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Sneddon's syndrome consists of livedo reticularis and cerebral vascular accidents with no evidence of systemic disease responsible for the livedo. The syndrome has been assimilated to a subgroup of systemic lupus erythematosus (SLE) with presence of antibodies directed against phospholipids. Recently, a significant increase in the frequency of cardiac valve diseases has been demonstrated in some SLE patients with livedo reticularis, cerebral vascular accidents and antiphospholipid antibodies. We report the case of a 26-year old woman who had been presenting for 6 years with idiopathic livedo reticularis. Her history was remarkable for the occurrence of 2 cerebral ischaemic accidents at the ages of 23 and 26 years, generalized convulsive seizures at 22 years, and hypertension of pregnancy with 2 miscarriages. Biopsy of the livedo showed normal histological patterns, but electron microscopy detected an obliterating endothelial proliferation and endothelial cells with numerous Weibel-Palade bodies. Laboratory signs of SLE, as well as antiphospholipid antibodies were absent. At the age of 26 years, cardiac abnormalities were heard at auscultation for the first time, and echocardiography showed that they were due to a fairly loose mitral stenosis. According to Burton's criteria our patient had all the typical features of Sneddon's syndrome. The finding of mitral stenosis--an emboligenic cardiopathy that is potentially responsible for cerebral vascular accidents--raises the problem of its relationship with Sneddon's syndrome. The association does not seem to be fortuitous, since our case is very similar to the cases of SLE or antiphospholipid antibody syndrome associated with cardiac valve lesions. However, this case is particular in that 6 years after the onset of the disease there was still no sign of SLE and of antiphospholipid antibodies.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Livedo reticularis, cerebrovascular disorders and mitral disease: a new cause of Sneddon's syndrome?]. 208 86

In this cross-sectional study, the authors evaluated 197 patients diagnosed with central retinal vein occlusion (CRVO) at the Wilmer Ophthalmological Institute between 1980 and 1985 to determine the risk of systemic disease and mortality. Complete follow-up information for mortality was obtained in 191 (97%). National Health Interview Survey (NHIS) patients and Wilmer cataract patients formed two comparison groups. The prevalence of hypertension was significantly elevated in the CRVO cases when compared with both comparison groups (P less than 0.03, 0.005). The prevalence of diabetes mellitus was increased in CRVO cases in comparison with the NHIS group (P less than 0.005). The prevalence of cerebrovascular or cardiovascular disease was the same for all three groups, as was overall mortality. Mortality was not increased in CRVO cases as compared with United States mortality rates.
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PMID:The risk for systemic vascular diseases and mortality in patients with central retinal vein occlusion. 225 27

The numerous findings discussed lead to the following conclusions: 1. The mesangial lesions, which may take a wide range of different forms, can be classified into two groups according to whether an underlying immunological pathomechanism is involved. Those that result from such a pathomechanism represent various types of glomerulonephritis. 2. Amongst these immunologically-mediated glomerulonephritides mesangioproliferative glomerulonephritis (and, of this group, IgA nephritis) is the most common. Membranoproliferative glomerulonephritis is the most severe of these diseases. Either may be idiopathic or secondary, or may occur in association with systemic disease. 3. The number of macrophages in the mesangial lesions in glomerulonephritis correlates with the severity of the glomerulonephritis, the localization of the immune complex deposits and the degree of proteinuria. If the immune complex deposits extend out of the mesangium into the subendothelial space, the number of macrophages is higher, the structural changes are more marked, and proteinuria is more severe. 4. Various pathomechanisms and nosologic entities can lead to mesangial lesions of the type seen in mesangioproliferative glomerulonephritis or membranoproliferative glomerulonephritis. On the other hand, the same entity may be associated with mesangial lesions of different severity, and consequently the prognosis varies. Differential diagnosis of the mesangial lesions, which represent heterogeneous nosologic entities, requires the use of light microscopic, immunohistochemical, and electron microscopic techniques. Exact diagnosis is necessary because of the differences in prognosis. 5. The course and prognosis of mesangial lesions are determined by immunological and nonimmunological factors. Long-term studies have demonstrated that prognostically relevant information can already be gained at the time of biopsy by the assessment of certain morphological features (e.g., immunohistological findings, severity of glomerulonephritis, the presence of focal/segmental lesions) and clinical parameters (e.g., proteinuria, hematuria, hypertension, and serum creatinine concentration). The decisive predictor of an unfavorable prognosis is the presence of interstitial fibrosis.
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PMID:[Clinical pathology of the glomerulus--from phenomenon to entity. The mesangial lesion]. 248 29

The presence of a lupus anticoagulant (LA) is paradoxically associated with a high incidence of arterial and venous thrombosis. In a patient with a lupus-like systemic disease, having received phenindione for 11 years, LA was discovered in association with recurrent deep venous thrombosis, a right atrial thrombus, coronary occlusion, arterial hypertension, thrombopenia, and anticardiolipin antibodies without anti-DNA antibodies. Renal cortical ischemia was detected by a tomographic scan. Renal biopsy showed glomerular ischemia and diffuse interstitial fibrosis. After a one-year anticoagulant and steroid therapy, LA has disappeared despite a high level of anticardiolipin antibodies, and renal function remains normal.
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PMID:[Renal cortex ischemia, right atrial thrombosis and coronary occlusion in anti-phospholipid antibody syndrome]. 251 17

Renal artery stenosis is an uncommon, but curable cause of systemic hypertension. The most common causes of stenosis are atherosclerosis, and fibromuscular dysplasia. Diagnosis may be difficult due to the lack of a suitable screening test, and a high index of suspicion needs to be maintained. Treatment may be with pharmacotherapy, renal angioplasty, or surgery and the choice of therapy needs to be tailored to the individual patient. Once a stenotic lesion is discovered in a hypertensive patient, the functional significance is not always clear cut. Fibromuscular dysplasia may be a systemic disease in some cases, and may affect the central nervous system.
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PMID:Renovascular hypertension and demyelimating disease in a young woman. 259 48

Oculo-oscillodynamography after Ulrich was performed in 27 patients suffering from glaucoma without hypertension (so-called low-tension glaucoma). Patients who had severe systemic disease or were receiving systemic medication which might influence IOP, and patients with narrow angle and an IOP higher than 22 mm Hg were excluded. In 85% of the patients a severe decrease in systolic ciliary perfusion pressure was found, whereas the systolic retinal and diastolic ocular perfusion pressures were significantly lower in almost 30% of the cases. Glaucoma without hypertension appears to be caused by the vascular change at the disk.
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PMID:[Oculo-oscillodynamography findings in glaucoma without hypertension]. 260 Dec 74

We report renal biopsy findings in 109 patients with unexplained renal impairment (serum creatinine greater than 0.15 mmol/l) and normal-sized non-obstructed kidneys. The most common histological lesions were interstitial nephritis, rapidly progressive glomerulonephritis and a variety of other types of glomerulonephritis. The groups could not be distinguished by the presence or absence of hypertension, haematuria, proteinuria, or features of systemic disease. However interstitial nephritis was found more frequently in patients presenting with one or none of these features and rapidly progressive glomerulonephritis in patients presenting with three or more. All four patients with none of these features had interstitial lesions. Fifty-two per cent of patients with interstitial nephritis improved and 60 per cent of the patients with rapidly progressive glomerulonephritis who received immunosuppressive treatment improved or remained stable with treatment. The benefits of a biopsy diagnosis were almost wholly confined to these two groups. Complications were recorded in nine patients - prolonged macroscopic haematuria in six and symptomatic perirenal haematomata in three. Six required blood transfusion. One required nephrectomy to control haemorrhage and subsequently died. Percutaneous renal biopsy is not without risk in patients with renal impairment but the benefits of diagnosing interstitial nephritis and rapidly progressive glomerulonephritis outweigh the disadvantages.
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PMID:Renal biopsy in patients with unexplained renal impairment and normal kidney size. 260 35

The clinical presentation and prognostic determinants in a group of 20 patients who presented a hemolytic-uremic syndrome for a period of 14 years are reviewed. Patients younger than 10 years old and those with and hemolytic-uremic syndrome due to a systemic disease, renal transplant or accelerated arterial hypertension were excluded from the study. The clinical picture consisted mainly of renal, hematological, hemorrhagic, neurologic cardiovascular, gastrointestinal and hepatic manifestations. It was note worthy the high incidence of malignant hypertension which frequently developed after admission. Thrombocytopenia and hemolysis were frequently not severe and of limited duration. Severe neurologic manifestations was the key determinant of immediate vital prognosis, while severe arterial hypertension was the most reliable marker of irreversible renal failure. In general, the clinical picture was very severe, with and elevated early mortality rate (5 patients) and a high rate of irreversible loss of renal function (five patients needed dialysis, and 4 remained with residual renal failure).
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PMID:[Clinical presentation and prognostic factors in primary hemolytic-uremic syndrome in adults]. 262 47

A prospective cross-sectional study of 84 foot lesions in 50 diabetic patients was done in a Nigerian teaching hospital over a three-year period (1982-1984) to assess factors that may influence the choice of treatment and treatment outcome. Age, gender, duration of diabetes, mode of treatment of diabetes and tobacco smoking did not influence whether or not a diabetic with a foot lesion will have major amputation, an unsatisfactory outcome of primary treatment, prolonged hospital stay or will die. Similarly, the presence of foot infections alone, microangiopathy (nephropathy, retinopathy), foot ischaemia alone or neuropathy alone had no relationship to poor prognostic indices. However, when these complications appeared in concert (neuropathy, ischaemia and infection) and when, at presentation, there was associated systemic disease (as shown by anaemia and leucocytosis), severe fasting hyperglycaemia, evident bone destruction and anaerobic superinfection, the outcome of treatment was adverse. In addition, hypertension and infection of the foot were related to need for major amputation. Poor long-term control did not influence prognosis adversely. We therefore suggest that the high morbidity seen with diabetic foot lesions could be reduced by optimizing glycaemic control, using combination antibiotic chemotherapy, vigorously correcting anaemia and encouraging early presentation of even mild lesions before underlying bone disease supervenes.
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PMID:Factors influencing the outcome of treatment of foot lesions in Nigerian patients with diabetes mellitus. 269 57

Thirty-seven untreated subjects with borderline or mild hypertension were studied to establish the prevalence and clinical characteristics associated with abnormal left ventricular filling in this disorder. Subjects were referred to this study because of casual office blood pressure measurements of greater than or equal to 140/90 mm Hg; all were less than 50 years old, had no other cardiovascular or systemic disease and had not received antihypertensive medication for at least 1 year. To precisely determine blood pressure, measurements were made over 30 min with the patient in the supine position and during awake hours with ambulatory monitoring. Left ventricular mass was determined echocardiographically, and Doppler echocardiography was used to assess left ventricular filling. No subject had increased left ventricular mass, but 8 (22%) of the 37 had abnormal left ventricular filling. All eight subjects with abnormal left ventricular filling had an ambulatory systolic blood pressure greater than 130 mm Hg and a supine systolic blood pressure greater than 122 mm Hg. Abnormal filling was not related to left ventricular mass or heart rate. In multivariate analysis, the degree of abnormal filling could best be predicted from a combination of age and supine systolic blood pressure (r = 0.69; p less than 0.001). This study suggests that in untreated early essential hypertension, abnormal left ventricular filling is present in greater than 20% of subjects, precedes detectable left ventricular hypertrophy and is related to age and prevailing level of blood pressure.
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PMID:Determinants of abnormal left ventricular filling in early hypertension. 279 87


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