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PubMed2001

Risk factors and mortality associated with calciphylaxis in end-stage renal disease.

BACKGROUND: We conducted a case control study to determine risk factors and mortality associated with calciphylaxis in end-stage renal disease. METHODS: Cases of calciphylaxis diagnosed between December 1989 and January 2000 were identified. Three controls were identified for each hemodialysis patient, with calciphylaxis matched to the date of initiation of hemodialysis. Laboratory data and medication doses were recorded during the 12 months prior to the date of diagnosis and at the time of diagnosis of calciphylaxis. Conditional logistic regression was used to identify risk factors for calciphylaxis. Cox proportional hazards models were used to estimate the risk of death associated with calciphylaxis. RESULTS: Nineteen cases and 54 controls were identified. Eighteen patients were hemodialysis patients, and one had a functioning renal allograft. Diagnosis was confirmed by skin biopsy in 16 cases. Women were at a sixfold higher risk of developing calciphylaxis (OR = 6.04, 95% CI 1.62 to 22.6, P = 0.007). There was a 21% lower risk of calciphylaxis associated with each 0.1 g/dL increase in the mean serum albumin during the year prior to diagnosis and at the time of diagnosis of calciphylaxis (OR = 0.79, 95% CI, 0.64 to 0.99, P = 0.037, and OR = 0.80, 95% CI, 0.67 to 0.96, P = 0.019, respectively). There was a 3.51-fold increase in the risk of calciphylaxis associated with each mg/dL increase in the mean serum phosphate during the year prior to diagnosis (95% CI, 0.99 to 12.5, P = 0.052). At the time of diagnosis of calciphylaxis, for each 10 IU/L increment in alkaline phosphatase, the risk of calciphylaxis increased by 19% (OR = 1.19, 95% CI, 1.00 to 1.40, P = 0.045). Body mass index, diabetes, blood pressure, aluminum, and higher dosage of erythropoietin and iron dextran were not independent predictors of calciphylaxis. Calciphylaxis independently increased the risk of death by eightfold (OR = 8.58, 95% CI, 3.26 to 22.6, P < 0.001). CONCLUSIONS: Female gender, hyperphosphatemia, high alkaline phosphatase, and low serum albumin are risk factors for calciphylaxis. Calciphylaxis is associated with a very high mortality.

Alkaline Phosphatase↗
PubMed2001

Transcutaneous oxygen tension in patients with calciphylaxis.

Calciphylaxis is a severe complication of chronic renal failure, confined almost exclusively to patients on dialysis therapy. Histological characteristics of calciphylaxis include small-vessel calcifications of skin, subcutaneous tissue, and visceral organs. These vascular changes promote tissue ischemia that often results in tissue necrosis. In this study, we investigated the extent of skin ischemia in patients with calciphylaxis by means of transcutaneous oxygen tension (TCPO(2)) measurement, a noninvasive test that accurately assesses skin oxygenation. TCPO(2) levels were measured in 21 patients with calciphylaxis and 21 age- and sex-matched patients without evidence of calciphylaxis (controls). TCPO(2) levels were measured bilaterally at the chest, anterior abdomen, and upper thigh while patients breathed room air and after a 30-minute exposure to 100% fraction of inspired oxygen (FIO(2)). Compared with controls, patients with calciphylaxis showed significantly lower TCPO(2) levels at each body region. In both controls and patients with calciphylaxis, lower TCPO(2) levels correlated with increased weight and use of hemodialysis. No correlation with serum parathyroid hormone (PTH), serum calcium, or serum phosphorus values was present, although 39% of the patients with calciphylaxis had markedly elevated PTH values (sixfold greater than normal; >300 pg/dL). Low TCPO(2) levels in patients with calciphylaxis were documented in body regions with and without skin lesions. In patients with calciphylaxis, extremely low TCPO(2) values (</=30 mm Hg while patients breathed room air) were present in 62% of the body regions with skin lesions and 26% of the body regions without lesions. Room-air TCPO(2) levels </=30 mm Hg were present in only 0.8% of the body regions of control patients. TCPO(2) levels obtained while patients breathed 100% FIO(2) remained lower in patients with calciphylaxis than in controls. In conclusion, TCPO(2) levels are abnormally low in patients with calciphylaxis, indicating that severe and diffuse skin ischemia exists, even at areas free of skin lesions. Low TCPO(2) values did not substantially increase with 100% FIO(2) in many patients with calciphylaxis, suggesting a fixed insufficiency of the skin vessels. This study shows that TCPO(2) measurements may allow rapid and noninvasive screening for skin ischemia before the development of skin lesions in patients with calciphylaxis.

Blood Gas Monitoring, Transcutaneous↗
PubMed2005

Calciphylaxis: a rare complication of patients who required parathyroidectomy for advanced renal hyperparathyroidism.

Calciphylaxis is a relatively rare but life-threatening complication in uremic patients. Clinical findings and prognosis were evaluated in six patients who developed calciphylaxis from a group of 1499 patients who underwent parathyroidectomy (PTx) for advanced renal hyperparathyroidism (HPT) in our department from July 1972 to July 2003. The frequency of calciphylaxis was 0.40% (6/1499). Two patients were women and four were men. The mean age was 50.5 years, and the mean duration of hemodialysis (HD) treatment was 14.0 years. In five of six patients, calciphylaxis was classified as distal type; in one case, as proximal type. In three patients, calciphylaxis was diagnosed at the time for PTx. In two patients, calciphylaxis was identified after PTx, although the serum parathyroid hormone (PTH) level was within the appropriate range for dialysis patients. In two patients, calciphylaxis improved after PTx, but two patients required leg and toe amputations after PTx. In one patient with the proximal type of calciphylaxis, the condition occurred when a high PTH level recurred after the initial PTx. The patient died as a result of a serious infection due to uncontrollable skin ulcers. Calciphylaxis is a rare complication in patients who require PTx for renal HPT. Especially the proximal type has a poor prognosis. High levels of the Ca x P product and/or PTH are risk factors. Therefore, this syndrome should be kept in mind and attention should be paid to reduce risk factors. It is important that PTx being performed at the right time in patients with advanced renal HPT refractory to medical treatment.

Adult↗
PubMed1997

Calciphylaxis in patients on hemodialysis: a prevalence study.

BACKGROUND: Calciphylaxis is characterized by painful, violaceous, mottled skin lesions (livedo reticularis) that may progress to tissue necrosis, nonhealing ulcers, gangrene, and potentially amputation, sepsis, or death. The prevalence and characteristics of patients who have calciphylaxis need further identification to predict which patients on dialysis may benefit from close monitoring or early surgical intervention. METHODS: All 242 patients undergoing hemodialysis in an outpatient unit were reviewed retrospectively during a 15-month cross-sectional study of the prevalence and characteristics of calciphylaxis. RESULTS: Ten patients (prevalence, 4.1%) had calciphylaxis. Patients with calciphylaxis were significantly younger (49 versus 60 years; p = 0.01), had undergone hemodialysis longer (80 versus 20 months; p < 0.0001), and had higher median serum calcium (9.7 versus 9.2 mg/dl; p = 0.03), phosphate (8.2 versus 5.7 mg/dl; p = 0.001), calcium phosphate product (81.5 versus 52.9; p = 0.0004), parathyroid hormone (1496 versus 138 pg/ml; p < 0.0001), and alkaline phosphatase levels (188 versus 89 IU/L; p = 0.0001). Bone surveys were positive in all 10 patients with calciphylaxis compared with 49 (21%) of the 232 patients without calciphylaxis (p < 0.0001). All patients who underwent parathyroidectomy for calciphylaxis had dramatic healing of the ulcers. CONCLUSIONS: The presence of calciphylaxis is higher among younger patients who had undergone longer periods of hemodialysis. Therefore this group of patients should be monitored aggressively and treated expeditiously for complications of secondary hyperparathyroidism.

Adult↗
PubMed2000

Calciphylaxis: is there a role for parathyroidectomy?

OBJECTIVE: Calciphylaxis, a rare disorder typically affecting renal failure patients, results in vascular calcification with subsequent skin necrosis, gangrene, and often death from sepsis. Parathyroid hormone is thought to act as a tissue sensitizer leading to these soft tissue changes. As such, parathyroidectomy is often advocated to control this complicated condition. A discussion of calciphylaxis does not exist in the otolaryngology literature, and head and neck surgeons performing parathyroidectomy should be aware of this phenomenon. This study evaluates the success of parathyroidectomy in reversing the ill effects of calciphylaxis in both our patient population and the literature. STUDY DESIGN: Retrospective study and review of the literature. METHODS: Five patients with calciphylaxis treated at our institution were evaluated for mortality, surgical and perioperative complications, wound healing, and predictors of patient outcomes. RESULTS: Two patients died from sepsis and infectious complications of their calciphylaxis shortly after surgery. Of the three survivors, two later died (15 and 18 mo after surgery) from causes not directly related to calciphylaxis. The other long-term survivor required partial amputation of a leg for osteomyelitis. There was one operative complication-- wound infection requiring antibiotic therapy, drainage, and packing. Postoperative hypocalcemia required treatment in two patients. Immediate perioperative survival was more likely in patients with leukocyte counts less than 20,000 cells/mL. CONCLUSIONS: Calciphylaxis is a serious disease and patients often succumb to sepsis and infectious complications. Patients with extremely high leukocyte counts from coexistent infections may have a worse prognosis. Although a conclusive effective therapy does not exist, parathyroidectomy can be safely performed and may benefit some patients with what is often an otherwise fatal disease. The literature to date generally confirms our findings. Key Words: Calciphylaxis, parathyroid hormone, parathyroidectomy, skin necrosis, chronic renal failure.

Aged↗
PubMed2006

Association between calciphylaxis and inflammation in two patients on chronic dialysis.

The pathogenesis of calciphylaxis, which has a rising incidence in the chronic dialysis population and a high mortality rate, is poorly understood. Abnormalities in the calcium-phosphorus-parathyroid axis are clinically related to calciphylaxis, but alone, they cannot explain this condition. Here, we present two patients who had chronic inflammatory conditions and hyperparathyroidism and who developed calciphylaxis. A 41-year-old white woman on hemodialysis following scleroderma, hepatitis C, liver transplant, and failed kidney transplant, developed progressive ulcerative lower extremity calciphylaxis lasting more than 3 years. She had evidence of severe hyperparathyroidism and elevated serum C-reactive protein (CRP). A 39-year-old white woman on continuous ambulatory peritoneal dialysis for 6 years for renal failure secondary to lupus nephritis, with sustained lupus activity during the dialysis period, developed rapidly progressing ulcerative calciphylaxis of the lower and upper extremities not responding to adequate treatment of hyperphosphatemia and hyperparathyroidism. Her condition culminated in death within 2 months of the appearance of the skin lesions. Her serum CRP was elevated on a sustained basis before the development of the calciphylaxis and rose to a very high level after appearance of the skin lesions. Inflammation may assist in the development of calciphylaxis through depression of serum levels of fetuin-A, an endogenous inhibitor of calcification that is also a negative acute-phase reactant. The interactions between inflammation-mediated changes in the levels of endogenous inhibitors of calcification and abnormalities in calcium-phosphorus metabolism merit intensive study in the future as potential mechanisms of calciphylaxis.

Adult↗
PubMed1996

Calcification of polyurethanes implanted subdermally in rats is enhanced by calciphylaxis.

Calcification complicates the use of the polymer polyurethane in cardiovascular implants. To date only costly experimental circulatory animal models have been useful for investigating this disease process. In this paper we report that polyurethane calcification in rat subdermal implants is enhanced by overdosing with a vitamin-D analog. The calcification-prone state, known as calciphylaxis, was induced in 4-week old rats by oral administration of a vitamin-D analog, dihydrotachysterol. We studied two commercially available polyurethanes (Biomer and Mitrathane) and two proprietary polyurethanes (PEU-2000 and PEU-100). PEU-100 is unique because it is derivatized with ethanehydroxy-bisphosphonate (EHBP) for calcification resistance. Polyurethane calcium and phosphate levels and morphological changes due to calciphylaxis were compared with those of control rat subdermal explants in 60-day studies. Increased polyurethane mineralization was observed due to calciphylaxis with 60-day rat subdermal explants of Biomer, Mitrathane, and PEU-2000 (calcium levels, respectively, 4.13 +/- 0.56, 18.61 +/- 2.73, and 3.37 +/- 0.22 microgram/mg, mean +/- standard error) as compared to control explants (calcium levels, respectively, 1.22 +/- 0.1, 12.57 +/- 0.86, and 0.20 +/- 0.86 microgram/mg). The study also demonstrated that with 60-day implants calciphylaxis had no side effects on somatic growth and serum calcium levels. Explant surface morphology of these polyurethane explants examined by scanning electron microscopy, back scattering electron imaging coupled with energy dispersive X-ray spectroscopy, and light microscopy demonstrated the presence of predominantly surface-oriented calcification. PEU-100, derivatized with 100 n.moles/ mg of EHBP, resisted calcification with explant calcium levels 0.51 +/- 0.01 (calciphylaxis) and 0.38 +/- 0.01 (control) microgram/mg. It is concluded that calciphylaxis enhances superficial polyurethane calcification in rat subdermal implants and that an EHBP-modified polyurethane resists calcification despite calciphylaxis. Rat subdermal implants using calciphylaxis may be generally useful for evaluating the calcification potential of various biomedical polymers.

Animals↗
PubMed1998

A case control study of proximal calciphylaxis.

The purpose of this investigation was to describe the clinical presentation of nine patients with calciphylaxis involving the proximal lower extremities or trunk and to compare the clinical characteristics of these patients with those of 347 hemodialysis patients from the same geographic area. Patients were identified primarily through a computer search of pathology records, identifying patients with the term "calciphylaxis" in the biopsy report. All patients had pathologic specimens consistent with calciphylaxis. All the calciphylaxis patients were white and were markedly obese. While two patients had markedly elevated parathyroid hormone levels, most patients did not show severe derangements of calcium phosphate metabolism compared with other dialysis patients. A logistic regression model identified body mass index and low serum albumin 3 months before diagnosis as being highly associated with a diagnosis of calciphylaxis. Diabetes mellitus and parameters of calcium-phosphate metabolism were not significantly associated with proximal calciphylaxis. These findings suggest that white race, morbid obesity, and poor nutritional status are associated with proximal calciphylaxis in dialysis patients.

Aged↗
PubMed2006

Fulminant pulmonary calciphylaxis and metastatic calcification causing acute respiratory failure in a uremic patient.

Calciphylaxis is a rare and life-threatening disorder characterized by small-vessel mural calcification with intimal proliferation, fibrosis, and thrombosis, resulting in tissue ischemic necrosis. Although it has been viewed as a systemic disease involving mainly the dermis, subcutaneous fat, or muscle, calciphylaxis of other organs rarely is reported. We describe the case of a 25-year-old uremic woman who rapidly developed massive pulmonary calcification that led to acute respiratory failure after the initiation of hemodialysis therapy. Chest radiography and high-resolution computed tomography showed typical pulmonary calcification. Pulmonary calciphylaxis and metastatic calcification were confirmed further by lung tissue biopsy. No skin or muscle calciphylaxis was discovered. Despite multiple factors precipitating pulmonary calciphylaxis in this patient, we speculate that hemodialysis was the main culprit in accelerating the development of fulminant pulmonary calciphylaxis and metastatic calcification. Alteration in the local environment from an acid to an alkaline condition and a relatively high dialysate calcium level in the presence of systemic hyperphosphatemia are believed to have facilitated the deposition of calcification. This case highlights the importance of "visceral calciphylaxis" and early identification of its causes.

Acute Disease↗
PubMed2005

Calciphylaxis: a rare association with alcoholic cirrhosis. Are deficiencies in protein C and S the cause?

Calciphylaxis is a rare condition of induced systemic hypersensitivity in which tissues respond to appropriate challenging agents with a sudden local calcification. It is characterized by acute calcium deposition in the medial layer of small and intermediate dermal vasculature that can lead to epidermal ischemia, ulceration, and necrosis. Calciphylaxis typically occurs in patients with end-stage renal disease who are undergoing dialysis and who have secondary hyperparathyroidism. Even in this population the incidence is less than 1%. The cause of calciphylaxis is unknown. However, it has been suggested that deficiencies of protein C and protein S may play a role in the pathophysiology of this disorder. Our patient is the fourth with cirrhosis to be reported to have developed calciphylaxis and adds further evidence that low levels of these anticoagulant factors may be an important etiologic factor for development of calciphylaxis. This report should alert the clinician that calciphylaxis occurs in patients with cirrhosis and should stimulate further research concerning the possible role of protein C and protein S deficiency in calciphylaxis.

Calciphylaxis↗
PubMed2001

Calciphylaxis associated with cholangiocarcinoma treated with low-molecular-weight heparin and vitamin K.

Calciphylaxis is a rare disorder of small-vessel calcification and cutaneous infarction associated with chronic renal failure. Rare cases of calciphylaxis not associated with chronic renal failure have been reported with breast cancer, hyperparathyroidism, and alcoholic cirrhosis. To our knowledge, we report the first case of calciphylaxis without chronic renal failure associated with cholangiocarcinoma and the first attempt to treat calciphylaxis with vitamin K. A 56-year-old woman presented with necrotic leg ulceration. She was treated initially with low-molecular-weight heparin, with no effect. A coagulation work-up showed vitamin K deficiency. During vitamin K therapy, the patient had fulminant progression of the calciphylaxis. She died, and an autopsy showed metastatic cholangiocarcinoma. Thrombosis and protein C deficiency have been implicated in the pathophysiology of calciphylaxis. Functional protein C deficiency may be one of several factors contributing to the development of calciphylaxis. Vitamin K therapy was ineffective in our patient and may have been detrimental.

Adenocarcinoma↗
PubMedDate not supplied

Calcium use increases risk of calciphylaxis: a case-control study.

OBJECTIVE: To investigate the risk factors for the development of calciphylaxis in renal failure, a poorly understood and often fatal condition characterized by calcium deposition in tissues. DESIGN: Retrospective case-control study. SETTING: University hospital peritoneal dialysis center. PATIENTS: Eight continuous ambulatory peritoneal dialysis (CAPD) patients with calciphylaxis were identified in a 3-year period. We matched up to five controls for dialysis modality and length of time on dialysis with each case. STATISTICS: Multivariate conditional logistic regression analysis for matched case-controls. MAIN OUTCOME MEASURES: Laboratory data and demographics were collected as well as cumulative calcium and vitamin D ingestion over the year prior to disease onset. RESULTS: All the patients were female, versus only 38% (14/37) of controls (p < 0.0001). While not statistically significant, a majority of the patients were diabetic [62.5% (5/8) vs 32% (12/37)]. Peak and average levels of serum calcium, phosphate, calcium x phosphate product, parathyroid hormone (PTH), albumin, iron, total iron-binding capacity (TIBC), and ferritin were not significantly different in cases compared with controls. The use of calcitriol alone or with calcium carbonate was not found to be a significant risk factor for the development of calciphylaxis. In a multivariate analysis, iron intake seemed to be protective, contrary to previous reports, while the use of calcium carbonate was associated with a strong trend to increased risk of calciphylaxis development (odds ratio = 1.029/g and 1.011/g calcium ingested per month, at 1 and 2 - 3 months prior to calciphylaxis development; p = 0.0556 and 0.0565, respectively). CONCLUSION: These data, although limited by the small numbers of index cases, suggest that calcium ingestion is a risk factor for calciphylaxis. The increased use of calcium salts as a phosphate binder in recent years might explain the apparent increased incidence of calciphylaxis in our and other centers. The preponderance of female diabetics among cases reported elsewhere was confirmed in our study.

Calciphylaxis↗
PubMed2006

Calciphylaxis: natural history, risk factor analysis, and outcome.

BACKGROUND: Calciphylaxis is characterized by ischemic cutaneous ulceration, high mortality, and ineffective treatment. METHODS: We conducted a retrospective study of 64 patients with calciphylaxis (including 49 dialysis patients age- and sex-matched to 98 dialysis controls). RESULTS: The estimated 1-year survival rate of calciphylaxis was 45.8%. Risk factors for calciphylaxis included obesity, liver disease, systemic corticosteroid use, calcium-phosphate product more than 70 mg(2)/dL(2), and serum aluminum greater than 25 ng/mL. Survival rates were similar for 16 patients who received parathyroidectomy and 47 who did not. An estimated 1-year survival rate of 61.6% was observed for 17 patients receiving surgical debridement compared with 27.4% for the 46 who did not (P = .008). LIMITATIONS: The study was limited by its retrospective design and there was no control group for the 15 nondialysis cases. CONCLUSIONS: Calciphylaxis is multifactorial and usually fatal. Prevention of calciphylaxis may include correction of risk factors identified in this study. Surgical debridement was associated with improved survival, but parathyroidectomy was not.

Adult↗
PubMed1999

Calciphylaxis associated with metastatic breast carcinoma.

Calciphylaxis is a rare disorder associated with calcification of small- and medium-sized blood vessels, and progressive skin necrosis usually seen in the setting of end-stage renal disease (ESRD) and secondary hyperparathyroidism. It has also been observed in primary hyperparathyroidism, hypercalcemia of malignancy (extensive bony metastasis of breast cancer), and an isolated case reported with end-stage liver disease. We report an unusual case of calciphylaxis associated with metastatic breast carcinoma in the absence of renal or parathyroid disease. Calciphylaxis has generally been associated with end-stage renal disease and hyperparathyroidism. One previous case report described calciphylaxis occurring in a patient with metastatic adenocarcinoma of the breast and hypercalcemia. Our case represents the second reported case of calciphylaxis associated with osteolytic, metastatic breast cancer. Although ESRD with secondary hyperparathyroidism is the most common presentation of calciphylaxis, this case demonstrates that other conditions that alter normal calcium metabolism must be considered in the differential diagnosis.

Bone Neoplasms↗
PubMed2003

Penile calciphylaxis: analysis of risk factors and mortality.

PURPOSE: Penile calciphylaxis is a rare condition resulting in infection and gangrene. Most cases are associated with systemic calciphylaxis. The pathophysiology, diagnosis and management of penile calciphylaxis as a distinct entity have received little attention. We reviewed the literature to increase understanding of this disease. MATERIALS AND METHODS: A retrospective review of the literature was performed after treating a case of penile calciphylaxis. Patient characteristics, presentation, serum chemistry studies, management and outcomes are reported. RESULTS: A total of 34 cases of penile calciphylaxis were identified in the literature including our patient. Average patient age was 58 years. All patients had end stage renal disease, and diabetes mellitus was a co-morbidity in 76%. Additional areas of gangrene beyond the genitalia were found in two-thirds of patients. Average calcium phosphate product was 78.5 mg.2/dl.2 (range 20.6 to 52.5) and mean parathormone level was 553 pg./ml. (10 to 65). Parathyroidectomy was performed in 8 patients. All patients were treated with either local débridement/wound care or partial/total penectomy. Survival was better in patients who underwent parathyroidectomy (75%) than in those treated with local débridement or penectomy alone (28%). The overall mortality associated with this disease was 64% with a mean time to death of 2.5 months. CONCLUSIONS: Penile calciphylaxis is a result of medial calcification and fibrosis of blood vessels. The co-morbidity and mortality associated with this disease are extremely high. Secondary hyperparathyroidism and an increased calcium phosphate are characteristic and require aggressive medical management. Surgical management of penile lesions and parathormone is controversial. Our review suggests that parathyroidectomy may improve survival and that survival is independent of the type of local treatment for the penile lesions.

Arteries↗
PubMed1998

Calciphylaxis: a syndrome of skin necrosis and acral gangrene in chronic renal failure.

BACKGROUND: Calciphylaxis is a rare condition of rapidly extending ischemic skin necrosis or acral gangrene of fingers, toes or penis in patients with chronic renal failure. It may be accompanied by extensive metastatic calcification of soft tissues. Histology of infarcted tissues shows prominent medial calcification and intimal hyperplasia of subcutaneous arteries and/or digital arteries, respectively. The pathogenesis of calciphylaxis is only poorly understood. Most patients have hyperparathyroidism and an elevated calcium-phosphate-product, which is thought to be a major pathogenetic factor of calciphylaxis. PATIENTS AND METHODS: All published cases of calciphylaxis including nine of own (155 patients in total) from 1936 through 1996 were reviewed and subjected to statistical meta-analysis (Fisher's exact test). RESULTS: Proximal locations of necrosis (thighs, buttocks, trunk) carried an unfavourable prognosis (63% mortality) compared to distal locations (calves, forearms, fingers, toes, penis) with 23% mortality (p < 0.0001). Parathyroidectomy was associated with a favourable outcome (p < 0.004). Diabetics with chronic renal failure had acral gangrene in 61% compared to 34% of the non-diabetic calciphylaxis-patients (p < 0.007). CONCLUSIONS: The present analysis of all published cases of calciphylaxis is limited by patient selection and publication bias. The unfavourable prognosis of patients with proximal necrosis is impressive and might justify an early and aggressive treatment in such cases. However, the general benefit of parathyroidectomy remains debatable. Hyperparathyroidism should be managed primarily by conservative means. Parathyroidectomy should be reserved for patients with very high parathyroid hormone level and calcium-phosphate-product or with a rapidly progressive disease.

Calciphylaxis↗
PubMed2001

The development of the pathophysiological concept of calciphylaxis in experiment and clinic.

An attempt to clarify some pathophysiological aspects of calciphylaxis from both experimental and clinical points of view is presented. Until now, we cannot explain precisely various forms of calcification, affecting either soft tissues or biomaterial as well, only on the basis of metastatic or dystrophic calcification including the pure topical, local aspects. Therefore, the biological phenomenon of calciphylaxis can be useful in searching the ways for the inhibition of soft tissues and biomaterial calcification. The experiments with experimental progeria have also made it possible to study the so-called reversed calciphylaxis. The systemic reversed calciphylaxis, or 'anacalciphylaxis' was shown in the authors experiments with the total artificial heart as a very effective factor in the inhibition of mineralization of the TAH driving diaphragms. The discovery of the ubiquitous organ regulator of calcification, osteopontin, can greatly contribute to further elucidation of the calciphylactic mechanisms in general, and also to the prevention of calcifying lesions. It is pointed to some clinical states, predominantly the end-stage renal disease with hemodialysation where many authors pay attention to calciphylaxis as a clinical entity in a series of such cases. Calciphylaxis has been described in dermatology, nephrology and other clinical disciplines. In the area of attempts to prevent the ectopic organ calcification and also mineralization of biomaterials, a vast field of stimulation of endogenic inhibitors of calcification remains open.

Journal Article↗
PubMed2004

An unusual case of calciphylaxis.

BACKGROUND: Cutaneous calciphylaxis is a rare disorder that occurs most frequently in patients with end-stage renal disease (ESRD), those on hemodialysis, and renal transplant recipients. It is frequently associated with hyperparathyroidism and a markedly elevated calcium-phosphate product, and it carries a high mortality rate. The usual clinical presentation is of painful, stellate necrosis of the thighs or buttocks, often in the setting of livedo reticularis. Death usually results from septicemia. OBJECTIVE: This report documents an unusual case of recurrent, self-limiting calciphylaxis in the setting of a patient with ESRD and discusses the clinical and pathologic features of this potentially very fatal disorder. METHODS AND RESULTS: A 52-year-old woman presented with a greater than one-year history of relapsing and remitting, exquisitely painful, necrotic, numular plaques on the abdomen, breast, and arm. This patient had a markedly elevated calcium-phosphate product and parathyroid hormone level. The diagnosis of calciphylaxis was made by wedge biopsy of the most recent plaque, revealing calcification of medium-sized subcutaneous vessels and lobular capillaries with associated epidermal necrosis. CONCLUSIONS: This case demonstrates an unusual clinical variant of calciphylaxis that presented without the characteristic stellate necrosis or livedo reticularis that normally marks this condition and spontaneous resolution without incurring septicemia. Regardless of morphology, calciphylaxis should be considered in the differential diagnosis of painful, necrotic lesions occurring in the setting of ESRD.

Calciphylaxis↗