Cases reported "Calciphylaxis"

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1/34. 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.
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2/34. 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.
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3/34. 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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4/34. 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.
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5/34. calciphylaxis: a rare limb and life threatening cause of ischaemic skin necrosis and ulceration.

    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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6/34. calciphylaxis in the absence of renal failure.

    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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7/34. 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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8/34. 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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9/34. calciphylaxis with peau d'orange induration and absence of classical features of purpura, livedo reticularis and ulcers.

    calciphylaxis is an ill-defined syndrome that is commonly associated with chronic renal failure. Its heterogeneous clinical features include painful livedo reticularis-like purpuric patches and plaques, vesicles, irregularly shaped ulcers, and black eschars. Despite demonstration of extensive vascular arteriolar calcification in this syndrome, its exact pathogenesis remains unknown. Here, we report a case of calciphylaxis presenting with indurated plaques without the usual clinical picture of livedo reticulate purpura, ulcers or necrotic eschars. This case provides an opportunity to review the clinical spectrum of calciphylaxis and to discuss the therapeutic approaches and pathogenesis of this syndrome from deep intra-wall vascular calcification to the resulting infarctions of adjacent tissues.
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10/34. Painful ulcers in calciphylaxis - combined treatment with maggot therapy and oral pentoxyfillin.

    BACKGROUND: calciphylaxis is a rare syndrome developing predominantly in female patients suffering from end-stage renal disease (ESRD) with secondary hyperparathyroidism. skin lesions begin as superficial patches that quickly progress to painful necrotic ulcers. Histopathological findings are calcification of small arteries and arterioles and infarction of subcutis and skin. The prognosis of calciphylaxis is poor due to an increased risk of systemic infection. methods: We report on a 50-year-old woman suffering from calciphylaxis. Initial treatments were not tolerated due to pain and therefore the patient was treated with maggot therapy and 800 mg/day of oral pentoxyfillin. RESULTS: Over a period of 6 months a complete remission of her skin lesions was achieved. CONCLUSION: patients suffering from ulcers due to calciphylaxis may benefit from the use of maggot therapy, which cleanses ulcers and prevents systemic infection.
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