1/110. Dystrophic calcification of the fetal myocardium.Intramural cardiac masses were detected antenatally in three fetuses by echocardiography. The masses were initially thought to be rhabdomyomas. All three pregnancies were terminated and histology showed dystrophic calcification in all, with no evidence of tumour. Therefore, dystrophic calcification of the fetal myocardium may have a similar appearance to single or multiple rhabdomyomas. This should be considered when counselling parents after detection of masses in the fetal heart, particularly when considering the risk of associated tuberous sclerosis.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/110. Phlebosclerosis of the colon with positive anti-centromere antibody.A 56-year-old woman with symptoms of chronic bowel disease presented a peculiar calcification of the mesenteric vein of the ascending to transverse colon on barium enema study. The resected colon was hard and black. Histo-pathologic examinations demonstrated fibrous change of the colon with a calcified and hyaline-deposited mesenteric vein. No cell infiltration was observed. These findings were compatible with phlebosclerosis and also with systemic sclerosis. Positive anti-centromere antibody and Raynaud's phenomenon, hallmarks of a variant systemic sclerosis, the crest syndrome were observed. We therefore speculated that the pathogenesis of the phlebosclerosis of the colon is related to the crest syndrome.- - - - - - - - - - ranking = 8keywords = sclerosis (Clic here for more details about this article) |
3/110. calcinosis cutis and intestinal pseudoobstruction in a patient with adult onset Still's disease associated with recurrent relapses of disordered coagulopathy.adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown origin, characterized by a typical spiking fever, evanescent salmon-colored rash, polyarthralgia, and myalgia. calcinosis cutis and gastrointestinal involvement have rarely been noted in AOSD. We herein describe a 54-year-old woman who demonstrated repeated disseminated intravascular coagulation (DIC), and adult respiratory distress syndrome (ARDS), associated with AOSD. The patient also revealed a remarkable degree of digital calcinosis cutis and intestinal pseudoobstruction. A connective tissue disease, such as systemic sclerosis, might have been the underlying factor in the latter two symptoms.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
4/110. osteosclerosis, hypoplastic nose, and proptosis (Raine syndrome): further delineation.We describe a newborn girl with a lethal sclerosing bone dysplasia leading to prenatal skeletal alterations and microcephaly, proptosis, hypoplastic nose and midface, small jaw, cleft palate, hypertrophied gums, intracranial calcifications, and generalized osteosclerosis. There is a remarkable similarity between our patient and six previously reported infants subsequently categorized as having a distinct entity: Raine syndrome. Autosomal recessive inheritance is postulated based on parental consanguinity in several of the previous cases and in our patient.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
5/110. The CRST syndrome (calcinosis, Raynaud's phenomenon, sclerodactyly and telangiectasia).The CRST syndrome is defined, and its relation to systemic sclerosis described. The literature referring to the condition is reviewed and a further case reported. Clinical and diagnostic features of the telangiectasia are emphasised, particularly in comparison with those of the similar condition, hereditary haemorrhagic telangiectasia.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
6/110. Using coronary calcification scanning in the clinical practice of preventive cardiology.Preventive therapies such as cholesterol reduction significantly reduce the risk of acute coronary events. Diagnostic tools that identify asymptomatic coronary atherosclerosis would permit initiation of aggressive preventive therapies at an earlier stage of coronary disease. Histologic and angiographic data demonstrate that coronary calcium has a very high sensitivity for the presence of coronary plaque. Therefore, coronary calcification can be regarded as a marker for coronary atherosclerosis. Coronary calcium scanning has been suggested as a tool for identification of a high-risk asymptomatic patient group. It can be utilized to guide the aggressiveness of risk factor modification and therapeutic preventive interventions toward those at higher risk for future events. Based on the available data, we review the clinical use of coronary calcium scanning in preventive cardiology.- - - - - - - - - - ranking = 2keywords = sclerosis (Clic here for more details about this article) |
7/110. Case report: stent placement in support of intravascular ultrasound in a woman with atypical lower abdominal aortic stenosis.Localized stenosis confined to the distal abdominal aorta near the bifurcation is an atypical manifestation of atherosclerosis, particularly in a woman. We report the case of a middle-aged woman who presented with a focal stenosis accompanied by heavy calcification in the distal abdominal aorta. The lesion was successfully treated by Palmaz stent placement under intravascular ultrasound guidance.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
8/110. Dramatic worsening of vascular calcifications after kidney transplantation in spite of early parathyroidectomy.vascular calcification is a common feature in chronic dialysis patients, but their clinical significance is debated and the role of kidney transplantation (TP) in the natural history of their development has received scanty attention. We will describe a case of dramatic worsening of vascular calcifications during TP in a young patient in spite of early and successful parathyroidectomy (PTX), and will discuss other causes which might be putatively linked to vascular damage during the time of TP. A 37-year-old man on regular dialytic treatment (RDT) for 11 years, received his first cadaveric transplantation in January 1993. He underwent PTX 6 months after TP because of the lack of decreasing in parathyroid hormone values despite normal graft function. Although PTX was effective, a dramatic worsening was evident in large as well as in medium and small-sized arteries during the following three years of TP. In February 1997, few months after starting dialysis again because of the recurrence of his primary membranoproliferative glomerulonephritis (MPGN), the patient experienced myocardial infarction followed by aorto-coronary bypass (right coronary artery and anterior descending coronary artery) and leg "claudicatio". Though a role for parathyroid hormone in vascular disease has been commonly accepted, the case here reported clearly shows that blunting parathyroid gland activity may be unable to avoid the worsening of a process of vascular disease during the time of TP. Many other factors--linked to the time of TP--may be involved in vascular diseases, such as nephrotic syndrome, dyslipidemia, hypertension and drugs. In the case of our patient, a clear cut risk factor for his progressive atherosclerosis can be designated hyperlipidema and other disturbancies secondary to a nephrotic syndrome due to relapse of MPGN, together with persistent hypertension. This is the first case report in the English literature which clearly demonstrates that TP may add fuel to the fire of vascular disease also in young people and even in the absence of parathyroid hyperactivity, perhaps on the basis of a favorable genetic background. Furthermore, the history of our patient demonstrates that vascular calcifcation heralds major cardiovascular diseases.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
9/110. Pseudohypertension in a patient with diffuse scleroderma.Pseudohypertension is the artifactual elevation of blood pressure that occurs secondary to noncompressible blood vessels. It has been described in patients with uremia, diabetes mellitus, and severe atherosclerosis. If unrecognized, the condition may lead to inappropriate and potentially harmful therapy. We report a case of pseudohypertension in a 65-year-old man with diffuse scleroderma. His blood pressure as assessed by conventional sphygmomanometry was at least 240/135 to 145 mm Hg. Intra-arterial blood pressure was found to be 107/52 mm Hg. The severe rise in blood pressure as measured by sphygmomanometry led to the concern of scleroderma renal crisis and potentially harmful therapy. Intra-arterial pressure monitoring confirmed the presence of pseudohypertension, however. This is the first reported case of pseudohypertension in a patient with diffuse scleroderma.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
10/110. Neuropathology of Raine syndrome.We present three cases of Raine syndrome occurring in siblings of consanguineous parents. Raine syndrome is characterised by generalised osteosclerosis with craniofacial anomalies and intracranial calcifications. So far, only nine cases have been reported, and no evaluation of the distribution and extent of the cerebral mineralisations, as well as their impact on the surrounding tissue, has been undertaken yet. In our cases, calcifications were unevenly distributed throughout the central nervous system, not associated with neuronal loss or dystrophic events and appeared mostly as single calcospherites within the neuropil with occasional confluent deposits at advanced gestational age. There was intense perifocal microgliosis around single immature calcospherites, as well as mild astrogliosis around and within the confluent lesions, in which occasional macrophages could be found. Rarely, mineralisations occurred in blood-vessel walls, mainly affecting basal ganglia. Preferential sites of calcification were parietal and occipital periventricular white matter and corpus callosum, while frontal lobes were mildly affected. The cortex, temporal lobes as well as internal capsule, brain stem, cerebellum, leptomeninges, pituitary gland and choroid plexus were devoid of mineralisations. The subcortical grey matter was moderately involved in the putamen and pallidum, mildly in the caudate nucleus and subependymal germ cell matrix and not at all in the thalamus, Ammon's horn, amygdala and substantia nigra. The distribution of mineral deposits was thus inversely correlated to regional blood circulation and capillary density, with calcifications being concentrated in more sparsely perfused areas but lacking in highly vascularised tissue. This inverse relationship between mineralisation and regional blood flow was reflected in the varying distribution of calcospherites in grey and white matter as well as in the white matter of different lobes.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
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