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We report the first case of a successful transapical transcatheter treatment of a giant pseudoaneurysm originating from a rupture of the mitroaortic fibrosa that occurred 3 months after a Bentall procedure in a 81-year-old male patient. Because of the age of the patient and the location of the leak at the mitroaortic fibrosa, the risk of a conventional ascending aorta reoperation was considered too high, and a transcatheter approach was chosen. A transapical puncture was performed with a left minithoracotomy followed by a catheterization of the pseudoaneurysm neck and an 8-mm Amplatzer (St. Jude Medical, Saint Paul, MN, USA) device was delivered, resulting in a successful complete endovascular exclusion of the pseudo-aneurysmal sac., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Unlabelled: Since heterozygous familial hypercholesterolemia (HeFH) is a disease that exposes the individual from birth onwards to severe hypercholesterolemia with the development of early cardiovascular disease, a clear consensus on the management of this disease in young patients is necessary. In Belgium, a panel of paediatricians, specialists in (adult) lipid management, general practitioners and representatives of the FH patient organization agreed on the following common recommendations. 1. Screening for HeFH should be performed only in children older than 2 years when HeFH has been identified or is suspected (based on a genetic test or clinical criteria) in one parent.2. The diagnostic procedure includes, as a first step, the establishment of a clear diagnosis of HeFH in one of the parents. If this precondition is satisfied, a low-density-lipoprotein cholesterol (LDL-C) levelabove 3.5 mmol/L (135 mg/dL) in the suspected child is predictive for differentiating affected from non-affected children. 3. A low saturated fat and low cholesterol diet should be started after 2 years, under the supervision of a dietician or nutritionist.4. The pharmacological treatment, using statins as first line drugs, should usually be started after 10 years if LDL-C levels remain above 5 mmol/L (190 mg/dL), or above 4 mmol/L (160 mg/dL) in the presence of a causative mutation, a family history of early cardiovascular disease or severe risk factors. The objective is to reduce LDL-C by at least 30% between 10 and 14 years and, thereafter, to reach LDL-C levels of less than 3.4 mmol/L (130 mg/dL)., Conclusion: The aim of this consensus statement is to achieve more consistent management in the identification and treatment of children with HeFH in Belgium., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

In ulcerative colitis, the T helper type 2 proinflammatory cytokine Interleukin-13 (IL-13) contributes as effector cytokine to the epithelial changes associated with disturbed epithelial barrier function. This study aimed to investigate the underlying mechanisms in a colonic epithelial cell culture model. For studying these epithelial features in response to proinflammatory cytokines epithelial apoptosis was investigated by TdT-mediated X-dUTP nick end labeling (TUNEL) staining in HT-29/B6 cell monolayers. In contrast to interferon-gamma, IL-13 significantly upregulated the apoptotic rate of cells, which was intensified by simultaneous exposure to tumor necrosis factor-alpha. That this has a direct functional influence on epithelial barrier was shown by the caspase inhibitor N-benzyloxycarbonyl-Val-Ala-Asp, which inhibited IL-13 induced apoptosis induction and concomitantly reversed the decrease in epithelial resistance by approximately 50%. Direct evidence for apoptotic rosettes at corresponding sites of barrier defects in the epithelium was obtained by conductance scanning. In addition, the pore-forming tight junction protein claudin-2 was found to be upregulated at protein and mRNA level. In conclusion, IL-13 disturbs intestinal barrier function through mechanisms including apoptosis induction and alteration of tight junction protein composition.

Macrophages and dendritic cells are heterogenous and highly plastic bone marrow-derived cells that play major roles in renal diseases. We characterized these cells using immunohistochemistry in 55 renal biopsies from control patients or patients with glomerulonephritis as an initial step towards postulating specific roles for these cells in kidney disease. In proliferative glomerulonephritis numerous CD68 positive (pan monocyte, macrophage and dendritic marker) cells were found in both glomeruli and the tubulointerstitial space, however, a myeloid dendritic cell marker (DC-SIGN) was identified only in the tubulointerstitium. A significant number of plasmacytoid dendritic cells (identified as BDCA-2 positive cells) were seen at sites of interstitial inflammation, including follicular aggregates of inflammatory cells. Langerin positive cells (a marker of Langerhans' cells) were detectable but rare. The area of either CD68 or DC-SIGN positive interstitial cells correlated with serum creatinine. Low levels of DC-SIGN, DC-LAMP and MHC class II mRNA were present in the tubulointerstitial space in controls and increased only in that region in proliferative glomerulonephritis. We demonstrate that the CD68 positive cells infiltrating the glomerulus lack dendritic cell markers (reflecting macrophages), whereas in the tubulointerstitial space the majority of CD68 positive cells are also DC-SIGN positive (reflecting myeloid dendritic cells). Their number correlated with serum creatinine, which further emphasizes the significance of interstitial DCs in progressive glomerular diseases.

Local B-cell infiltrates play a role in tissue fibrosis, neolymphangiogenesis, and renal allograft survival. We sought to characterize the B-cell infiltrates, factors involved in B-cell recruitment, and lymphangiogenesis in renal interstitial injury (ie, acute and chronic interstitial nephritis and chronic IgA nephropathy). CD20-positive B cells formed a prominent part of the interstitial infiltrating cells. Together with CD3-positive T cells, the CD20-positive B cells formed larger nodular structures. CD10-positive pre-B cells were rare, and the majority were mature CD27-positive B cells. Proliferating B cells were detected within nodular infiltrates. The level of mRNA expression of the chemokine CXCL13 was increased and correlated with CD20 mRNA in the tubulointerstitial space. CXCL13 protein was predominantly found at sites of nodular infiltrates, in association with CXCR5-positive B cells. Furthermore, sites of chronic interstitial inflammation were associated with a high number of lymphatic vessels. B-cell infiltrates form a prominent part of the interstitial infiltrates both in primary interstitial lesions and in IgA nephropathy. CXCR5-positive B cells might be recruited via the chemokine CXCL13 and seem to contribute to the formation of intrarenal lymphoid follicle-like structures. These might represent an intrarenal immune system.

A novel DNA sequence belonging to a new genotype of TT virus (TTV) was detected by long-distance PCR in the serum of a chronically HCV-infected patient. The isolate was designated KAV according to the patient's initials. Extending the sequence to full length revealed a 3705-nt viral genome, which is about 100 nucleotides shorter than the other TT-viruses. KAV showed common features with the TTV family, such as the organization of open reading frames and conserved noncoding regions. The largest open reading frame of KAV (ORF 1) was about 40 aa shorter than that of other TT-viruses. Overall sequence homology with known TTV isolates was less than 66%. Phylogenetic analysis poses KAV in one major group with three recently published TTV sequences. So KAV can be considered as a new genotype of the TTV family (provisionally designated genotype 28).

Differentiating FH from other causes of hypercholesterolemia has important clinical and therapeutic implications but is often not possible by standard clinical criteria. As accumulation of cholesterol in tendon is generally considered as pathognomonic of FH, we evaluated the sensitivity and specificity of clinical and ultrasonographic tendon characteristics using the data of 127 genetically ascertained FH and 160 controls with various lipid profiles. Upon clinical examination, none of the controls and 29% of FH individuals (17% FH women and 38% FH men) presented with xanthomata in Achilles tendons, but no female and only 6% of male FH patients also showed xanthomata in the extensor tendon of the hand. Amongst all possible quantitative parameters (thickness, breadth, section and roundness) of Achilles tendon (AT) measured by ultrasonography, the thickness presented the best receiver operating curves. AT thickness above 5.8 mm was the most useful threshold for diagnosis of FH, procuring sensitivity of 75% and specificity of 85%. Analysis of variation of AT thickness with age and sex indicated that this clinical criterion performed better in females older than 45 and in males under 45. In patients carrying the APOB-R3500Q mutation, AT-thickness appeared significantly less important compared with those carrying LDLR mutations. In conclusion, this study recommends identification of possible FH individuals amongst hypercholesterolemic patients using a criteria of AT-thickness over 5.8 mm eventually associated with a specific genetic test for APOB-R3500Q mutation.

The peroxidation step of lipid transormation is considered to be essential in the pathogenesis of atherosclerosis. Although data concerning the mechanisms by which lipid peroxidation occurs in vivo are scarce, several lines of evidence suggest that some endogenous and exogenous compounds with antioxidant activity could have some beneficial effects in the prevention of atherosclerosis. Ascorbic acid (vitamin C) and alpha-tocopherol (vitamin E) act as the most important hydrophilic and lipophilic antioxidants, respectively in vivo. Accordingly, animal and human studies suggest that these compounds may have some preventive effect against the development of clinical coronary heart disease. Many plant phenols and flavonoids may be important dietary antioxidants and it has been speculated that these compounds in red wine or in the Mediterranean diet could explain the 'French paradox'. Several studies show that antioxidants such as probucol and butylated hydroxytoluene can inhibit development of atherosclerotic lesions in Watanabe and cholesterol-fed rabbits. Some drugs such as beta-blockers, calcium antagonists, hypolipodemic drugs,...appear to have at least in vitro antioxidant effects but the clinical relevance of these properties remains unkonwn. Moreover, some interventions aimed to decrease the LDL-oxidative susceptibility have not been shown to attenuate atherogenesis when cholesterol levels remain markedly elevated.

Familial hypercholesterolemia (FH) results from an inherited functional defect of the low density lipoprotein (LDL) receptor and is complicated by premature atherosclerosis. FH diagnosis is obtained by sophisticated techniques or is suggested by clinical criteria. We have developed a technique based on flow cytometry for the measurement of DiI-labeled LDL uptake in human peripheral blood T lymphocytes left for 2 days in a lipoprotein-deficient culture medium. Flow cytometry allowed us to discriminate the uptake of DiI-LDL by T lymphocytes subpopulation from the uptake by the whole mononuclear population using a T cell-specific anti-CD3 antibody. The method appeared to be highly specific for the receptor-mediated pathway of LDL uptake as DiI-LDL uptake was inhibited in the presence of a 10-fold excess of unlabeled LDL and by EDTA. A good relationship was found between the uptake of DiI-LDL and 125I-labeled LDL degradation. The test was applied in three groups of patients: patients with normal cholesterol levels, patients with heterozygous FH, and patients with high cholesterol levels but without clinical criteria of FH. The mean fluorescence intensities were 23.1 +/- 8.9, 6.3 +/- 1.7, and 17.1 +/- 3.5 (mean +/- standard deviation), respectively. The ability to measure the fluorescence in T lymphocytes improved the discrimination between FH and non-FH subjects when compared with values obtained from the whole mononuclear cell population. These results suggest that our method could be useful for LDL receptor defects screening.

The incidence of hypoxic hepatitis was prospectively studied for 1 year in a group of high-risk patients suffering from low cardiac output in a coronary care unit. Hypoxic hepatitis, defined as an increase in serum aminotransferase activity of at least 20 times the upper limit of normal without any other cause for hepatic necrosis, was observed in 20 patients. This represents 2.6% of the 766 patients admitted to the unit during this period and 21.9% of the 91 patients suffering from low cardiac output. Clinical, biological and hemodynamic data were compared between 20 patients with low cardiac output and hypoxic hepatitis, and 48 patients with low cardiac output but without hypoxic hepatitis who survived more than 24 h. In these two groups of patients, hepatic blood flow was measured by galactose clearance at low concentration. Patients with hypoxic hepatitis exhibited a higher central venous pressure (90% versus 38%-p < 0.001) as well as a lower hepatic blood flow (867 +/- 377 ml/min versus 1429 +/- 644 ml/min-p = 0.001). In conclusion, although it is considered a rare hepatic disorder, hypoxic hepatitis is frequent in patients with low cardiac output admitted to the coronary care unit, and is associated with a decrease in hepatic blood flow and passive hepatic venous congestion.

Peroxisomes (microbodies) have been localized in the cytoplasm of various cells, mainly the hepatocytes and the renal tubular cells. Various enzymes were found in the granular matrix and sometimes in the nucleus ; they were involved in the production and the degradation of hydrogen peroxide, in regulation of the energetic processes of the cell, and in the oxidation of fatty acids. Several xenobiotics and drugs such as hypolipidemic agents clofibrate-like, have induced in rodents a proliferation of peroxisomes and frequently, the appearance of malignant tumors, in men, the precise role of peroxisomes in the physiology of the cell, their link with the hypolipidemic action of some drugs and the development of cancer remain to be established.

An ultracentrifugation technique for isolation of the various lipoprotein fractions using a vertical rotor is described. By the use of a multiple discontinuous density gradient, very low density lipoproteins, low density lipoproteins and high density lipoproteins are sharply separated, without contamination of other lipoproteins or albumin. Centrifugation time is 80 min. The densities, electrophoretic mobilities, electron microscopic appearance and chemical composition are those of the expected classes of lipoproteins. Two different gradients are used to enhance the separation of very low density lipoproteins from low density lipoproteins on one hand and high density lipoproteins from infranatant on the other.

Intralipid is a fat emulsion which is widely used for intravenous nutrition in very ill patients. In order to know more about the capacity of these patients to metabolize exogenous triglycerides, the plasma activities of lipoprotein lipase (LPL), hepatic lipase (HL) and lecithin: cholesterol acyltransferase (LCAT), the key enzymes in the metabolism of serum lipoproteins were measured by a radioisotope technique in 23 critically ill patients and 20 patients with recent major surgery. Compared with normal volunteers, the activities were significantly decreased. On the other hand, the capacity to clear intravenously given Intralipid (0.1 g/kg), expressed as fractional removal rate (K2), was retained in patients. It is suggested that the measurement of K2 could not be useful to evaluate the capacity of Intralipid administration to satisfy the metabolic needs and also that its utilization must be reevaluated in terms of potential harmful effects.