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Objective: Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid metabolism which arises due to deficient or absent activity of lysosomal α-galactosidase A (α-Gal A). This may be associated with increased left ventricular (LV) wall thickness and may mimic the morphological features of hypertrophic cardiomyopathy. The purpose of this study was to define the ratio of occurrence of FD to the manifestation of unexplained left ventricular hypertrophy (LVH). Methods: We studied a prospectively assembled a consecutive cohort of 190 patients with unexplained LVH on echocardiography. The criterion for LVH diagnosis was a maximum LV wall thickness of 13 mm or greater. All patients were tested for mutations in the GLA gene. Results: The majority of patients were male (n=119, 63%) and the mean patient age was 47.2+-15 years. In 190 patients diagnosed with LVH, we identified 2 patients (1.05%) with documented GLA mutations [c.427G>A (p.A143T)(p.Ala143Thr)] and [c.937G>T (p.D313Y)(p.Asp313Tyr)]. After the family screening, 3 additional patients with FD were identified in 2 families, including 5 individuals who are now receiving enzyme replacement therapy. Conclusion: We identified 2 index patients with FD and unexplained LVH. Cardiologists should, therefore, be aware of FD in cases of unexplained LVH. Family screening is crucial for the earlier identification of unaffected new patients who may benefit from enzyme replacement therapy.

Background and Aims Anemia is one of the most important complications in chronic renal disease and inadequate erythropoietin production is the most important reason for anemia. Fibrosis of renal interstitium may be associated with inadequate synthesis of erythropoietin and anemia. In our study, we investigated the relationship between the severity of interstitial fibrosis (IF) in patients with primary glomerulonephritis (PGN) and anemia parameters. Method A total of 2794 patients who were recorded to the database of the Turkish Society of Nephrology, Glomerular Diseases (TSN-GOLD) Working Group between May 2009 and June 2019 were included in our national multicenter (44 centers) study. Patients aged 16 years or more with documented biopsy were included in the study. Patients were divided into four groups according to IF severity. IF was not detected in the interstitial area, was defined as none, if fibrosis was present in less than 25% of the area, defined as mild, if fibrosis was present in 25-50%, defined as moderate and if more than 50%, defined as severe. Anemia was defined as hemoglobin Results In the study, 57% of the patients were male and their mean age was 41.0 ± 14.3 years. The mean eGFR was 82.7 ± 36.9 ml / min / 1.73 m2 and the hemoglobin level was 13.1 ± 1.9 g/dl. General characteristics of patients with and without anemia are given in Table 1. While 41.1% of patients had no IF, 41.2% had mild, 14.1% had moderate and 3.5% had severe IF. When the patients were divided into four groups according to the severity of IF, the mean hemoglobin and hematocrit levels were significantly different between the groups (Figure 1). Anemia was present in 960 patients (34.4%) (Figure 2). Anemia rate was significantly higher in the patients with IF than patients without IF (36.8% vs 30.9%, p = 0.001). Anemia rate was %25.1 in patients with eGFH≥60 ml/min/1.73m2 and 56% in patients with eGFR Conclusion Approximately one-third of PGN patients had anemia at the time of biopsy and the frequency of anemia increases in patients with IF. The presence of IF is an independent risk factor associated with the development of anemia in PGN. The elucidation of the etiopathogenesis of anemia in PGN patients may play an important role in the follow-up and treatment of patients. Studies on IF can play a critical role.

Background and Aims The largest data on the epidemiology of primary glomerular diseases (PGD) are obtained from the databases of countries or centers. Here, we presented the extended results of the Primary Glomerular Diseases Study of the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD)Working Group. Method The data of patients who underwent renal biopsy and diagnosed as primary glomerular disease were recorded in the database prepared for the study. Between May 2009 and May 2019, a total of 4399 patients from 47 centers were evaluated. Basal data of 3875 patients were analyzed after exclusion of those lacking light microscopy and immunofluorescence findings. Results The mean age was 41.5 ± 14.9 years. Of the patients, 1690 were female (43.6%) and 2180 (56.3%) were male. Nephrotic syndrome was the most common biopsy indication (51.7%). This was followed by asymptomatic urinary abnormalities (18.3%) and nephritic syndrome (17.8%). The most common PGH was IgA nephropathy (25.7%), followed by membranous nephropathy (25.6%) and FSGS (21.9%). The mean total number of glomeruli per biopsy was 17 ± 10. Mean baseline systolic blood pressure was 130 ± 20 mmHg and diastolic blood pressure was 81 ± 12 mmHg. Median proteinuria was 3300 (IQR: 1467-6307) mg / day, mean serum creatinine, estimated GFR and albumin values were 1.4 ± 1.5 mg / dl, 80.7 ± 39.1 ml / min and 3.2 ± 0.9 g / dl, respectively. Conclusion In Turkey, the incidence of IgA nephropathy patients have become more common than membranous nephropathy among PGD patients diagnosed with renal biopsy.

Background The largest data on the epidemiology of primary glomerular diseases (PGDs) are obtained from the databases of countries or centers. Here, we present the extended results of the Primary Glomerular Diseases Study of the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) Working Group. Methods Data of patients who underwent renal biopsy and received the diagnosis of PGD were recorded in the database prepared for the study. A total of 4399 patients from 47 centers were evaluated between May 2009 and May 2019. The data obtained at the time of kidney biopsy were analyzed. After the exclusion of patients without light microscopy and immunofluorescence microscopy findings, a total of 3875 patients were included in the study. Results The mean age was 41.5 ± 14.9 years. 1690 patients were female (43.6%) and 2185 (56.3%) were male. Nephrotic syndrome was the most common biopsy indication (51.7%). This was followed by asymptomatic urinary abnormalities (18.3%) and nephritic syndrome (17.8%). The most common PGD was IgA nephropathy (25.7%) followed by membranous nephropathy (25.6%) and focal segmental glomerulosclerosis (21.9%). The mean total number of glomeruli per biopsy was 17 ± 10. The mean baseline systolic blood pressure was 130 ± 20 mmHg and diastolic blood pressure was 81 ± 12 mmHg. The median proteinuria, serum creatinine, estimated GFR, and mean albumin values were 3300 (IQR: 1467–6307) mg/day, 1.0 (IQR: 0.7–1.6) mg/dL, 82.9 (IQR: 47.0–113.0) mL/min and 3.2 ± 0.9 g/dL, respectively. Conclusions The distribution of PGDs in Turkey has become similar to that in other European countries. IgA nephropathy diagnosed via renal biopsy has become more prevalent compared to membranous nephropathy.

When total correction is not possible in infants who have a cyanotic congenital heart disease, creation of a palliative aortopulmonary shunt is essential. A central aortopulmonary shunt is preferable, because of its technical and hemodynamic advantages. Overcirculation, thrombosis, and stenosis of the shunt are the main postoperative sequelae that necessitate urgent reintervention. Percutaneous transcatheter closure of aortopulmonary shunts can eliminate the need for reoperation and substantially decrease postoperative morbidity and mortality rates. We report our successful transcatheter closures of central aortopulmonary shunts in a 3-month-old infant and a 15-year-old girl, with use of an Amplatzer Duct Occluder II and an Amplatzer Vascular Plug I, respectively. To our knowledge, this is the first report of the transcatheter closure of central aortopulmonary shunts with these 2 devices.

WOS: 000429628000003 PubMed ID: 29578201 Objective: To report clinical and procedural characteristics of twelve patients who received a covered stent for the treatment of aortic coarctation and concurrent patent ductus arteriosus (PDA). Methods: A single center database was retrospectively evaluated to obtain data of patients with combined aortic coarctation and PDA. We selected patients in whom a covered stent was used for the treatment of both pathologies. The stent length was chosen so as to cover the entire length of the lesion from healthy to healthy tissue and also cover the ampulla of PDA. Results: The median age of the patients was 15 (range, 6.5-35) years. The diameter of the coarctated segment increased from a median of 8.4 (range, 2.6-10.8) mm to 16 (range, 9-24) mm (p

Background: One of the most important side effects of contrast pharmaceutical agents, which are used very common in routine radiology practice, is contrast induced nephropathy. Even ischemia, oxidative stress and osmolality related cytotoxic effects are considered, the molecular mechanisms underlying this pathology have not been identified completely yet. Objectives: The aim of the current study was to reveal the role of oxidative stress and antioxidant enzymatic defence mechanisms in the aetiopathogenesis of contrast-induced nephropathy. We also studied possible alleviating effects of N-acetylcysteine (NAC), a potent antioxidant, to obtain extra information regarding the molecular mechanisms underlying this pathology. Materials and Methods: This is an clinical-experimental study, This study was conducted of Istanbul/Turkey between September 15, 2012 and April 15, 2013. Three groups of male rats were randomly set up as a control group (C), a 100 mg/kg intraperitoneal NAC + 7 mL/kg contrast agent group (N + CIN) and a 7 mL/kg intraperitoneal contrast agent group (CIN). They were placed in individual metabolic cages 48 hours after agent administration to obtain 24-hour urine samples. Renal function tests (albumin, urea, creatinine, total protein) were conducted, oxidative stress parameters (Cu, Zn superoxide dismutase activity - Cu, Zn-SOD; advanced oxidation protein products - AOPP; protein carbonyls - PCO; total thiol groups - T-SH; and lipid hydroperoxides -LHP) were measured and tissues were analysed histopathologically. Results: Compared with the control group, groups CIN and N + CIN had significantly higher urea and LHP levels (P < 0.05 and P < 0.001, respectively) and significantly lower Cu, Zn-SOD activity and creatinine clearance (P < 0.05). There was no statistically significant difference between the groups in PCO or AOPP levels despite differences in descriptive statistics. Conclusions: Contrast-agent-induced nephropathic changes are more closely related to the magnitude of lipid peroxidation than protein oxidation.

This descriptive study investigated the relationship between depression and perceptions of sexuality in people with type II diabetes. The study population comprised all patients who came for check-ups at the polyclinic for diabetes at Istanbul Okmeydani Training and Research Hospital between November 2010 and February 2011. One hundred patients were willing to participate in the study and met the participant criteria. Socio-demographic characteristics, perceptions of sexuality, and other factors related to diabetes were evaluated from information provided by participants. The Beck Depression Inventory-II (BDI-II) was used to evaluate depression. Results revealed an average depression score of 16.34 ± 8.06. The average level of depression was higher in women, those whose level of education was low, and the unemployed. Of the participants, 53% reported that diabetes has affected their sex life adversely. There was no relationship between level of depression and aspects of the illness. However, level of depression was related to sexual satisfaction. Findings suggest that depression and sexually related problems should be addressed when providing care for people diagnosed with type II diabetes.

WOS: 000345626000016, PubMed ID: 25269407, The aim of our study was to delineate the demographic and clinical properties of primary glomerular diseases of adult population in our country in the light of global knowledge. All over the country, a total of 25 centers entered data between May 2009 and July 2012 to the database created by 'Glomerulonephritis Study Group' of Turkish Society of Nephrology. Demographic and clinical characteristics, specific diagnoses of glomerular diseases and biopsy findings recorded to the database were analyzed. Among the 1,274 patients, who had renal biopsy within the defined time period, 55 % were male and 45 % were female. The mean age was 40.8 +/- A 14.6 years. The most frequent indication for biopsy was nephrotic syndrome (57.8 %), followed by nephritic syndrome including rapidly progressive glomerulonephritis (16.6 %) and asymptomatic urinary abnormalities (10.8 %). The most frequent primary glomerular disease was membranous nephropathy (28.8 %), followed by focal segmental glomerulosclerosis (19.3 %) and IgA nephropathy (17.2 %). The presented study displayed important data about the epidemiology of primary glomerular diseases among adults in our country. The predominance of membranous nephropathy in contrast to other countries, in which the most frequent etiology is IgA nephropathy, seems to be due to differences in the indications for renal biopsy., Turkish Society of Nephrology, Financial supports have been obtained from Turkish Society of Nephrology and logistic support from the authors' affiliation departments. None of authors have conflict of interest.