Orhan Ozbek

Blue Rubber Bleb Nevus Syndrome: Successful Treatment With Sirolimus

Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder with characteristic vascular malformations of the skin, gastrointestinal system, and, less often, other organ systems. The characteristic cutaneous lesions consist of deep-blue, soft, rubbery blebs, which are easily compressible. The most serious complication is abundant gastrointestinal bleeding. We describe the case of an 8-year-old girl with diagnosed BRBNS who had multiple venous malformations all over her body, importantly, throughout the gastrointestinal tract, mouth, esophagus, stomach, small bowel, and colon. She presented with recurrent massive gastrointestinal bleeding and soft tissue hematoma despite prednisolone and α-interferon therapy. We started low-dose sirolimus as an antiangiogenic agent. The vascular masses were reduced rapidly and there was no gastrointestinal bleeding and muscular hematoma after sirolimus therapy. There was no drug adverse reaction at 20-month follow-up. To the best of our knowledge, this is the first report related to the use of sirolimus in a patient with BRBNS.

The Relationship between Oxidative Stress, Inflammation, and Atherosclerosis in Renal Transplant and End-Stage Renal Disease Patients

Objectives: Cardiovascular risk is increased in the early stages of chronic kidney disease (CKD) and is also found to be ongoing in renal transplant (Rtx) patients. As a sign of atherosclerosis, increased carotid intima–media thickness (CIMT) has been widely accepted as a strong predictor of cardiovascular disease (CVD) and mortality in the end-stage renal disease (ESRD) patients. Ischemia-modified albumin (IMA), pentraxin-3 (PTX-3), and neutrophil-to-lymphocyte ratio (NLR) were introduced as oxidative stress and inflammatory biomarkers in ESRD. The role of Rtx in terms of atherogenesis, oxidative stress, and inflammation is still unclear. We aimed to investigate the relationship between IMA, PTX-3, NLR, and CIMT in Rtx patients without overt CVD and to compare these results with those obtained from healthy subjects and ESRD patients receiving hemodialysis (HD) and peritoneal dialysis (PD). Design and methods: Cross-sectional analysis in which CIMT measurements, NLR, and serum PTX-3 and IMA levels were assessed in 18 Rtx patients (10 females; mean age: 40.0 ± 13.3 years), 16 PD patients (7 females; 40.2 ± 12.9 years), 14 HD patients (8 females; 46.6 ± 10.7 years), and 19 healthy subjects (9 females; 36.9 ± 8.9 years). Results: IMA, PTX-3, and high-sensitive C-reactive protein (hs-CRP) levels, NLR, and CIMT of Rtx patients were found to be significantly higher compared with healthy subjects ( p = 0.04, p < 0.0001, p < 0.005, p = 0.005, and p = 0.005, respectively). IMA level was positively correlated with hs-CRP and PTX-3 levels, NLR, and CIMT when all participants were included (r = 0.338, p = 0.005; r = 0.485, p < 0.0001; r = 0.304, p = 0.013; and r = 0.499, p < 0.0001, respectively). Conclusion: There has been ongoing inflammation, oxidative stress, and atherosclerosis in Rtx patients.

The Relationship between Epicardial Adipose Tissue and Malnutrition, Inflammation, Atherosclerosis/Calcification Syndrome in ESRD Patients

BACKGROUND AND OBJECTIVES Malnutrition, inflammation, atherosclerosis/calcification (MIAC) and endothelial dysfunction are the most commonly encountered risk factors in the pathogenesis of cardiovascular disease in ESRD patients. Epicardial adipose tissue (EAT) is the true visceral fat depot of the heart. The relationship between CAD and EAT was shown in patients with high risk of coronary artery disease. In this study, we aimed to investigate the relationship between EAT and MIAC syndrome in ESRD patients. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS Eighty ESRD patients and 27 healthy subjects enrolled in this cross-sectional study. EAT and coronary artery calcification score were measured by a multidetector computed tomography (MDCT) scanner. Patients with serum albumin <3.5 mg/dl were defined as patients with malnutrition; those with serum C-reactive protein level >10 ng/dl (normal range, 0-5 ng/dl) had inflammation; and those with CACS >10 had atheroscleosis/calcification. RESULTS Total CACS and EAT measurements were significantly higher in ESRD patients when compared with healthy subjects. There was a statistically significant relationship between EAT and CACS in ESRD patients (r = 0.48). EAT measurements were higher in PD patients than HD patients. Twenty-four of the patients had no component, 31 had one component, 17 had two components, and nine had all of the MIAC components. EAT was found to be significantly increased when the presence of MIAC components increased. EAT was positively correlated with age, body mass index, and presence of MIAC. These parameters were also found as independent predictors of increased EAT. CONCLUSIONS We found a relationship between EAT and components of MIAC syndrome in ESRD patients.

The Relationship between Epicardial Adipose Tissue and Coronary Artery Calcification in Peritoneal Dialysis Patients

Background: Atherosclerosis, endothelial dysfunction, coronary artery calcification (CAC), and left ventricular hypertrophy are the most commonly encountered risk factors in the pathogenesis of cardiovascular disease in end-stage renal disease patients. Epicardial adipose tissue (EAT) is the true visceral fat depot of the heart. The relationship between coronary artery disease and EAT has been shown in healthy subjects and patients with a high risk of coronary artery disease. In the present study, we aimed to investigate the relationship between EAT and CAC in peritoneal dialysis (PD) patients. Patients and Methods: Forty-five PD patients (18 females, 27 males, with a mean age of 50.6 ± 15 years) and 25 healthy subjects (12 females, 13 males, with a mean age of 52.4 ± 10.7 years) were enrolled in the study. EAT and CAC score (CACS) measurements were performed by a multidetector computed tomography scanner. Results: EAT of the PD patients was significantly higher than that of the healthy subjects (p = 0.02). When patients were divided into two subgroups (group 1: CACS ≤10, n = 20; group 2: CACS >10, n = 25), EAT was also significantly higher in group 2 patients than in group 1 patients and healthy subjects. Age and EAT were also found to be correlated with CACS ≧10. Conclusion: There is a relationship between the anatomic assessment of coronary artery lesions by multidetector computed tomography and EAT in PD patients. This relationship might be attributed to increased inflammation and proinflammatory cytokines in uremic patients.

Epicardial Adipose Tissue and Coronary Artery Calcification in Diabetic and Nondiabetic End-Stage Renal Disease Patients

Background/aims: Atherosclerosis, coronary artery calcification, diabetes mellitus, inflammation, endothelial dysfunction, and left ventricular hypertrophy are the most commonly encountered risk factors in the pathogenesis of cardiovascular disease in end-stage renal disease (ESRD) patients. Epicardial adipose tissue (EAT) is the true visceral fat depot of the heart. The relationship between coronary artery disease (CAD) and EAT was shown in healthy subjects and patients with high risk of CAD. To date, there is not enough data about EAT in diabetic and nondiabetic ESRD patients. Therefore, we aimed to investigate the EAT and coronary artery calcification score (CACS) in diabetic and nondiabetic ESRD patients and healthy subjects. Methods: Sixty ESRD patients (17 diabetic, 43 nondiabetic ESRD patients) and 20 healthy subjects were enrolled in the study. EAT and CACS were performed by a 64-slice multidetector computed tomography scanner. Results: There were no differences in age, gender, body mass index, predialysis systolic and diastolic blood pressure levels, biochemical parameters including serum low-density lipoprotein and high-density lipoprotein cholesterol, triglycerides, and C-reactive protein between healthy subjects, diabetic, and nondiabetic ESRD patients. Total CACSs and EAT measurements were significantly higher in diabetic ESRD patients when compared with nondiabetic ESRD patients and healthy subjects. There was statistically significant relationship between EAT and CACS in ESRD patients (p < 0.0001, r = 0.48). Conclusion: In conclusion, we found a significant increase in terms of EAT and CACS in diabetic ESRD patients when compared with nondiabetic ESRD patients and healthy subjects.

Soluble TWEAK independently predicts atherosclerosis in renal transplant patients

BackgroundCardiovascular risk is increased in the early stages of chronic kidney disease (CKD) and also found to be ongoing in renal transplant (Rtx) patients. As a sign of atherosclerosis, increased carotid intima-media thickness (CIMT) has been widely accepted as a strong predictor of cardiovascular disease (CVD) and mortality in CKD patients. A novel markers, soluble tumor necrosis factor-like weak inducer of apoptosis (sTWEAK) and neutrophil-to-lymphocyte ratio (NLR) were introduced as potential markers in inflammatory disorders including CKD. The role of Rtx in terms of atherogenesis is still unclear. We aimed to investigate the relationship between sTWEAK, NLR and CIMT in Rtx patients without overt CVD and to compare these results with those obtained from healthy subjects.MethodsCross-sectional analysis in which CIMT measurements, NLR and serum TWEAK levels were assessed in 70 Rtx patients (29 females; mean age, 40.6 ± 12.4 years) and 25 healthy subjects (13 females, mean age; 37.4±8.8 years).ResultssTWEAK levels were significantly decreased (p=0.01) and hs-CRP, NLR and CIMT levels of Rtx patients were significantly increased compared to healthy subjects (p<0.0001, p=0.001, p<0.0001, respectively). sTWEAK was also found to be decreased when eGFR was decreased (p=0.04 between all groups). CIMT was positively correlated with sTWEAK and NLR in Rtx patients (r=0.81, p<0.0001 and r=0.33, p=0.006, respectively). sTWEAK was also positively correlated with NLR (r=0.37, p=0.002). In the multivariate analysis only sTWEAK was found to be an independent variable of increased CIMT.ConclusionsTWEAK might have a role in the pathogenesis of ongoing atherosclerosis in Rtx patients.

Complementary use of NBCA with the Amplatzer vascular plug for embolization of a high-flow traumatic hepatic arteriovenous fistula.

Arterioportal fistula (APF) is a rare vascular disorder and may cause severe complications such as portal hypertension. APF may be congenital, posttraumatic, or iatrogenic. Today, transarterial embolization is being accepted as the first choice in the treatment of these lesions. We presented a traumatic fistula between the hepatic artery (HA), the portal vein (PV), and the hepatic vein (HV) which was diagnosed by multidetector computed tomography (MDCT) and treated endovascularly using the Amplatzer Vascular Plug (AVP) and N-butyl-cyanoacrylate (NBCA).

The Association between Myocardial Iron Load and Ventricular Repolarization Parameters in Asymptomatic Beta-Thalassemia Patients

Previous studies have demonstrated impaired ventricular repolarization in patients with β-TM. However, the effect of iron overload with cardiac T2* magnetic resonance imaging (MRI) on cardiac repolarization remains unclear yet. We aimed to examine relationship between repolarization parameters and iron loading using cardiac T2* MRI in asymptomatic β-TM patients. Twenty-two β-TM patients and 22 age- and gender-matched healthy controls were enrolled to the study. From the 12-lead surface electrocardiography, regional and transmyocardial repolarization parameters were evaluated manually by two experienced cardiologists. All patients were also undergone MRI for cardiac T2* evaluation. Cardiac T2* score <20 msec was considered as iron overload status. Of the QT parameters, QT duration, corrected QT interval, and QT peak duration were significantly longer in the β-TM group compared to the healthy controls. T p − T e and T p − T e dispersions were also significantly prolonged in β-TM group compared to healthy controls. (T p − T e)/QT was similar between groups. There was no correlation between repolarization parameters and cardiac T2* MRI values. In conclusion, although repolarization parameters were prolonged in asymptomatic β-TM patients compared with control, we could not find any relation between ECG findings and cardiac iron load.

The predictive value of CRP levels on future severe renal disease in overweight and obese subjects without diabetes mellitus and hypertension.

Background:Obesity and related disorders have a high prevalence all over the world. Increased C-reactive protein (CRP) value in obese individuals and its potential adverse effects have been reported. Here we have investigated the relationship between CRP levels and renal functions in nondiabetic, nonhypertensive, overweight, and obese individuals. The aim of this study was to evaluate the predictive value of CRP levels on future severe renal disease. Methods:One hundred sixty individuals were included in the study. They were grouped as normal weight, overweight, and obese. Anthropometric measurements, renal function tests, and serum hsCRP values were obtained. Mean values were compared and correlation analysis was performed. Results:Significant differences were detected between the groups according to body mass index, waist circumference (WC), and body fat percentage. There was a significant difference with respect to creatinine clearance (CC). Difference in the mean urinary albumin excretion (UAE) was significant between normal-weight and overweight subjects. There was a linear increase in serum CRP values in parallel to the increase in body weight; mean values were significant between groups. A positive correlation was detected between CC and body mass index and WC, and there were significant correlations between CRP and anthropometric measurements, CC and UAE. Conclusions:This study showed that increased CRP levels in nondiabetic, nonhypertensive, overweight, and obese individuals could possibly associated with impaired renal functions that might be originating from endothelial dysfunction. Determination of cutoff levels of CRP, as in cardiovascular diseases, may be useful for early estimation and prevention of renal diseases.

The relationship between atherogenic index of plasma and epicardial adipose tissue in hemodialysis and peritoneal dialysis patients

Abstract Introduction: Epicardial adipose tissue (EAT) is the true visceral fat depot of the heart. The relationship between coronary artery disease and EAT was shown in end-stage renal disease (ESRD) patients. One of the established risk factor in this population is dyslipidemia. We aimed to determine the relationship between atherogenic index of plasma (AIP) and EAT in ESRD patients. Methods: This was a cross-sectional study involving 76 ESRD patients receiving PD or HD for ≥6 months and 42 healthy subjects. EAT was measured by using an electrocardiogram-gated 64-multidetector computed tomography (MDCT). Atherogenic index of plasma was calculated as the logarithmically transformed ratio of the serum trigliseride to HDL-cholesterol. Results: The etiology of ESRD patients was diabetic nephropathy (n = 16), chronic glomerulonephritis (n = 10), hypertensive nephropathy (n = 23), polycystic kidney disease (n = 7), nephrolithiasis (n = 5) and unknown (n = 15). There were no differences with respect to the following variables between ESRD patients and healthy subjects: age; sex; BMI; predialysis levels of DBP; serum levels of albumin, HDL-cholesterol and hemoglobin. However, ESRD patients had higher serum levels of trigliseride, hs-CRP and AIP when compared to healthy subjects. There was a statistically significant relationship between EAT, BMI and AIP in ESRD patients (r = 0.42, p < 0.001 and r = 0.25, p = 0.028, respectively). The stepwise linear regression analysis revealed that age, as well as BMI were independent predictors of EAT. Conclusion: We found a relationship between EAT as defined by MDCT and AIP in ESRD patients. Further clinical and experimental studies are needed.