Immunosuppressive treatment results in patients with primary IgA nephropathy in Turkiye; the data from TSN-GOLD working group.

BACKGROUND: Immunoglobulin A (IgA) nephropathy (IgAN) treatment consists of maximal supportive care and, for high-risk individuals, immunosuppressive treatment (IST). There are conflicting results regarding IST. Therefore, we aimed to investigate IST results among IgAN patients in Turkiye. METHOD: The data of 1656 IgAN patients in the Primary Glomerular Diseases Study of the Turkish Society of Nephrology Glomerular Diseases Study Group were analyzed. A total of 408 primary IgAN patients treated with IST (65.4% male, mean age 38.4 ± 12.5 years, follow-up 30 (3-218) months) were included and divided into two groups according to treatment protocols (isolated corticosteroid [CS] 70.6% and combined IST 29.4%). Treatment responses, associated factors were analyzed. RESULTS: Remission (66.7% partial, 33.7% complete) was achieved in 74.7% of patients. Baseline systolic blood pressure, mean arterial pressure, and proteinuria levels were lower in responsives. Remission was achieved at significantly higher rates in the CS group (78% vs. 66.7%, p = 0.016). Partial remission was the prominent remission type. The remission rate was significantly higher among patients with segmental sclerosis compared to those without (60.4% vs. 49%, p = 0.047). In the multivariate analysis, MEST-C S1 (HR 1.43, 95% CI 1.08-1.89, p = 0.013), MEST-C T1 (HR 0.68, 95% CI 0.51-0.91, p = 0.008) and combined IST (HR 0.66, 95% CI 0.49-0.91, p = 0.009) were found to be significant regarding remission. CONCLUSION: CS can significantly improve remission in high-risk Turkish IgAN patients, despite the reliance on non-quantitative endpoints for favorable renal outcomes. Key predictors of remission include baseline proteinuria and specific histological markers. It is crucial to carefully weigh the risks and benefits of immunosuppressive therapy for these patients.

Could mesangial C3 deposition be an independent prognostic marker in immunoglobulin A nephropathy?

BACKGROUND: Immunoglobulin A nephropathy (IgAN) is a common primary glomerulonephropathy. There is evidence that mesangial C3 deposition plays a role in the development of the disease. The aim of this study was to examine the effect of C3 deposition on the prognosis of IgAN patients. METHOD: The study included 1135 patients with biopsy-confirmed IgAN from the database of the Turkish Nephrology Association Glomerular Diseases Working Group (TSN-GOLD). Patients were excluded from the study if they were aged < 18 or > 75 years or if C3 staining had not been performed in the immunofluorescent analysis. C3 deposition was defined as an immunofluorescence intensity of C3 ≥ 2 + within the mesangium. The primary endpoints were the development of end-stage renal disease, a 30% decrease in glomerular filtration rate compared to the basal value or an elevation in proteinuria to a nephrotic level (3.5 gr/day). RESULTS: Mesangial C3 deposition was observed in 603 (53.1%) patients. No statistically significant difference was found at baseline between the groups with and without mesangial C3 deposition, as for age, sex, BMI, proteinuria level, or the presence of hypertension. In the follow-up period with a mean duration of 78 months, no significant difference was found between the two groups regarding the primary endpoints (p = 0.43). A significant correlation between C3 deposition and segmental glomerulosclerosis (S1) according to the Oxford MEST-C classification was found (p = 0.001). CONCLUSION: Although a correlation was observed between mesangial C3 deposition and the S1 MEST-C classification, mesangial C3 deposition was not a prognostic factor in IgAN.

Sarcopenia and cardiovascular risk indices: Its impact on cardiovascular events and mortality in dialysis patients.

BACKGROUND: Sarcopenia is a common complication in end-stage renal disease. Low muscle strength and muscle mass are risk factors for cardiovascular disease and mortality in patients undergoing dialysis. We studied the relation between sarcopenia and pre-atherosclerotic markers and its effect on cardiovascular events and death in dialysis patients. METHODS: We measured muscle strength, muscle mass, carotid intima-media thickness, and pulse wave velocity in 106 patients. Sarcopenia was diagnosed according to the EWGSOP-2 suggestions. Patients with low muscle strength and low muscle mass were considered sarcopenic. The follow-up period for cardiovascular events and mortality was 24 months. RESULTS: The mean age and dialysis duration were 57.4 ± 16.6 and 6.5 ± 4.9 years, respectively. Of all patients, 53 (50%) were male and 70 (66%) were on hemodialysis treatment. Sarcopenia and low muscle strength were seen in 47.1% and 88.7%, respectively. Hemodialysis patients were more likely to be sarcopenic than peritoneal dialysis patients (p = 0.001). Ferritin and Kt/V levels were higher, and body mass index was lower significantly in sarcopenic patients (p < 0.001). There was no significant difference in carotid intima-media thickness and pulse wave velocity measurements between the groups (p = 0.62 and p = 0.68, respectively). There was no statistically significant difference in cardiovascular events and mortality in cases with and without sarcopenia (p = 0.43 and p = 0.17, respectively). CONCLUSION: There was no association between sarcopenia and pre-atherosclerotic markers, cardiovascular events, and all-cause mortality in dialysis patients. Techniques to detect low muscle strength and muscle mass need standardization, and new specific cut-off levels must be defined for dialysis patients.

Characteristics and Sensitization Risk Factors in Kidney Transplant Wait List Candidates: Panel Reactive Antibodies Status Is Crucial for Successful Kidney Allocation Systems in Turkey.

OBJECTIVES: Kidney transplant is the treatment of choice for end-stage renal disease. Because of the insufficient supply of donor organs for transplant, the number of patients on the transplant wait list is increasing. We analyzed demographic and clinical factors including sensitization status of patients on the kidney transplant wait list in our center. MATERIALS AND METHODS: Patients on the kidney transplant wait list at Ankara University School of Medicine by July 2018 were evaluated. Data on demographics, comorbidities, treatment characteristics, and immunologic properties were collected. RESULTS: The study included 528 kidney transplant candidates whose mean time on the deceased donor organ wait list was 57 ± 47 months. Enlisted patients were aged 53 ± 13 years, and 95% of them were on dialysis. Dialysis vintage was longer and percentage of patients who had anti-HLA antibodies was higher in women than men (P = .004 and P < .001, respectively). Levels for median fluorescence intensity were higher in women compared with men (class I, P < .001; and class II, P = .011). Transfusion (P < .001), pregnancy (P = .001), transplant (P < .001), longer dialysis vintage (P = .021), and longer time on wait list (P = .001) were associated with anti-HLA antibody positivity. Multiple regression analysis revealed that a history of transplant and blood transfusion were independent risk factors of a positive panel reactive antibodies. CONCLUSIONS: In our kidney transplant candidates on the wait list, sensitization by transplant has a significant impact on development of anti-HLA antibodies. Updates of the organ allocation system to consider sensitized candidates and strategies to expand the deceased donor organ pool and donation rates are needed to increase the rate of deceased donor kidney transplant in Turkey.

Clinical and pathologic features of primary membranous nephropathy in Turkey: a multicenter study by the Turkish Society of Nephrology Glomerular Diseases Working Group.

BACKGROUND: We aimed to evaluate the features of primary membranous nephropathy (MNP) in Turkish people. METHODS: This is a retrospective analysis of patients with biopsy-proven primary MNP. We obtained the data collected between 2009 and 2019 in the primary glomerulonephritis registry of the Turkish Society of Nephrology Glomerular Diseases Study Group (TSN-GOLD). Patients with a secondary cause for MNP were excluded. Clinical, demographic, laboratory, and histopathological findings were analyzed. RESULTS: A total of 995 patients with primary MNP were included in the analyses. Males constituted the majority (58.8%). The mean age was 48.4 ± 13.9 years. The most common presentation was the presence of nephrotic syndrome (81.7%) and sub nephrotic proteinuria (10.3%). Microscopic hematuria was detected in one-third of patients. The median estimated glomerular filtration rate (eGFR) was 100.6 mL/min/1.73 m2 (IQR, 75.4-116.3), and median proteinuria was 6000 mg/d (IQR, 3656-9457). Serum C3 and C4 complement levels were decreased in 3.7 and 1.7% of patients, respectively. Twenty-four (2.4%) patients had glomerular crescents in their kidney biopsy samples. Basal membrane thickening was detected in 93.8% of cases under light microscopy. Mesangial proliferation and interstitial inflammation were evident in 32.8 and 55.9% of the patients, respectively. The most commonly detected depositions were IgG (93%), C3 complement (68.8%), and kappa and lambda immunoglobulin light chains (70%). Although renal functions were normal at presentation, vascular, interstitial, and glomerular findings were more prominent on biopsy in hypertensive patients. No significant effect of BMI on biopsy findings was observed. CONCLUSIONS: Despite some atypical findings, the main features of primary MNP in Turkey were similar to the published literature. This is the largest MNP study to date conducted in Turkish people.

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.

The diastolic function abnormalities in hemodialysis session: a two-dimensional speckle tracking echocardiographic (2DSTE) study.

Hemodialysis (HD) decreases preload and its acute effect on the diastolic function is still controversial even with the introduction of new non-volume-dependent tissue Doppler echocardiographic indices. This study is designed to evaluate these acute changes following HD sessions. We enrolled 39 patients receiving standard thrice weekly HD for more than 6 months and performed two dimensional speckle tracking echocardiography (2DSTE) and tissue Doppler studies with a standard cardiac ultrasound device shortly before and after HD. We observed significant changes in most of the transmitral flow and tissue Doppler echocardiographic parameters after HD. The left atrial volume index, left ventricular mass index, mitral E, mitral E/A, and lateral annular E/é levels decreased after HD (p: < 0.001, p: 0.026, p: < 0.001, p: 0.011, p: < 0.001, respectively). Medial á, medial myocardial performance index (MPI), medial s, lateral s, and lateral MPI values increased significantly after HD (p: 0.049, p: 0.007, p: 0.001, p: < 0.001, p: 0.01, respectively). Diastolic parameters like diastolic strain ratio early diastole (DSRE), diastolic strain ratio late diastole (DSRA), E/DSRE, and E/DSRA did not change significantly after HD (p: 0.716, p: 0.117, p: 0.114, and p: 0.211, respectively). The global longitudinal strain value obtained with 2DSTE worsened after HD (- 18.4 ± 4.0 before vs - 15.9 ± 5.4 after HD, p: 0.011). Transmitral flow and tissue Doppler parameters changed significantly after HD while the change in 2DSTE findings was not significant. The diastolic measurements made with 2DSTE may be less volume and cardiac preload dependent compared to conventional echocardiography and this may explain the difference.

Cytomegalovirus infection in patients with glomerular diseases treated with cyclophosphamide: a single-center prospective study.

PURPOSE: Cytomegalovirus infection is an important complication in immunocompromised patients. As few studies have shown that cyclophosphamide treatment is a risk factor for cytomegalovirus infection in patients with glomerulonephritis, we aimed to describe the frequency and risk factors of cytomegalovirus infection in glomerulonephritis patients treated with cyclophosphamide. METHODS: We prospectively recruited 43 cytomegalovirus seropositive patients with glomerulonephritis treated with cyclophosphamide. We screened all patients for viral DNA monthly during treatment. Patients were compared for age, sex, glomerular pathology, renal function and clinical status regarding development of cytomegalovirus infection before and after the treatment. RESULTS: Cytomegalovirus infection was detected in 10 (23.3%) patients, most commonly within the first 2 months of cyclophosphamide treatment. All patients recovered without any cytomegalovirus-related complications. Patients with cytomegalovirus infection had higher serum creatinine (4.2 ± 3.2 vs. 1.9 ± 1.8 mg/dl, p = 0.006) and lower estimated glomerular filtration rate (29 ± 11 vs. 65 ± 8 ml/min/1.73 m2, p = 0.016) at diagnosis compared with cytomegalovirus infection non-occurred patients. In addition, number of patients presented with rapidly progressive glomerulonephritis were higher in cytomegalovirus infection group (80.0% vs. 27.3%, p = 0.007). Moreover, cytomegalovirus infection was associated with prolonged hospital stay (54 ± 7 vs. 29 ± 6 days, p = 0.027). CONCLUSION: Cytomegalovirus infection is a common complication in glomerulonephritis patients treated with cyclophosphamide in this prospective study. Routine monitoring and prophylaxis should be considered for these high-risk patients.

The relationship between glomerular IgG staining and poor prognostic findings in patients with IgA nephropathy: the data from TSN-GOLD working group.

BACKGROUND: Galactose-deficient IgA1 (Gd-IgA1) has an increased tendency to form immunocomplexes with IgG in the serum, contributing to IgAN pathogenesis by accumulating in the glomerular mesangium. Several studies showed that glomerular IgG deposition in IgAN is an important cause of mesangial proliferation and glomerular damage. This study aims to determine the association of the positivity of IgG and the intensity of IgG staining with a poor renal prognosis. METHODS: A total of 943 IgAN patients were included in the study. Glomerular IgG staining negative and positive patients were compared using Oxford classification scores, histopathological evaluations, proteinuria, eGFR, albumin, blood pressures. IgG positive patients were classified as (+), (++), (+++) based on their staining intensity, and the association with the prognostic criteria was also evaluated. RESULTS: 81% (n = 764) of the patients were detected as IgG negative, while 19% (n = 179) were positive. Age, gender, body mass index, blood pressure, proteinuria, eGFR, uric acid values were similar in IgG positive and negative patients who underwent biopsy (p > 0.05). Intensity of glomerular IgG positivity was not found to be associated with diastolic and systolic blood pressure, urea, uric acid, age, eGFR, albumin, proteinuria (p > 0.05 for all, r = - 0.084, r = - 0.102, r = - 0.006, r = 0.062, r = 0.014, r = - 0.044, r = - 0.061, r = - 0.066, r = 0.150, respectively). There was no difference for histopathological findings between IgG (+), IgG (++), IgG (+++) groups (for all, p > 0.05). CONCLUSION: Glomerular IgG negativity and positivity detected by routine IFM in IgAN patients is not associated with poor renal prognostic risk factors.

Infectious Complications in Patients with Primary Glomerulonephritis over 10 Years: A Single-Center Experience in Turkey.

INTRODUCTION: Infections can play an important role in the mortality and morbidity of patients with glomerulonephritis. However, the frequency of infectious complications in primary glomerulonephritis and their burden to the healthcare managements are not clear. METHODS: We evaluated the infectious complications in patients with biopsy-proven focal segmental glomerulosclerosis, membranous glomerulonephritis, IgA nephropathy, minimal change disease, membranoproliferative glomerulonephritis, and chronic glomerulonephritis during the last 10 years in a single center. We recorded the demographic, clinical, and laboratory characteristics; treatment modalities; infectious episodes; and infection-related mortality and morbidity of the patients. RESULTS: Of the patients, 154 (63.6%) received immunosuppressive treatment and 88 (34.4%) were followed up under conservative treatment. Overall, 118 infectious episodes were noted in 64 patients, with an infection rate of 0.20 per patient-year. Total infectious complications were higher in the immunosuppressive group than in the conservative group (42.1 vs. 23.3%, p = 0.005). Infection-related hospitalizations were also higher in the immunosuppressive group (p = 0.01). The most frequently infected area was the lungs (15.7%). Although bacterial infections were the most common in both groups, 14.9% of the immunosuppressive group had cytomegalovirus (CMV) replication. Age >50 years (OR 2.19, p = 0.03), basal serum albumin <2.5 g/dL (OR 2.28, p = 0.02), cyclophosphamide (OR 2.43, p = 0.02), and cyclosporine (OR 2.30, p = 0.03) were independently associated with experiencing infectious episodes. CONCLUSIONS: Because of high seropositivity for CMV in Turkey, it might be a wise approach to use prophylactic antiviral drugs in patients treated with immunosuppressive treatments. Close monitoring of patients with primary glomerulonephritis, especially those treated with immunosuppressive therapy, is important for reducing infection-related morbidity and mortality.

Demographic, clinical and laboratory characteristics of rapidly progressive glomerulonephritis in Turkey: Turkish Society of Nephrology-Glomerular Diseases (TSN-GOLD) Working Group.

BACKGROUND: In our study, diagnostic and demographic characteristics of patients diagnosed with RPGN by biopsy, clinical and laboratory findings in our country were investigated. METHODS: Data were obtained from the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) Working Group database. Demographic characteristics, indications for biopsy, diagnosis of the glomerular diseases, comorbidities, laboratory and biopsy findings of all patients were recorded. According to their types, RPGN patients were classified as type 1 (anti-GBM related), type 2 (immuncomplex related) and type 3 (pauci-immune). RESULTS: Of 3875 patients, 200 patients with RPGN (mean age 47.9 ± 16.7 years) were included in the study which constitutes 5.2% of the total glomerulonephritis database. Renal biopsy was performed in 147 (73.5%) patients due to nephritic syndrome. ANCA positivity was found in 121 (60.5%) patients. Type 1 RPGN was detected in 11 (5.5%), type 2 RPGN in 42 (21%) and type 3 RPGN in 147 (73.5%) patients. Median serum creatinine was 3.4 (1.9-5.7) mg/dl, glomerular filtration rate was 18 (10-37) ml/min/1.73m2 and proteinuria 2100 (1229-3526) mg/day. The number of crescentic glomeruli ratio was ratio 52.7%. It was observed that urea and creatinine increased and calcium and hemoglobin decreased with increasing crescentic glomerular ratio. CONCLUSIONS: Our data are generally compatible with the literature. Advanced chronic histopathological findings were prominent in the biopsy of 21 patients. Early biopsy should be performed to confirm the diagnosis of RPGN and to avoid unnecessary intensive immunosuppressive therapy. In addition to the treatments applied, detailed data, including patient and renal survival, are needed.

Characteristics of primary glomerular diseases patients with hematuria in Turkey: the data from TSN-GOLD Working Group.

PURPOSE: Hematuria is one of the most common laboratory findings in nephrology practice. To date, there is no enough data regarding the clinical and histopathologic characteristics of primary glomerular disease (PGD) patients with hematuria in our country. METHODS: Data were obtained from national multicenter (47 centers) data entered into the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) database between May 2009 and June 2019. The data of all PGD patients over the age of 16 years who were diagnosed with renal biopsy and had hematuria data were included in the study. Demographic characteristics, laboratory and biopsy findings were also recorded. RESULTS: Data of 3394 PGD patients were included in the study. While 1699 (50.1%) patients had hematuria, 1695 (49.9%) patients did not have hematuria. Patients with hematuria had statistically higher systolic blood pressure, serum blood urea nitrogen, creatinine, albumin, levels and urine pyuria. However, these patients had statistically lower age, body mass index, presence of hypertension and diabetes, eGFR, 24-h proteinuria, serum total, HDL and LDL cholesterol, and C3 levels when compared with patients without hematuria. Hematuria was present 609 of 1733 patients (35.8%) among the patients presenting with nephrotic syndrome, while it was presented in 1090 of 1661 (64.2%) patients in non-nephrotics (p < 0.001). CONCLUSION: This is the first multicenter national report regarding the demographic and histopathologic data of PGD patients with or without hematuria. Hematuria, a feature of nephritic syndrome, was found at a higher than expected in the PGDs presenting with nephrotic syndrome in our national database.

Epidemiological features of primary glomerular disease in Turkey: a multicenter study by the Turkish Society of Nephrology Glomerular Diseases Working Group.

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.

Acute stunning effect of hemodialysis on myocardial performance: A three-dimensional speckle tracking echocardiographic study.

The effects of acute changes during hemodialysis (HD) on the myocardium are not yet known. The invention of three-dimensional speckle tracking echocardiography (3DSTE) has offered clinicians a new method to assess the movements of ventricular segments simultaneously in three spatial directions. The aim of this study was to evaluate the effect of first weekly standard HD process on the left ventricle (LV) and right ventricle (RV) global and regional myocardial function in patients with normal left ventricle ejection fraction using 3DSTE-derived indices. Patients (n=38) receiving maintenance HD in our clinic who have no known cardiovascular disease are examined just before and after a HD session using 3DSTE. Demographic and comorbidity data, renal replacement treatment characteristics, and laboratory test results are recorded. 3DSTE analysis is performed to calculate the LV global longitudinal, circumferential area and radial peak systolic strain, as well as RV septum and free-wall longitudinal strain and fractional area change. Patients are aged 52.8 ± 13.6 years and 52.6% of them are male. Mean dialysis duration is 56 months. The LV strain values of the patients changed markedly before and after HD (GLS: -14.2 ± 5.2, -11.1 ± 4.6 [P < .001], GCS: -14.8 ± 4.2, -12.4 ± 5.28 [P < .009]; GRS: 41.5 ± 16, 33.3 ± 16.5 [P = .003]; AREA -24.7 ± 7.2, -20.1 ± 7.6 [P = .001], respectively). We could not demonstrate any improvement in RV strain values before or after HD. LV strain values are positively correlated with blood pressure variability during the dialysis sessions. LV function is preserved better after HD in patients on beta or calcium channel blocker therapy compared to those who do not use these agents (P < .001, P < .01, respectively). HD treatment results in deterioration in all LV strain directions but not in RV. Strain assessment may improve vascular risk stratification of patients on chronic HD.

Fabry Disease Screening in Patients With Kidney Transplant: A Single-Center Study in Turkey.

OBJECTIVES: Fabry disease is a rare X-linked multisystemic lysosomal storage disorder of the glycosphingolipid metabolic pathway. Nephropathy is one of the most important complications of Fabry disease, and patients with classical phenotype are at risk of developing endstage kidney disease. In this study, we investigated the use of screening for Fabry disease in kidney transplant recipients at our center. MATERIALS AND METHODS: We screened 301 kidney transplant recipients with functioning grafts. Analyses for α-galactosidase A gene mutation were performed in all female and male kidney transplant recipients. We also measured leukocyte α-galactosidase A enzyme activity in patients with identified GLA mutation. RESULTS: In 301 kidney transplant recipients, mean age was 42.9 ± 12.5 years, and the number of male patients was 180 (60%). Mean time after transplant was 79 ± 56 months, and estimated glomerular filtration rate was 66.8 ± 21 mL/min/1.73 m². One male patient who was diagnosed with Fabry disease before kidney transplant was also evaluated (mutation in the α-galactosidase A gene, c.1093_1101dup [p.Tyr365_lle367dup]). In 2 female patients, p.A143T (c.427G>A) mutation of unknown significance and p.D313Y (c.937G>T) heterozygous mutation were identified; however, leukocyte ?-galactosidase A enzyme activity was normal in these patients (63.7 and 67.3 nmol/h/mg protein). In the patient diagnosed with Fabry disease, family screening revealed 4 additional affected family members. DISCUSSIONS: Although prevalence was shown to be low in our center (1/301 patients; 0.33%), screening studies in kidney transplant recipients may help to detect new patients before they develop life-threatening complications such as renal involvement.

Biomarkers of Cardiovascular Disease in Patients With Ankylosing Spondylitis.

OBJECTIVES: This study aims to evaluate the inflammatory status and clinical and vascular alterations using tonometry and B-mode sonography in patients with ankylosing spondylitis (AS). PATIENTS AND METHODS: The study included 71 AS patients (57 males, 14 females; mean age 40.1±10.8 years; range, 29 to 51 years) without cardiovascular disease and 30 healthy controls (24 males, 6 females; mean age 41.2±9.3 years; range, 32 to 50 years). We evaluated participants' clinical, biological and functional status along with laboratory data and measured both the arterial stiffness using carotid-femoral pulse wave velocity (PWV) measured by applanation tonometry and carotid intima-media thickness (CIMT) as a preclinical atherosclerosis marker. RESULTS: The mean disease duration of AS patients was 10.6±4.2 years. CIMT (p=0.03) and PWV (p=0.04) data showed significant differences between AS patients and healthy controls. Multiregression analysis showed that PWV correlated with age (r2=0.42; p=0.03) and disease duration (r2=0.31; p=0.04), while CIMT correlated with disease duration (r2=0.37; p=0.03) and Bath Ankylosing Spondylitis Disease Activity Index (r2=0.3; p=0.04). CONCLUSION: This study demonstrated an increase in early preclinical atherosclerosis in AS patients without cardiovascular (CV) disease compared to healthy controls. Therefore, screening AS patients with noninvasive methods for atherosclerosis and subclinical vasculopathy would allow us to take primary prevention measures. We found that the major determinant for increased CV risk was the disease duration, while there was no difference between different treatment modalities.

Profile of renal AA amyloidosis in older and younger individuals: a single-centre experience.

OBJECTIVE: In epidemiological studies of amyloid A (AA) amyloidosis from Turkey, the most frequently cause was familial Mediterranean fever (FMF) and it occurs generally in young age population. However, there are no sufficient data regarding aetiology, clinical presentation and prognosis of renal AA amyloidosis in advanced age patients. In this study, we aimed to investigate demographic, clinical presentation, aetiology and outcomes of adults aged 60 years or older patients with biopsy-proven renal AA amyloidosis. METHODS: This is a retrospective study involving 53 patients who were diagnosed with AA amyloidosis by kidney biopsy from 2006 to 2016. In all patients, kidney biopsies were performed due to asymptomatic proteinuria, nephrotic syndrome and/or renal insufficiency. The patients were separated into two groups on the basis of age (group I: ≥60 years and group II: <60 years). Outcomes of patients in terms of the requirement of renal replacement therapy and mortality were recorded. RESULTS: In patients with group I, the causes of AA amyloidosis were as follows: FMF 16 (50%), bronchiectasis 7 (23%), chronic osteomyelitis 2 (6%), inflammatory bowel disease 2 (6%), rheumatoid arthritis 2 (6%), ankylosing spondylitis 1 (3%) and unknown aetiology 2 (6%). The underlying disorders of AA amyloidosis in group II patients were as follows: FMF 17 (81%), Behcet's disease 1 (5%) and unknown aetiology 3 (14%). No statistically significant differences were detected between two groups with regard to systolic and diastolic blood pressures, albumin, proteinuria and lipids. The combination of chronic kidney disease and nephrotic syndrome was the most common clinical presentation in group I (73%) and group II (43%) (p = .05). Compared to the group II, estimated glomerular filtration rate was significantly lower in group I at the time of kidney biopsy (p = .003). At 12-month follow-up, 61% of the group I and 33% of the group II developed end-stage kidney disease requiring dialysis, while 11% of the group I died. CONCLUSION: Our results indicated that renal AA amyloidosis is a rare disease in advanced age patients. At baseline and follow-up period, advanced age patients had worse kidney disease and outcomes.

Clinical Correlates of Ambulatory Blood Pressure Phenotypes at a Tertiary Care Hospital in Turkey.

BACKGROUND/AIMS: Hypertension and its complications are major public health issues worldwide due to their association with high cardiovascular morbidity and mortality. Despite significant progress in health, the prevalence of hypertension is increasing. Ambulatory blood pressure monitoring (ABPM) is becoming increasingly important for the management of hypertension. In this study, we aimed to investigate the clinical and laboratory correlates of ambulatory blood pressure (ABP) phenotypes at a tertiary care hospital in Turkey. METHODS: The characteristics of 1053 patients were retrospectively obtained from the hospital database. Hypertension was defined as patients with office blood pressure (BP) ≥140/90 mmHg and/or previously diagnosed hypertension and/or the use of antihypertensive medication. According to the office BP and ABPM results patients were identified namely: (1) sustained normotensive (SNT) patients (both office BP and ABPM were normal), (2) sustained hypertensive (SHT) patients (both office BP and ABPM were high), (3) masked hypertensive (MHT) patients (office BP were normal, but ABPM were high), (4) white coat hypertensive (WCHT) patients (office BP were above limits, but ABPM were normal). RESULTS: A total of 1053 patients were included to the study (female/male: 608/445 and mean age 55 ± 15 years). The mean age of patients with hypertension was significantly higher than without hypertension (p< 0.0001). Hypertension was more frequent in females (p=0.009). The rates of history of diabetes mellitus (DM), hyperlipidemia (HL), and chronic kidney disease (CKD) were higher in patients with hypertension (p< 0.0001). Among patients with hypertension (n=853, 81%), ABPM results showed that 388 (45%) of patients had SHT, 92 (11%) had MHT, and 144 (17%) had WCHT, whereas 229 (27%) had SNT. Patients with MHT were significantly older than patients with SNT (p=0.025). The prevalence of SHT was higher in men than in women, whereas the prevalence of WCHT was higher in women than in men (p< 0.0001). There was no significant difference between 4 groups with regard to body mass index (p=0.142), a history of DM (p=0.189) and smoking status (self-reported) (p=0.306). Patients with SHT had the highest prevalence of history of hypertension, HL and CKD (p< 0.0001). Among patients without hypertension, 26 (13%) of patients had MHT and none of those patients was on antihypertensive treatment. CONCLUSION: Potential usages of ABPM in Turkey may include screening of high risk individuals who have traditional cardiovascular risk factors. It also provides clinicians valuable information on abnormal ABP phenotypes. Future studies are needed to clarify the risk factors of different ABP phenotypes and to evaluate the role of ABPM on detection and control of hypertension.

Is Sclerostin Level Associated with Cardiovascular Diseases in Hemodialysis Patients?

BACKGROUND/AIMS: The objective of this study is to evaluate the relation between sclerostin, arterial stiffness, and cardiovascular events (CVE) in hemodialysis patients (HD). METHODS: Sclerostin level and carotid-femoral pulse wave velocity (PWV) in 97 HD patients and sclerostin level in 40 controls were measured. RESULTS: Sclerostin level was significantly higher in patients than in controls. Sclerostin associated positively with age, male gender, cardiovascular disease, statin use, BMI, and PWV while negatively with alkaline phosphatase, parathormone (PTH), Kt/V, cinacalcet and vitamin D use in univariable correlation analyses. Sclerostin associated positively with male gender and statin use but negatively with PTH in multivariate regression analyses. During observation, 30 fatal or nonfatal CVEs were observed. While univariate correlation analysis showed a positive association between PWV and sclerostin, there was no relation between the two in multivariate regression analysis. CONCLUSION: Further studies are needed to understand the role of sclerostin in predicting PWV changes in HD patients.