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The phenomenon of calciphylaxis is rare, but potentially fatal. It has been recognised for a long time in patients with chronic renal failure with secondary hyperparathyroidism. Disturbed calcium and phosphate metabolism can result in painful necrosis of skin, subcutaneous tissue and acral gangrene. Appearance of the lesions is distinctive but the pathogenesis remains uncertain. The beneficial effects of parathyroidectomy are controversial. However, correction of hyperphosphataemia or occasionally hypercalcaemia is imperative. Fulminant sepsis as a consequence of secondary infection of necrotic and gangrenous tissue is a frequent cause of patient morbidity and mortality.
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PMID:Calciphylaxis. 1152 12

Calciphylaxis is a rare, painful, necrotizing skin condition that occurs most frequently in patients with chronic renal failure who are receiving dialysis. These patients commonly have secondary hyperparathyroidism. Treatment involves a multidisciplinary approach. Surgical wound debridement, local wound care, pain control, and nutritional support are the primary care issues that must be addressed. The outcome is often poor, with the fatal outcome often resulting from sepsis.
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PMID:Calciphylaxis in the patient with chronic renal failure. 1183 81

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 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 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

Calcinosis cutis, one of the rare manifestations of systemic calcinosis, is characterized by the deposition of calcium and phosphate salts in the skin. Metastatic calcinosis, usually a late complication of chronic renal failure, arises from increased calcium or phosphate levels in the serum or both. Both sexes and all ages may be affected; however, cutaneous involvement is uncommon, particularly in children. We present the youngest patient, to our knowledge, with end-stage renal disease and cutaneous metastatic calcification resulting from secondary hyperparathyroidism. A 2-month-old infant presented to the pediatric service with anuria and uremia. A renal biopsy specimen showed chronic tubulo-interstitial nephritis. Indurated, firm, tender reddish papules were localized to both lower limbs, and extensive irregular nodules and plaques with ulceration and white stony contents were localized to the right upper limb. Topical antibiotic ointment was applied to the skin lesions to prevent secondary infection. However, acute peritonitis developed during peritoneal dialysis, and death occurred as a result of sepsis.
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PMID:Extensive skin calcifications in an infant with chronic renal failure: metastatic calcinosis cutis. 1678 Apr 69


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