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PubMed1986

Progressive vascular calcification with necrosis of extremities in hemodialysis patients: a possible role of iron overload.

Progressive vascular calcification with ischemia and gangrene of the extremities occurs rarely in uremic patients, patients undergoing maintenance dialysis, and following renal transplantation. In this paper we present two additional patients on chronic hemodialysis who developed this syndrome in association with severe secondary hyperparathyroidism. Fulminant gangrene led to the death of the first patient, while in the second, multiple amputations had to be performed after parathyroidectomy. In both patients, evidence of iron overload due to multiple blood transfusions was present and iron was histologically demonstrated in a calcification area in one case. The possibility of iron overload as a "challenger" for systemic calciphylaxis is discussed.

Acute Disease↗
PubMed1998

Breakdowns on the path of chronic illness: opportunities for learning.

An unusual case of calciphylaxis, presenting at the onset of end-stage renal disease and evolving into an extended and arduous hospital stay, is described. The medical approach to this case is addressed briefly, but the main focus of this paper is to describe, in the words of various participants, the events and interactions that occurred and to learn from this description how our management of such cases breaks down. When confronted by difficult circumstances, it is common for us to react emotionally in ways that are automatic and based on our own personal histories and behavior patterns. Such automatic reactions prevent us from seeing and understanding what we really need to know about a given situation and leave us vulnerable to discouragement and internal suffering when clinical events do not go well (A. Nierenberg, personal communication, April 1998). The result is often exasperation with patients and families, as well as emotionally laden interactions that do not forward problem solving. In retrospect, the appearance of such breakdowns is not only predictable in the course of chronic illness, but offers us the opportunity to observe our automatic reactions, to re-evaluate our approach, and to redesign our actions. We have written this review, not to find error or blame, but rather to emphasize that we are learning to view these breakdowns as signals first to step back from our automatic reactions and then to listen and communicate clearly as a means to navigating the best pathway through difficult and discouraging clinical challenges.

Calciphylaxis↗
PubMed1996

Cutaneous gangrene due to hyperparathyroidism secondary to chronic renal failure (uraemic gangrene syndrome)

Cutaneous and subcutaneous gangrene are serious sequelae of secondary or tertiary hyperparathyroidism which may accompany chronic renal failure. Based on analysis of the present typical case and a retrospective survey of similar cases in the literature, we propose the term 'uraemic gangrene syndrome' for this association. These skin lesions in chronic renal failure patients represent the most serious clinical manifestation of calciphylaxis, a condition originally described by Selye. The appearance of early skin lesions should be regarded as an indication for subtotal parathyroidectomy. The association of cutaneous gangrene with vascular calcification was first described by Bryandt and White in 1989, and was termed by these authors as 'gangrenous calcification'. A total of 80 cases of this rare complication of chronic renal disease had been reported up to early 1994. A retrospective review of these reports has strongly suggested a close interrelationship between renal failure, secondary hyperparathyroidism, vascular calcification and cutaneous gangrene. The present report concerns a characteristic case to draw attention to this syndrome which is accompanied by serious, predominantly cutaneous, changes.

Adult↗
PubMed1998

Cutaneous necrosis from calcific uremic arteriolopathy.

Calcific uremic arteriolopathy (calciphylaxis) is an uncommon complication of chronic renal failure that is associated with high morbidity and mortality. We report 16 patients (13 female) who presented between 1985 and 1996. All patients developed painful livido reticularis that progressed to cutaneous necrosis and ulceration (11 cases on the proximal extremities and five cases on the distal extremities). Two patients with predominately distal leg disease survived; the cause of death in the other 14 patients was sepsis (six patients), withdrawal from dialysis (three), cardiac arrest (three), and gastrointestinal hemorrhage (two). Mesenteric ischemia from intestinal vascular calcification occurred in two cases. Clinical factors identified included the use of warfarin therapy in seven cases and significant weight loss (>10% body weight) in seven cases in the 6 months preceding the development of calcific uremic arteriolopathy. Skin pathology was studied in 12 cases, with all showing calcific panniculitis and small vessel calcification. Electron microscopic spectral analysis of the mineral content of the calcific lesions in the subcutaneous tissue showed only calcium and phosphorous. In two cases, substitution of low molecular weight heparin for warfarin therapy resulted in clinical improvement. Current theories of pathogenesis and treatment are reviewed. This study confirms the high morbidity and mortality of calcific uremic arteriolopathy producing ischemic tissue necrosis while drawing attention to significant weight loss and warfarin therapy as risk factors for the development of ischemic tissue necrosis. Hyperbaric oxygen therapy warrants further study.

Adult↗
PubMed1976

[Cystinosis, hyperparathyroidism and metastatic calcifications (author's transl)].

An 11-year-old boy died from uraemia in the course of cystinosis. In addition to the renal lesions common to this disease he had also developed nodular hyperplasia of the parathyroids and widespread extra-osseous calcifications in lungs, kidneys, stomach, the muscular arteries, skin and subcutaneous tissue. The calcifications and other features suggest that the hyperparathyroidism was a "tertiary" one, although this term is not generally accepted. The unusual cutaneous calcifications were probably due to calciphylaxis. As in this case, hyperparathyroidism and calcinosis can develop into a "second disease" which, due to the cystinosis, may follow a remarkably autonomous course.

Arteries↗
PubMed2002

[Ultrasonic diagnosis of calciphylactic lesions].

Calciphylaxis is a serious disease most commonly occurring in patients with end-stage renal disease characterized by systemic medial calcification of the arteries and tissue necrosis due to ischaemia. Secondary infections contribute to the high mortality of this disease. Diagnosis is suggested by characteristic skin lesions, calcifying septal panniculitis on deep skin biopsy and detection of calcifications in conventional radiology. We are able to demonstrate the early diagnosis with high resolution high frequency ultrasound prior to the occurrence of typical skin lesions facilitating early treatment.

Body Mass Index↗
PubMed1996

The uremic gangrene syndrome: improved healing in spontaneously forming wounds following subtotal parathyroidectomy.

Patients with end-stage renal disease often demonstrate retarded healing of surgical wounds, but the basis for spontaneous wound formation in these patients is less well understood. We report our experience with four patients with a unique clinical entity previously described as the uremic gangrene syndrome (also known as calciphylaxis) that involves spontaneously forming and insidiously progressive wounds of the skin and soft tissue in uremic patients with hyperparathyroidism. The importance of recognizing this phenomenon relates to the potential benefit to wound-healing efforts resulting from subtotal parathyroidectomy and adjustment of serum calcium and phosphate levels when severe hyperparathyroidism is present. Disrupted parathyroid homeostasis as a mechanism for soft-tissue ischemia and subsequent infarction is supported by wound biopsies demonstrating microarterial calcification. As experts in factors resulting in refractory wounds, plastic surgeons need be aware of this peculiar vulnerability for spontaneously forming wounds in uremic patients. Clinical and laboratory findings, success with wound treatment in four patients, and currently popular pathophysiologic mechanisms are discussed.

Adult↗
PubMed2005

Calcifying panniculitis following subcutaneous injections of nadroparin-calcium in a patient with osteomalacia.

Calcifying panniculitis is a rare form of calcinosis cutis belonging to the spectrum of calciphylaxis that has almost invariably been described in patients with severe renal disturbances. We report a patient with osteomalacia without chronic renal failure, who developed calcifying panniculitis following subcutaneous administration of nadroparin-calcium. Light microscopy studies of biopsy specimens revealed multiple foci of microcalcification within the adipose lobules, in the interadipocyte spaces, in connective tissue septa and in the media of small arteries in the subcutis. The patient had an elevated level of intact parathyroid hormone, whereas the calcium-phosphorus product was normal. The lesions slowly resolved upon discontinuation of nadroparin. We conclude that calcifying panniculitis is a rare complication associated with the subcutaneous administration of nadroparin-calcium that may rarely also occur in the absence of renal disturbances. Low molecular weight calcium-containing heparins should probably be used with caution in the presence of hyperparathyroidism.

Anticoagulants↗
PubMed2003

The serum protein alpha 2-Heremans-Schmid glycoprotein/fetuin-A is a systemically acting inhibitor of ectopic calcification.

Ectopic calcification is a frequent complication of many degenerative diseases. Here we identify the serum protein alpha2-Heremans-Schmid glycoprotein (Ahsg, also known as fetuin-A) as an important inhibitor of ectopic calcification acting on the systemic level. Ahsg-deficient mice are phenotypically normal, but develop severe calcification of various organs on a mineral and vitamin D-rich diet and on a normal diet when the deficiency is combined with a DBA/2 genetic background. This phenotype is not associated with apparent changes in calcium and phosphate homeostasis, but with a decreased inhibitory activity of the Ahsg-deficient extracellular fluid on mineral formation. The same underlying principle may contribute to many calcifying disorders including calciphylaxis, a syndrome of severe systemic calcification in patients with chronic renal failure. Taken together, our data demonstrate a critical role of Ahsg as an inhibitor of unwanted mineralization and provide a novel therapeutic concept to prevent ectopic calcification accompanying various diseases.

Animals↗
PubMed2002

Distal calcific uremic arteriolopathy in a hemodialysis patient responds to lowering of Ca x P product and aggressive wound care.

Calcific uremic arteriolopathy (CUA; calciphylaxis), is reported in approximately 4% of patients receiving hemodialysis, and is characterized by skin lesions that may include firm plaques or subcutaneous nodules. The syndrome has been associated with the use of calcium-containing phosphate binders, high serum phosphorus levels, and elevated calcium x phosphorus (Ca x P) product. This report describes a 73-year-old white male with chronic renal failure due to diabetes mellitus and hypertension, who had been on home hemodialysis for 3 years. He developed CUA after an acute elevation in serum phosphorus (8.1 mg/dl) and Ca x P product (84.2), with painful skin lesions that rapidly progressed to become circumferentially located around the entire lower left extremity. The patient declined amputation, opting for a treatment approach that included aggressive management of phosphorus and calcium, more frequent dialysis, and rigorous wound care. All calcium-containing phosphate binders were discontinued. The patient was switched from calcitriol to paricalcitol, a less calcemic form ofvitamin D replacement therapy, from which he was slowly weaned. Dialysis dose and frequency was also increased to 4 hours, 6 times weekly. The patient was given sevelamer hydrochloride (Renagel)--a calcium-free phosphate binder--with meals at an initial dose of 6.4 g/day. After 5 months, the dose was increased to 8 g/day, with additional dietary counseling to restrict phosphorus intake. At this point, serum phosphorus decreased to 4.9 mg/dl and calcium levels had fallen to 8.5 mg/dl, compared to 9.5 - 10.4 mg/dl prior to diagnosis of CUA with an overall decline in the Ca x P product. Significant healing of the lesions was noted at 8 months following diagnosis, with near-total healing by 12 months. Our studies support that lowering of elevated serum phosphorus, calcium, and Ca x P product, together with aggressive wound care may contribute to the successful outcome of patients with CUA.

Aged↗
PubMedDate not supplied

Painful cutaneous lesions, renal failure and urgent parathyroidectomy.

We describe two patients with end stage renal failure who presented with painful skin lesions, which rapidly progressed to become necrotic and gangrenous. The diagnosis was calciphylaxis, a rare disorder due to calcification and luminal fibrosis of small and medium sized cutaneous and systemic vessels. Both patients had tertiary hyperparathyroidism. An urgent parathyroidectomy was performed on one patient, which relieved her symptoms; the other required local surgery but refused parathyroidectomy and died.

Calciphylaxis↗
PubMed1992

[Calcifying panniculitis].

We report a case of calcifying panniculitis which occurred in a 67-year old woman suffering from chronic renal failure and primarily involved anticoagulant injection sites. Calcifying panniculitis is a rare condition belonging to the spectrum of calciphylaxis first described by Selye in 1962. This disease seems to imply necessarily a specific morbid background including renal failure together with calcium-phosphate metabolism impairment. Following the action of a so-called "challenger" (in the present case calcium heparinate [Calciparine], a subcutaneously administered anticoagulant drug), the lesions start as painful subcutaneous nodules soon turning into plaques of necrosis. The area involved mainly includes the abdomen and thighs, due to the preferential distribution of fat and the classical use of such regions for subcutaneous injections. In our patients, histological examination disclosed an initial involvement of subcutaneous fat vessels mimicking mediacalcosis, soon followed by calcium-phosphate deposits within interadipocyte spaces. An electron microscopy study confirmed the presence of calcium crystals within the cytoplasms of some connective tissue cells and the extracellular matrix. From a review of the literature, it seems reasonable to normalize the calcium-phosphate product by reducing hyperparathyroidism as a first therapeutic step. Then, wide surgical excision of the necrotic areas should be performed to rule out any possibility of self worsening of the lesions and provide the best chance of healing without superinfection. The use of diphosphonates on pathophysiological grounds is discussed.

Aged↗
PubMed1992

Experimental calcification in rat submandibular gland.

The present study describes the phenomenon of calciphylaxis, rapid calcification due to treatment with sensitizer dihydrotachysterol (DHT) and challenging agent 5-hydroxytryptamine (5-HT) in the rat submandibular gland (SMG) in terms of light and electron microscopy, and histochemistry. For biophysical analysis of the calcified bodies, X-ray microanalysis (XMA) and X-ray powdered diffraction methods were used. The calcified lesions in the salivary glands were histologically divided into 3 types: type 1, calcification of basal membranes in duct-like structures; type 2, granular calcified materials with remarkable necrotic changes in cell, containing 3 kinds of small vesicular structures observed in electron microscopy; and type 3, von Kossa's positive structures containing needle-like crystalline and electron-dense amorphous materials. Con A and UEA-1 lectin staining reactions were strong in the type 1 and 2 lesions. These findings suggest that the calcification matrix may contain mannose, fucose and glucose. The X-ray microanalysis of calcified materials revealed the magnesium whitelockite pattern, the type 3 displayed high quantities of Ca, P, and Mg ions comparing with the type 1 and 2, and the X-ray diffraction showed the hydroxyapatite pattern. We suggest that the above changes may be categorized as dystrophic calcification due to necrotic alterations brought about by the hypercalcaemic condition.

Animals↗
PubMed1989

Acute hyperparathyroidism with systemic calcinosis. Report of a case.

A patient with a huge mediastinal parathyroid adenoma had an acute hypercalcemic crisis. The patient exemplifies the many pitfalls in diagnosis and management of this unusual complication. Postoperatively the patient further developed severe calciphylaxis with calcinosis cutis and systemic and pulmonary calcinosis, a most rare condition.

Acute Disease↗
PubMed1986

[Mast cells in calciphylactic pancreatitis].

In the pancreas of female rats (180 g body mass) mast cells were seen with histological and histochemical methods in the periductular and perivasal connective tissue in a less number; they were absent in the interacinar region totally. Compared to it they were relatively numerous in the peripancreatic investing tissue. Under calciphylactic conditions (H. Selye) i. e. after sensitization with Vitamin D2 and infiltration of Ferric dextran only calcifies the interstitium of the pancreatic gland, but not the peripancreatic tissue. It is concluded that mast cells in the calciphylactic reaction of the pancreas are of an inferior importance, a mastocalciphylaxis does not exist, but in the sense of a simple calciphylaxis the collagenous connective tissue undergoes a calcifying reaction.

Animals↗
PubMed1996

Proximal cutaneous necrosis in association with chronic renal failure.

We report two patients with renal failure who developed painful symmetrical ulceration over the proximal thighs and abdominal wall. Histological examination of the ulcerated areas revealed intravascular calcification. This phenomenon has been termed calciphylaxis and is a rare complication of renal failure. The pathogenesis is poorly understood. However, abnormalities of calcium/phosphate metabolism and of coagulation are important.

Calciphylaxis↗
PubMed2003

Nephrogenic fibrosing dermopathy with systemic involvement.

BACKGROUND: There is a growing literature regarding sclerotic and panniculitic cutaneous conditions seen in patients with end-stage renal disease (eg, calciphylaxis and soft tissue calcification). Nephrogenic fibrosing dermopathy (NFD) is a recent designation to describe cutaneous findings in patients with end-stage renal disease who developed sclerotic plaques with scleromyxedema-like histologic features. Soft tissue calcification is rare in patients with NFD and systemic involvement has not been reported. OBSERVATIONS: We describe a patient with end-stage renal disease who developed diffuse indurated woody plaques consistent with NFD in association with soft tissue calcification with catastrophic sequelae. A deep excisional biopsy specimen from the patient revealed thickened collagen bundles in the reticular dermis, plump bipolar spindle cells, and increased mucin. Focally, there were zones of calcium deposition in dermal collagen without vessel calcification. Autopsy of the patient revealed extensive fibrosis and calcification of the diaphragm, psoas muscle, renal tubules, and rete testes. The patient died 11 months after developing NFD. CONCLUSION: A subset of patients with NFD may have significant systemic involvement.

Calcinosis↗