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PubMed2002

The involvement of matrix glycoproteins in vascular calcification and fibrosis: an immunohistochemical study.

Calcification and fibrointimal proliferation are associated with advanced complicated atherosclerosis in large arteries but may also occur in smaller vessels, resulting in ischaemic tissue necrosis. This study investigates whether the mechanisms of calcification and intimal fibrosis are similar in vessels of different sizes. The localization of osteopontin (OPN), matrix Gla protein (MGP), thrombospondin-1 (TSP-1), and cartilage oligomeric matrix protein (COMP) was investigated in three types of human vascular lesions: atherosclerosis, chronic vascular rejection (CVR) in renal allografts, and calcific uraemic arteriolopathy (calciphylaxis). These lesions were chosen as they affect different sized blood vessels and they exhibit a fibroproliferative intimal reaction, with or without calcification, resulting in luminal obliteration and ischaemic complications. OPN, MGP, TSP-1, and COMP were not detected in normal blood vessels. However, OPN and MGP were expressed at sites of calcification within atherosclerotic lesions and in microvessels in calciphylaxis, suggesting that calcification in different sized vessels may occur by a common mechanism. These proteins were not detected in areas of fibrointimal proliferation. In contrast, TSP-1 was localized primarily within the fibrous tissue of atherosclerotic lesions and was also expressed in the expanded fibrous intima of arteries showing CVR. COMP was localized primarily within the fibrous tissue under the lipid core of the majority of advanced atherosclerotic lesions. TSP-1 and COMP were also detected in areas of microcalcification in atherosclerotic lesions and TSP-1 was detected adjacent to areas of calcification in calciphylaxis. However, neither TSP-1 nor COMP was localized to calcific foci within these lesions. The localization of OPN, MGP, TSP-1, and COMP to pathological, but not normal arterial intima supports a pathogenetic role for these proteins in the development of vascular fibrosis and calcification. Modulation of their production and activity may offer a novel approach to the therapy of a number of vascular diseases.

Adult↗
PubMed1984

Cutaneous calciphylactic reactions in the mouse and the rat and the effects of diphosphonates on the reaction in the rat.

Calciphylaxis is a local tissue calcific reaction at the site of an injection of challenger substance given a critical time period after the oral administration of a sensitizer substance such as dihydrotachysterol (DHT), vitamin D or parathormone. Cutaneous calciphylaxis is readily induced in the rat but not in the mouse and this may be because, in the latter, the challenger substance is absorbed rapidly by macrophages. In the rat the administration of 500 micrograms/0.1 ml of DHT followed after 24 h by the subcutaneous (SC) injection of ferric chloride (FeCl3) (30 micrograms/0.1 ml) is followed rapidly by calcification of the SC site. There is an early transient acute inflammatory reaction with the incrustation of collagen fibres by the iron salt and an apparent exudation of calcium and phosphate ions from the bloodstream. These ions also become associated with collagen fibres. Two days after injection macrophages and multinucleated giant cells become the dominant cells. Calciphylaxis is a useful experimental model of ectopic calcification and is associated with an initial hypercalcaemia. The diphosphonates ethane-1-hydroxy-1, 1-diphosphonate (EHDP) and dichloromethylene diphosphonate (Cl2MDP) are effective inhibitors of the calciphylactic reaction when administered prior to the initiation of the experimental procedure.

Animals↗
PubMed2003

Metastatic calcification.

Calciphylaxis is a rare, painful, and debilitating disease of widespread metastatic calcification that progresses to ischemic tissue necrosis. It occurs almost exclusively in patients with end-stage renal disease and secondary hyperparathyroidism. Reticulated, violaceous, mottled patches commonly appear on the lower extremities and progress to cordlike nodules and necrotic ulcers with overlying eschars. Septicemia, which may occur after secondary infection of the ulcers, is the most common cause of death. Treatment with phosphate-binding antacids, total or partial parathyroidectomy, and avoidance of local tissue trauma remain the mainstays of therapy, although a uniform cure for calciphylaxis remains elusive. Prognosis for patients with calciphylaxis is dismal, even after surgical intervention. Early diagnosis and treatment is critical to prevent progression of the disease process.

Calcinosis↗
PubMed2002

Intestinal ischemia and peripheral gangrene in a patient with chronic renal failure.

Gastrointestinal complications are common in patients with renal failure and result in significant morbidity and mortality. Systemic calciphylaxis is an uncommon complication of renal failure, characterized by disseminated intravascular calcification and associated with progressive vascular compromise. We describe the case of a 63-year-old woman who presented with abdominal pain, elevated transaminases, and skin manifestations consistent with a vasculitic process. Hand films and skin biopsies showed extensive vascular calcification, and a computerized tomography scan confirmed colonic perforation and disseminated visceral vascular calcification. Histologic analysis of the resected skin and colonic tissues revealed extensive ischemic damage and mural calcification of medium to large vessels. Gastrointestinal involvement has been reported in only 3 prior cases of calciphylaxis; consequently, gastroenterologists are often unaware of this disease entity and may fail to recognize it, even in patients with the classical presentation. Prompt diagnosis is crucial, as parathyroidectomy may result in clinical improvement in up to two thirds of patients who present with elevated parathyroid hormone levels.

Abdominal Pain↗
PubMed2002

Necrotic skin lesions in a dialysis patient: a multifactorial entity.

A female dialysis patient with a consistently high serum calcium phosphate product presented with large necrotic skin lesions with ulcers. The clinical course was highly suggestive of calciphylaxis. Parathyroidectomy was followed by the healing of the lesions. New skin lesions appeared following relapse of hyperparathyroidism. Her clinical records included a long past of hypertension, which was the cause of her renal failure. She had a limited walking range and previously had presented bilateral ulcers of vascular origin. This case presents a type of lesion which bears a serious prognosis in dialysis patients. The clinical context and the presentation of the lesions are compatible with multiple etiology: vascular lesions and calciphylaxis. The documented longitudinal follow-up illustrates the importance of treating the different factors known to participate in the appearance of skin lesions in dialysis patients. Particularly, it stresses the benefit of performing parathyroidectomy, even if the parathyroid hormone level is not in the range normally accepted as requiring surgical removal of parathyroid glands.

Aged↗
PubMedDate not supplied

[Isolated peripheral arterial ischaemia and medullary neurostimulation: case report].

Isolated peripheral arterial ischaemia (IPAI) is an unusual pathology of dialysis and peritoneal patients which represents the first sign of a complication of uraemia known as calciphylaxis. Recent studies have revealed an increased incidence of this complication. Risk factors are known but there is no consensus on them: elevated CaxP product, female gender, elevated serum parathormone. We present here the case of a 65-year-old man with 21-year history of dialysis, distal isolated ulceration and without any signs of severe vasculopathy. Our clinical diagnosis was calciphylaxis. In this case, the role of early PTX is not clear and the use of steroids is recommended only in non-ulcerating cases. The therapy gives good results but not in all patients. Electrical stimulation of the posterior roots of the spinal cord is an alternative approach to this case. We hypothesised that the electrical action, through cutaneous vasodilatation of afferent dorsal fibres and release of calcitonin gene-releasing protein, determines the release of prostaglandin E sub 2 that may positively affect the proliferation and activity of epidermal fibroblasts.

Afferent Pathways↗
PubMed1991

Proximal cutaneous necrosis associated with small vessel calcification in renal failure.

Cutaneous necrosis with microvascular calcification is a rare and serious complication of chronic renal failure and has been given the sobriquet of 'calciphylaxis'. We describe four dialysis-dependent patients with proximal cutaneous necrosis who presented with this distinctive clinical syndrome. All of the patients were women aged between 40 and 68, and all developed widespread livedo reticularis followed by painful subcutaneous nodules which progressed to eschar-like lesions of the skin. Microvascular calcification was seen on radiographs of the limbs, especially at the sites of the cutaneous lesions. Serum phosphorus concentrations were increased in all the patients (maximally to between 2 and 3.6 mmol/l), but serum calcium concentrations were mildly increased in only two patients and only one patient had hyperparathyroidism. Histological examinations of skin biopsies in two patients showed cutaneous infarcts. Three patients died despite a reduction in serum phosphorus concentration and one patient improved. The proximal form of 'calciphylaxis' constitutes a distinct syndrome which can be recognized clinically.

Adult↗
PubMed1989

Polyamine biosynthetic decarboxylases in muscles of rats with different experimental myopathies.

The activities of the two polyamine biosynthetic decarboxylases (PBD), L-ornithine decarboxylase (ODC) and S-adenosyl-L-methionine decarboxylase (SAMD), have been measured in quadriceps femoris of rats killed at different times after the induction of calciphylaxis- or serotonin(5-HT)-induced myopathy. Decreases in both PBD levels were observed at early times after both myotoxic treatments. Subsequent progressive increases in both enzyme levels were observed to nearly control values by 4 days after 5-HT administration. In the 5-HT-treated rats, the effects on the myocardial PBD activities were different from those in skeletal muscle, with no effect on ODC but much on SAMD, when rats were killed shortly after 5-HT injection. These results demonstrate that the time-course of the changes in PBD activities in quadriceps femoris mirrors quite well the successive occurrence of degenerative and regenerative processes during the calciphylaxis-induced myopathy and the 5-HT-induced myopathy; it is 5-HT that is mainly responsible for the decreases in PBD levels observed in both experimental myopathies, since dihydrotachysterol alone was without any effect on PBD activity levels and 5-HT alone was effective; myocardial ODC reacts more slowly to 5-HT than quadriceps femoris ODC.

Adenosylmethionine Decarboxylase↗
PubMed2004

Near-total parathyroidectomy is beneficial for patients with secondary and tertiary hyperparathyroidism.

BACKGROUND: Despite several options for the extent of parathyroidectomy in secondary and tertiary hyperparathyroidism, <5% of patients are referred for surgical procedures. The purpose of this study was to identify beneficial outcomes of near-total parathyroidectomy (NTPTX) in this population. METHODS: NTPTX (a vascularized parathyroid remnant is left in situ, and cryopreservation is performed) was completed in 142 patients with secondary (n = 93 patients) and tertiary (n = 49 patients) hyperparathyroidism, and was guided by intraoperative intact PTH (IOPTH). RESULTS: One hundred thirty-six of 142 patients (96%) remain cured at a mean follow-up time of 23 +/- 26 months. Six patients had missed mediastinal (n = 4 patients) or cervical (n = 2 patients) parathyroid glands. No patient required re-resection of a cervical parathyroid remnant. One patient had hypoparathyroidism and underwent autografting with cryopreserved tissue. NTPTX reduced parathyroid mass (4526 +/- 4515 mg to 77 +/- 56 mg) and IOPTH (1171 +/- 1252 pg/mL to 101 +/- 127 pg/mL). Fatigue and musculoskeletal pain resolved promptly after operation. Patients with tertiary hyperparathyroidism experienced an improvement in bone density of 3.6% per year. NTPTX achieved full healing of extremity ulcers without amputations or perioperative death in 22 patients with calciphylaxis. CONCLUSIONS: NTPTX effectively treats secondary and tertiary hyperparathyroidism in >95% patients. IOPTH monitoring and cryopreservation are valuable procedural adjuncts. Limb salvage in calciphylaxis, symptomatic relief, and improved bone density are substantial benefits to recommend this procedure to patients with renal hyperparathyroidism.

Adult↗
PubMed2004

Traumatic calcinosis cutis in a dialysis patient.

A patient with end-stage renal disease and refractory hyperparathyroidism was evaluated for acute-onset thickening and hardening of the skin of the lower extremities. Her clinical course and physical examination findings were consistent with the recently described entity of nephrogenic fibrosing dermopathy. However, skin biopsy results showed metastatic and dystrophic calcification, without calcific uremic arteriolopathy (calciphylaxis). The patient reported a history of self-inflicted trauma; the authors postulate that trauma, in the setting of hyperparathyroidism and an elevated serum calcium phosphorous product, resulted in the subcutaneous deposition of calcium salts. To the authors' knowledge, this is the first report of metastatic and dystrophic calcification, without calciphylaxis, in a patient with refractory hyperparathyroidism. This case underscores both the rich variety of skin conditions seen in patients undergoing dialysis and recent developments in the field of dermatologic disorders associated with end-stage renal disease.

Calcinosis↗
PubMed2004

Fatal calcific uraemic arteriolopathy (CUA): a case report and review of the literature.

Calciphylaxis, now better known as Calcific uraemic arteriolopathy (CUA), is an uncommon condition characterised by small vessel calcification and occlusion with resultant painful violaceous skin lesions that typically ulcerate to form non-healing gangrenous ulcers. The syndrome is usually found in patients with renal failure. In this report we describe a 61 year old lady who developed lower limb ulceration secondary to calciphylaxis and discuss the current treatment options for this serious condition.

Calcinosis↗
PubMed1998

Editorial

As clinical dermatologists, we are all striving to achieve the highest possible accuracy in our clinical acumen and diagnostic skills. Over the past decade, one relatively simple advance, epiluminescence microscopy with the use of the dermatoscope, has significantly contributed to our diagnostic skills in the detection of benign versus pigmented lesions. In the paper by Kawabata and Tamaki, these authors delineate distinctive dermatoscopic features of acral lentiginous melanoma in situ, and contrast this with melanocytic nevi. The restructuring of healthcare delivery systems by third party payers and governmental programs is impacting on the pattern of our medical practices. In Canada, this has limited access to widespread use of techniques such as Mohs' micrographic surgery. The article by Arlette and colleagues has further supported the well-established studies indicating that Mohs' micrographic surgery for high-risk skin cancers has a dramatic benefit. Healthcare restructuring has also led to a decreased number of trainees in a number of subspecialties, including dermatology. This decrease in manpower has been an impetus to look at alternative forms of care for underserviced areas. Telemedicine, the use of telecommunications technology to provide healthcare services over a distance, has been examined as one attempt at solving this problem. In the Point-Counterpoint articles, we have two distinct views on the future of telemedicine as it applies to dermatology. Over the past decade, there have been dramatic advances in our understanding at a molecular nature of various disease processes. This rapid development has translated into a large number of therapies. Regulatory agencies such as the Food and Drug Administration in the United States, or the Health Protection Branch in Canada, are caught between demands to bring effective therapies to the market in an expedited fashion, and yet establish efficacy and maintain safety of new therapeutic entities. This occurs by a multistaged approval process. During the early phases, exposure is limited in order to accumulate preliminary data on pharmacology and toxicity. In the Critical Appraisal CME series, Muglia and DiGiovanna describe early testing processes in Phase 1 clinical trials. Calciphylaxis is a severe disease associated with calcification of the skin, subcutaneous tissue and potentially, internal organs. While the disease itself is relatively uncommon, the manifestations are quite distinctive. In this issue of the Journal, we have a review of calciphylaxis from Richard Worth, as well as a preamble by Dr. Goodall and a case report by Kalaaji et al. illustrating the consequences of this rare but distinctive entity.

Journal Article↗
PubMed2005

Vascular calcifications in chronic kidney disease: are there new treatments?

Cardiovascular disease is extremely common in patients with end-stage renal disease (ESRD) and accounts for at least 50% of deaths among these patients. Vascular calcifications (VC) have been recently implicated as a possible cause of this excess cardiovascular mortality. Medial calcification is a striking feature of vascular disease in patients with ESRD. The traditional view that VC is a degenerative and passive process has been seriously challenged, based on strong evidence suggesting that VC is an active and highly regulated process similar to bone formation. Different data support the notion that elevated levels of phosphorus and/or other uremic toxins may play an important role by transforming vascular smooth muscle cells into osteoblast-like cells, which can produce bone matrix proteins. This nidus can then mineralize if the balance of pro-mineralizing factors outweighs inhibitory factors. The advent of newer noninvasive screening tests have generated great interest for screening patients with ESRD for vascular calcifications. Control of serum phosphorus with sevelamer, a recently developed non-calcium, non-aluminum phosphate binder, have attenuated or arrested progression of coronary artery and aortic calcifications compared to treatment with calcium-based binders. Amino bisphosphonates, have shown to completely inhibit soft tissue calcifications, calciphylaxis and prevent death in animal models. The first generation bisphosphonate, etidronate, reduces the progression of coronary artery calcifications patients receiving long-term hemodialysis and intravenous pamidronate has produced a rapid improvement of calciphylaxis. In conclusion, VC is a widespread phenomenon in patients with ESRD with important cardiovascular consequences. A better understanding of the processes of VC is leading to therapies to retard or improve this phenomenon and will probably have an important impact on patient mortality.

Animals↗
PubMed1995

Progressive ischemic gangrene in dialysis patients: a clinicopathological correlation.

The syndrome of progressive ischemic gangrene (PIG) of the extremities was examined over 3.5 years in patients undergoing maintenance dialysis (MD) in Kuwait and was compared to that in a similar age group (> 40 years) in the general population. The incidence of PIG in MD patients was 15.4/1000 person years of observation (PYO) versus 0.086/1000 PYO in the general population. Patients with diabetes mellitus were found to be at particular risk. PIG developed in 41.4/1000 PYO of diabetic patients who received MD, compared to 7.1/1000 in nondiabetic patients on MD and 0.14/1000 in diabetics without renal disease. The clinical, biochemical, radiological, and histological findings in the 8 patients who developed PIG while on maintenance dialysis (MD) are presented. Two patients had severe hyperparathyroidism and their histological findings were consistent with systemic calciphylaxis. Histological examination, in the remaining patients, showed severe calcified atherosclerosis. Intimal hypertrophy was common especially in patients with long duration on dialysis. The three lesions produced a variable degree of luminal narrowing and were associated with arterial thrombosis. None of the patients showed evidence of iron deposition even in those with systemic calciphylaxis and excessive iron stores. Our study indicated a high incidence of PIG in patients undergoing MD, especially in those with diabetes mellitus. These findings constitute a cogent argument in favor of early parathyroidectomy in selected cases and concern with long-term consequences of atherosclerosis in this patient population.

Aged↗
PubMed1963

EFFECT OF CHEMICAL THYMECTOMY ON SKIN HOMOGRAFTS. A PRELIMINARY REPORT.

In an attempt to combine the results obtained by Miller (mice thymectomized at birth accepted homograft at six weeks of age) and those obtained by Selye (selective calcification of the cortex of the thymus with calciphylaxis), calcification of the thymus was produced by the combined injection of dihydrotachysterol and triamcinolone, in non-inbred Sprague-Dawley and hooded, eight-week-old rats. Six days after the beginning of treatment, full-thickness skin homografts were performed on the rats.Homografts exchanged between two rats with complete calcification of the thymus cortex were accepted for an extended period of time, which in the oldest rats at the time of writing was seven months. Homografts exchanged between rats with incomplete calcification of the thymus resulted in a prolonged homograft survival with final rejection within a period of three weeks. Homografts exchanged between rats that were not treated, surgically thymectomized at the same age as the treated animals, or treated with only one of the two substances used for thymus calcification, resulted in rejection in the average time of eight days.

Allografts↗
PubMed2006

[Vascular emergencies in dermatology].

Life-threatening vascular complications of the skin are rare and usually reflect underlying systemic diseases. Lesions are sudden in onset, become necrotic and may be severe or life-threatening. Effective management requires an understanding of the underlying medical condition, such as arterial thrombosis, temporal arteritis, calciphylaxis and purpura fulminans. In contrast, the acute painful symptoms of erythromelalgia often lead to an urgent dermatologic consultation but pose no acute risk.

Acute Disease↗
PubMed1995

The strength of a calcified tissue depends in part on the molecular structure and organization of its constituent mineral crystals in their organic matrix.

High-voltage electron-microscopic tomographic (3D) studies of the ultrastructural interaction between mineral and organic matrix in a variety of calcified tissues reveal different crystal structural and organizational features in association with their respective organic matrices. In brittle or weak pathologic or ectopic calcifications, including examples of osteogenesis imperfecta, calciphylaxis, calcergy, and dermatomyositis, hydroxyapatite crystals occur in various sizes and shapes and are oriented and aligned with respect to collagen in a manner which is distinct from that found in normal calcified tissues. A model of collagen-mineral interaction is proposed which may account for the observed crystal structures and organization. The results indicate that the ultimate strength, support, and other mechanical properties provided by a calcified tissue are dependent in part upon the molecular structure and arrangement of its constituent mineral crystals within their organic matrix.

Animals↗
PubMed2004

Breast necrosis following harvest of internal mammary artery.

The use of internal mammary artery is well established not only in cardiac surgery for coronary artery bypass grafting but also in plastic surgery for breast reconstruction with free autologus tissue. The complications that follow harvest of internal mammary artery for heart surgery usually relate to the wound. This case report documents the first case of complete breast infarction following such a procedure in a patient who had no prior history of breast pathology. The histology of the breast showed extensive calcification of the intima and media of small to medium sized vessels, a condition called calciphylaxis, which is largely unknown outside the fields of nephrology and dermatology.

Breast↗