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Juxta-articular erosions and calcifications in patients with chronic renal failure.

The frequency of subperiosteal erosions and soft tissue calcifications was found to be about 36 and 33 per cent, respectively, in a series of 241 patients with chronic renal failure. In patients on chronic hemodialysis and in renal transplant recipients, subperiosteal juxta-articular erosions combined with soft tissue calcifications were observed in the same localization in 53 joints, most commonly in small joints of the hands. The calcifications appeared before the erosions, suggesting that the calcifications induced the erosions.

Adolescent↗

Children with irreversible brain damage associated with hypothyroidism and multiple intracranial calcifications.

Children who develop clinical hypothyroidism in early childhood have various degrees of irreversible brain damage, albeit less severe than cases detected by neonatal screening test for hypothyroidism in the first months of the life. We report three patients with hypothyroidism of childhood onset after a normal neonatal thyroid-stimulating hormone screening who showed deceleration in linear growth, spasticity in the lower limbs with deformity, mild intellectual impairment, and multiple calcifications in the basal ganglia and subcortical areas. The neurologic symptoms were not progressive but were irreversible in spite of thyroxine treatment. Motor disturbances commonly observed in postnatal-onset hypothyroidism are similar to those of cerebral palsy. Specific distribution of intracranial calcifications may result from metabolic derangement as a result of hypothyroidism, although the mechanism of calcification is not fully understood. We emphasize the need to re-evaluate thyroid function in diplegic patients with specific intracranial calcifications but normal neonatal thyroid-stimulating hormone screening.

Basal Ganglia Diseases↗

Effects of double cross-linking technique on the enzymatic degradation and calcification of bovine pericardia.

The strength, resorption rates, and biocompatibility of collagenous biomaterials are profoundly influenced by the method of cross-linking. The in vitro and in vivo calcification and enzymatic degradation of bovine pericardia (BP) after a series of surface modifications were studied as a function of exposure time. Collagenase degradations of modified BP were monitored by scanning electron microscopy and tensile strength measurements. Bovine pericardium was modified by a combination of different tissue fixatives such as glutaraldehyde (GA), carbodiimide (EDC), diisocyanate (HMDIC), and polyethylene glycol (PEG). GA-PEG-EDC-PEG and GA-PEG-HMDIC-PEG combination treated BP retained maximum stability in collagenase digestion compared to GATBP. In vitro calcification studies and in vivo rat subcutaneous implantations of modified pericardium have shown substantial reduction in the calcification of double cross-linked BP with PEG modification. Further, the biocompatibility aspects of pericardial tissues were established by platelet adhesion and octane contact angle. It seems that cross-links involving amino and carboxyl residues may provide new ways of controlling biodegradation and calcification.

Animals↗

Myocardial calcification as a rare cause of congestive heart failure: a case report.

Myocardial calcification is a manifestation of either metastatic or dystrophic calcium deposition in the myocardium. Dystrophic calcification of the myocardium is most commonly seen in long-term survivors of substantial myocardial infarctions. Current literature has reported only 3 cases of myocardial calcification with normal coronary arteries. We present a case of an 80-year-old woman with multiple admissions over a 5-year period for congestive heart failure. She was found to have a normal left ventricular ejection fraction and normal coronary arteries on left heart catheterization. A high resolution computed tomography (CT) study of the chest revealed extensive left ventricular myocardial calcifications, which were not present 4 years earlier on CT. The patient's history and clinical presentation revealed no etiologic factors for her calcified myocardium.

Aged, 80 and over↗

Is tear calcium an indicator of ocular calcification in patients with chronic renal failure?

BACKGROUND: In patients with chronic renal failure (CRF) on regular dialysis treatment, limboconjunctival degeneration and calcifications are common. The mechanisms of ectopic conjunctival and corneal calcification remain largely speculative. The aim of the present work was to study tear calcium levels in patients with chronic renal failure, which could alter calcium excretion patterns, and to examine whether the calcium level in tear fluid is of more diagnostic importance than static calcium levels in blood. METHODS: Tear calcium levels were measured in 25 patients with chronic renal failure, compared with 14 normal subjects. Conjunctival and corneal calcium deposits were graded by the Porter and Crombie classification. RESULTS: While 11 of the 25 patients had no clinically apparent calcium deposits, six had Grade 1 or 2, and eight had Grade 3 and over limboconjunctival calcification. Mean tear calcium levels were 1.436 +/- 0.165 mg/dl in the patients and 1.307 +/- 0.155 mg/dl in the normal group. The difference was not significant (P = 0.572). No correlation was found between the grade of the calcium deposits and tear calcium levels. CONCLUSIONS: Tear calcium level has no diagnostic importance for the development of ocular calcification in chronic renal failure.

Adult↗

Calcification and proteoglycan accumulation following balloon angioplasty and stenting.

PURPOSE: To examine in a porcine model if a correlation exists between calcification and the hyperplastic response of arteries to balloon angioplasty and stenting. METHODS: Eleven Sinclair miniature swine on an atherogenic diet underwent balloon angioplasty or endovascular stenting in nondiseased external iliac arteries using standard procedures. Ninety days postoperatively, the animals were euthanized, and histological sections of the dilated, stented, and control arteries were examined for evidence of calcification and increased proteoglycan accumulation. RESULTS: An increase in proteoglycan accumulation and the loss of integrity of the internal elastic lamina were observed in both the dilated and stented arteries. Diffuse calcification was seen in the media/neomedia of both groups, and large calcium salt deposits were observed in the dilated arteries at the site of the internal elastic lamina and near the stent struts in stented arteries. CONCLUSIONS: Internal elastic lamina loss of integrity appears to be related to an increase in proteoglycans through the formation of a neointima, and the arterial hyperplastic response to balloon angioplasty and stenting seems to lead to calcification.

Angioplasty, Balloon↗

Graft calcifications and dysfunction following liver transplantation.

BACKGROUND: The molecular events, following ischemia and reperfusion (I/R) of the liver during transplantation are largely unknown. There is evidence that apoptotic and necrotic events may take place, and occasionally result in primary graft dysfunction. We herein report two cases, where significant I/R injury correlated with the development of liver calcification and primary liver dysfunction. CASE PRESENTATION: Both patients with clinical and biochemical evidence of primary graft dysfunction demonstrated calcification at light and electron microscopy levels. In addition, one patient had macroscopic evidence of calcification on cross-sectional imaging. Both patients died secondary to the sequelae of the graft dysfunction. CONCLUSIONS: Severe I/R-induced injury to the liver, clinically leads to graft dysfunction. This is due to advanced apoptotic and/or necrotic events at the hepatocyte level that may, on the most severe form, lead to calcification. The study of microcalcification at the early posttransplant period could provide insight in the events taking place following significant ischemia/reperfusion-induced injury to the graft.

Adult↗

Calcification of the intervertebral discs and curvature of the radius and ulna: a radiographic survey of Finnish miniature dachshunds.

The vertebral column of 124 randomly selected miniature dachshunds, representing 4.5% of the population registered by the Finnish Kennel Club during the years 1988 to 1996, were radiographed. The front legs were also radiographed in order to evaluate the curvature of the radius and ulna. Calcified discs were found in 75.9% of the longhaired miniature dachshunds and in 86.7% of the wirehaired ones. The occurrence of signs associated with IDD was 16.5% in longhaired and 15.6% in wirehaired miniature dachshunds. The occurrence of signs of IDD in dogs with calcified discs was 20.0% and 17.9% in longhaired and wirehaired miniature dachshunds, respectively. In dogs without calcifications only one dog showed signs of IDD. The curvature of the radius and the ulna did not differ between the dogs with signs of IDD and the healthy ones, or between the dogs with and without intervertebral calcifications. Our results indicate that radiographic eradication based on the presence of intervertebral calcifications is not suitable for breeding purposes for the Finnish miniature dachshund population because the percentage of dogs without calcifications is small.

Animals↗

Tumoral calcinosis presenting with eyelid calcifications due to novel missense mutations in the glycosyl transferase domain of the GALNT3 gene.

CONTEXT: Familial tumoral calcinosis (TC) is a rare autosomal recessive disorder characterized by metastatic calcifications, often periarticular. Biochemical findings include hyperphosphatemia, high 1,25-dihydroxyvitamin D levels, and elevated tubular maximum for phosphate reabsorption per deciliter of glomerular filtrate (TmP/GFR). TC is caused by biallelic mutations of the genes encoding either fibroblast growth factor 23 (FGF23) or uridine diphosphate-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 3 (GalNAc transferase 3 or GALNT3). OBJECTIVE: The objective was to identify mutations in FGF23 or GALNT3 responsible for a mild TC phenotype by DNA sequencing and to determine serum FGF23 levels by ELISA. PATIENTS OR OTHER PARTICIPANTS: The subject was a 25-yr-old Caucasian woman with eyelid calcifications and biochemical features of TC. RESULTS: Eyelid biopsy revealed superficial dermis calcifications. There was no history of metastatic calcifications, mineral homeostasis abnormalities, or renal dysfunction. Biochemistry revealed normal levels of calcium, creatinine, PTH, and 25-hydroxyvitamin D, with elevated phosphorous, TmP/GFR, and high normal 1,25-dihydroxyvitamin D levels. Intact FGF23 was undetectable (< 3 pg/ml), whereas C-terminal FGF23 was elevated (698.2 RU/ml). Mutation detection revealed compound heterozygosity for two novel mutations in the glycosyl transferase domain of the GALNT3 gene. CONCLUSION: Previously reported GALNT3 mutations in TC have been null mutations. This study shows that missense mutations affecting the glycosyl transferase domain of GalNAc transferase 3 also cause TC. Elevated C-terminal FGF23 fragments with undetectable intact FGF23 suggest that the mutant enzyme lacks the ability to glycosylate FGF23 and that glycosylation by GalNAc transferase 3 is necessary for secretion of functional full-length FGF23.

Adult↗

Calcification in brainstem gliomas.

Calcification in brainstem gliomas is an unusual histologic and and radiologic finding. To date, radiologic evidence of calcification in these tumors has not been reported. The two patients described here had evidence of calcification on either computerized tomography (CT) or plain skull films. Increasing availability of CT scans may provide a more accurate estimate of the incidence of calcification in these tumors.

Adolescent↗

Dystonia and calcification of the basal ganglia.

We studied a family with dominantly inherited dystonia and intracranial calcification. Thirty-seven members were examined; 13 were affected by dystonia that was segmental in most patients, affecting especially the voice, face, neck, and limbs. Intracranial calcification involved the putamen, pallidum, cerebral white matter and cortex, and cerebellar nuclei. Several patients had both dystonia and calcification, but others had either dystonia or calcification alone.

Adult↗

Progressive intracranial calcification in dihydropteridine reductase deficiency prior to folinic acid therapy.

Hyperphenylalaninemia in infants and children may be caused by a deficiency of dihydropteridine reductase (DHPR). Recommended therapy includes folinic acid as a source of tetrahydrofolate, a phenylalanine-restricted diet, and both dopamine and serotonin precursors. We report a child with progressive basal ganglia and other subcortical calcifications prior to the use of folinic acid. Six other reported cases of DHPR deficiency demonstrated similar calcifications prior to folinic acid therapy. Since this pattern of calcification also resembles that seen in CNS folate deficiency caused by both congenital folate deficiency and that which is methotrexate-induced, we propose that intracranial calcification in DHPR deficiency is caused by inadequate CNS tetrahydrofolate and may be prevented by the use of folinic acid. Our patient achieved excellent seizure control following the use of folinic acid, suggesting either a direct or indirect anticonvulsant effect of this compound in patients with DHPR deficiency.

Brain↗

Spontaneous calcific embolization to the supraclinoid internal carotid artery from a regurgitant bicuspid aortic valve.

Calcific brain embolization leading to stroke is rarely recognized. We report a case of spontaneous embolization to the right cerebral circulation from a severely regurgitant, calcified bicuspid valve. Bicuspid aortic valves constitute the most common congenital heart abnormality, and have a tendency to become stenosed, regurgitant, calcified, or infected. The presence of heavy calcification in a noninfected bicuspid valve may lead to dislodgement of calcific embolic material which is not necessarily heralded by acute valvular rupture. Accurate localization of the calcific embolus to the right supraclinoid internal carotid artery was provided by spiral CT imaging in this case.

Adult↗

Familial idiopathic brain calcification with autosomal dominant inheritance.

We report a family with brain calcification, predominantly in the basal ganglia, and no evident cause such as abnormal calcium or phosphorus metabolism. The proband, a 48-year-old man, had intellectual decline, parkinsonism, and mild cerebellar ataxia. He had bilateral and symmetric calcification of the basal ganglia, thalamus, dentate nucleus, cerebral cortex, subcortical white matter, and hippocampus on CT. Calcified areas showed low- or high-intensity signals on MRI T1-weighted images, and low-intensity signals on MRI T2-weighted images. Two sons and both parents, all asymptomatic, also showed calcification of the basal ganglia, suggesting an autosomal dominant inheritance. Familial idiopathic brain calcification is a rare disorder with less than 20 previously reported families. Twelve families with autosomal dominant inheritance showed a relatively homogeneous clinical picture, which may represent a distinct clinical entity. Mental deterioration, parkinsonism, and cerebellar ataxia appear in adult life and progress gradually. CT imaging, rather than MRI, is a simple and useful means to screen family members for this condition.

Brain Diseases↗

Effect of etidronate on aortic calcification and bone metabolism in calcitriol-treated rats with subtotal nephrectomy.

The present study was undertaken to determine the effects of etidronate (ED) on calcitriol-induced aortic calcification and bone metabolism in rats with renal failure. Severe aortic calcification was induced by treatment with calcitriol for 3 weeks in rats in which 5/6 of the kidneys were removed (SNx group). Treatment of ED (10 mg/kg) together with calcitriol after subtotal nephrectomy (SNx) significantly inhibited thoracic and abdominal aortic calcification 3 weeks after the operation; however, ED (2 mg/kg) was ineffective. The serum levels of osteocalcin and pyridinoline decreased in ED (10 mg/kg) treated-renal failure rats compared with SNx rats. Total bone mineral density (BMD) in the SNx group was lower than that in the sham group, in which animals were treated with calcitriol after a sham operation. The total BMD value in the ED (10 mg/kg)-treated group was similar to that in the SNx group, whereas the levels of cancellous BMD were low in the ED (10 mg/kg)-treated rats. Our data show that ED at a dosage that suppresses bone metabolism markedly inhibits vascular calcification in rats with renal failure.

Amino Acids↗

Enchondromata with annular calcification in association with fibrous dysplasia.

Annular or ring calcification is described in conjunction with fibrous dysplasia in four of 110 patients culled from the Bristol Bone Tumour Registry. This feature results from endochondral calcification and ossification within cartilage rests or chondromata developing pari passu with fibrous dysplasia. Statistically the condition is more likely to be present in the polyostotic form of fibrous dysplasia (17%) than in the monostotic form (circa 1%). All patients were male and the diagnosis of fibrous dysplasia was established between the time of birth and the age of 16. Ring calcification was first detected between the ages of three and 16 years and persisted into adult life. Calcification almost exclusively involved the lower limbs particularly around the knee and above the ankle. In the three polyostotic cases rapid and gross extentsion of the cartilaginous component of the disease occurred at the time of the adolescent growth spurt. When present in a patient with fibrous dysplasia the appearance are typical and signify and associated benign enchondromatous process. No suspicion of chondrosarcoma should arise.

Adolescent↗

Case report: soft tissue and perivisceral calcification occurring in an infant: a case of brown fat necrosis.

Fat necrosis is a well described cause of widespread subcutaneous calcification occurring in a young infant. In this condition the radiographic demonstration of soft tissue calcification is often dramatic but is clinically irrelevant since the diagnosis is usually evident without recourse to radiology. Visceral fat necrosis and calcification, in the absence of hypercalcaemia, have been reported occasionally in association with subcutaneous fat necrosis. We report a case with calcification largely confined to deep perivisceral sites without clinical signs of subcutaneous fat necrosis.

Adipose Tissue, Brown↗

Classification of ductal carcinoma in situ by image analysis of calcifications from digital mammograms.

There is evidence to suggest that non-comedo cases of ductal carcinoma in situ (DCIS) may have a lower risk of progressing to invasive disease than comedo DCIS and may be managed with less radical treatment. Most cases of DCIS present as calcifications on the mammogram and, due to differences in the tumour architecture, comedo calcifications often have a characteristic linear or branching appearance. In this study, computer methods were developed to identify the comedo cases using the imaging features of individual calcifications and of calcification clusters. Classifier performance was measured using the area under the receiver operating characteristic (ROC) curve and was optimized by systematic testing of many different combinations of these features. On a test-set of 42 cases the computer achieved a ROC curve area of 0.91 using six combined cluster features. These results are encouraging given the observed overlap in appearance between the comedo and non-comedo cases.

Breast Diseases↗