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Results for “Calciphylaxis”

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PubMed1996

Proximal calciphylaxis in four insulin-requiring diabetic hemodialysis patients.

We present four cases of proximal calciphylaxis in end-stage renal disease patients treated with hemodialysis. All patients were diabetic and developed lesions in areas that had previously served as sites of insulin injection. We review the presentation, pathogenesis, pathology, prognosis, and treatment of this devastating condition. Finally, we hypothesize that subcutaneous injection of insulin may play a pathogenic role in the development of proximal calciphylaxis.

Abdomen↗

PubMed2000

Calciphylaxis in a patient without renal failure or elevated parathyroid hormone: possible aetiological role of chemotherapy.

Calciphylaxis is a rare, often fatal disease characterized clinically by progressive cutaneous necrosis and ulceration, and histologically by vascular calcification and thrombosis. It has been described in association with end-stage renal disease, after initiation of dialysis, following renal transplantation, and in patients with hyperparathyroidism. We present the first case of calciphylaxis occurring in a patient with both normal renal function and parathyroid hormone level and discuss the possible aetiological role of chemotherapy-induced functional protein C and protein S deficiency.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed2003

Calciphylaxis in a patient with alcoholic liver disease in the absence of renal failure.

Calciphylaxis is a rare, potentially life-threatening syndrome characterized by progressive microvascular and superficial soft tissue calcification, usually seen in patients with chronic renal failure. We describe an unusual case of calciphylaxis in a patient with alcoholic liver disease and normal renal function who responded well to conservative wound care.

Calciphylaxis↗

PubMed2001

Calciphylaxis: a poor prognostic indicator for limb salvage.

INTRODUCTION: Calciphylaxis (calcific uremic arteriolopathy) is a rare complication of end-stage renal disease in patients with secondary hyperparathyroidism. Subcutaneous skin nodules and extensive tissue necrosis with vascular calcification characterize this process. DESIGN AND SETTING: The design of the study included case series and literature review. The study was set in a tertiary care hospital. PATIENTS: Four patients presented over an 8-month period with extensive tissue loss and the subsequent diagnosis of calciphylaxis. Two of these patients were revascularized. One underwent primary amputation, and the final patient died before revascularization. The mortality rate for this group was 75%. All patients had significant vascular complications outside the area of initial presentation. CONCLUSIONS: These individuals represent a subset of patients who may not benefit from revascularization or may require extensive regulation of divalent metabolism before consideration for revascularization.

Aged↗

PubMedDate not supplied

Intensive tandem cryofiltration apheresis and hemodialysis to treat a patient with severe calciphylaxis, cryoglobulinemia, and end-stage renal disease.

This is the first report on tandem cryofiltration apheresis (CFA) and hemodialysis (HD). A 44 year old white man with Type II mixed cryoglobulinemia, hepatitis C virus (HCV), severe skin lesions, and end-stage renal disease (ESRD) on maintenance hemodialysis was air-transferred for CFA, which is only available at our medical center. The patient failed to respond to high dose steroids, immunosuppression, intravenous immunoglobulin (IVIG), and plasma exchange for the treatment of his cryoglobulinemia, and he failed alpha-interferon therapy for his HCV. On arrival, he was also found to have severe calciphylaxis secondary to ESRD with generalized, painful skin ulceration, necrosis, and penile gangrene. To treat both conditions, intensive, tandem CFA/HD was initiated. He received extensive wound care and surgical debridement. To prevent pressure ulcers and worsening of skin lesions, he was placed on the FluidAir (Kinetic Concepts Inc., San Antonio, TX) controlled air bed. The patient received 18 tandem CFA/HD treatments, and four extra HD treatments in one month. Sodium citrate was used as an anticoagulant for the CFA procedure. His plasma cryoglobulin (CG) level dropped from 6,157 to 420 microg/ml, and his calciphylaxis also improved. The CFA effectively removed 93% of CG, without significant removal of IgG, IgM, IgA, albumin, and fibrinogen. No albumin or fresh frozen plasma (FFP) was required as replacement fluid for CFA. No citrate toxicity or evidence of complement activation with the cryofilter was observed. The entire CFA procedure time (3(1/2) hours) was considered. Intensive, tandem CFA/HD was performed in a critically ill patient with no apparent adverse consequences.

Adult↗

PubMed2001

Control of serum phosphorus: implications for coronary artery calcification and calcific uremic arteriolopathy (calciphylaxis).

There is mounting evidence that elevated serum phosphorus is an important cardiovascular risk factor in patients with end stage renal disease. Recent work has shown that vascular smooth muscle cells have the ability to undergo osteoblastic differentiation and produce an environment conducive to mineralization. Serum phosphorus is an important stimulator of this process and the adverse cardiovascular effects of hyperphosphatemia are most likely mediated via its ability to enhance the development of vascular calcification. Arterial calcification, whether it is intimal or medial in location, is a strong independent risk factor for cardiovascular morbidity and mortality. Both coronary artery calcification and calciphylaxis are prototypical examples of arterial calcification that have been associated with poor phosphate control. Furthermore, several investigators have recently suggested that the prescription of large doses of calcium to achieve phosphate control may augment, rather than diminish, the risk of vascular calcification. This is more likely to be true in the presence of low turnover bone disease, a diagnosis difficult to make with routine laboratory testing. A brief review of the molecular biology of vascular calcification supports the concept that warfarin administration may exacerbate the calcific process, particularly in the setting of hyperphosphatemia, as has been reported in patients with calciphylaxis. Recognizing the consequences of poor phosphate control, it is time to adopt strict target levels that aim to normalize serum phosphorus levels. The available evidence supports that this control should not be achieved through the use of supraphysiologic doses of supplemental calcium.

Arterioles↗

PubMed2003

Early recognition and treatment of calciphylaxis.

Calciphylaxis is a condition with a high mortality rate that is often found in patients with renal failure. It is characterized by soft tissue calcification and painful skin ulceration. A serum calcium-phosphorus product of more than 60 mg2/dl2 indicates great risk for calciphylaxis. The diagnosis is made on the basis of an incisional biopsy showing calcification of the small, subcutaneous arteries.

Aged↗

PubMed2001

Fulminant and relentless cutaneous necrosis with excruciating pain caused by calciphylaxis developing in a patient undergoing peritoneal dialysis.

A 50-year-old Japanese female with chronic renal failure who had been on continuous ambulatory peritoneal dialysis developed fulminant systemic cutaneous necrosis that began as painful livedo reticularis-like skin lesions on her thighs. Because of disseminated vascular calcification within the muscular layer of her lower limbs, we eventually diagnosed her with calciphylaxis. The skin necrosis progressed rapidly, and she died of sepsis and pneumonia on the 53rd hospital day. In addition to her long-lasting severe hyperparathyroidism and extremely elevated serum phosphorus and calcium levels, mechanical, frictional stimulation inflicted on the local skin and administration of corticosteroids were suspected to have precipitated the calciphylaxis. Our lack of awareness of this disease in its early stages resulted in our missing the chance to do a parathyroidectomy that might have changed the course. It is important to know the clinical features of this rare disease in order to make a diagnosis as early as possible.

Calciphylaxis↗

PubMed2001

Calciphylaxis in a patient with end-stage renal disease.

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.

Aged↗

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

Calciphylaxis in moderate renal insufficiency: changing disease concepts.

Calciphylaxis is a rare but frequently fatal complication in patients with end-stage renal disease. Original concepts regarding groups at risk for the disease, predisposing factors, and associated morbidity have changed significantly in the past few years as more cases are reported. We present a patient who developed fatal calciphylaxis in the setting of moderate renal insufficiency to illustrate some of the evolving concepts in this disease process.

Aged↗

PubMed2001

Acute respiratory failure due to "pulmonary calciphylaxis" in a maintenance haemodialysis patient.

Calciphylaxis is a rapidly developing, fatal process of vascular calcium deposition with prominent cutaneous manifestation. We treated a long-term haemodialysis patient who developed an analogous disorder limited to the lungs. A 57-year-old man was admitted for initiation of peritoneal dialysis because limited cardiac reserve precluded further haemodialysis. He was treated successfully for pneumonia until hypoxia and progressive hypercalcaemia developed. (99m)Tc-methylene disphosphonate scintigraphy showed diffusely increased pulmonary uptake. Death supervened despite aggressive and successful treatment of hypercalcaemia. Autopsy studies included immunohistochemistry and morphometric studies of bone. Alveolar capillary walls showed diffuse calcium deposition. Both gross and microscopical findings differed from those of typical metastatic calcification in dialysis patients. Immunoreactivity for parathyroid hormone-related protein was present in the lesions. Bone histomorphometry indicated mild osteitis fibrosa. Pneumonia is believed to have caused local synthesis of parathyroid hormone-related protein that, along with high calcium x phosphorus product, contributed to calcium deposition. By analogy with the cutaneous process we termed the deposition "pulmonary calciphylaxis".

Acute Disease↗

PubMed1995

Calciphylaxis in a chronic hemodialysis patient with protein S deficiency.

Vascular calcifications are common in uremic patients whereas calciphylaxis is rare. We report the case of a 45-year-old woman on chronic hemodialysis since 1977. She had a subtotal parathyroidectomy in 1985, aortic and mitral valve replacement in 1986, and has been treated since then with nicoumalone. In June 1991, she presented with repeated, painful cutaneous necrosis suggesting panniculitis. A skin biopsy showed lobular panniculitis and evidence of calciphylaxis. There was an obvious biological hyperparathyroidism. Protein C functional level was in the normal range whereas protein S functional level was low, i.e. 42%. The patient underwent cervical surgery to remove two parathyroid glands, and daily hemodialysis sessions. Despite this treatment, cutaneous necrosis progressed with superinfection. A few weeks later, the patient died from a septic shock after a myocardic infarction. Necropsy was not performed.

Calciphylaxis↗

PubMed1998

Calcific cerebral embolism in systemic calciphylaxis.

Calciphylaxis represents a rare complication of end-stage renal disease with hyperparathyroidism. We report the case of a 26-year-old woman with systemic calciphylaxis secondary to chronic renal failure who developed mitral annular calcification and a right middle cerebral artery stroke. The high-density lesion seen on CT scan of the brain probably represents a calcified cerebral embolus originating from the mitral valve.

Adult↗

PubMedDate not supplied

Physical and chemical analyses of the mineral substance during the development of two experimental cutaneous calcifications in rats: topical calciphylaxis and topical calcergy.

In subcutaneous calcinoses induced in rats by topical calciphylaxis and topical calcergy, the ultrastructural aspects related to the evolution of calcified deposits were previously described. In the present study by means of X-ray diffraction, infrared spectrometry and electron spin resonance, different biophysical analyses are performed on the mineral substance deposited during development of calciphylaxis and calcergy. A rapid evolution of the calcium phosphate deposits into hydroxyapatite was noticed along the first 20 days of the calcinoses; from then, there is no important modification even at the later stages, and the characteristics of the mineral substance are mostly similar to that of bone tissue. The concentration of trace elements such as Mg2+ and Fe2+, is found higher in cutaneous calcinoses than in bone tissue.

Animals↗

PubMed1995

A dialysis patient with systemic calciphylaxis exhibiting rapidly progressive visceral ischemia and acral gangrene.

Systemic calciphylaxis is a rare, poorly understood syndrome of progressive peripheral ischemic necrosis and medial arterial calcification in patients with end-stage renal disease. We report a patient with this syndrome which developed following corticosteroid administration and who ultimately required amputation of the four extremities. Furthermore, cerebral, myocardial, splenic, and intestinal infarctions also developed in parallel with the increment of visceral arterial calcification. No evidence of noticeable hyperparathyroidism or elevation of serum calcium-phosphate product was observed. We speculated that, in addition to diabetes mellitus and chronic renal failure while undergoing dialysis therapy, the administration of corticosteroids might act synergistically to cause calciphylaxis.

Calciphylaxis↗

PubMed2001

An unusual presentation of calciphylaxis due to primary hyperparathyroidism.

We present the case of a 69-year-old woman with calciphylaxis due to primary hyperparathyroidism. A 0.5-g parathyroid adenoma was surgically removed, which resulted in complete recovery of the patient. Review of the literature revealed 7 other cases of calciphylaxis due to primary hyperparathyroidism and showed that prompt surgical removal of the autonomous parathyroid gland lesion results in clinical recovery of calciphylactic skin lesions.

Adenoma↗

PubMed1998

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 pruritus 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.

Adult↗