Ertuğrul Erken

Assessment of myocardial repolarisation parameters in patients with familial Mediterranean fever

Summary Background: Familial Mediterranean fever (FMF) is a chronic, recurrent auto-inflammatory disease characterised by self-terminating attacks of fever and sterile polyserositis. The main cause of death in auto-inflammatory diseasesis cardiovascular events. Additionally, auto-inflammatory diseases have potential effects on the myocardial repolarisation parameters, including the T-wave peak-to-end (Tp-Te) interval, cTp-Te interval (corrected Tp-Te) and the cTp-Te/ QT ratio. The aim of this study was to analyse the efficacy of myocardial repolarisation alterations in anticipation of cardiovascular risks in patients with FMF. Methods: This study included 66 patients with FMF and 58 healthy control subjects. Tp-Te and cTp-Te intervals and the cTp-Te/QT ratio were measured from the 12-lead electrocardiogram. Results: In electrocardiographic parameters, analysis of QT, QT dispersion, corrected QT (QTc) and QTc dispersion were similar between the groups. The Tp-Te and cTp-Te intervals and Tp-Te/QT and cTp-Te/QT ratios were significantly prolonged in FMF patients. Multivariate linear regression analyses indicated that erythrocyte sedimentation rate was an independent predictor of a prolonged cTp-Te interval. Conclusions: Our study revealed that when compared with control subjects, Tp-Te and cTp-Te intervals and cTp-Te/QT ratio were increased in FMF patients.

A RARE PRESENTATION OF CYTOMEGALOVIRUS INFECTION IN A RENAL TRANSPLANT RECIPIENT: PNEUMONIA ACCOMPANIED BY AORTIC ANEURYSM INFECTION/DISSECTION

DOI : 10.26650/IUITFD.397135 Sitomegalovirus (CMV) enfeksiyonu, bobrek nakli alicilari icin onemli bir morbidite ve mortalite nedeni olabilmektedir. Genellikle CMV sendromu olarak adlandirilan bir klinik tabloya yol acmakla birlikte, invaziv doku tutulumuna da yol acabilmektedir. En cok tutulan organlar akcigerler ve gastrointestinal sistemdir. Kardiyovaskuler tutulum ise nadirdir ve siklikla ateroskleroz ve transplant arter stenozu ile birliktedir. Bu yazida; bobrek nakli alicisinda CMV enfeksiyonu ile iliskili aort anevrizmasi enfeksiyonu/diseksiyonu ve eslik eden pnomoni nedeniyle takip ettigimiz nadir bir olguyu sunmayi amacladik.

Periton Diyalizi Hastalarının Periton Sıvısında Trefoil Faktör 3 Peptid Düzeyleri ve İlişkili Faktörler

OBJECTIVE: TFF3 is a small peptide hormone secreted from mucous producing cells and many epithelial cells. TFF3 inhibits apoptosis, promotes migration and facilitates restoration against injury. In our cross-sectional study, TFF3 levels in peritoneal fluids of peritoneal dialysis (PD) patients were measured and associated factors were investigated. MATERIAL and METHODS: Peritoneal fluid after a 12-hour dwell and concurrent serum samples of 48 chronic PD patients were collected. Serum and peritoneal fluid TFF3 levels were measured by ELISA. The SPSS15.0 package was used for statistical analysis of database files. RESULTS: The study included 48 patients (men/women; 24/24, mean age: 51.6∓13.9 years) with a median PD vintage of 43 months (3-200). Median effluent TFF3 level was 17.07 ng/ml (2.38-99.4). There was no relationship between the number of peritonitis episodes and TFF3 levels. There was a positive correlation between effluent TFF3 levels and PD vintage (r=0.349, p<0.015). There was also a positive correlation between effluent TFF3 and serum PTH. Median serum TFF3 was 1.56 ng/ml (0.7911.05). There was no association between serum TFF3 levels and clinical features. After multivariate analysis, the only association was between effluent TFF3 and PD vintage. CONCLUSION: Effluent TFF3 levels increasing with dialysis vintage may be related to local production or peritoneal transport.

Cardiac disease in familial Mediterranean fever

Familial Mediterranean fever (FMF) is an autoinflammatory disease manifested by inflammatory attacks of peritonitis, pleuritis, pericarditis accompanied by fever and arthritis. Mutations of MEFV gene results in pyrin dysfunction, which causes uncontrolled interleukin-1 beta production and triggers the inflammatory attacks. Inflammation persists even during attack-free periods in one-third of the FMF patients. Findings of elevated proinflammatory cytokine patterns during remission as well as inflammatory attacks indicate the continuous subclinical disease activity and inflammation. Chronic inflammation was thought to be related to the cardiovascular risk in FMF patients. Main cardiac manifestations reported in FMF are pericarditis, idiopathic recurrent pericarditis, pericardiac tamponade, coronary heart disease and abnormal cardiovascular reactivity. Cardiac involvement in FMF may often be related to secondary AA amyloidosis. Deposition of amyloid may lead to cardiovascular morbidity and mortality in FMF patients. Associations of several vasculitic disorders such as Immunoglobulin A-associated vasculitis, polyarteritis nodosa and Behcet’s disease are common in FMF. Appropriate prophylactic treatment with colchicine is recommended to prevent from cardiovascular risks. For those resistant to colchicine, IL-1 inhibitor agents can be used. Associated vasculitis should be treated with immunosuppressive agents. This review article aims to compile information about cardiac disease in FMF and refer to recent studies on the topic.

MANNOSE BINDING LECTIN (MBL) GENE POLYMORPHISMS AND THEIR RELATIONS WITH CLINICAL FEATURES IN PATIENTS WITH FAMILIAL MEDITERRANEAN FEVER (FMF)

Amac: Kompleman aktivasyonunun lektin yolaginda rol oynayan ve dogal immun sistemin bir parcasi olan mannoz baglayici lektin (MBL), cesitli patojenlerin mannan gruplarinin uyarisi ile aktive olur. MBL genindeki bazi polimorfizmler (orn. kodon 52, kodon 54 polimorfizmleri) MBL’nin serum duzeylerinde degisikliklere yol acarak, infeksiyon hastaliklarina yatkinliga neden olabildigi gibi, bazi otoimmun ve inflamatuvar hastaliklarin patogenezine de katkida bulunabilir. Bu calismada, ailesel Akdeniz atesi (AAA) hastalarinda MBL geni kodon 52 ve kodon 54 polimorfizmlerinin sikligini ve basta sekonder amiloidoz olmak uzere, hastaligin klinik ozellikleri ile olasi iliskilerini arastirmayi amacladik. Gerec ve Yontem: Yuzelli-yedi AAA hastasinda ve hastalarla akrabalik iliskisi olmayan benzer demografik dagilimdaki 150 saglikli kontrolde MBL genindeki R52C C>T ve G54D G>A polimorfizmleri sekanslama yontemi ile arastirildi. AAA hastalarinin MEFV geni analizleri, klinik ozellikleri ve ataksiz donemdeki serum CRP duzeyleri kaydedildi. Genetik sonuclar ile klinik ve laboratuvar bulgular arasindaki olasi iliskiler incelendi. Bulgular: MBL geni R52C C>T polimorfizmi hastalarin %12,7’sinde, kontrol grubunun %10,6’sinda saptandi. G54D G>A polimorfizmi hastalarin %26,8’sinde, kontrollerin %26,7’sinde saptandi. Polimorfizm sikligi acisindan, iki grup arasinda anlamli fark bulunamadi (p=0,79 ve 0,98). Incelenen MBL geni polimorfizmleri ile hastalarin cesitli klinik ozellikleri (or. amiloidoz, ates, kolsisin yaniti, MEFV mutasyonlari) arasinda anlamli iliski bulunamadi. AAA hastalarinin ortalama CRP degeri 4,90±6,72 mg/dL olup, ataksiz donemde serum CRP duzeyi normalden yuksek (>0,8 mg/dL) olan hastalarda MBL kodon 52 polimorfizmi sikligi %25,2, kodon 54 polimorfizmi sikligi %14,8 oraninda bulundu. AAA hastalarinda yuksek CRP duzeyine gore kodon 52 ve kodon 54 polimorfizmi sikliklari farkli bulunmadi (p=0,399). AAA hastalarinda M694V mutasyonu ile amiloidoz arasinda (p=0,002) ve M694I mutasyonu ile kolsisin direnci arasinda (p=0,016) anlamli iliski saptandi. Sonuc: AAA hastalarin ataksiz donemdeki CRP duzeyleri ve tasidiklari klinik ozellikler ile MBL polimorfizmleri arasinda anlamli iliski bulunamamasi, AAA hastalarinin proinflamatuvar durumda oldugunu ve MBL aracili mekanizmalarin bu sureclere katkisinin olmadigini dusundurmektedir. Olgularimizda M694I ile kolsisin direnci arasinda anlamli iliski saptanmis olmasi dikkate deger bir bulgudur. Yine olgularimizda M694V ile amiloidoz arasinda anlamli iliski bulunmasi onceki literatur bulgulari ile uyumludur ve M694V’nin hastalik siddeti ve prognozu icin onemli oldugu gorusunu desteklemektedir.

Acute Kidney Injury After Scorpion Sting

Scorpion sting cases are mostly seen in the southern provinces in our country. Scorpion venom might comprise neurotoxic, cardiotoxic and even nephrotoxic contents. Occurrence of nephropathy is a rare complication of scorpion sting. A 27-year-old male was admitted with acute kidney injury after scorpion sting. The etiology was probably associated with hypovolemia. key words: Scorpion, Sting, Acute kidney injury

Aortic Flow Propagation Velocity in Patients with Familial Mediterranean Fever: an Observational Study

Background and Objectives Systemic inflammation has an important role in the initiation of atherosclerosis, which is associated with arterial stiffness (AS). Aortic flow propagation velocity (APV) is a new echocardiographic parameter of aortic stiffness. The relationship between systemic inflammation and AS has not yet been described in patients with familial Mediterranean fever (FMF). We aimed to investigate the early markers of AS in patients with FMF by measuring APV and carotid intima-media thickness (CIMT). Subjects and Methods Sixty-one FMF patients (43 women; mean age 27.3±6.7 years) in an attack-free period and 57 healthy individuals (36 women; mean age 28.8±7.1 years) were included in this study. The individuals with atherosclerotic risk factors were excluded from the study. The flow propagation velocity of the descending aorta and CIMT were measured to assess AS. Results APV was significantly lower (60.2±16.5 vs. 89.5±11.6 cm/sec, p<0.001) and CIMT was significantly higher (0.49±0.09 vs. 0.40±0.10 mm, p<0.001) in the FMF group compared to the control group. There were significant correlations between APV and mean CIMT (r=-0.424, p<0.001), erythrocyte sedimentation rate (ESR) (r=-0.198, p=0.032), and left ventricle ejection fraction (r=0.201, p=0.029). APV and the ESR were independent predictors of FMF in logistic regression analysis (OR=-0.900, 95% CI=0.865-0.936, p<0.001 and OR=-1.078, 95% CI=1.024-1.135, p=0.004, respectively). Mean CIMT and LVEF were independent factors associated with APV in linear regression analysis (β=-0.423, p<0.001 and β=0.199, p=0.017, respectively). Conclusion We demonstrated that APV was lower in FMF patients and is related to CIMT. According to our results, APV may be an independent predictor of FMF.

Evaluation of cisterna chyli diameter with MRI in patients with chronic kidney disease

To evaluate cisterna chyli (CC) diameter with magnetic resonance imaging (MRI) in patients with chronic kidney disease (CKD).

Single nucleotide polymorphism of adiponectin +276 G/T is associated with the susceptibility to essential hypertension in a Turkish population

ABSTRACT Background: It is well known that arterial stiffness is associated with hypertension. Recent studies have shown that adiponectin +276 G/T, ACE I/D, AGTR1 A1166C, and eNOS E298D polymorphisms are likely to be risk factors for arterial stiffness. In this study, we aimed to investigate possible associations between these single-nucleotide polymorphisms (SNPs) and essential hypertension in a Turkish population. Methods: The study population consisted of 170 patients who were diagnosed with essential hypertension and 170 sex- and age-matched controls. Genotyping of adiponectin +276 G/T, ACE I/D, AGTR1 A1166C, and eNOS E298D SNPs were performed using real-time polymerase chain reaction and commercially produced kits. Results: The percentage of the adiponectin +276 T allele carriers was significantly higher in the patients with hypertension (33%) than in the controls (25%, p < 0.011). Through multiple logistic regression analysis, the adiponectin +276 T allele carrier was found to be associated with an increased risk of hypertension (TT vs. GG and TG: odds ratio = 3.318, p = 0.014, 95% confidence interval: 1.269–8.678). The genotype distributions or allelic frequencies of ACE I/D, AGTR1 A1166C, and eNOS E298D SNPs did not significantly differ between the patients with hypertension and the controls. Conclusion: The present study demonstrated that the adiponectin +276 G/T SNP is likely to be a risk factor for essential hypertension in a Turkish population.

Killer Cell Immunoglobulin-Like Receptor (KIR) Genotype Distribution in Familial Mediterranean Fever (FMF) Patients

Background Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disease predominantly affecting Mediterranean populations. The gene associated with FMF is the MEFV gene, which encodes for a protein called pyrin. Mutations of pyrin lead to uncontrolled attacks of inflammation, and subclinical inflammation continues during attack-free intervals. Killer cell immunoglobulin-like receptor (KIR) genes encode HLA class I receptors expressed by NK cells. The aim this study was to look for immunogenetic determinants in the pathogenesis of FMF and find out if KIR are related to susceptibility to disease or complications like renal amyloidosis. Material/Methods One hundred and five patients with FMF and 100 healthy individuals were involved in the study. Isolated DNA from peripheral blood was amplified by sequence specific PCR probes and analyzed by Luminex for KIR genotypes. Fisher Exact test was used to evaluate the variation of KIR gene distribution. Results All patients and healthy controls expressed the framework genes. An activator KIR gene, KIR2DS2, was significantly more frequent in FMF patients (p=0.036). Renal amyloidosis and presence of arthritis were not associated with KIR genes and genotype. KIR3DL1 gene was more common in patients with high serum CRP (p=0.016). Conclusions According to our findings, we suggest that presence of KIR2DS2, which is an activator gene for NK cell functions, might be related to the autoinflammation in FMF. The potential effect of KIR genes on amyloidosis and other clinical features requires studies with larger sample sizes.