Cases reported "Calciphylaxis"

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1/8. Healing of skin necrosis and regression of anticardiolipin antibodies achieved by parathyroidectomy in a dialyzed woman with calcific uremic arteriolopathy.

    AIM: To present the impact of parathyroidectomy on the spontaneous healing of necrotic lesions of the skin of the lower leg and on anticardiolipin antibodies regression in a 68-year-old female dialyzed patient with hyperparathyroidism and calcific-uremic arteriolopathy (CUA). methods: After the occurrence of initial lesions of the lower leg skin, the intact parathyroid (iPTH) level, calcium (Ca) and phosphorus (P) product were measured, and on two occasions at 6-week intervals, the titer of anticardiolipin antibodies was determined, followed by a clinical monitoring of the progress of necrotic skin lesions. Two months after the occurrence of the skin lesions, the patient's right leg was amputated below the knee due to gangrene, and a histopathological analysis of the skin tissue sample of the amputated lower leg was made. After parathyroidectomy, iPTH, Ca x P product were measured, and on two occasions at 6 weeks' intervals, anticardiolipin antibodies titer was determined, followed by a clinical monitoring of lesions of the left lower leg skin. RESULTS: Before parathyroidectomy, iPTH level and Ca x P product were increased, as well as IgG anticardiolipin antibody titer measured on two occasions 6 weeks apart. The histopathological analysis of the skin tissue sample of the amputated right lower leg showed mural calcification of artery walls and thrombotic occlusions of small arteries, arterioles, and dermal capillaries, in addition to epidermolysis. A week after parathyroidectomy, iPTH level and Ca x P product were within normal range. Two measurements 6 weeks apart revealed no anticardiolipin antibodies. Eight weeks after parathyroidectomy, spontaneous healing of necrotic skin lesions of the left lower leg was observed. CONCLUSION: Regression of anticardiolipin antibodies, normalization of Ca x P product, and healing of the skin lesions after parathyroidectomy all pointed to the elevated PTH level as a crucial factor in the pathogenesis of CUA.
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keywords = arteriole
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2/8. Three-dimensional analysis of a calciphylaxis plaque: clues to pathogenesis.

    BACKGROUND: calciphylaxis is a rare, life-threatening disorder associated with chronic renal failure, presenting with ulcerating plaques leading to death by sepsis in 60% of patients. Calcification of subcutaneous arterioles, thromboses, and extravascular calcification have been demonstrated in incisional biopsy specimens. However, the sequence of these pathologic events is unknown. OBJECTIVE: We examined a calciphylaxis plaque to document the wave of pathologic change from its center to its periphery. methods: A calciphylaxis plaque was excised postmortem from a female patient. It was examined histologically along 12 radii from the center of the lesion to its periphery. RESULTS: Calcification of small subcutaneous vessels was present in all histologically abnormal sections and extended further peripherally than extravascular calcification by up to 3.0 cm and further than subcutaneous thrombosis by up to 1.5 cm. CONCLUSION: Vascular mural calcification is an early and essential process in the development of a calciphylaxis plaque.
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ranking = 1
keywords = arteriole
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3/8. 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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ranking = 1
keywords = arteriole
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4/8. calciphylaxis in chronic, non-dialysis-dependent renal disease.

    BACKGROUND: calciphylaxis cutis is characterized by media calcification of arteries and, most prominently, of cutaneous and subcutaneous arterioles occurring in renal insufficiency patients. CASE REPORT: A 53-year-old woman with chronic cardiac and renal failure complained of painful crural, non-varicosis ulcers. She was hospitalized in an immobilized condition due to both the crural ulcerations and the existing heart-failure state (NYHA III-IV) having pleural and pericardial effusions, atrial fibrillation and weight loss of 30 kg over the past year. Despite normalization of calcium-phosphorus balance and improvement of renal function, the clinical course of crural ulcerations deteriorated during the following 3 months. After failure of surgical debridements, multiple courses of sterile-maggot therapy were introduced at a late stage to stabilize the wounds. The patient died of recurrent wound infections and sepsis paralleled by exacerbations of renal malfunction. CONCLUSIONS: The role of renal disease in vascular complications is discussed. Sterile-maggot debridement may constitute a therapy for the ulcerated calciphylaxis at an earlier stage, i.e. when first ulcerations appear.
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ranking = 1
keywords = arteriole
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5/8. Bone morphogenic protein-4 expression in vascular lesions of calciphylaxis.

    calciphylaxis is characterized by an extensive media-calcification of cutaneous and subcutaneous arterioles and capillaries. Recent studies have provided evidence that vascular calcification is a process with similarities to bone metabolism. Bone morphogenic protein-4 (BMP-4) is physiologically involved in bone development and repair. The presence of BMP-4 in atherosclerosis and in sclerotic heart valves led us to suggest that BMP-4 is also involved in calciphylaxis. A 47-year-old male patient developed end-stage renal failure due to chronic glomerulonephritis. He has had two kidney transplants with an immunosuppressive regimen consisting of cyclosporine A and steroids. He was admitted to our hospital because of an increase in serum creatinine (Cr) and he subsequently developed progressive dermal ulcerations. A skin biopsy led to the diagnosis of calciphylaxis. immunohistochemistry for BMP-4 of a skin specimen from our patient showed strong cytoplasmic immunoreactivity of intradermal cells with clear spatial association to arterioles and hair follicles. Whereas there are identified inhibitors and promoters of vascular calcification, the presence of BMP-4 has not been demonstrated in calcific uremic arteriolopathy. In contrast to atherosclerosis, BMP-4 in calciphylaxis cannot be found in vascular media, but in intradermal cells at the border of arterioles and hair follicles. Therefore, in calciphylaxis BMP-4 can play the role of a cytokine, a growth factor or a media-calcification promoter.
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ranking = 3
keywords = arteriole
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6/8. Mysterious calciphylaxis: wounds with eschar--to debride or not to debride?

    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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ranking = 2
keywords = arteriole
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7/8. A case report comparing various radiological tests in the diagnosis of calcific uremic arteriolopathy.

    BACKGROUND: Calcific uremic arteriolopathy (CUA) is a rare necrotizing skin condition characterized by calcification in arterioles, leading to ischemia and skin ulcerations. This disease affects 1% to 4% of patients with chronic kidney disease and has a reported mortality rate up to 80%. The diagnosis of CUA is based on clinical judgment suggested by the characteristic skin lesions. Although skin biopsy is the gold standard for establishing the diagnosis, it is performed infrequently because of poor healing and risk for secondary infections. methods: In this case report, we compare the ability of various radiological tests to show arteriolar calcifications of patients with CUA. Our patient had biopsy-proven CUA manifesting as chronic nonhealing ulcers of the calves. She underwent soft-tissue x-ray of the affected extremities and high-resolution (0.5-mm slice) computed tomographic (CT) scanning with 3-dimensional image reconstruction. We also used a dedicated mammography machine to obtain images of the patient's calves. Images were compared based on the ability to show small-vessel calcification. RESULTS: Plain soft-tissue x-ray showed mildly increased soft-tissue density and very few calcified vessels, whereas CT showed few calcified small- and medium-sized arterioles. Diffuse calcification of small arterioles in a mesh-like pattern was shown by means of the mammography technique. CONCLUSION: Simple, safe, and inexpensive x-ray imaging using the mammography technique was superior to plain soft-tissue x-ray and 3-dimensional CT in showing the hallmark arteriolar calcifications of patients with CUA. Thus, we propose a possible role for this technique in diagnosing CUA.
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ranking = 3
keywords = arteriole
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8/8. Benign nodular calcification and calciphylaxis in a haemodialysed patient.

    Several types of soft tissue calcification can be detected from radiographic evaluation of patients with end-stage renal failure. The factors that predispose to such calcification include an increase in CaxP product in serum, the degree of secondary hyperparathyroidism, the level of blood magnesium, the degree of alkalosis, and the presence of local tissue injury. Three major varieties include calcification of medium-sized arteries, periarticular or tumoral calcification and visceral calcification. calciphylaxis is a phenomenon consisting of acute ischemic necrosis in presence of calcification of dermohypodermic arterioles. It occurs mostly in chronic renal failure patients with secondary or tertiary hyperparathyroidism with a persistently elevated calcium-phosphorus product. There are few options in treating calciphylaxis and the outcome is generally poor. The authors report the case of a haemodialised patient with benign nodular calcification and calciphylaxis. The coexistence of both entities in the same patient has never been described.
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ranking = 1
keywords = arteriole
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