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Calciphylaxis is a rare, severe complication of secondary hyperparathyroidism. Patients present with painful, violaceous, mottled skin lesions of the upper and lower extremities, which become necrotic and produce nonhealing ulcers. Gangrene of fingers and toes frequently requires amputation, produces nonhealing wounds, and can lead to sepsis and death. We reviewed the clinical course of five patients with calciphylaxis treated in our institution. The three men and two women (aged 47 to 72 years) had secondary hyperparathyroidism from chronic renal failure. All patients had severe pruritus, painful ulcers, and severe hyperphosphatemia with elevated serum calcium-phosphate product (greater than 12 mmol2/L2), but the serum parathyroid hormone levels were only moderately elevated. Most patients had medical calcification of medium and small blood vessels, and some had soft-tissue calcification visible on roentgenography. Treatment consisted of local wound care, antibiotics, phosphate-binding agents, and parathyroidectomy. Two patients died of uncontrollable sepsis. The three survivors had dramatic improvement of pain and ulcers after parathyroidectomy. Calciphylaxis is a limb- and life-threatening complication of secondary hyperparathyroidism. Diagnosis can be made by recognizing the characteristic painful skin lesions, ulcers, and gangrene of the digits, and patients should be treated with subtotal parathyroidectomy.
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PMID:Calciphylaxis in secondary hyperparathyroidism. Diagnosis and parathyroidectomy. 192 21

Calciphylaxis occurs in renal transplant patients more frequently than in chronic hemodialysis patients. It can occur in a patient with normal renal function who is also normocalcemic. Early parathyroidectomy can lead to healing of the skin lesions and prevent death of sepsis.
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PMID:Post-renal transplant calciphylaxis: successful treatment with parathyroidectomy. 633 43

Calciphylaxis, a syndrome of disseminated calcification found in chronic renal failure patients with secondary hyperparathyroidism, results in soft tissue calcification and vascular medial calcinosis leading to subsequent ischemic tissue necrosis. It is a rarely occurring condition in which patients present with painful, violaceous, mottled lesions of the extremities and/or trunk that progress to skin and subcutaneous tissue necrosis, non-healing ulcers, and gangrene. We reviewed the clinical course of seven patients (aged 24-69) with calciphylaxis treated at our institution over a 4-year period (October 1988-June 1992). All seven patients underwent parathyroidectomy, with a mean time of 8 weeks (range 3-20 weeks) between the onset of calciphylactic symptoms and parathyroidectomy. Four patients died, three secondary to wound-related sepsis. Of the three survivors, two healed soft tissue lesions primarily. The other required extremity amputation and wound excision before healing. Neither anatomical location of the soft tissue lesions nor post-parathyroidectomy serum calcium and phosphorus levels had any bearing on wound healing or mortality. Lesion severity at the time of parathyroidectomy appeared to best correlate with clinical course. Although treatment with phosphate-binding antacids, total or subtotal parathyroidectomy, and avoidance of challengers such as Vitamin D or local tissue trauma remain the mainstays of therapy, the uniform cure for calciphylaxis remains elusive. Prognosis for patients with calciphylaxis is dismal, even following late surgical intervention. Earlier recognition of the signs and symptoms of calciphylaxis should lead to timely parathyroidectomy in the hopes of ameliorating the symptoms and preventing or retarding its progressive sequelae.
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PMID:Calciphylaxis: early recognition and management. 830 50

Calciphylaxis is a rare and life-threatening complication that is estimated to occur in 1% of patients with ESRD each year. Typically, extensive microvascular calcification and occlusion/thrombosis leads to violaceous skin lesions, which progress to nonhealing ulcers and sepsis. Secondary infection of skin lesions is common, often leading to sepsis and death. The lower extremities are predominantly involved (roughly 90% of patients). Patients with skin involvement over the trunk or proximal extremities have a poorer prognosis. Although most calciphylaxis patients have abnormalities of the calcium:phosphate axis or elevated levels of parathyroid hormone, these abnormalities do not appear to be fundamental to the pathophysiology of the disorder, and the etiology of calciphylaxis remains unclear. Recently, functional protein C deficiency has been hypothesized to cause a hypercoagulable state that could induce thrombosis in small vessels, with resulting skin ischemia, necrosis, and gangrene. The lack of understanding of the pathophysiology of the disease results in treatments that are equally unsatisfactory. Patients who undergo parathyroidectomy have a tendency to improve, but the prognosis for the disease is poor and mortality remains high.
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PMID:Calciphylaxis in chronic renal failure. 882 11

Calciphylaxis is a rare but potentially life-threatening complication in chronic renal failure. It is characterized by ischemic tissue necrosis primarily of the skin. The typical histopathologic finding is microvascular calcification with endovascular fibrosis. Patients typically present with violaceous, mottled and painful lesions which tend to progress to non-healing ulcers and necrosis. Most frequently the lower extremities are involved in a symmetric fashion but the trunk may also be affected. Sepsis from superinfection of the lesions accounts for the high mortality of this disease which is of importance for dermatologists and nephrologists alike. 61-year-old female patient developed lesions of calciphylaxis on both calves two years after beginning hemodialysis to treat renal failure due to diabetic glomerulosclerosis. We discuss aspects of the pathogenesis of calciphylaxis, as well as diagnosis, treatment and means of prevention, and review the current literature.
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PMID:[Calciphylaxis: ischemic tissue necrosis in chronic renal failure. Case report and review of the literature]. 1066 38

Calciphylaxis (uraemic gangrene syndrome) is a rare complication of chronic renal failure and secondary hyperparathyroidism. Patients present with painful purple skin lesions which undergo necrosis and ulceration. The histology is specific. There is medial calcification with intimal hyperplasia and thrombosis of the lumen of small sized arteries in the underlying subcutaneous tissue. Death frequently arises from overwhelming sepsis. Early recognition of this condition and prompt parathyroidectomy can lead to rapid relief of symptoms and ulcer healing and may be life saving.
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PMID:Calciphylaxis: a rare limb and life threatening cause of ischaemic skin necrosis and ulceration. 1073 37

We report a patient with severe bilateral leg ulceration that was resistant to treatment. A biopsy confirmed the cause as calciphylaxis. Calciphylaxis refers to a syndrome of calcium deposition in the small and intermediate dermal vasculature which can lead to epidermal ischaemia, ulceration and necrosis. Most cases occur in those with chronic renal failure and secondary hyperparathyroidism. We describe the rare presentation of calciphylaxis in a patient with normal renal function and primary hyperparathyroidism who had many classical features. Unfortunately she developed gangrene, sepsis and died.
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PMID:Calciphylaxis in the absence of renal failure. 1101 92

Calciphylaxis is a rare and life-threatening condition of progressive cutaneous necrosis secondary to small and medium-sized vessel calcification. It is seen almost exclusively in patients with end-stage renal disease and secondary hyperparathyroidism. We experienced a case of 67-year-old man with calciphylaxis that manifested with characteristic skin lesions, pathologic findings, and laboratory changes. His skin lesions began as painful erythematous patches and subsequently progressed to necrotic ulcers with eschars on the distal aspect of the extremities. Pathologically, calcification was found in small and medium-sized blood vessels in the deep dermis and subcutaneous tissue. His serum calcium was 9.5 mg/dL, phosphorus was 7.8 mg/dL, and nPTH was 99.9 pg/mL. The patient had been treated with surgical debridement and other supportive treatment. However, he eventually underwent an amputation below the right knee and died from sepsis.
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PMID:Calciphylaxis in a patient with end-stage renal disease. 1143 66

Calciphylaxis is a rare disease that is caused by calcium deposition in medium and small sized vessels of the skin causing ischemic necrosis. These lesions often become infected and cause septicemia. We report a unique case of penile and scrotal calciphylaxis that rapidly progressed to Fournier's gangrene. We review the etiology of calciphylaxis and also its management.
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PMID:Calciphylaxis of the penis: a unique cause of Fournier's gangrene. 1171 34

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


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