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1/24. 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.
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ranking = 1
keywords = calciphylaxis
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2/24. 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.
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ranking = 1
keywords = calciphylaxis
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3/24. Spectrum of complications related to secondary hyperparathyroidism in a peritoneal dialysis patient.

    The index patient is a 23-year-old female with end-stage renal disease (ESRD) secondary to chemotherapeutic agents. Continuous cycling peritoneal dialysis (CCPD) has been the renal replacement therapy for the past 5 years since a failed cadaveric renal transplant. Past medical history was significant for diabetes mellitus, hypertension, anemia, bilateral subclavian vein thrombosis with superior vena cava syndrome, secondary hyperparathyroidism, leukemia (at age 8), and hyperlipidemia. On presentation, soft tissue nodules were noted in the anterolateral surfaces of the legs. After 3 months of continued low-calcium-dialysate CCPD, calcitriol, and oral phosphate binders, a 2 x 3 cm nodule was noted on the posterior aspect of the thorax at the scapula. The only complaint at this time was shoulder pain at the acromioclavicular joint. Radiological examination revealed a 3 x 4 cm soft tissue opacity in the superior segment of the left lower lobe laterally. Despite a prior subtotal parathyroidectomy, phosphate binders, and calcitriol, the parathyroid hormone levels continued to increase, with development of tumoral calcinosis, worsening renal osteodystrophy, and calciphylaxis. Computed tomography examination revealed extensive soft tissue calcification consistent with tumoral calcinosis. An ulcerative lesion (1 cm) developed on the lateral aspect of the upper thigh owing to warfarin necrosis versus calciphylaxis. At this time, the phosphate binder was changed from calcium acetate to sevelamer hydrochloride. Aggressive wound treatment and aggressive calcium and phosphate control added to the treatment regimen has resulted in healing of the single ulcer and a decrease in the size of the tumoral lesions. In conclusion, early recognition and aggressive treatment of calciphylaxis can result in reduced morbidity and mortality from calciphylaxis in ESRD patients.
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ranking = 2
keywords = calciphylaxis
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4/24. Severe necrotizing mastopathy caused by calciphylaxis alleviated by total parathyroidectomy.

    calciphylaxis is a complication caused by secondary hyperparathyroidism in patients with chronic renal failure. These patients may present with clinical findings of ischemic necrosis involving the skin and muscle resulting in subsequent gangrene and vascular calcifications. We report a rare case of necrotizing mastopathy caused by calciphylaxis in a 70-year-old female with end-stage renal disease whose symptoms resolved with a total parathyroidectomy.
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ranking = 2.5
keywords = calciphylaxis
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5/24. calciphylaxis, a poorly understood clinical syndrome: three case reports and a review of the literature.

    Systemic calcification syndromes are a recognized complication occurring in some patients with end-stage renal disease (ESRD) and secondary hyperparathyroidism. These patients develop severe livedo reticularis and subcutaneous tissue lesions progressing to frank necrosis and ultimately large areas of eschar. Clinically this syndrome is known as calciphylaxis; these lesions are usually resistant to aggressive debridement, systemic antibiotics, and revascularization procedures. We report three patients with somewhat different clinical presentations but all sharing a common link of exquisitely painful leg ulcers initially being treated as ischemic lesions or venous stasis-type ulcerations. These three patients were diagnosed with calciphylaxis on the basis of clinical, biochemical, and histopathological criteria. Two patients underwent parathyroidectomy late in the progression of their disease, with some resolution of their ulcerative lesions.
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ranking = 1
keywords = calciphylaxis
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6/24. calciphylaxis: what nurses need to know.

    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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ranking = 1
keywords = calciphylaxis
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7/24. Subtotal parathyroidectomy: a possible treatment for calciphylaxis.

    calciphylaxis is a rare disorder in patients with chronic renal failure that is characterized by ischemic necrotic skin lesions. The prognosis is grave and mortality is high (80%). The precise mechanism of calciphylaxis is still unknown, but in addition to chronic renal failure, elevated parathyroid hormone levels appear to play a role. The role of parathyroidectomy in treating affected patients is questionable. In this article, we describe the case of a patient with chronic renal failure who developed rapidly progressive subcutaneous calcifications and ulcerations in the lower extremities. These lesions regressed following subtotal parathyroidectomy. We also review the literature on calciphylaxis, with a focus on treatment options.
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ranking = 3
keywords = calciphylaxis
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8/24. Metastatic calcification.

    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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ranking = 1
keywords = calciphylaxis
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9/24. Development of proximal calciphylaxis with penile involvement after parathyroidectomy in a patient on hemodialysis.

    Elevated parathyroid hormone (PTH) levels and hyperphosphatemia are thought to be associated with the development of calciphylaxis. We report a patient on hemodialysis who developed proximal calciphylaxis with consistently low PTH levels after parathyroidectomy. A 31-year-old man was admitted to our hospital because of abdominal skin ulcerations. calciphylaxis spread to the penis, and simultaneous progressive lung calcification was evident on chest X-ray, suggestive of pulmonary calciphylaxis on 99mTc-methylene disphosphonate scintigraphy. The patient died of respiratory failure despite intensive treatment including hyperbaric oxygen therapy. This is the first report of a patient on hemodialysis who developed calciphylaxis involving the penis after parathyroidectomy.
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ranking = 4
keywords = calciphylaxis
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10/24. Traumatic calcinosis cutis in a dialysis patient.

    A patient with end-stage renal disease and refractory hyperparathyroidism was evaluated for acute-onset thickening and hardening of the skin of the lower extremities. Her clinical course and physical examination findings were consistent with the recently described entity of nephrogenic fibrosing dermopathy. However, skin biopsy results showed metastatic and dystrophic calcification, without calcific uremic arteriolopathy (calciphylaxis). The patient reported a history of self-inflicted trauma; the authors postulate that trauma, in the setting of hyperparathyroidism and an elevated serum calcium phosphorous product, resulted in the subcutaneous deposition of calcium salts. To the authors' knowledge, this is the first report of metastatic and dystrophic calcification, without calciphylaxis, in a patient with refractory hyperparathyroidism. This case underscores both the rich variety of skin conditions seen in patients undergoing dialysis and recent developments in the field of dermatologic disorders associated with end-stage renal disease.
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ranking = 1
keywords = calciphylaxis
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