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[Tumorous calcinosis in hemodialysis: anatomo-clinical study apropos of 3 cases].

Three patients (2 females, 1 male) with a mean age of 51.4 years receiving long term hemodialysis affected by tumoral calcinosis were analysed. Clinical, radiological and pathological features were evaluated and pathogenic were reviewed. The joints involved int the cases presented in this report were the hip shoulder and finger. The lesions were bilateral in shoulder. An increased calcium-phosphorus product (Ca x P) was observed in all patients with secondary hyperparathyroidism in one case. Surgery was carried out in all patients. No relapse of the tumoral calcinosis was observed after surgery. The most important pathogenic factor involved in uremic tumoral calcinosis is an increase in calcium-phosphorus product (Ca x P) not necessarily related to hyperparathyroidism. Therefore, maintaining the calcium x phosphate product within the normal range appears to be the most important factor ito prevent the appearance of uraemic tumoral calcinosis.

Adult↗

Dystrophic cardiac calcinosis in mice: genetic, hormonal, and dietary influences.

Mice of five inbred strains (BALB/c, C3H, C3Hf, DBA/2, and C57BL/6) of both sexes, mated and virginal, were examined for the incidence, severity, and location of dystrophic cardiac calcinosis (DCC) at various ages. Three hybrids, B6C3F1, C3B6F1, and CC3F1 of both sexes, all mated, were likewise studied. Excepting DBA/2, females of the inbred strains acquired the lesion at a much earlier age than males; DCC appeared in young DBA/s mice of both sexes. DCC in BALB/c mice was almost exclusively epicardial and occurred with equal frequency and severity in mated males and females, with higher incidence but lesser extent in virginal females. The occurrence was highest, the degree most severe, and the location exclusively myocardial in C3H and C3Hf mated females, irrespective of parity, whereas virginal females of these strains were entirely free of disease even after administration of exogenous progesterone. Involvement of males, also myocardial, was relatively minimal, especially in C3Hf mice. Over half the DBA/2 mice were affected, regardless of sex or mating; calcinosis appeared in the epicardium and/or myocardium, predominantly in the myocardium. Strain C57BL/6 was completely devoid of the lesion, as were the two hybrids thereof, B6C3F1 and C3B6F1. The hybrid of BALB/c and C3H showed a high incidence of minimal involvement, exclusively myocardial and limited to breeding females, indicating dominance of the C3H gene(s). Renal calcinosis was uncommon among BALB/c mice but was frequently found in C3H, C3Hf, and DBA/2 strains. Pulmonary calcinosis was rare and limited to C3H and C3Hf female breeders. Mated C3H females fed increasing amounts of fat showed a concomitant rise in incidence and severity of the cardiac lesions. Progression of the lesion from necrotic myocardial fibers to fibrocalcific masses is illustrated, as is formation of the renal deposits.

Aging↗

[Beneficial effect of high-dose etidronate on periarticular calcinosis associated with arterial and periarticular basic calcium phosphate (BCP) crystal deposition disease].

Basic calcium phosphate (BCP) deposition disease is a crystal-induced inflammation syndrome. We report a beneficial effect of high-dose etidronate, one of the bisphosphonates, on periarticular calcinosis in a case of arterial and periarticular BCP crystal deposition disease. A 55-year-old woman was referred to St. Marianna University Hospital because of a 30-year history of recurrent acute periarthritis. The X-ray photographs revealed periarticular calcinosis of the hand and foot, wrist, and knee joints without bony erosions and the calcification of carotid and popliteal arteries. The biopsied specimen from periarticular tissue showed Alizalin-red and von Kossa staining-positive crystals. With the diagnosis of BCP crystal-induced periarthritis, she has been treated with colchicine and probenecid. This regimen was partially effective in terms of a decrease in the frequency of periarthritic attack. Despite the treatment, periarticular calcinosis increased in size and number. She was treated with 800 mg of etidronate 3 months after the etidronate therapy; periarticular calcinosis was diminished although arterial calcification was unchanged. This case suggests that high-dose etidronate might be useful for heterotopic calcification associated with rheumatic diseases.

Calcinosis↗

Tumoral calcinosis in infants: a report of three cases and review of the literature.

Tumoral calcinosis is the deposition of calcium crystals and salts in the periarticular soft tissues. It commonly is seen as a complication of renal dialysis or in patients with a hereditary disposition. Occasionally, it is seen as an isolated condition with no known cause. Tumoral calcinosis usually affects adults and rarely, children. This disorder is extremely uncommon in infants. Only 13 cases have been reported. In this study three additional infants with tumoral calcinosis are reported, bringing the total to 16 cases. These 16 cases provide the opportunity to compare tumoral calcinosis in infants (younger than 18 months) with the disease in older patients. Although histologic and radiologic features in infants and adults are identical, some clinical features differ. In almost all infants, the disease develops without any predisposing factors, such as a family history. Surgical removal of lesions in infants is curative. By contrast, lesions in adults tend to recur after surgery.

Bone Neoplasms↗

Effect of ethane-I-hydroxy-I, I-diphosphonate on arterial calcinosis induced by hypervitaminosis D: a morphologic investigation.

The present study was undertaken to examine changes in vascular ultrastructure of rats subjected to hypervitaminosis D with or without treatment with ethane-I-hydroxy-I, I-diphosphonate (EHDP). Five groups of rats were studied. Untreated rats were given 0.9% NaCl i.p. Sham-treated rats were given vehicle (corn oil). Treated rats were given ergocalciferol (75,000 IU i.p.) dissolved in vehicle with or without EHDP (5 mM/100 g body-weight i.p.). Rats which had been given ergocalciferol without EHDP developed hypercalcemia and demonstrated significant arterial calcinosis. A similar degree of calcinosis was not observed in rats given ergocalciferol with EHDP. EHDP appeared to inhibit arterial calcinosis; however, it did not affect plasma calcium levels. This suggests that EHDP might delay calcium influx into the cell and thereby prevent calcium overload. Our findings support the suggestion that EHDP therapy can be an effective treatment for the inhibition of dystrophic arterial calcinosis.

Animals↗

Tumoral calcinosis: seasonal biochemical studies and chemical studies of eyelid lesion.

We recently described (Arch Ophthalmol 1988; 106:725-6) the presence of unique calcific lesions in the eyelids of a young woman with a history of hyperphosphatemic tumoral calcinosis. Here we document that no immediate family members showed similar lesions and that none was hyperphosphatemic. Dental roentgenography revealed characteristic abnormalities in the patient that confirmed the clinical diagnosis of tumoral calcinosis. Seasonal biochemical studies demonstrated persistently increased concentrations of phosphorus and 1,25-dihydroxyvitamin D in her serum. A calcific eyelid excrescence removed from the patient, studied by x-ray diffraction, was found to consist of crystals of hydroxyapatite. Microprobe analysis indicated the major elements in the deposit to be Ca, P, S, and Cl, just as in the periarticular deposits found in tumoral calcinosis. The Ca concentration in the patient's tear fluid, measured by atomic absorption spectrometry, was within the range found in tears of healthy volunteers. Phosphorus was undetectable (less than 30 mumol/L) in tears of the patient and the volunteers. These findings suggest that the eyelid lesions represent a new manifestation of the pathological process that produces the characteristic periarticular calcific masses of tumoral calcinosis.

Adolescent↗

[Calcinosis of the scrotum].

A critical review of the present classification of the calcinosis cutis is done and the difficulties to make it more precise are pointed out: it is hard to put together diseases totally different that have in common only calcium deposits in the skin; there is a lot of confusion in the current terminology; some authors don't recognize certain entities and; in a few times their own definitions are not respected. A cause of scrotal calcinosis (SC) secondary to epidermoid cysts calcification is presented. The authors believe that many cases classified as idiopathic calcinosis of the scrotum can be in reality late stages of a dystrophic calcification. They suggest that serial cuts in the histopathology examination of every lesion of SC should be performed in order to establish the existence or not of any epithelial remnant. Considering the fact that the treatment is surgical and mostly due to esthetic reasons, they also suggest to follow, if possible, a significant number of patients from an early to a late stage of the disease; only in this way it will be possible to know about the natural history of the scrotal calcinosis.

Adult↗

Ectopic calcinosis possibly due to 1 alpha (OH) vitamin D3 in a patient with systemic lupus erythematosus.

A 30-year-old woman with systemic lupus erythematosus (SLE) developed ectopic calcinosis. She had been receiving prednisolone since 1980 with the addition of vitamin D3 in 1986. Despite this therapy, her renal function had gradually deteriorated. Right gonalgia was noted in September 1991. X-ray findings revealed calcinosis of the arteries of the femur, poplitea, cubitus, hands, and feet. Her finger pads and joint sacs were also involved. Calcinosis seen in SLE has only rarely been reported, and that observed in association with vitamin D intoxication or arteriosclerosis has a different distribution of calcium deposits. The use of vitamin D3 in our patient with renal disability may have induced calcinosis with a unique distribution.

Adult↗

The effects of warfarin on calcinosis in a patient with systemic sclerosis.

We describe the use of low doses of warfarin to treat calcinosis in a patient with systemic sclerosis or CREST syndrome. Our patient had Raynaud's phenomenon, skin sclerosis of the neck and the distal surface of the elbows, and pitting ulcers and scarification of the fingers as well as cutaneous calcinosis. After beginning warfarin, no calcium containing substance was discharged from the fingertip ulcers. There was no tendency to bleed and activated partial thromboplastin time and prothrombin time were normal. Sequential radiographs of the hands showed that calcinosis had improved. Since there seem to be few adverse effects, the use of warfarin in patients with calcinosis warrants further study.

Calcinosis↗

Fulminant metastatic calcinosis with cutaneous necrosis in a child with end-stage renal disease and tertiary hyperparathyroidism.

Metastatic calcinosis is a common feature of chronic renal failure. Its first manifestations are bone demineralization and non-visceral and/or visceral calcification with mostly mural deposits in arteries and arterioles. It is initially characterized by hyperphosphataemia followed by secondary or tertiary hyperparathyroidism. Cutaneous involvement is a rare complication. Histologically, the lesions show vascular calcification with ischaemic skin necrosis. Extreme cases may produce calcinosis cutis (calciphylaxis), i.e. disseminated calcification of the subcutaneous tissue and dermis in the form of hard painful cutaneous nodules and plaques with subsequent ulceration. Metastatic calcinosis is a disease affecting adults, while the dystrophic or idiopathic type can develop in children. We present the case of a 6-year-old boy with end-stage renal disease, attributed to congenital renal hypoplasia, and accompanied by secondary hyperparathyroidism. He developed fulminant tertiary hyperparathyroidism and metastatic calcinosis of the lungs, as well as cutaneous necrosis of the buttocks and legs, subsequent to calcification of arteries and arterioles. A maternal renal transplant failed to function. The serum parathormone, calcium and phosphate levels could not be controlled by maintenance dialysis, phosphate binders and calcitriol. Total parathyroidectomy without autotransplantation of parathyroid tissue rapidly returned the serum parathormone, calcium and phosphate levels to normal. In addition, topical treatment using merbromine solution and hydrocolloid dressings, healed the ulcers with significant scar formation, within 2.5 months after parathyroidectomy. A renewed increase of the calcium x phosphate product, 2 months after parathyroidectomy, was attributed to mobilization of calcium compounds from the viscera, as confirmed by a chest X-ray.

Calcinosis↗

Periarticular ectopic calcinosis probably due to 1 alpha-OH-vitamin D3 therapy, and successful treatment with bisphosphonate compound in a patient with systemic lupus erythematosus.

We describe the case of a 25-year-old woman who developed soft tissue ectopic nephritis. Ectopic calcinosis rarely occurs in systemic lupus erythematosus (SLE) patients. This is the first detailed case report of metastatic ectopic calcinosis, one of two categories of ectopic calcinosis, probably due to 1 alpha-OH-vitamin D3 therapy. We administered disodium 3-amino-1-hydroxypropylidene-1, 1-bisphosphonate pentahydrate, a second-generation bisphosphonate, to decrease the patient's serum calcium level, and subsequently observed a dramatic decrease in severity of the ectopic calcinosis along with decreases in both the serum calcium level and the (serum calcium level)x(serum phosphate level) index. We suggest that 1 alpha-OH vitamin D3 should be used in cases of lupus nephritis with great caution.

Adult↗

[Non-metastatic calcinosis localized in the prepuce. Clinicopathological study of a case and review of the literature].

Penis calcinosis is a rare pathology and only two previous cases have been reported in literature. We describe the clinicopathologic features of a case of nodular foreskin calcinosis in a 25-year-old man. The patient's history resulted negative for local trauma, inflammatory disorders or metabolic diseases. The mass measured up to 2 cm and was histologically constituted by multiple intradermic calcium deposits, whose deepest ones were surrounded by epithelioid histiocytes and multinucleated giant cells, with no evidence of any epithelial structures around none of them. These features were consistent with a non-metastatic calcinosis, likely idiopathic, even though also dystrophic calcinosis, observed at its end-stage, may show the same microscopic aspect. The exact idiopathic/dystrophic nosology is briefly discussed.

Adult↗

[Perforating milia-like idiopathic calcinosis of the extremities in a patient with Down syndrome].

INTRODUCTION: In 1989 a new type of calcinosis cutis has been described in association with Down's syndrome. This is the milia-like idiopathic calcinosis cutis, which is characterized by milia-like papulae generally located on the limbs (especially hands and feet) and sometimes associated with syringomas around lesions or on the eyelids. OBSERVATION: A 6 year old trisomic girl had about ten round shaped hard white-yellowish papules with a diameter of 2-3 mm on both palms of her hands. The biological balance and immunologic tests gave normal values. The histopathologic pattern was compatible with calcinosis cutis circumscripta associated with the transepidermal elimination phenomenon. Calcified sweat ducts were not observed at the von Kossa staining. Moreover, histology did not evidence any syringomas around the lesions. DISCUSSION: Our observation does not sustain the presently more spread pathogenetic interpretation, according to which eccrine ductal structures could have an active role in the formation of calcium deposits, since histology did not show any calcified eccrine ducts. Therefore, in our opinion, milia-like calcinosis associated with Down's syndrome should be classified among the idiopathic forms.

Calcinosis↗

Scrotal calcinosis. Dystrophic calcification of epidermoid cysts.

Scrotal calcinosis, consisting of solitary or multiple circumscribed deposits of calcium within the dermis of the scrotum, has generally been assumed to be idiopathic. We have recently examined three patients in whom scrotal epidermoid cysts in varying stages of inflammation coexisted with scrotal calcinosis. Some cysts showed calcification of their keratin contents with little evidence of active inflammation. Other cysts showed rupture of their epithelial walls associated with the presence of keratin fibers, granulomatous inflammation, and calcium granules in the surrounding dermis. It seems that so-called idiopathic scrotal calcinosis may represent the end stage of dystrophic calcification associated with the inflammation of scrotal epidermoid cysts.

Adult↗

Fine-needle aspiration cytology of soft-tissue calcinosis presenting as an enlarging mass in the chest wall.

Calcinosis cutis is an uncommon condition, and calcific deposits in patients with end-stage renal disease are now somewhat rare, due to improvement in management. Since calcific deposits may clinically resemble a tumor, it is feasible to investigate them by fine-needle aspiration cytology (FNAC). A 52-yr-old male with a history of end-stage renal failure presented with a 2-cm mass in the left chest wall. Within 6 mo the mass enlarged to nearly four times its original size. In view of clinical suspicion of malignancy, FNAC was performed. Cytopreparations showed abundant calcium, indicative of soft-tissue calcinosis. The case is interesting, since the known history of end-stage renal failure enabled the FNAC diagnosis of soft-tissue calcinosis.

Biopsy, Needle↗

Calcinosis cutis: diagnosis by aspiration cytology--a case report.

Calcinosis cutis is characterized by the deposition of calcium salts in the subcutaneous tissues of the body. Metastatic calcifications can occur in the body in hyperparathyroidism and end-stage renal disease. Calcifications can also occur in a variety of other clinical settings and can be subjected to fine-needle aspiration (FNA). Calcinosis cutis was diagnosed by FNA in a 20-yr-old male who presented with a solitary subcutaneous nodule near the ankle, on the lateral malleolus. Smears showed amorphous granular material consistent with calcium, and occasional histiocytes. The presence of amorphous calcium salts along with histiocytes in the appropriate clinical setting is diagnostic of calcinosis cutis. The diagnosis was confirmed on histology. Diagn. Cytopathol. 1999;21:200-202.

Adult↗

Surgical management of calcinosis cutis universalis in systemic lupus erythematosus.

Calcinosis cutis is common in several connective tissue diseases but rare in systemic lupus erythematosus (SLE). A 43-year-old woman with a 12-year history of SLE who presented with calcinosis cutis at the time of SLE diagnosis developed a large, ulcerated, draining mass on her left hip. The lesion was excised and skin was grafted, with an excellent early result. The clinical variants and mechanisms of ectopic calcification are discussed, as well as the proposed medical therapies for calcinosis cutis.

Adult↗

Tumoral calcinosis-like lesion of the proximal linea aspera.

Tumoral calcinosis is presently a poorly defined disease. In its classic form, it consists of multiple large foci of benign mineralization in the soft tissue adjacent to bone near large joints. Patients are generally of African descent and are adolescents or young adults at presentation. Both metabolic and traumatic etiologies have been proposed. We report six adult Caucasian patients with lesions that pathologically resembled tumoral calcinosis. All lesions were small (less than 3 x 3 cm) and were located along the proximal linea aspera of the femur. All patients presented with pain. Because of the atypical patient population and the unusual size and location of the lesions, we refer to this process as a "tumoral calcinosis-like lesion." A typical radiographic appearance and location, together with appropriate clinical history, can strongly suggest this diagnosis.

Aged↗