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Calcifying/ossifying synovial sarcoma shows t(X;18) with SSX2 involvement and mitochondrial calcifications.

AIMS: Synovial sarcoma with extensive calcification and ossification is a rare variant, the ultrastructural, cytogenetic and molecular analysis of which has not been reported previously. METHODS AND RESULTS: A large mass in the shoulder of a 20-year-old male patient led to a deformity of the chest wall, thus supporting the hypothesis that this is a slowly growing variant of synovial sarcoma. Nevertheless, the patient developed metastatic lung disease 7 months after resection. On histology, the monophasic spindle cell proliferation was in several areas obscured by the massive calcification and ossification. Immunohistochemistry showed keratin, epithelial membrane antigen, vimentin and CD99 expression. The cytogenetic analysis revealed a single t(X;18)(p11.2; q11.2), typical for synovial sarcoma. Additional fluorescence in-situ hybridization revealed SSX2 involvement. At the ultrastructural level, prominent needle-shaped intramitochondrial crystals were present, both in the cytoplasm and in the extracellular matrix. CONCLUSION: The presence of the t(X;18) with SSX2 involvement definitively characterizes this tumour as a variant of synovial sarcoma. In addition, the needle-like mitochondrial calcifications give a possible clue to the pathogenesis of the extensive metaplastic ossification and calcification.

Adult↗

Sevelamer attenuates the progression of coronary and aortic calcification in hemodialysis patients.

BACKGROUND: Cardiovascular disease is frequent and severe in patients with end-stage renal disease. Disorders of mineral metabolism may contribute by promoting cardiovascular calcification. METHODS: We conducted a randomized clinical trial comparing sevelamer, a non-absorbed polymer, with calcium-based phosphate binders in 200 hemodialysis patients. Study outcomes included the targeted concentrations of serum phosphorus, calcium, and intact parathyroid hormone (PTH), and calcification of the coronary arteries and thoracic aorta using a calcification score derived from electron beam tomography. RESULTS: Sevelamer and calcium provided equivalent control of serum phosphorus (end-of-study values 5.1 +/- 1.2 and 5.1 +/- 1.4 mg/dL, respectively, P = 0.33). Serum calcium concentration was significantly higher in the calcium-treated group (P = 0.002), and hypercalcemia was more common (16% vs. 5% with sevelamer, P = 0.04). More subjects in the calcium group had end-of-study intact PTH below the target of 150 to 300 pg/mL (57% vs. 30%, P = 0.001). At study completion, the median absolute calcium score in the coronary arteries and aorta increased significantly in the calcium treated subjects but not in the sevelamer-treated subjects (coronary arteries 36.6 vs. 0, P = 0.03 and aorta 75.1 vs. 0, P = 0.01, respectively). The median percent change in coronary artery (25% vs. 6%, P = 0.02) and aortic (28% vs. 5%, P = 0.02) calcium score also was significantly greater with calcium than with sevelamer. CONCLUSIONS: Compared with calcium-based phosphate binders, sevelamer is less likely to cause hypercalcemia, low levels of PTH, and progressive coronary and aortic calcification in hemodialysis patients.

Adult↗

A new neurological mutant rat with symmetrical calcification of Purkinje cells in cerebellum.

A new neurological mutant has been found in the inbred F344 strain of rats. The mutation is inherited as an autosomal recessive trait and is manifest clinically by a hesitant and wobbling gait with asynergic limbs and slight tremor. These symptoms begin at 16-18 days of age and remain essentially constant thereafter. Histologic examination revealed severe degeneration of the Purkinje cells and symmetrical calcification in these and in their dendritic branches in the cerebellar cortex. Such calcified Purkinje cells were intensely stained with the periodic acid-Schiff (PAS) method. PAS-positive substances in the Purkinje cells and extending diffusely over the lesioned sites in the molecular layer were also evident before calcification took place. We have named this neurological mutant the Cerebellar Calcification (CC) rat with the gene symbol cc. This offers a new animal model for the study of the Purkinje cell degeneration and intracranial calcification.

Animals↗

Synergistic effect of released aspirin/heparin for preventing bovine pericardial calcification.

Calcification is a frequent cause of the clinical failure of bioprosthetic heart valves fabricated from glutaraldehyde pretreated bovine pericardium (GATBP). Aspirin, a potent antiplatelet drug, and heparin, an anticoagulant, are commonly used for postimplant complications such as thrombosis and thromboembolism. Aspirin and heparin were embedded in chitosan/polyethylene vinylacetate co-matrix to develop a prolonged release form. The effect of these drugs towards the bioprosthetic calcification was investigated by in vitro and in vivo models. In vitro and in vivo evaluation suggest that the released aspirin/heparin from the co-matrix had a synergistic effect in inhibiting GATBP calcification. In vivo subcutaneous co-implantation was performed with PEG-20,000 grafted bovine pericardium (PEG-GABP), aspirin, and heparin. Biochemical, histological, and scanning electron microscopic evaluation of retrieved samples demonstrated a significant reduction in calcium deposition and alkaline phosphatase activity on PEG-GABP compared to GATBP. It seems that the aspirin/heparin combination synergistically inhibits the pericardial calcification in addition to their antithrombotic function.

Alkaline Phosphatase↗

Pathological mineralizations: calcifications and Si-bearing particles in soft tissues and their eventual relationship to different prostheses.

Polarizing microscopy (PM), scanning electron microscopy (SEM), x-ray dispersive analysis (EDAX), x-ray diffraction (XRD), and infrared spectrometry (IR) were used to study the following pathological mineralizations: calcifications and silicon(Si)-bearing mineralizations in cerebral tissue from an epileptic child; traces of Si-bearing particles in periprosthesic mammarian tissue, and calcifications in capsular mammarian tissue from a patient with a silicone gel mammarian implant, and 2 calcium-bearing compounds, a typical apatitic calcification, and a nonphosphorous-bearing calcification in arterial tissues. In this tissue we also found Si-bearing particles due to an artifact from glassware.

Aged↗

Choroidal calcifications in patients with Gitelman's syndrome.

Gitelman's syndrome is a renal tubular disorder characterized by a sodium and chloride reabsorption defect in distal tubular cells that determines hypokalemia, metabolic alkalosis, hypomagnesemia, and low calcium excretion. The presence of choroidal calcifications was sought in five patients with Gitelman's syndrome by ophthalmic examination, fluorescein angiography, indocyanine green angiography, and ocular ultrasonography. Calcifications observed in the choroid of two patients were shown by ultrasonography in both patients. Ophthalmic and fluorangiographic examinations detected this alteration in one of the two subjects. Chondrocalcinosis was found in one patient with choroidal calcifications. These findings suggest that precipitation of calcium salts can occur in the choroidal tissue of patients with Gitelman's syndrome. Deposits appeared to be well seen by ultrasonography because of their depth in ocular tissues. Sclerochoroidal calcifications may be favored by the low calcium excretion, which is associated with normal intestinal calcium absorption in patients with Gitelman's syndrome.

Adolescent↗

Changes of coronary calcification after kidney transplantation.

BACKGROUND: Coronary artery calcification (CAC) in patients with end-stage renal disease is driven by uremia and increased serum calcium and phosphate levels. Improvement in calcium-phosphate homeostasis and uremia by kidney transplantation therefore might favorably influence CAC. METHODS: We measured the extent of CAC by using multidetector computed tomography in 31 patients immediately after transplantation and at 6 and 12 months' follow-up. Baseline and follow-up measurements were compared, and the effect of atherogenic factors on CAC progression was determined by means of multivariate regression analysis. RESULTS: Mean total Agatston score increased significantly from baseline to 6 months (716 +/- 980 [SD] versus 916 +/- 1,307; P < 0.001), but remained unchanged at 12 months' follow-up (890 +/- 1,263; P = not significant). Progression of calcification was present only in patients with a baseline total Agatston score higher than 10. In these patients, the score increased from 964 +/- 1,028 to 1,234 +/- 1,385 (P < 0.001) at 6 months and remained stable thereafter (1,199 +/- 1,338; P = not significant). Duration of pretransplantation dialysis treatment and smoking were identified as independent predictors of posttransplantation CAC progression. Conversely, changes in calcium and phosphate levels were not associated with calcification. CONCLUSION: This study shows that CAC progresses during the early posttransplantation course, but slows between 6 and 12 months after transplantation. The extent of early calcification is influenced mainly by dialysis treatment duration and smoking.

Adult↗

Coronary artery calcification and chronically decreased GFR in living kidney donors.

BACKGROUND: In the presence of decreased glomerular filtration rate (GFR), the risk of morbidity and mortality caused by cardiovascular disease (CVD) is increased markedly. Increased coronary artery calcification (CAC) is proposed as a pathogenetic link between CVD and chronic kidney disease. We examined the frequency and severity of CAC in living kidney donors to test the hypothesis that decreased GFR is associated with increased CAC. METHODS: We used multidetector spiral computed tomography to examine CAC in 101 living kidney donors and 99 age- and sex-matched healthy control subjects without diabetes and a history of coronary artery disease. The extent of calcification was measured by means of the Agatston score. GFR was calculated by using the abbreviated Modification of Diet in Renal Disease formula. The frequency of risk factors for coronary artery disease was compared in kidney donors and controls, and the relation between kidney donors' clinical characteristics and the presence or absence of CAC was examined. RESULTS: CAC frequency and mean calcification scores were similar between kidney donors (13.9%; 4.5 +/- 22.6) and controls (17.2%; 13.2 +/- 89.2). CAC was not associated with decreased GFR, and the correlation between CAC and GFR was not statistically significant. Kidney donors with calcification were more likely to be older (P = 0.003) and male (P = 0.001). Age- and sex-adjusted analysis showed an association between greater parathormone levels (odds ratio, 1.023; 95% confidence interval, 1.001 to 1.045; P = 0.037) and CAC in kidney donors. CONCLUSION: A mild decrease in GFR without the presence of diabetes does not seem to be associated with increased CAC. These findings need to be confirmed in different and larger study populations.

Calcinosis↗

[Calcification of the left atrium in mitral disease].

Calcification in the wall of the left atrium is a certain sign of rheumatic heart disease; in nearly all cases it is associated with combined mitral valve disease and auricular fibrillation. The cause of the calcification is probably repeated attacks of endocarditis. Similar to the cases described in the literature, four out of five our own patients were in the end-stages of chronic rheumatic heart disease. In four patients there was clinical evidence of mitral valve disease, whereas in the fifth patient, the clinical findings suggested a ventricular septal defect. Calcification in the dilated left atrium indicated the presence of an additional mitral valve abnormality. The typical appearances of atrial calcification are illustrated. The importance of its pre-operative demonstration is discussed on the basis of the relevant literature.

Arrhythmias, Cardiac↗

[Symmetrical cortical calcification of the cerebrum following pre-natal encephalitis (author's transl)].

Purely cortical cerebral calcification without involvement of the cerebellum was observed radiologically in a 3-month old girl; the calcification was extensive and symmetrical. During a three year period of observation, she showed severe psychomotor retardation, spasticity, microcephaly, a secondary scoliosis, subluxation of the hips and fits. These changes have only been observed so far following pre-natal herpes encephalitis. Following this condition there may also be periventricular calcification. Therefore it is assumed that the extensive cortical calcification is due to a virus, but not necessarily a specific one and that it depends on the age of the foetus at the time of the infection.

Brain Diseases↗

[Spiral CT for the detection of coronary artery calcifications and the prognosis of coronary heart disease--a comparison with fluoroscopy and coronary angiography].

PURPOSE: Evaluation of clinical relevance of coronary calcifications detected by helical CT. Evaluation of congruence with fluoroscopy (FS) and coronary angiography. Comparison with studies reporting on application of ultrafast computed tomography. MATERIALS AND METHODS: 40 patients underwent helical CT (2 mm slice thickness, table-feed 3 mm/s). Coronary angiography and FS were performed in usual technique. Stenosis and calcifications were evaluated semiquantitatively. RESULTS: 28 of 28 stenoses > or = 75% were detected via helical CT by evaluation of calcification (FS: 19). One non-detected solitary stenosis was calculated as 40%. FS did not detect 4 cases with 2-vessel and 3-vessel disease each one case with 1-vessel disease, and one case with a main stem stenosis. Sensitivity of helical CT was 97% (FS: 66%). For the evaluation of stenosis III degree (> or = 75%), sensitivity was 100% (FS: 58%), specificity was 77% (FS: 48%). CONCLUSION: Helical CT is more sensitive for haemodynamically relevant stenoses than FS. Helical CT is suitable as a substantial non-invasive diagnostic procedure in coronary heart disease. In certain cases, e.g. if the patient suffers from additional risks, helical CT should be done prior to coronary angiography. Coronary calcifications found incidentally in symptomatic patients at chest CT warrant further cardiological investigation.

Adult↗

Fetal myocardial calcification associated with maternal cocaine use.

Two cases of fetal myocardial calcification confirmed postnatally are reported. In contrast to other reports, both infants survived with resolution of calcification by 6 and 12 months. Diagnostic investigations failed to confirm the presence of congenital infection. Both pregnancies were complicated by early cocaine use leading to the speculation that myocardial necrosis with subsequent calcification related to the toxic and/or vascular effects of cocaine was responsible. The finding of fetal myocardial calcification on prenatal ultrasound should prompt a search for causes, which may include cocaine exposure.

Adult↗

Cerebral calcifications and cerebellar hypoplasia in two children: clinical, radiologic and neuropathological studies--a separate neurodevelopmental entity.

Two siblings with cerebral calcifications are described, clinically characterized by the early onset of general regression and epileptic seizures, followed by cerebral blindness and spastic tetraplegia. No remarkable biochemical abnormalities were found. Death ensued before the age of seven years. Radiology of the skull in the second case showed widespread calcifications, situated periventricularly, in the basal nuclei and in the cerebellum. In both cases postmortem examination revealed widespread calcifications, corpus callosum hypoplasia and hydrocephalus "a vacuo". Moreover retrocerebellar leptomeningeal "cysts" and hypoplasia of the cerebellum were found. A diffuse lack of axons appeared to be the main cause of the poor staining of the white matter. The diagnostic criteria are neurological regression, progressive calcification, hydrocephalus and hypoplasia of the corpus callosum and cerebellum. Five similar cases were found in the literature. Our cases and those from the literature probably belong to a separate entity, originally published by Laubenthal et al (1940).

Brain Diseases↗

Infantile familial encephalopathy with cerebral calcifications and leukodystrophy.

Two sets of siblings, in two different families, presenting with congenital and progressive neurological disorders, cerebral calcifications and leukodystrophy are reported. In the first family, the diagnosis of brain calcifications in two infants was based on skull X-rays; in the second family, ultrasound scans showed hyperechoic areas in the basal ganglia and periventricular white matter in both infants. Neuropathological studies confirmed the calcifications and revealed severe abnormalities of the white matter with GFAP positive gliosis. Electron micrographs showed large astrocytes with an increased amount of glial filaments. In the group of idiopathic non arteriosclerotic cerebral calcifications, these four cases may represent a separate entity with possible autosomal recessive inheritance.

Age Factors↗

[Extraosseous calcification in juvenile dermatomyositis. The ineffectiveness of EHDP].

A now 20-year-old man was first diagnosed as having dermatomyositis when aged 12 years. At that time he had muscular weakness and heart failure followed a year later by heterotopic calcifications. For over seven years he was treated with corticoids, at times also with azathioprine or methotrexate. Nonetheless the calcifications progressed. Because of the extensive calcifications, predominantly of the extremities, he is severely disabled and has been confined to a wheel-chair. For one year he was treated with EHDP (1-hydroxyethylidenediphosphonate), up to 16 mg/dl per day. In addition he received prednisolone (10 mg/d) and azathioprine (100 mg/d). But radiologically there has been further slight progression of the calcifications, indicating that EHDP has been ineffective in this patient.

Adult↗

[Mitral ring calcification in dialysis patients. Echocardiographic diagnosis and etiological factors].

Echocardiography for assessment of presence of mitral ring calcification was performed in 96 dialysis patients. The control group consisted of 1758 consecutive patients without renal disease. For the first time it was shown that dialysis patients with mitral ring calcification (group I) had aortic valve sclerosis, renal osteopathy and peripheral angiopathy more frequently than dialysis patients without mitral ring calcification (group II). There were no significant differences between both groups for calcium, phosphate, calcium-phosphate product, magnesium, parathormone and lipids. There were equal numbers of hypertensive patients in both groups. In comparison to patients without renal disease dialysis patients had calcifications of the mitral ring more frequently (P less than or equal to 0.001) an at an earlier age.

Adolescent↗

Extraspinal tendon and ligament calcification associated with long-term therapy with etretinate.

Isotretinoin, a synthetic retinoid that has been prescribed for over 500,000 patients with cystic acne, has been associated with both spinal hyperostosis and a disorder similar to diffuse idiopathic skeletal hyperostosis. We describe a syndrome of tendon and ligament calcification, primarily in extraspinal locations, that we have observed after long-term therapy for psoriasis and disorders of keratinization with etretinate, another synthetic retinoid. Of 38 patients who had received etretinate (average dose, 0.8 mg per kilogram of body weight per day; average duration, 60 months), 32 (84 percent) had radiographic evidence of extraspinal tendon and ligament calcification. The most common sites of involvement were the ankles (29 patients [76 percent]), pelvis (20 patients [53 percent]), and knees (16 patients [42 percent]); spine involvement was uncommon in this group of etretinate-treated patients. Involvement tended to be bilateral and multifocal. Fifteen (47 percent) of the 32 affected patients had no bone or joint symptoms at the sites of radiographic abnormality. Thus, tendon and ligament calcification can occur without vertebral involvement as well as in association with it (for example, as part of the spectrum of diffuse idiopathic skeletal hyperostosis). We have identified extraspinal tendon and ligament calcification as a toxic effect that is commonly associated with long-term etretinate therapy.

Adult↗

Metastatic calcification of the nasal septum presenting as an intraoral mass: a case report with a review of the literature.

Metastatic calcification is a pathologic condition characterized by deposition of calcified product in otherwise normal tissues as a result of hyperphosphatemia with or without concurrent hypercalcemia. Metastatic calcification presenting clinically as an oral lesion is extremely rare. To date, only 7 cases of metastatic calcification involving the oral soft tissues have been described. This report describes a case of metastatic calcification of the nasal septum presenting as a mass of the anterior maxillary vestibule in a patient with end-stage renal disease. The case reported is only the second example with nasal septum involvement. A brief review of the clinical and histopathologic features of previously reported intraoral cases is also presented.

Calcinosis↗