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Results for “Calciphylaxis”

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At least 19 recordsLinked to original sources
PubMed1990

Skin necrosis associated with acquired protein C deficiency in patients with renal failure and calciphylaxis.

PURPOSE: To determine if the natural anticoagulant protein C plays a role in the pathogenesis of systemic calciphylaxis, a syndrome characterized by extensive vascular and soft tissue calcification and skin necrosis, which is similar to that seen in warfarin-induced skin necrosis. PATIENTS AND METHODS: The study population included five patients with end-stage renal disease and systemic calciphylaxis undergoing hemodialysis, 12 patients without evidence of calciphylaxis undergoing dialysis, eight patients with nephrotic syndrome, and eight normal healthy volunteers. Protein C antigen levels were measured by rocket immunoelectrophoresis, and functional activity was quantitated by a chromogenic assay and an anticoagulant assay utilizing the venom of Agkistrodon contortrix. RESULTS: Skin biopsy specimens of involved areas in three patients showed thrombotic occlusion of venules identical to that seen in warfarin-induced skin necrosis. Protein C antigen levels were normal in all groups. However, protein C activity was significantly reduced as measured by chromogenic (p less than 0.01) or anticoagulant assays (p less than 0.01) in patients with calciphylaxis compared with the other three groups. CONCLUSION: These findings suggest that hypercoagulability due to functional protein C deficiency may contribute to thrombosis, resulting in skin necrosis and digital gangrene in systemic calciphylaxis.

Adult↗
PubMed1991

Calciphylaxis in secondary hyperparathyroidism. Diagnosis and parathyroidectomy.

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.

Aged↗
PubMed1990

Calciphylaxis induced by iron-citrate sorbitol.

The author describes an experimental model of calciphylaxis induced by iron citrate sorbitol in young dogs. The lesions became apparent in form of nodular white and hard papules 0.1-0.5 cm in diameter 5-7 d after subcutaneous administration of iron. Microscopically a granulomatous reaction of the dermis and hypodermis are visible and also some associated modifications, as adipose cysts and fibrillar densification. From histochemical points of view a highly positive von Kossa reaction is observed on the adipocyte's membrane, on collagen fibers or as a compact extensive material. The significance of the morphohistochemical appearances is discussed as a dynamic process which leads to calciphylaxis of the skin and then to a granulomatous reaction of foreign body type. We suggest that this experimental model of calciphylaxis could serve as an explanation for some calcifications observed in human pathology.

Animals↗
PubMed1992

Calciphylaxis in a hemodialysis patient: appearance after parathyroidectomy during a psoriatic flare.

Chronic renal failure patients are prone to soft tissue calcifications. A phenomenon of acute ischemic skin necrosis and dermohypodermic arteriolar medial calcification has been described recently in patients with chronic renal failure and secondary hyperparathyroidism (HPT). This phenomenon, termed calciphylaxis, occurs in response to certain factors, the most important of which appears to be an elevated blood calcium-phosphate product. Accordingly, parathyroidectomy in addition to normalization of calcium-phosphate product has been proposed as the only effective therapeutic approach for this condition. We describe a case of chronic renal failure with severe secondary HPT in which the patient developed calciphylaxis 4 days after the appearance of a psoriatic flare. Four months before, a subtotal parathyroidectomy was performed for severe HPT and at the time the ulcerations appeared, blood calcium-phosphate product was correct. Etiological and physiopathological aspects of calciphylaxis are discussed.

Calciphylaxis↗
PubMed1989

[Clinical aspects of metabolic nephropathies, interstitial nephritis and urolithiasis in calciphylaxis].

The problem of abacterial nephropathies associated with impaired calcium metabolism is discussed from the standpoint of membranous pathology. The data are presented on the similarity of the calciphylaxis pathogenesis described for the first time by H. Selye to calcium nephrolithiasis. The disease is viewed as the result of pronounced modification of the phospholipid layer of cell membranes under the influence of peroxidation, phospholipase activity and other processes induced by calcium-regulating hormones (parathyroid hormone, D3 and calcitonin). It is assumed that the clinical manifestations of renal pathology during calciphylaxis depend on the hereditary and environmental factors that determine the gravity of membranous pathology of renal cells. Recommendations as to the treatment and prevention of renal pathology during calciphylaxis based on the results of the authors' own experience are given.

Calcinosis↗
PubMed1992

Widespread cutaneous and systemic calcification (calciphylaxis) in patients with the acquired immunodeficiency syndrome and renal disease.

BACKGROUND: Calciphylaxis is a form of widespread calcification that may occur in patients with renal disease and hyperparathyroidism. The skin is often affected secondary to vascular compromise and ischemia. Patients infected with the human immunodeficiency virus (HIV) are predisposed to renal failure and thus may develop this serious complication. OBJECTIVE: The purpose of this article is to describe two cases of fatal calciphylaxis in patients with the acquired immunodeficiency syndrome and renal disease. METHODS: Two patients were examined clinically and through the performance of serologic studies and skin biopsies. RESULTS: Both patients had a widespread livedo reticularis-like eruption, which rapidly progressed to cutaneous gangrene. Patient 1 had elevated serum calcium and phosphorus levels whereas patient 2 had normal values but had markedly elevated serum parathyroid hormone levels. Biopsy specimens in both cases showed vascular thrombosis and soft tissue calcification. CONCLUSION: Widespread calcification may develop in patients with HIV infection and renal failure and, in some cases, calcium and phosphorus levels may be normal.

Acquired Immunodeficiency Syndrome↗
PubMed1992

Systemic calciphylaxis presenting as a painful, proximal myopathy.

A renal transplant patient who presented with a painful, proximal myopathy due to systemic calciphylaxis is described. The myopathy preceded the characteristic skin and soft tissue necrosis. Systemic calciphylaxis should be considered in a dialysis or a renal transplant patient presenting with a painful proximal myopathy even in the absence of necrotic skin lesions.

Calciphylaxis↗
PubMedDate not supplied

Experimental skin calciphylaxis induced by iron citrate sorbitol in young dogs.

An experimental model of skin calciphylaxis using iron citrate sorbitol is presented. There were used for the experiment 12 young dogs sensitized with D3 vitamin and then injected with 0.2 ml Jectofer on the internal face of the shank. Macroscopic lesions become evident after 2-3 days last drug was administered. Nodular calcifications occurred after 7-8 days as white, hard and irregular lesions, when sectioned, presenting dissociable crystals. From the microscopic point of view fatty cysts, dermic granuloma and an amorphous irregular material are described. In the early period Perls stain is positive and becomes negative after 3 or 4 days. Von Kossa reaction is positive after 4 or 5 days and alizarin S after 7. Degenerative lesions of elastic fascicles are noticed and discussed in relationship with localization of calcium salts. On the basis of these data the possible succesion of skin calciphylaxis steps is discussed, but many things remain unknown.

Animals↗
PubMed1987

[Calcifying panniculitis associated with renal insufficiency: a tissue calciphylaxis syndrome].

Among the cutaneous manifestations of hyperparathyroidism, cases of panniculitis with calcification of the adipose tissue and necrosis of the skin have recently been reported, the mechanism incriminated being calciphylaxis, as defined by Selye on the basis of experiments. Experimental calciphylaxis consists of local or systemic calcium deposits followed by inflammatory necrosis or sclerosis. The deposits are induced by "provoking" or precipitating factors (metal salts, albumin, traumas) after a phase of sensitization (to parathyroid hormone, vitamins D2 or D3, dihydrotachysterol), provided a critical period is allowed between these two phases; the duration of that period depends on the experimental conditions. The case reported here concerns a 64-year old obese and diabetic woman who had presented with hard and tender nodosities and plaques in her abdominal and crural panniculi, ending in extensive and hyperalgesic necrosis (fig. 1 and 2). The panniculitis had occurred in a peculiar context: at the end of an episode of renal failure complicated with secondary hyperparathyroidism (serum PTH 12.9 mIU/ml; N = 1.5-4.4 mIU/ml) with moderate increase to 5,000 of the P x Ca product. Histological examination of a nodule of the thigh disclosed multiple foci of microcalcification (fig. 3, 4, 5) within the adipose lobules, in the interadipocyte spaces, in connective tissue septa and in the adventitia of small vessels (positive Von Kossa reaction). Electron microscopy showed dense calcium deposits between adipocytes, in subcutaneous septa (fig. 6, 8) and in more or less damaged vascular walls (fig. 9). Within the microfibrillar and granular fundamental substance, microcrystals looking like hydroxyapatite crystals (fig. 7) conglomerated into pincushion-like formations becoming increasingly denser and more compact.(ABSTRACT TRUNCATED AT 250 WORDS)

Abdominal Muscles↗
PubMed1987

Ultrastructural study of the long-term development of two experimental cutaneous calcinoses (topical calciphylaxis and topical calcergy) in the rat.

Skin calcification induced by topical calciphylaxis was provoked by a subcutaneous injection of iron chloride in rats previously sensitized by dihydrotachysterol. A cutaneous topical calcergy was induced by an injection of potassium permanganate. An electron-microscopical study of the long-term evolution of both these models of calcification was made. After the initial stages, mineralization of the connective tissue continued by a secondary nucleation process without matrix vesicles. The mineral composed of needle-like structures, apatite in nature, was mainly deposited between and around collagen fibrils, and showed various arrangements in calcified plaques. Intrafibrillar calcification was rarely observed and appeared only in the later stages. The extension of calcified deposits then stopped. Finally, there was a fragmentation of the mineralized area which was progressively surrounded by uncalcified collagen fibrils. A demineralization process, caused by cells such as macrophages and multinucleated giant cells, rather than a resorption of the calcified deposits, was noted. It is important to emphasize that, in both models of ectopic calcification, an evolution toward ectopic ossification was never observed, which is perhaps due to the absence of extensive resorption mechanisms.

Animals↗
PubMed1977

The effects of various diphosphonates on a rat model of cardiac calciphylaxis.

Seven diphosphonate analogs were treated for their effects on myocardial and cardiovascular degeneration and calcification in an experimental model of cardiac calciphylaxis. A single oral dose of dihydrotachysterol (DHT) administered to rats induced myocardial and vascular degeneration, focal myocarditis and vasculitis, and myocardial and vascular mineralization. The results demonstrated a considerable variation among the various diphosphonates in their ability to block the pathological changes observed in this model. Ethane-1-hydroxy-1,1-diphosphonate (EHDP) was the most effective diphosphonate in reducing myocardial and vascular degeneration and calcification, whereas diphosphonates such as ethane-1-amino-1,1-diphosphonate (EADP) and hydroxymethylene diphosphonate (HMDP) had little or no effect compared to saline controls. For those diphosphonates which were effective, e.g., EHDP, the tissue-protective effects were observed whether the rats were treated with drug prior to the administration of DHT, or whether drug treatment commenced after DHT administration. The results are discussed in terms of the known biological properties of the diphosphonate drugs.

Animals↗
PubMed1988

A case of calciphylaxis. Case report.

A young woman on hemodialysis with hyperparathyroidism suddenly developed areas of skin necrosis caused by arterial calcification. Parathyroidectomy apparently arrested this process. Seven months after cadaver kidney transplantation, following reparation of a graft artery stenosis, necrosis spread anew from the wound gradually extending to large areas. The patient died and autopsy revealed calcification in many internal organs. The case fits the description of systemic calciphylaxis a syndrome now described in humans but originally experimentally induced in rats.

Adult↗
PubMed1988

Epidermal and follicular calciphylaxis.

A patient with secondary hyperparathyroidism due to chronic renal failure developed epidermal and follicular calcification without concomitant dermal calcification in the lesions of toxic epidermal necrolysis. We propose that the combination of keratinocyte necrosis and an elevated serum calcium-phosphorus product resulted in epidermal calciphylaxis.

Adult↗
PubMed1990

Calciphylaxis and systemic calcinosis. Collective review.

The phenomenon of calciphylaxis as defined by Selye is a condition of hypersensitivity that results in acute local calcification of various organs, with a whole host of morbid processes. Nephrocalcinosis and cutaneous calcifications have long been recognized in patients with chronic renal failure, but they have not often been reported in acute hyperparathyroidism or other causes of calcium-phosphate metabolism aberrations. The pathogenesis is not clear, and both the sensitizer and challenging agent in the hypersensitivity theory are often elusive, though hypercalcemia is the most consistent factor. The clinical features vary according to the organs affected and often mimic a gamut of more common conditions. Treatment is primarily supportive, with specific measures only possible when a sensitizer or challenger is identified. Correction of the hypercalcemia is, however, imperative. It is important to recognize this rare condition to avoid the more serious end results often reported.

Calcinosis↗
PubMed1990

[Systemic calciphylaxis and acquired perforating disease in a uremic patient].

We show the case of a patient suffering from a chronic renal failure in hemodialysis. He had a ischemic necrosis that was quickly progressive in his fingers and toes. It was necessary to amputate them. Diffuse vascular calcifications were recorded so radiologically than pathologically. PTH and phosphocalcic product were raised. Parathyroidectomy was practised with a quick initial improvement but immediately new distal ischemic lesions and keratotic papules with histologic perforation, that became necrosed, appeared in his buttocks and inferior extremities. The coexistence of acquired perforating disease and calciphylaxis in uremic patients has not been reported until now.

Calciphylaxis↗
PubMed1990

[Idiopathic arterial calcification: its link with calciphylaxis].

The attempt to establish the connection of the idiopathic arterial calcification (IAC) with calciphilaxy is made on the basis of the published data analysis and 11 observations of IAC in children. Apart from known morphological manifestations of IAC, the authors found mast cells in the cellular infiltrate, vesicular transformation of smooth cells in the arteries affected. This together with the elastic membrane changes in the arteries and massive iron and oxyapatite deposits in the arterial wall suggests the development of calciphylactic reaction. The similarity of the morphological and clinical manifestations, and IAK therapy in the clinics and experiment is shown. Special emphasis is made on the possibility of the calciphylaxy development in children under conditions of the vitamin D wide use for the prevention of rickets and iron preparations use for anemia treatment. The conclusion is drawn on the multicausal character of IAK and possibility to distinguish congenital, acquired and combined forms of the disease. The necessity of through analysis of each case of IAK is stressed in order to reveal iatrogenic factors capable to produce sensibilization and/or to provoke the disease.

Animals↗
PubMedDate not supplied

[Cutaneous calcinosis induced by topical calciphylaxis in rats. I. Ultrastructural aspcts].

Skin calcification induced by topical calciphylaxis was provoked by a subcutaneous injection of iron chloride in rats previously sensitized by D.H.T. and studied by electron microscope. The evolution of the calciphylatic response can be resumed as follows: -- early appearance of numberous granules in the mitochondria of the cells of the connective tissue; their inorganic nature was revealed by microincineration and it is highly probable that they are a reserve of mineral ions; -- between the 6 hr, and the 18 hr, stages the interfibrillar matrix contained rounded vesicular structures, enclosed by a triple mambrane, which must surely be of cellular origin; they progressively incorporated more and more mineral particles which confirm their primordial role in the initial phases of mineralization; -- from the 14 hr. stage onward, mineral rodlets composed of chains of little dots appeared in the intercellular matrix and then on the collagen fibrils, probably in contact with the mucopolysaccharides; -- starting at the 24 hr, stage, the mineralization progressed without a vesicular intermediary forming increasingly dense and widespread plaques; the presence of needles of homogenous aspect indicated the appearance of a crystalline mineral deposit; an intrafibrillar mineral deposit becomes visible later. These results are compared with those obtained previously in other studies of experimental or pathological calcinosis and of mineralization of skeletal tissues.

Animals↗