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PubMed1982

Experimental hydroxyapatite synovial and articular cartilage calcification: light and electron microscopic studies.

Intraarticular calciphylaxis with cartilage and synovial calcification was produced in rabbits by oral administration of a single dose of dehydrotachysterol followed by an intraarticular injection of ferrous chloride. Synovial membrane apatite deposits were seen in the interstitium, on collagen fibers, and within cell vacuoles. In contrast, long-term administration of dehydrotachysterol alone induced only articular cartilage calcification. Such calcification was limited to the mid and deep zone of cartilage. Most calcium deposits were apatite-like crystals. Acute crystal-associated inflammation was not demonstrated in these specimens with crystals sequestered in synovial or cartilage tissue. Further studies on these models will examine the relation of apatite to joint disease.

Animals↗

PubMed1983

Crystal populations in human synovial fluid. Identification of apatite, octacalcium phosphate, and tricalcium phosphate.

Defatted joint fluid pellets from 4 patients with the "Milwaukee shoulder" syndrome, an aspirate of milky fluid from subcutaneous tissue of a girl with dermatomyositis/calcinosis, and 2 rabbit synovial membranes calcified by calciphylaxis were studied by Fourier transform infrared analysis. This method permits sequential electronic subtraction ("stripping") of known patterns of highly characterized reference compounds from an unknown sample pattern. Collagen and hydroxyapatite, with carbonate partially (1-3%) substituted for phosphate, and to a lesser extent hydroxyl, were found in all samples. Octacalcium phosphate (OCP) was found in all samples except that from a patient with synovial chondromatosis which contained tricalcium phosphate (TCP). Sulfate was found in 2 of the shoulder joint fluid specimens. An unidentified organic phase was present in all samples. This study confirms recent observations by others that pathologic calcifications contain several mineral phases. These data raise the possibility that hydroxyapatite nucleation may be controlled by hydrolytic alteration of precursor calcium phosphate phases such as OCP or TCP. Studies of the biologic effects of synthetic calcium phosphate crystals must take into account the existence of several crystalline phases in natural pathologic calcifications.

Animals↗

PubMed1982

Calcification of basal ganglia, postoperative hypoparathyroidism and extrapyramidal, cerebellar, pyramidal motor manifestations.

A patient is described who 32 years after thyroidectomy developed a chronic progressive syndrome due to hypoparathyroidism, with multiple extrapyramidal signs (faciobuccolingual dyskinesias, choreiform and athetotic movements of upper extremities, tremor of hands, cogwheel phenomenon), cerebellar manifestations (dysarthria, dysgraphia, mild gait ataxia), and pyramidal signs as well as an organic psychosis and epilepsy. A CT scan showed calcification of the basal ganglia. Therapy for hypoparathyroidism produced partial regression of the disorder. To explain the physiopathologic mechanism of the syndrome, Selye et al. advanced an attractive hypothesis of calciphylaxis, which assumes that a sensitizer and a challenger together induce development of calcification. In the case reported calcification involved the periventricular region and was not limited to the basal ganglia. It is suggested that either a sensitizer or a challenger may enter the periventricular tissue via the ependyma.

Aged↗

PubMed1991

Calcergy in murine progressive ankylosis.

Murine progressive ankylosis (MPA) is characterized by periarticular ossification and joint ankylosis. We studied calcergy and calciphylaxis in MPA. Calcergy represents a chemical attraction between heavy metal salts and apatite followed by hydroxyapatite deposition around collagen. Mice were injected subcutaneously with FeCl2, PbAcetate, and saline, and sacrificed at intervals between 5 minutes and 14 days. Section of skin and subcutaneous tissues were stained with Von Kossa's stain. No mice developed calcifylaxis. PbAcetate produced calcergy in all mice. MPA mice exhibited calcergy qualitatively and quantitatively the same as normal mice suggesting abnormal calcergy is not responsible for joint ankylosis in MPA.

Animals↗

PubMed1978

Mineral substance of bone tissue and of experimental cutaneous calcinosis in rats: chemical analysis and ESR study.

The evolution of the mineral constituents of subcutaneous calcinosis induced in rats by topical calciphylaxis was studied by the method of quantitative chemical analysis, and after treatment with excited gases by electron spin resonance (ESR) analysis. Chemical data show that the genesis of the subcutaneous calcinosis does not significantly alter the concentration of Ca, P, F, CO3, Mg, and Fe in the mineral phase of the femoral bone of calciphylactic rats. In the calcinosis an important increase of the fluoride concentration is noticed in function of the time after challenging. There is also a high concentration of Mg2+ ions in the early stages of the experimental calcification. Iron injected for the challenging is continuously present in the calciphylactic tissue after this treatment. This suggests that subcutaneous calcinosis might be a means of fixing certain heavy metal ions. After treatment with excited gases, the proportions of the trapped CO33- and O3- radicals are of the same order of magnitude in calciphylactic tissue after 12 days and observations in bone mineral. These suggest that after 12 days the mineral of the calciphylactic tissue has a crystalline state close to that of bone.

Animals↗

PubMed1999

[Treatment of secondary and tertiary hyperparathyroidism--surgical viewpoints].

Nearly all patients with chronic renal failure exhibit some degree of secondary hyperparathyroidism (sHPT), defined as parathyroid hyperplasia and elevated serum parathyroid hormone (PTH) levels. Despite improvements in the medical management of patients with sHPT continue to develop progressive bone disease manifested by osteitis fibrosa cystica, soft tissue calcification and myopathy, pruritus, bone and joint pain and calciphylaxis may accompany the bone disorder. When medical therapy fails, parathyroidectomy becomes necessary. This is not sufficiently explained by the failure to administer calcitriol to control serum phosphate and calcium concentration or to deliver sufficient dialysis. The continuous increase of the proportion of patients exhibiting severe uncontrolled HPT with increasing time of dialysis points to a more basic underlying biological problem; an even higher proportion of patients shows also nodular, rather than diffuse hyperplasia. It was commonly believed that after restoration of normal renal function with successful transplantation, the hyperplastic parathyroid glands would involute and return to normal function state. After renal transplantation some patients continue to have a HPT. This disease entity is recognized and termed as tertiary Hyperparathyroidism (tHPT). After establishing a diagnosis of hyperparathyroid bone disease, in patients with sHPT and tHPT a parathyroidectomy (PTX) frequently becomes necessary to decrease the mass of the hyperplastic parathyroid tissue. The surgical procedure remains controversial. Some surgeons prefer subtotal PTX, others prefer total PTX with autotransplantation of a small amount of tissue to the arm, because the transplanted tissue can be removed in the event of a recurrent HPT. Successful surgical intervention for sHPT and tHPT significantly reduces preoperative symptoms and leeds to restoration of bone disease and therefore supports PTX for patients with s and tHPT. In our experience total PTX with autograft has proven to be a satisfactory procedure. Subtotal PTX is also an effective procedure and the choice of operative technique should be left to the surgeon.

Chronic Kidney Disease-Mineral and Bone Disorder↗

PubMed1990

Calcifying panniculitis associated with polycystic kidney disease and chronic renal failure.

Tender erythematous nodules and plaques developed on the abdomen and legs of two women, 45 and 48 years of age, with hyperparathyroidism as a result of chronic renal failure caused by polycystic kidney disease. The histopathologic findings of these lesions were characterized by a calcifying panniculitis. Laboratory tests disclosed normal calcium levels and hyperphosphatemia with an elevated level of calcium phosphate product. Calciphylaxis appeared to be the mechanism of soft tissue calcification in these patients.

Calcinosis↗

PubMed1992

Sclerosing peritonitis with gross peritoneal calcification: a case report.

We report the case of a patient on dialysis for 13 years, including continuous ambulatory peritoneal dialysis (CAPD) for 11 years, who developed sclerosing peritonitis with gross peritoneal calcification. The patient first presented with abdominal pain in January 1990, when peritoneal calcification was detected for the first time. Her symptoms settled spontaneously and 1 year later she presented with acute peritonitis and adynamic ileus. The peritonitis settled with antibiotics and Tenchkoff catheter removal, but the ileus persisted. She was commenced on long-term parenteral nutrition, but never recovered useful bowel function. After 8 weeks of hemodialysis and total parenteral nutrition, a further laparotomy for an acute abdomen showed what appeared to be extensive bowel infarction and peritoneal calcification. She died several days later. Of significance, peritoneal calcification was first noted on x-ray and computed tomography (CT) scan while the patient was still largely asymptomatic and before peritoneal ultrafiltration capacity was significantly impaired. Unlike other reported cases of calcifying peritonitis, sclerosing peritonitis was present and calcification was far more extensive. It was not associated with factors such as frequent infective peritonitis or acetate dialysate. Calciphylaxis was not present nor was there any abnormality of calcium-phosphate metabolism. The outcome of this case suggests that patients with recurrent or persistent bowel symptoms on long-term CAPD should have early abdominal x-ray or CT scanning to exclude sclerosing peritonitis or bowel calcification. If present, consideration should be given to transferring the patient to another therapeutic dialysis modality if possible.

Adult↗

PubMed1997

Other forms of vasculitis and pseudovasculitis.

Behçet's syndrome can involve all sizes and kinds of blood vessels. There is an association between arterial involvement and venous thrombosis. Pulmonary arterial aneurysms and neurological involvement have a definite influence on mortality. Male sex and young age are indicators of a more severe disease course. Immunosuppressive treatment early in the disease may affect the long term prognosis favourably. Patients with familial Mediterranean fever may develop manifestations of vasculitis. The most common associations are with Schönlein-Henoch purpura and polyarteritis nodosa. In some patients the diagnosis of vasculitis precedes that of familial Mediterranean fever. Kawasaki disease, although rare, can be seen in adults. The coronary sequela of childhood disease can affect the prognosis later in life. Many conditions, like myxoma, cholesterol embolism, calciphylaxis may mimic vasculitic syndromes. These conditions should always be kept in mind because their pathophysiology and treatment are different from true vasculitides.

Animals↗

PubMedDate not supplied

Regulation of cardiovascular calcification.

Vascular calcification is highly correlated with cardiovascular disease (CVD) and is a significant predictor of cardiovascular events, especially in high risk patients such as the end stage renal disease (ESRD) population. Vascular calcification can lead to serious problems including valve stenosis, decreased vascular compliance, calciphylaxis, and even sudden death. However, the contribution of vascular calcification to progression of atherosclerosis is unknown and needs more study. Biochemical, histological, and genetic studies indicate that vascular calcification is actively regulated and involves both positive and negative modulators. Several nonmutually exclusive theories to account for vascular calcification based on current studies are discussed.

Animals↗

PubMed1996

Panniculitis: recent developments and observations.

Dermatopathologists rarely greet a biopsy of panniculitis with total confidence that a specific, definitive diagnosis will be rendered. As with many other areas in dermatopathology, our understanding of the pathogenesis of many forms of panniculitis is incomplete. This article examines a subset of panniculitis primarily from a pathogenetic standpoint, with the intention of providing a differential diagnosis for those cases in which ischemic changes are seen in the subcutis. The diverse group of conditions evoked by this approach also shares the distinction of having been the focus of nosologic and causative controversy, both historically and currently. In particular, stasis-associated sclerosing panniculitis, vascular calcification-cutaneous necrosis syndrome (calciphylaxis), oxalosis, and nodular vasculitis-erythema induratum are examined in depth. Erythema nodosum and variants, other granulomatous panniculitides, and panniculitides showing cytophagocytosis are also discussed with current perspectives.

Diagnosis, Differential↗

PubMed2001

Secondary hyperparathyroidism in chronic renal failure: pathogenic and clinical aspects.

Secondary hyperparathyroidism occurs early in the course of chronic renal failure. Early in the course, a deficit of calcitriol and an abnormality in the calcium sensor receptor may be the important factors; later, with advanced renal failure, hyperphosphatemia becomes an additional important pathogenic factor. Important clinical problems in dialysis patients are as follows: (1) hyperphosphatemia, which contributes to high morbidity and mortality of dialysis patients (a clinical approach to maintaining a normal serum phosphorus is essential and is discussed in this review); (2) a high calcium X phosphorus product leading to coronary artery calcifications (factors leading to calcifications are also discussed); and (3) calciphylaxis, which has been observed with increasing frequency in these patients (an increase in the total calcium load may be the most important pathogenic factor leading to this syndrome). Therapeutic considerations regarding the use of new vitamin D analogues devoid of a hypercalcemic and/or a hyperphosphatemic effect are also mentioned.

Animals↗

PubMed2001

Panniculitis. Part II. Mostly lobular panniculitis.

UNLABELLED: The second part of our review of panniculitis summarizes the clinicopathologic features of the mostly lobular panniculitides. Erythema induratum of Bazin (nodular vasculitis) represents the most common variant of lobular panniculitis with vasculitis, although controversy persists about the nature of the involved vessels. Mostly lobular panniculitides without vasculitis comprise a series of disparate disorders. These include sclerosing panniculitis that results from chronic venous insufficiency of the lower extremities; panniculitis with calcification of the vessel walls such as calciphylaxis and oxalosis; and inflammatory diseases with crystals within the adipocytes such as sclerema neonatorum, subcutaneous fat necrosis of the newborn, and poststeroid panniculitis. Connective tissue diseases, such as systemic lupus erythematosus and dermatomyositis, pancreatic diseases, and alpha(1)-antitrypsin deficiency may also show a mostly lobular panniculitis with characteristic histopathologic features. Lobular panniculitis may also be an expression of infections, trauma, or factitial causes involving the subcutaneous fat. Lipoatrophy refers to a loss of subcutaneous fat due to a previous inflammatory process involving the subcutis, and it may be the late-stage lesion of several types of panniculitis. In contrast, lipodystrophy means an absence of subcutaneous fat with no evidence of inflammation and often the process is associated with endocrinologic, metabolic, or autoimmune diseases. Finally, cytophagic histiocytic panniculitis is the term that has been used to describe two different processes: one is inflammatory, a lobular panniculitis, and the other one is neoplastic, a subcutaneous T-cell lymphoma. The only common feature of these two different processes is the presence of cytophagocytosis in the lesions. (J Am Acad Dermatol 2001;45:325-61.) LEARNING OBJECTIVE: At the completion of this learning activity, participants should be familiar with the pathogenesis, clinical manifestations, histopathologic findings, and treatment options for the most frequent variants of the lobular panniculitides.

Humans↗

PubMed2003

Primary hyperoxaluria in a 27-year-old woman.

Primary hyperoxaluria is a rare autosomal recessive disorder resulting in precipitation of insoluble oxalate crystals in the joints, kidneys, heart, eyes, and skin. Two thirds of patients have calcium oxalate nephrolithiasis by age 5 years and 80% die of renal failure by age 20 years. Rarely, the disease will present in adulthood, with the onset of symptoms occurring as late as the sixth decade. We present a 27-year-old woman with end-stage renal disease who presented to the dermatology department for the evaluation of a reticular rash shortly after beginning peritoneal dialysis. Associated symptoms included arthralgias and episodic acral cyanosis. Previous kidney and skin biopsy specimens revealed crystalline deposition, however, the diagnosis of primary hyperoxaluria was not entertained until an atrial mass was found to have the same crystalline material. This report reviews primary hyperoxaluria and underscores the importance of recognizing the disease as a cause of renal failure in a patient with livedo reticularis and skin lesions resembling calciphylaxis. Early recognition of the disease is important because combined liver-kidney transplantation may achieve long-term survival.

Adult↗

PubMed1981

Finger gangrene caused by small artery occlusive disease.

Available evidence indicates that about one third of all patients presenting with localized finger gangrene developed the condition due to intrinsic occlusions of the small arteries of the hand and fingers caused by one of a variety of systemic diseases. We have treated 35 such patients in the past seven years. A variety of diagnostic tests allowed the establishment of the diagnosis of connective tissue disease in 14 patients, hypersensitivity angiitis in 13 patients, arteriosclerosis in five patients, and myeloid metaplasia, calciphylaxis, and carcinoma in one patient each. Treatment with cold and tobacco avoidance, vasodilators, and local debridement produced good results without amputation in 30 patients. Five patients required partial phalangeal amputation. These results suggest that appropriate diagnostic tests will allow an accurate diagnosis in all patients, and that the natural history is that of spontaneous improvement without major tissue loss. In our experience, surgical sympathectomy plays no role in the treatment of these patients.

Adolescent↗

PubMed2003

Musculoskeletal manifestations of chronic renal failure.

Musculoskeletal problems remain among the main limitations of the quality of life of renal failure patients, in particular of those treated with long-term maintenance dialysis. Renal osteodystrophy continues to receive great attention. The mechanisms of uremic skeletal resistance to parathormone (PTH) are further investigated. The assay used for the dosage of "intact PTH" has been found to detect 7-84 fragments with an inhibitory effect on the action of the whole hormone. A decrease in the density of PTH receptor on osteoblasts is another recently evidenced factor. Investigations of the recently described RANK-RANKL system have demonstrated an increase in serum osteprotegerin levels, which, together with the two above-mentioned abnormalities, may explain bone resistance to PTH. These are important advances in the understanding of renal osteodystrophy as skeletal resistance to PTH appears to play an important part in the pathophysiology of secondary hyperparathyroidism and of adynamic bone disease. Because of this skeletal resistance, it has been recommended for several years that serum PTH level be monitored and kept twofold to threefold above the upper value of the normal level to maintain normal bone turnover in dialysis patients. Relative hypoparathyroidism has recently been found to be associated with increased spontaneous fracture rate and mortality, so this recommendation appears to hold adequate, despite the demonstration that serum PTH levels in this range are a poor predictor of bone turnover and that chronic parathyroid gland hyperplasia is likely to favor parathyroid gland autonomization. Recent publications have insisted on the role that hyperphosphatemia plays not only in the development of secondary hyperparathyroidism, but also of vascular, especially coronary, calcification and as a predictor of mortality. This "silent killer" of uremic patients is one of the main targets for therapeutic intervention. Extensive use of calcium-containing phosphate binders has been recently criticized as calcium overload appears to favor vascular calcification. Sevelaner (RenaGel) is a calcium- and aluminum-free phosphate binder that is an important advance in the management of renal osteodystrophy, especially in patients with extraskeletal calcification and hypercalcemia. The use of vitamin D derivatives has also raised concern because they enhance calcium and phosphorus absorption and reduce bone turnover. New metabolites with fewer hypercalcemic effects have been developed. Calcium-sensing receptor agonists are stimulating interest and are likely to take an important place in the future management of renal osteodystrophy. Uremic myopathy has received recent attention. Impaired muscle capillary oxygen transfer has been identified as a pathophysiologic factor, and progressive resistance training has been shown to improve the condition. Finally, a new entity, nephrogenic fibrosing dermopathy, has been described, which must be distinguished from calciphylaxis and scleromyxedema.

Clinical Trials as Topic↗

PubMedDate not supplied

Acute hypercalcemia of the heart ("bony heart").

Cardiac abnormalities as a sign of hyperparathyroidism are common. A spectacular pitfall of peracute extended myocardiac hypercalcemia is reported. The history of a 30-year-old woman included symptoms such as insufficiency of the kidneys since childhood, secondary hyperparathyroidism, and hemodialysis for approximately 4 years. After kidney transplantation, the patient died from progressive heart failure. Three days before she died, CT showed a nearly white heart, and myocardial scintigraphy revealed a total infarction. The autopsy revealed a heart of normal size but with a weight of 590 g and with nearly bony texture. The histologic examination showed extended calcifications of the entire myocardium, thus explaining these findings. Laboratory photographs and electron microscopic images will be demonstrated. The metabolic pathogenesis of tertiary hyperparathyroidism and calciphylaxis is discussed. "Malignant" progression after kidney transplantation is stressed.

Acute Disease↗

PubMed2001

Consequences of hyperphosphatemia and elevated levels of the calcium-phosphorus product in dialysis patients.

Control of serum phosphorus levels is a central goal in the management of patients with chronic renal failure. Inadequate control of serum phosphorus leads to elevated levels of the calcium-phosphorus product. This plays a pivotal role in vascular calcification, cardiovascular disease, calciphylaxis, and death. Elevated phosphorus and elevated levels of the calcium-phosphorus product are both significant predictors of cardiovascular mortality, at phosphorus and calcium-phosphorus product levels that were considered safe until recently. A lowering of levels such that phosphorus is maintained between 2.2 and 5.5 mg/dl, calcium-phosphorus product is below 55 mg(2)/dl(2), and serum calcium is at 9.2-9.6 mg/dl, respectively, might well be the goal of therapeutic management strategies.

Bone and Bones↗