1/9. role of lipids in the progression of renal disease in systemic lupus erythematosus patients.Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease marked by immune-complex mediated lesions in small blood vessels of various organs, especially the kidneys, although other factors may also be implicated in the pathogenesis of the disease. This article focuses on the role of lipids in the progression of glomerular, vascular and tubulo-interstitial lesions in two patients with lupus nephritis associated with pronounced hyper- and dyslipidemia. The pathogenesis of progressive glomerulosclerosis in both patients appears to be multifactorial. In addition to immune complex mediated lupus glomerulonephritis, progressively active in the first patient, severe nephrotic-range persistent proteinuria, arterial hypertension associated with hyperfiltration and hyperperfusion injuries and, to a minor extent, hyper- and dyslipidemia were observed. Immunological and non-immunological factors were shown to contribute to the development of tubulo-interstitial lesions. In both patients, in addition to local immune deposits, prominent tubulo-interstitial lipid deposits were probably causally related to both hyperlipidemia and the increased permeability of the glomerular filtration barrier. Tubular lesions were highlighted by intracytoplasmic lipid droplets as well as small cleft-like spaces found to be impacted in the tubular lumina. They were seen to penetrate tubular epithelial cells and eventually lodge in the interstitium, surrounded by mononuclear cell infiltrates and foam cells. In both patients, hypertensive angiopathy and extraglomerular vascular immune deposits were demonstrated. In addition, in the second patient, arteriolar and small arterial hyaline was found at the age of 28 years to be full of lipids and calcium precipitates, suggesting a peripheral atherosclerosis-like process which never occurs as a natural age-related condition. In conclusion, all parts of the nephron may be involved in the pathogenetic process causally related or influenced by hyper- or dyslipidemia. Associated either with endothelial cell injury and consequent insudation of lipids in the vascular walls, glomerular filtration barrier injury with hyperfiltration, or tubulo-interstitial lipid deposition, the mechanism of tissue damage by lipids in all parts of the nephron shares similarities with the pathogenesis of systemic atherosclerosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/9. Acute two-vessel coronary closure in a patient with Down's syndrome.Myocardial infarctions are rare in patients with Down's syndrome. This paper reports an unusually aggressive presentation of two-vessel simultaneous coronary occlusion during an intended percutaneous intervention. Since survival in patients with Down's syndrome is improving, encounters with late (and perhaps unusual) sequelae of coronary artery disease are expected to increase.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
3/9. Electrophysiological changes in lipaemia retinalis.PURPOSE: To report the electrophysiologic findings in a patient with lipaemia retinalis. DESIGN: Observational case report. methods: An 11-year-old girl with diabetes had hyperlipidemia and presented for ophthalmologic consultation. Fundus examinations revealed milky discoloration of retinal vessels in both eyes. Laboratory testing disclosed hyperglycemia and markedly elevated level of triglycerides and cholesterol. Although the visual acuity was not affected, the electroretinograms showed decrease amplitudes of a- and b-wave in both cone and rod responses. RESULTS: After correction of blood sugar and lipids with insulin and diet control, the lipaemia retinalis resolved, and the impaired ERG response reversed within 1 week. CONCLUSIONS: Lipaemia retinalis usually resolves rapidly without visual impairment after correction of hyperlipidemia. The reversible deficits of electroretinogram response in lipaemia retinalis are reported for the first time. High level of triglycerides or cholesterol or both may impair retinal function. The mechanism of an impaired electroretinogram remained speculative.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/9. Spontaneous late thrombolysis of an occluded saphenous vein graft subsequent to acute myocardial infarction treated with percutaneous coronary intervention to the native culprit vessel.A 67-year-old male with prior history of myocardial infarction and coronary artery bypass grafting (individual vein grafts to the left anterior descending artery [LAD] and right coronary artery) presented with an acute anterior ST elevation myocardial infarction and cardiogenic shock. The vein graft to the LAD was occluded with heavy thrombus burden and there was severe native CAD. Given the degree of thrombus burden and other anatomic considerations, percutaneous intervention with stenting was performed to the native proximal LAD. Three months later, after complaining of atypical chest pain, repeat angiogram revealed a spontaneous widely patent vein graft to the LAD and occluded proximal LAD.- - - - - - - - - - ranking = 4keywords = vessel (Clic here for more details about this article) |
5/9. Management of cutaneous angiomyolipoma and its association with tuberous sclerosis.Although typically presenting renally, angiomyolipomas can rarely present in the skin. The tumors are composed of an admixture of blood vessels, smooth muscle and adipose tissue and are often seen in the setting of tuberous sclerosis. We describe a case of angiomyolipoma presenting on the thigh of a 50-year-old white female. This is the 17th reported case of cutaneous angiomyolipoma. As with all previously described cases, our patient did not present with the stigmata of tuberous sclerosis. angiomyolipoma should be considered within the differential for subcutaneous nodules and work-up for tuberous sclerosis should not be pursued when presenting in the skin.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/9. The retina in type 5 hyperlipoproteinemia.Of two patients with type 5 hyperlipoproteinemia, one exhibited lipemia retinalis with multiple retinal hemorrhages and intraretinal lipid extravasations. Postmortem examination showed hemorrhages in the inner retinal layers and lipid deposits largely in the outer plexiform layer. Lipid within the walls of the retinal vessels was demonstrated by light and electron microscopy. The second patient exhibited visual loss due to progressive obstruction of retinal vessels with white material presumed to be lipid. Findings were confined to one eye.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
7/9. Immune complex hyperlipidemia induced by an apolipoprotein-reactive immunoglobulin a paraprotein from a patient with multiple myeloma. Characterization of this immunoglobulin.An antibodylike paraprotein has been isolated from a patient with multiple myeloma and autoimmune hyperlipoproteinemia. The paraprotein bound to apolipoprotein B (apo B)-containing lipoproteins that formed macromolecular aggregates, and globules thought to be aggregated complexes of lipoproteins and reactive immunoglobulins were observed circulating within the retinal blood vessels of this patient. This binding specificity permitted purification of the paraprotein from both the agglutinated immune complexes and from the plasma. The protein is an IgA, kappa-immunoglobulin which exists primarily in a polymeric state. Capillary immunoprecipitation demonstrated reactivity with very low density lipoproteins (VLDL) and low density proteins (LDL), but not with high density lipoproteins (HDL). Delipidated apo B and apo E, but not apo A or apo C, formed precipitates with this immunoglobulin. In using a radioimmunoassay format, the affinity of the immunoglobulin was greatest for VLDL and decreases sequentially for intermediate density lipoproteins and LDL. No binding occurred with a dispersion of LDL lipids or with HDL. Deglycosylation did not change the binding to LDL. The apolipoproteins b and E bound with similar affinity, but no binding occurred with apo A-I or apo A-II. Weak binding appeared to occur with apo C. This paraprotein immunoprecipitated apo B-containing lipoproteins from all classes of vertebrates tested. Displacement of the lipids of LDL by Triton X-100 resulted in the formation of an apo B-Triton complex which, however, did not bind to the immunoglobulin; apparently the binding site on apo B was lost. Upon enzymatic digestion with the IgA-specific protease from streptococcus sanguis the immunoglobulin was cleaved into Fc and Fab fragments, and the binding of LDL occurred only with the latter, consistent with the behavior of an immunoglobulin. The immunoreactivity of this paraprotein with apo B and apo E raises the interesting possibility that it may be binding to a site on these apolipoproteins which is reactive with the apo B, E receptor of the plasma membrane, a site which is conserved throughout the vertebrate phylum.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/9. A new life-long hemorrhagic disorder due to excess plasminogen activator.A life-long bleeding disorder is described, characterized by hemorrhage occurring after surgery, injury, or dental extraction, and finally by spontaneous intracerebral bleeding. No abnormality of platelet function or plasma coagulation was demonstrable, but grossly enhanced overall fibrinolytic activity was present. The patient had, additionally, a hyperlipidemia with gross arterial atheroma and a family history of myocardial infarction but not of any hemorrhagic disorder. Laboratory studies led to the conclusion that the enhanced fibrinolysis was due to consistently greatly raised levels of a plasma plasminogen activator physically and immunologically related to that in human tissues and blood vessel endothelium. No deficiency of any known inhibitor of fibrinolysis was detected. Free plasmin was not detectable in functional assays but continuous intravascular plasmin generation clearly occurred as evidenced by presence of plasmin-alpha 2-antiplasmin complexes and of fibrin/fibrinogen-related antigens. Excessive production of plasminogen activator appeared to have occurred throughout life and to be independent of the hyperlipidemia. The pathologically increased fibrinolytic activity may have accounted for the complete absence of detectable thrombotic vascular occlusion at autopsy despite extensive arterial disease with severe narrowing of coronary and cerebral arteries.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/9. Phytosterolaemia, xanthomatosis and premature atherosclerotic arterial disease: a case with high plant sterol absorption, impaired sterol elimination and low cholesterol synthesis.A fourth case is described in which phytosterolaemia, earlier diagnosed as familial hypercholesterolaemia, was associated with normocholesterolaemia, hypersplenism and premature atherosclerotic arterial disease requiring a three-vessel coronary bypass at the age of 29 years. During a follow-up of 5 years 22-26% and 27-30% of serum and bile sterols were plant sterols, respectively. In addition to campesterol and beta-sitosterol, stigmasterol and a fourth major plant sterol, tentatively identified as avenasterol, were found in bile, and in free and esterified forms in all serum lipoproteins. Analysis of faecal steroids and measurement of biliary lipid secretion indicated that in addition to enhanced absorption of plant sterols their decreased biliary secretion contributed to the development of phytosterolaemia. Impaired biliary cholesterol secretion was compensated for by a markedly reduced cholesterol but normal bile acid synthesis and resulted in bile undersaturated with respect to cholesterol, in a reduced intestinal cholesterol pool and in a very low faecal excretion of cholesterol as neutral sterols. Cholestyramine brought about a modest increase in cholesterol elimination as bile acids, increased cholesterol synthesis as evidenced by the sterol balance value and the increased cholesterol precursors squalene and methyl sterols in plasma and bile, and reduced the plasma cholesterol by 21% and plant sterols by 16%, but had no effect on the biliary composition of main sterols.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |