Clinical and histopathological characteristics of primary focal segmental glomerulosclerosis in Turkish adults.

The data regarding primary FSGS (pFSGS) from different parts of the world differ. While the prevalence of pFSGS has been increasing in Western countries like the USA, it follows an inconsistent trend in Europe and Asia and a decreasing trend in Far Eastern countries such as China in the last two decades. There are undetermined factors to explain those national and geographic discrepancies. Herein, we aimed to reveal the current prevalence with clinical and histopathological characteristics of pFSGS in Turkish adults. This study includes the biopsy-proven pFSGS patients data recorded between 2009 and 2019, obtained from the national multicenter primary glomerulonephritis registry system of the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) database. 850 of the 3875 primer glomerulonephritis patients(21.9%) have pFSGS. The mean age is 40.5 ± 14.2 and 435 (51.2%) of patients are male. Nephrotic syndrome is the most common biopsy indication (59.2%). 32.6% of patients have hematuria, 15.2% have leukocyturia and 7.8% have both. Serum creatinine, albumin, and proteinuria are 1.0 mg/dL (IQR = 0.7-1.4) mg/dl, 3.4 ± 0.9 g/dl, 3400 mg/day(IQR, 1774-5740), respectively. Females have lower mean arterial pressure (- 2.2 mmHg), higher eGFR (+ 10.0 mL/min/1.73 m2), and BMI (+ 1.6 kg/m2) than males. Thickened basal membrane(76.6%) and mesangial proliferation (53.5%) on light microscopy are the major findings after segmental sclerosis. IgM (32.7%) and C3 (32.9%) depositions are the most common findings on immunofluorescence microscopy. IgM positivity is related to lower eGFR, serum albumin, and higher proteinuria. The prevalence of pFSGS is stable although slightly increasing in Turkish adults. The characteristics of the patients are similar to those seen in Western countries.

Overlooked factor in the etiology of pruritus in hemodialysis patients: Ultrafiltration volume.

INTRODUCTION: This study investigated the relationship between ultrafiltration (UF) volume and pruritus severity based on the idea that skin perfusion and inflammatory changes occur in dialysis patients with high UF volume. MATERIALS AND METHODS: This observational study included 392 patients. Patients filled out the Numerical Rating Scale, Verbal Rating Scale, and Visual Analogue Scale, showing the severity of pruritis. UF volumes in the last 12 sessions were recorded and averaged. RESULTS: The rate of patients with pruritis was between 59.4% and 67.5% in the three scales. In three pruritis scales, the severity of pruritis, age, body mass index (BMI), UF volume, and UF volume/body weight ratio were positively correlated. UF volume/body weight ratio, age, and BMI were independent predictors of pruritis severity. CONCLUSION: Limiting interdialytic weight gain may be an important treatment approach in pruritus control.

Can eculizumab be an option in traditional treatment-resistant ulcerative colitis?

Atypical/complement-mediated hemolytic uremic syndrome (A-HUS/CM-HUS) is a hereditary or sporadic disease with thrombotic microangiopathy (TMA). Diarrhea is a trigger that can cause attacks of CM-HUS. Although there are opinions that complement system activation plays a role in intestinal inflammation in patients with inflammatory bowel disease, the association of TMA with inflammatory bowel disease (IBD) has rarely been reported. In our case, a CM-HUS case that developed without an additional triggering factor in the course of ulcerative colitis (UC) was successfully treated with eculizumab, and then UC remission was also achieved. In this context, we would like to point out that the irregularities in the alternative pathway of the complement system may cause clinical findings in extra-renal organs, and the complement system may also play a role in the pathogenesis of inflammatory bowel disease. In addition, we think that our case may guide further studies on the usability of anti-complement therapies in treating patients with IBD who are resistant to conventional treatments.

Trends of primary glomerular disease in Turkey: TSN-GOLD registry report.

BACKGROUND: Although several renal biopsy registry reports have been published worldwide, there are no data on primary glomerular disease trends in Turkey. METHODS: Three thousand eight-hundred fifty-eight native kidney biopsy records were assessed in the Turkish Society of Nephrology Primary Glomerulopathy Working Group (TSN-GOLD) Registry. Secondary disease and transplant biopsies were not recorded in the registry. These records were divided into four periods, before 2009, 2009 to 2013, 2013-2017, and 2017-current. RESULTS: A total of 3858 patients (43.6% female, 6.8% elderly) were examined. Nephrotic syndrome was the most common biopsy indication in all periods (58.6%, 53%, 44.1%, 51.6%, respectively). In the whole cohort, IgA nephropathy (IgAN) (25.7%) was the most common PGN with male predominance (62.7%), and IgAN frequency steadily increased through the periods (× 2 = 198, p < 0.001). MGN was the most common nephropathy in the elderly (> 65 years), and there was no trend in this age group. An increasing trend was seen in the frequency of overweight patients (× 2 = 37, p < 0.0001). Although the biopsy rate performed with interventional radiology gradually increased, the mean glomeruli count in the samples did not change over the periods. CONCLUSIONS: In Turkey, IgAN is the most common primary glomerulonephritis, and the frequency of this is increasing.

Are Kidney Donor Risk Index/Kidney Donor Profile Index Scores Predictor of Future Graft Function?

Kidney donor profile index (KDPI) is a method developed to assess donor kidney quality for cadaveric transplants. We investigated the relationship between kidney donor risk index (KDRI) and KDPI scores of cadaveric transplants in the last five years in our clinic and current graft conditions. Forty people (12 females, 28 males; mean age, 46.35 + 8.94 years), who underwent cadaveric transplantation between January 2013 and March 2018, were participated in this study. At the same time, the KPDI and KDRI scores of the donor's kidneys were calculated and then compared with that of the recipients' data. The mean KDRI value of donors was 1.11 ± 0.33 and the median KDPI value was 60.50%. KDRI/KDPI scores were found to be high in women (P = 0.021) and, when the posttransplant findings of the recipients were evaluated by bivariate correlation analysis KDRI/KDPI scores were positively correlated with the day when creatinine (Cr) values started to fall off spontaneously (r = 0.457, P = 0.003). It was observed that recipients who were transplanted from the donors with high KDRI/KDPI scores had higher Cr levels (P <0.01) and this relationship did not change over time (P = 0.115). When the donors were grouped as the standard-criteria donor and expanded-criteria donor (ECD); we found that the relationship between KDRI/KDPI, creatinine, and time was more significant in the ECD group (P = 0.04). KDRI/KDPI scores are closely related to graft outcomes especially in recipients that have kidneys from marginal donors. Therefore, evaluation of KDRI/KDPI scores might be required in marginal donors.

The Relationship Between Visceral Adiposity Index and Epicardial Adipose Tissue in Patients with Type 2 Diabetes Mellitus.

INTRODUCTION AND AIM: Cardiovascular diseases remain the most common cause of morbidity and mortality in patients with diabetes. Epicardial adipose tissue (EAT), visceral fat depot of the heart, was found to be associated with coronary artery disease in cardiac and non-cardiac patients. Increased visceral adiposity is associated with proinflammatory activity, impaired insulin sensitivity, increased risk of atherosclerosis and high mortality. In the present study we aimed to investigate the relationship between EAT and visceral adiposity index (VAI) in patients with diabetes. METHODS: This was a cross-sectional study involving 128 patients with type 2 diabetes mellitus (73 females, 55 males; mean age, 54.09+±+9.17 years) and 32 control subjects (23 females, 9 males; mean age, 50.09+±+7.81 years). EAT was measured by using a trans-thoracic echocardiograph. Parameters such as waist circumference (WC), body mass index (BMI), triglyceride and high density lipoprotein (HDL) cholesterol were used to calculate VAI. RESULT: EAT and VAI measurements were significantly higher in patients with diabetes when compared to control subjects. In the bivariate correlation analysis, VAI was positively correlated with uric acid level (r=0.214, p=0.015), white blood cell count (r= 0.262, p=0.003), platelet count (r=0.223, p=0.011) and total cholesterol levels (r= 0.363, p<0.001). Also, VAI was found to be the independent predictor of EAT. CONCLUSION: Simple calculation of VAI was found to be associated with increased EAT in patients with type 2 diabetes.

Irritable bowel syndrome frequency and related factors in hemodialysis patients.

INTRODUCTION: Irritable bowel syndrome (IBS) is a functional bowel disease that is common in society, does not threaten life, impairs quality of life, and causes serious economic losses. Gastrointestinal system complaints and especially IBS are common in patients with chronic kidney disease. It has also been shown that psychiatric diseases are more common in patients with IBS. In this study, we aimed to determine the frequency of IBS in hemodialysis patients and to investigate the factors associated with IBS. METHODS: In this cross-sectional study, the questionnaire prepared to evaluate depression, anxiety, and abdominal pain was administered face-to-face to 686 patients by the same researcher in seven dialysis centers; 404 patients without exclusion criteria were included in the study. The diagnosis of IBS was made according to Rome IV criteria. A multivariate logistic regression model was used to identify factors that are significantly related to IBS. FINDINGS: In 69 (17.1%) of the patients included in the study, symptoms were consistent with IBS. Binominal logistic regression analysis was performed to evaluate the effect of age, dialysis duration, diabetes, proton pump inhibitor, non-steroidal anti-inflammatory drugs, calcium acetate use, Hamilton depression and anxiety scores associated with IBS in the presence of IBS of the participants. The logistic regression model was statistically significant, χ2 (3) = 69.748, P < 0.001. Independent risk factors for IBS in hemodialysis patients were determined as anxiety, long-term dialysis treatment, and using calcium acetate as a phosphorus binder. DISCUSSION: In hemodialysis patients, IBS occurs approximately twice as often as in a healthy population. Independent risk factors for IBS in hemodialysis patients are anxiety, long-term dialysis treatment, and using calcium acetate as a phosphorus binder.

Assessment of abdominal aortic calcification at different stages of chronic kidney disease.

PURPOSE: Vascular calcifications that may cause cardiovascular disease are highly prevalent in chronic kidney disease (CKD). In this study, we aimed to determine abdominal aorta calcifications (AAC) in predialysis and hemodialysis patients by lateral lumbar radiography and to investigate factors that were associated with the calcifications. METHODS: Two hundred and fifty-nine adult chronic hemodialysis patients, 300 predialysis CKD patients and 60 healthy subjects with normal kidney function as a control group were enrolled in the study. Lateral lumbar radiography was used to measure AAC. Calcified deposits of the abdominal aorta wall at the level of the first through fourth lumbar vertebrae were graded by a 24-point scoring system. RESULTS: AAC prevalence (AAC score ≥1) was significantly different in hemodialysis, predialysis and control groups (71.8, 45.7 and 33.3 %, respectively; p < 0.001). AAC prevalence in CKD stages 1, 2, 3, 4 and 5 predialysis patients was 26.6, 43.3, 40, 58.3 and 55 %, respectively. AAC scores of the hemodialysis group were higher than of the predialysis group (p < 0.001) and the control group (p < 0.001). AAC scores of the predialysis group were not higher than of the control group (p = 0.314). AAC scores of the hemodialysis group were significantly higher than of the control group (p < 0.001) and stage 1 (p < 0.001), stage 2 (p = 0.001) and stage 3 predialysis groups (p = 0.002). Age (p < 0.001), presence of diabetes mellitus (p < 0.001) and serum phosphorus levels (p = 0.011) were found to be independent predictors of calcification in the hemodialysis group. Age (p < 0.001), serum phosphorus levels (p = 0.007) and history of cardiovascular disease (p = 0.014) were found to be independent predictors of calcification in the predialysis group. CONCLUSIONS: Abdominal aortic calcification is highly prevalent in the hemodialysis population. Strict phosphorus control should be implemented to the predialysis and hemodialysis patients.

Common variable immunodeficiency in adults requires reserved protocols for long-term follow-up.

BACKGROUND/AIM: The aim of this study is to establish follow-up protocols for adult patients with common variable immunodeficiency (CVID) in a recently founded adult immunology clinic in the Central Anatolia Region of Turkey, where a clinical immunology center for adults was not available previously. MATERIALS AND METHODS: A total of 25 patients with CVID aged 18 years and older were included in this study. The file format consisted of 13 pages and was developed for the purpose of the study. Separate sections were designated for identity information, medical history, disease course, previous and current laboratory and imaging studies, follow-up plans, detection and management of complications/comorbidities, and treatment results. RESULTS: The mean age of the patients was 36.6 ± 13.4 years. The delay in diagnosis was 107 ± 95.6 months. In 92% of patients, initial symptoms resulting in admission to healthcare facilities were infections. Seventeen of 25 patients (68%) had bronchiectasis at the beginning of follow-up. CONCLUSION: Early identification of complications and comorbidities in patients with CVID will significantly improve quality of life and survival. Close observation and standardized protocols for follow-up are essential components of management.

Thrombotic thrombocytopenic purpura secondary to ABO group incompatible blood transfusion in a patient after cardiac surgery.

The triggers of secondary thrombotic thrombopcytopenic purpura (TTP) include drug toxicity, radiation and high-dose chemotherapy, angioinvasive infections, surgery and acute graft versus host disease. TTP secondary to surgery have been reported in a number of cases. Most of the cases have been occurred after open heart surgery. Extensive endothelial damage is held responsible as the initiating mechanism in postoperative TTP cases. However, there is no report of secondary TTP describing development owing to ABO incompatible blood transfusion. Here, we describe a patient who developed TTP after transfusion of ABO incompatible blood during hospitalization for bypass surgery. We also propose a hypothesis which may account for the possible underlying mechanism.

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.

Genotype-phenotype correlation in patients with familial Mediterranean fever in East Anatolia (Turkey).

AIMS: Familial Mediterranean fever (FMF) is an autosomal recessive hereditary disease. Clinical symptoms and findings (phenotypes) seen in this disease are generally fever, abdominal pain, and arthritis. Amyloidosis is also a significant complication. Phenotype-genotype correlations in FMF have not been conclusively resolved. The aims of this study were to find the most frequent mutation/genotype of FMF, as well as to investigate the role of genetic factors on the phenotype and on the development of amyloidosis in a population living in East Anatolia (Turkey). This study included 105 adult patients with FMF. DNA samples were obtained from peripheral blood lymphocytes of the patients. Mutations of the Mediterranean fever (MEFV) gene were analyzed with an FMF Strip Assay test kit (ViennaLab Labordiagnostika GmbH, Vienna, Austria). Patients were separated according to genotypes, and phenotypes were compared statistically by the chi-square test. RESULTS: The most frequent mutation was M694V (53%) and the most frequent genotype was M694V/M694V (26%). In total, 81% of the patients experienced abdominal pain, 76% had fever, and 22% had arthritis. Fever and arthritis were determined in similar ratios to other genotypes (76% and 19%, respectively) in the M694V/M694V genotype (74% and 29%, respectively) (p > 0.50 and p > 0.20, respectively). However, the patients without the M694V/M694V genotype (86%) had a higher abdominal pain ratio than did the patients with the M694V/M694V genotype (67%) (p <0.05). Renal amyloidosis was determined in 33% of both M694V/M694V and M680I(G/C)/M680I(G/C) homozygous groups and in 12% of the heterozygous groups (p < 0.02 and p < 0.00002, respectively). In other words, homozygous groups had higher ratios of renal amyloidosis. CONCLUSIONS: The most frequent mutation in FMF was M694V and the most frequent genotype was M694V/M694V. Fever, abdominal pain, arthritis, and renal amyloidosis were determined not only in patients with M694V/M694V genotype but also in other genotypes. Therefore, genotypes may not predict phenotypes in FMF. Renal amyloidosis was seen more frequently in homozygous genotypes.

Dilated cardiomyopathy in a patient with antibody-negative Goodpasture's syndrome and pulmonary relapse.

The Goodpasture's syndrome, also known as anti-GBM disease, is an uncommon disease, responsible for 20% of all cases of rapidly progressive glomerulonephritis. Anti-GBM antibodies are directed against non-collagenous domain (NC-1) of the alpha-3 chain of type IV collagen. When conventional ELISA assays are used, these antibodies can be detected in almost all the patients. Nevertheless, some reports have described antibody-negative relapsing disease. Some aggravating factors, namely, smoking, pulmonary infection and hypervolemia, may expose embedded antigenic target and may be responsible for the relapse. In addition, these antibody-negative relapses also respond to standard treatment, which comprises of plasma exchange, pulse steroids and cyclophosphamide. Herein, we report a patient who presented at the Selcuk University Meram School of Medicine, Meram, Konya, Turkey, with the pulmonary-renal syndrome. He was also found to have idiopathic dilated cardiomyopathy (DCM). To our knowledge, this is the first report describing co-existence of DCM and anti-GBM disease. There is growing evidence showing strong relation of both DCM and anti-GBM disease with HLA. Although not proven, this might have occurred in our patient. In our opinion, volume overload was facilitated by anuria and DCM and led to an antibody-negative pulmonary relapse. The relapse was treated just as the first episode and the patient improved satisfactorily.