Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) in a man with a diagnosis of X-linked reticulate pigmentary disorder (PDR)

Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare syndrome characterized by episodes of intestinal suboclusion caused by intestinal stenosis with superficial ulceration. Histological findings in the cases described in the literature are similar, even though they are not specific. The cause of the syndrome is as yet unknown. We report on the case of a 25-year-old male with a protein losing enteropathy (PLE) in the context of the CMUSE syndrome. At a later date the patient was also diagnosed with an X-linked recessive reticulate pigmentary disorder (PDR). The clinical features and tests which led to the diagnosis are described. The reported cases of CMUSE and of the gastrointestinal symptoms in the three families with PDR are reviewed in order to search for an association between these two entities. To date, there is no evidence to ascertain whether these two rare syndromes observed in the same patient are related or coincidental.     

Valor pronóstico a largo plazo del análisis completo de los índices de resonancia magnética cardiaca tras un infarto de miocardio con elevación del segmento ST

Introduction and objectives

A variety of cardiac magnetic resonance indexes predict mid-term prognosis in ST-segment elevation myocardial infarction patients. The extent of transmural necrosis permits simple and accurate prediction of systolic recovery. However, its long-term prognostic value beyond a comprehensive clinical and cardiac magnetic resonance evaluation is unknown. We hypothesized that a simple semiquantitative assessment of the extent of transmural necrosis is the best resonance index to predict long-term outcome soon after a first ST-segment elevation myocardial infarction.

Methods

One week after a first ST-segment elevation myocardial infarction we carried out a comprehensive quantification of several resonance parameters in 206 consecutive patients. A semiquantitative assessment (altered number of segments in the 17-segment model) of edema, baseline and post-dobutamine wall motion abnormalities, first pass perfusion, microvascular obstruction, and the extent of transmural necrosis was also performed.

Results

During follow-up (median 51 months), 29 patients suffered a major adverse cardiac event (8 cardiac deaths, 11 nonfatal myocardial infarctions, and 10 readmissions for heart failure). Major cardiac events were associated with more severely altered quantitative and semiquantitative resonance indexes. After a comprehensive multivariate adjustment, the extent of transmural necrosis was the only resonance index independently related to the major cardiac event rate (hazard ratio=1.34 [1.19-1.51] per each additional segment displaying >50% transmural necrosis, P<.001).

Conclusions

A simple and non-time consuming semiquantitative analysis of the extent of transmural necrosis is the most powerful cardiac magnetic resonance index to predict long-term outcome soon after a first ST-segment elevation myocardial infarction.Full English text available from: www.revespcardiol.org/en     

Cellular senescence determines endothelial cell damage induced by uremia

Renal dysfunction is closely associated with endothelial damage leading to cardiovascular disease. However, the extent to which endothelial damage induced by uremia is modulated by aging is poorly known. Aging can render endothelial cells more susceptible to apoptosis through an oxidative stress-dependent pathway. We examined whether senescence-associated to oxidative stress determines the injury induced by the uremia in endothelial cells.     

A phylogenetic study of hepatitis B virus in chronically infected Brazilian patients of Western and Asian descent

Background  Hepatitis B virus (HBV) causes one of the most important chronic viral infections worldwide. HBV is classified into eight genotypes whose epidemiology varies geographically. In Brazil, genotypes A, D, and F are more frequent, while in East Asia, genotypes B and C predominate. Several studies showed that immigrants retain the HBV infection pattern of their ancestral country. Purpose  To identify HBV genotypes infecting chronic carriers in Brazilian families of Western and Asian descent by Hepatitis B surface antigen gene sequencing and analyze the route of viral transmission by phylogenetic analysis of viral sequences. Methods  Eighty-seven people chronically infected with HBV were separated into two groups: Western descent (27) and Asian descent (60). Surface and pre-core/core genes were amplified from serum HBV-DNA and sequences were subjected to phylogenetic analysis. Results  HBV genotype A was found in 74% of Western subjects, while genotype C was found in 94% of Asian patients. Thirty-eight percent of Western families were infected with HBV with similar pre-core/core sequences, while only 25% of Asian families showed similarity in these sequences. Conclusions  Phylogenetical analysis of pre-core/core HBV gene suggested intra-familial transmission of HBV in 38% of Western families and 25% of Asian families. Analysis of HBsAg gene sequences helped to define the HBV genotype but did not allow inferring route of transmission as its sequences showed a smaller phylogenetic signal than pre-core/core sequences. Chronic HBV carriers of Asian descent born in or living in Brazil were infected with the same HBV genotype predominant in their ancestral country.     

Liver fibrosis in patients with chronic hepatitis C and persistently normal liver enzymes: influence of HIV infection

Summary. Liver fibrosis progress slowly in patients with chronic hepatitis C and persistently normal alanine aminotransferase (PNALT) compared to subjects with elevated aminotransferases. Differences in liver fibrosis according to human immunodeficiency virus (HIV) status in this population have not been examined. All patients with serum hepatitis C virus (HCV)‐RNA and PNALT who underwent liver fibrosis assessment using elastometry since 2004 at three different European hospitals were evaluated. Patients previously treated with interferon were excluded. PNALT was defined as ALT below the upper limit of normality in at least three consecutive determinations within the last 12 months. Fibrosis stage was defined as mild (Metavir F0–F1) if stiffness ≤7.1 kPa; moderate (F2) if 7.2–9.4 kPa; severe (F3) if 9.5–14 kPa, and cirrhosis (F4) if >14 kPa. A total of 449 HIV‐negative and 133 HIV‐positive patients were evaluated. HIV‐negative patients were older (mean age 51.8 vs 43.5 years) and more frequently females (63%vs 37%) than the HIV counterparts. Mean serum HCV‐RNA was similar in both the groups (5.9 vs 5.8 log IU/mL). Overall, 78.8% of the HIV patients were on HAART and their mean CD4 count was 525 (±278) cells/μL. In HIV‐negatives, liver fibrosis was mild in 84.6%; moderate in 8.7%, severe in 3.3% and cirrhosis was found in 3.3%. In HIV patients, these figures were 70.7%, 18.8%, 6%, and 4.5%, respectively. In the multivariate logistic regression analysis, older age (odds ratio or OR: 1.04; 95% confidence interval or CI: 1.02–1.07; P < 0.001) and being HIV+ (OR: 2.6; 95% CI: 1.21–5.85; P < 0.01) were associated with severe liver fibrosis or cirrhosis (F3–F4). Thus, severe liver fibrosis and cirrhosis are seen in 6.6% of the HCV‐monoinfected and in 10.5% of HCV‐HIV co‐infected patients with PNALT. Some degree of liver fibrosis that justifies treatment is seen in 15% of the HCV‐monoinfected but doubles to nearly 30% in HIV‐HCV co‐infected patients with PNALT.     

Comparison of the angiographic myocardial blush grade with delayed‐enhanced cardiac magnetic resonance for the assessment of microvascular obstruction in acute myocardial infarctions

Background: Both myocardial blush grade (MBG) and cardiac magnetic resonance (CMR) are imaging tools that can assess myocardial reperfusion after primary percutaneous coronary intervention (PCI) for acute myocardial infarction (AMI). Objectives: We studied the relation between MBG and gadolinium‐enhanced CMR for the assessment of microvascular obstruction (MVO) in patients with acute ST‐elevated myocardial infarction (STEMI) treated by primary PCI. Material and Methods: MBG was assessed in 39 patients with initial TIMI 0 STEMI successfully treated by PCI, resulting in TIMI 3 flow grade and complete ST‐segment resolution. These MBG values were related to MVO determined by CMR, performed between 2 and 7 days after PCI. Left ventricular (LV) volumes were determined at baseline and at 6‐month follow‐up. Results: No statistical relation was found between MBG and MVO extent at CMR (P = 0.63). Regarding MBG 0 and 1 as a sign of MVO, the sensitivity and specificity of these scores were 53.8 and 75%, respectively. In this study, CMR determined MVO was the only significant LV remodeling predicting factor (β = 31.8; P = 0.002), whatever the MBG status was. Conclusion: MBG underestimates MVO after an optimal revascularization in AMI compared with CMR. This study suggests the superior accuracy of delayed‐enhanced magnetic resonance over MBG for the assessment of myocardial reperfusion injury that is needed in clinical trials, where the principal endpoint is the reduction of infarct size and MVO. © 2009 Wiley‐Liss, Inc.     

Long-term successful percutaneous coronary intervention in factor VII deficiency

Factor VII deficiency (FVIId) is a congenital coagulation disorder with a wide spectrum of bleeding phenotypes. Percutaneous coronary intervention requires full anticoagulation during stent implantation to avoid acute coronary thrombosis and long-term dual antiplatelet therapy. Feasibility of percutaneous coronary intervention in FVIId is not described in literature. We present a successful case of percutaneous coronary intervention in a 55-year-old male with FVIId, discussing briefly the periprocedural handicaps (anticoagulation regimen and hemostasis at arterial puncture site) as the safety of long-term antiplatelet therapy, and future implications for recombinant FVIId administration in a patient with a previous coronary stent.