and death. This article will educate nurses to recognize this disease in hope of early treatment. The article includes information about disease history, pathogenesis, etiology, clinical manifestations, diagnosis, prevention, and current treatment, and, in addition, presents case study of a patient with calciphylaxis.
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PMID:Calciphylaxis: what nurses need to know. 1243 50

Calciphylaxis is a rare, painful, and debilitating disease of widespread metastatic calcification that progresses to ischemic tissue necrosis. It occurs almost exclusively in patients with end-stage renal disease and secondary hyperparathyroidism. Reticulated, violaceous, mottled patches commonly appear on the lower extremities and progress to cordlike nodules and necrotic ulcers with overlying eschars. Septicemia, which may occur after secondary infection of the ulcers, is the most common cause of death. Treatment with phosphate-binding antacids, total or partial parathyroidectomy, and avoidance of local tissue trauma remain the mainstays of therapy, although a uniform cure for calciphylaxis remains elusive. Prognosis for patients with calciphylaxis is dismal, even after surgical intervention. Early diagnosis and treatment is critical to prevent progression of the disease process.
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PMID:Metastatic calcification. 1459 75

Calciphylaxis is a confusing disease process that affects people with end-stage renal disease. The prognosis of this increasingly common condition is poor and mortality rates range from 60% to 80% related to wound infection, sepsis, and organ failure. Its presenting sign is skin necrosis related to calcification of the arteriole microvasculature. The disease is painful and debilitating, particularly due to the necrotic wounds. Aggressive wound care to prevent infection is vital when eschar does not protect the wound and drainage is present, but debridement is contraindicated for wounds covered with dry, noninfected eschars. The decision to debride is based on the patient's total clinical picture. Patients with calciphylaxis have poor healing potential due to ischemia and comorbidity factors such as diabetes mellitus, peripheral vascular disease, and obesity. The goal of care is prevention of infection and pain management. Some of the sensitizers and challengers responsible for the chemical imbalance leading to the arteriole calcification, as well as risk factors and clinical manifestations of calciphylaxis, are reviewed. A discussion of treatment focuses on wound care of stable necrotic ulcers and a case report illustrating the progression of calciphylaxis is presented.
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PMID:Mysterious calciphylaxis: wounds with eschar--to debride or not to debride? 1525 2

Calciphylaxis is a potentially life-threatening disease that occurs up to 4% of patients with chronic terminal renal failure and secondary hyperparathyroidism. Clinical symptoms are painful skin erythema and ischemic necrosis as a result of calcification of the small and medium-sized arteries of the subcutaneous tissues. The mortality of the disease is as high as 87% mainly due to sepsis from superinfection. We report on a 59-year-old patient with chronic renal failure and normal calcium-phosphate-product with leg ulcers as a rare manifestation of a calciphylaxis. In spite of an early parathyroidectomy and a temporary complete wound-healing the death because of calciphylaxis could not be prevented.
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PMID:[Calciphylaxis in terminal renal failure as a rare manifestation of leg ulcers]. 1618 43

Calciphylaxis presents like subcutaneous lesions with livedo reticularis leading to necrotic and painful ulcers, predominantly in the lower limbs and the abdomen. They initially simulate dermohypodermitis. Biology reveals secondary hyperparathyroidism, phosphocalcic metabolism abnormalities and state of hypercoagulability. Histological signs are constant: calcifications in the media of small and sub-cutaneous arteries, intimal hyperplasia and intravascular thrombosis. This complication occurs in 4% of end-stage renal disease patients. Its prognostic is awful with a rate of mortality of 60% due to sepsis. Treatment is based upon the normalization of phosphocalcic rates and local debridement.
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PMID:[Calciphylaxis: a severe but unrecognized complication in end-stage renal disease patients. A review of 2 cases]. 1624 22

Calciphylaxis may be considered a small vessel vasculopathy which is generaly associated with end-stage renal disease and hyperparathyroidism. The precise pathogenesis of the disease is not known. It needs sensitizers and challengers to occur. Steroids and immunosuppressive drugs including methotrexate are among those challenger agents. Calciphylaxis in collagen vascular diseases is rare. Only one case in rheumatoid arthritis was recently reported. Here we describe a case of calciphylaxis associated with active rheumatoid arthritis. This patient had active disease despite treatment of steroids and methotrexate for a long time. She died shortly after the diagnosis of calciphylaxis due to sepsis.
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PMID:Development of calciphylaxis after long-term steroid and methotroxate use in a patient with rheumatoid arthritis. 1635 57

'Calciphylaxis', a calcification syndrome associated with ischaemic cutaneous necrosis, is acquired naturally in humans in disease states. It is a life and limb-threatening complication, usually observed in patients with renal disease and secondary hyperparathyroidism, but known to occur in the absence of renal or parathyroid disease. The reported mortality rate, which ranges from 60-80%, relates to wound infection, sepsis and organ failure. It is a small-vessel vasculopathy, which is estimated to occur in about 4% of haemodialysis patients. Clinically, violaceous, reticulate areas of cutaneous necrosis and eschar may be evident, particularly in the extremities. In addition to the clinical picture, a raised calcium phosphorous product, an elevated parathyroid hormone level, radiographic evidence of vessel and soft-tissue calcification and the finding of mural calcification affecting small arteries and arterioles on histopathology help to confirm the diagnosis of this entity which generally has a poor prognosis. A high index of suspicion and an active multidisciplinary management approach, with rigorous attention to wound care and prevention of sepsis, are vital in the management of these patients. In this overview, we discuss the pathophysiology, clinical features and associations, risk factors, diagnosis and management issues relating to calciphylaxis.
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PMID:Calciphylaxis--a topical overview. 1668 74


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