Effects of different inspiratory muscle training protocols on functional exercise capacity and respiratory and peripheral muscle strength in patients with chronic kidney disease: a randomized study.

BACKGROUND: Pathological changes were observed in the diaphragm due to abnormal renal function in chronic kidney disease (CKD). Inspiratory muscle training (IMT) has been suggested for patients with CKD; however, the most appropriate intensity for IMT has not been determined. Therefore, this study aimed to investigate the effects of different IMT protocols on respiratory muscle strength, quadriceps femoris muscle strength (QMS), handgrip muscle strength (HGS), functional exercise capacity, quality of life (QoL), pulmonary function, dyspnoea, fatigue, balance, and physical activity (PA) levels in patients with CKD. METHODS: This randomized, controlled, single-blind study included 47 patients and they were divided into three groups: Group 1 (n = 15, IMT with 10% maximal inspiratory pressure (MIP)), Group 2 (n = 16, IMT with 30% MIP), and Group 3(n = 16; IMT with 60% MIP). MIP, maximal expiratory pressure (MEP), 6-min walking test (6-MWT), QMS, HGS, QoL, pulmonary function, dyspnoea, fatigue, balance, and PA levels were assessed before and after eight weeks of IMT. RESULTS: Increases in MIP, %MIP, 6-MWT distance, and %6-MWT were significantly higher in Groups 2 and 3 than in Group 1 after IMT (p < 0.05). MEP, %MEP, FEF25-75%, QMS, HGS, and QoL significantly increased; dyspnoea and fatigue decreased in all groups (p < 0.05). FVC, PEF, and PA improved only in Group 2, and balance improved in Groups 1 and 2 (p < 0.05). CONCLUSIONS: IMT with 30% and 60% MIP similarly improves inspiratory muscle strength and functional exercise capacity. IMT with 30% is more effective in increasing PA. IMT is a beneficial method to enhance peripheral and expiratory muscle strength, respiratory function, QoL and balance, and reduce dyspnoea and fatigue. IMT with %30 could be an option for patients with CKD who do not tolerate higher intensities. TRIAL REGISTRATION: This study was retrospectively registered (NCT06401135, 06/05/2024).

Impairments of functional exercise capacity, muscle strength, balance and kinesiophobia in patients with chronic kidney disease: a cross-sectional study.

BACKGROUND: Muscle weakness, balance, and functional capacity are affected in patients with chronic kidney disease (CKD) in dialysis. However, studies about kinesiophobia, peripheral and respiratory muscle strength, balance, exercise capacity, fatigue, and physical activity level in patients with CKD 3-4 are limited. The study aimed to compare the functional exercise capacity, peripheral and respiratory muscle strength, pulmonary function, balance, kinesiophobia, physical activity, fatigue, and dyspnea between patients with CKD 3-4 and controls. METHODS: This cross-sectional study included 43 patients and 45 controls. Functional exercise capacity [6-Minute Walking Test (6MWT)], peripheral and respiratory muscle strength, pulmonary function, dyspnea, fatigue, physical activity, balance [Berg Balance Scale (BBS)], and kinesiophobia were evaluated. RESULTS: Demographic characteristics were similar in patients [53(50-57) y, 26 M/17F] and controls [51(4.506-55) y, 33 M/12F] (p > 0.05). The 6MWT, respiratory and peripheral muscle strength, pulmonary function, physical activity, and BBS were significantly lower, and the level of dyspnea and kinesiophobia were higher in patients compared with controls (p < 0.05). CONCLUSIONS: Patients had impaired functional exercise capacity, upper and lower extremity muscle strength, respiratory muscle strength, pulmonary function, and balance, increased perception of dyspnea and kinesiophobia, and reduced physical activity level compared with controls. Patients should be directed to cardiopulmonary rehabilitation programs.

Thiol/disulfide homeostasis as a new oxidative stress marker in patients with Fabry disease.

This is the first study to show both dynamic thiol-disulfide balance and oxidative stress levels in patients with Fabry disease (FD). This prospective study consists of 30 FD patients and 30 healthy controls. Thiol and disulfide values of the study groups were evaluated using a new, cost-effective and fully automatic colorimetric method. A total of 60 subjects were included in the study. A statistically significant difference was found between the patient and control groups for native and total thiol levels (p < 0.001). In addition, disulfide levels were significantly higher in FD patients compared with the control group (p < 0.003). Native thiol levels showed significantly negative correlation with lysosomal globotriaosylceramide, total oxidant status (TOS), and oxidative stress index (OSI) levels. In addition, a positive correlation was found between disulfide/natural thiol and disulfide/total thiol ratios and TOS, OSI, and blood urea nitrogen. We found total antioxidant status levels were lower in the patient group compared to the control group, while TOS and OSI levels were higher and were statistically significant. This study highlights for the first time a novel, cost-effective and fully automated measurement of thiol-disulfide levels in patients with FD. Determination of thiol levels can make important contributions to understand the etiopathogenesis and follow-up of the disease in FD patients.

Acute Kidney Injury: Medical Causes and Pathogenesis.

Acute kidney injury (AKI) is a common clinical syndrome characterized by a sudden decline in or loss of kidney function. AKI is not only associated with substantial morbidity and mortality but also with increased risk of chronic kidney disease (CKD). AKI is classically defined and staged based on serum creatinine concentration and urine output rates. The etiology of AKI is conceptually classified into three general categories: prerenal, intrarenal, and postrenal. Although this classification may be useful for establishing a differential diagnosis, AKI has mostly multifactorial, and pathophysiologic features that can be divided into different categories. Acute tubular necrosis, caused by either ischemia or nephrotoxicity, is common in the setting of AKI. The timely and accurate identification of AKI and a better understanding of the pathophysiological mechanisms that cause kidney dysfunction are essential. In this review, we consider various medical causes of AKI and summarize the most recent updates in the pathogenesis of AKI.

Corneal endothelial cell density and microvascular changes of retina and optic disc in autosomal dominant polycystic kidney disease.

Purpose: Vascular endothelial dysfunction in autosomal dominant polycystic kidney disease (ADPKD) may affect the retinal vascular parameters due to structural similarities of kidney and retina. We aimed to evaluate the microvascular changes of retina and optic disc and also corneal endothelial cell density in patients with ADPKD. Methods: Forty-six eyes of 23 patients with ADPKD (Group 1), and 46 eyes of 23 sex- and age-matched healthy controls (Group 2) were included in this cross-sectional study. Demographic and ophthalmic findings of participants were collected. Corneal endothelial cell density (CECD) measurements were obtained by noncontact specular microscopy. Foveal retinal thickness, peripapillary retinal nerve fiber layer (RNFL) thickness, vessel density in different sections of the retina and optic nerve head were analyzed by optical coherence tomography angiography. Results: The mean ages were 41 ± 11 years for Group 1 and 39 ± 10 years for Group 2 (P = 0.313). CECD values were significantly lower in group 1 when compared to group 2 (2653 ± 306 cells/mm2 and 2864 ± 244 cells/mm2, respectively, P < 0.001). The foveal retinal thickness and RNFL thickness were similar, but superior quadrant thickness of RNFL was significantly lower in Group 1 than Group 2 (126 ± 14 µm vs. 135 ± 15 µm, P = 0.003). In Group 1, whole image of optic disc radial peripapillary capillary densities were significantly lower compared to Group 2 (49.4 ± 2.04%, and 50.0 ± 2.2%, respectively, P = 0.043). There was no significant difference regarding superficial, deep retinal vessel densities, foveal avascular zone and flow areas between the groups (P > 0.05 for all). Conclusion: Lower CECD values and decreased superior quadrant RNFL thickness, and microvascular densities of optic disc were revealed in patients with ADPKD. Evaluation of CECD and retinal microvasculature may be helpful in the management of these patients.

Determinants of Outcomes of Acute Kidney Injury: Clinical Predictors and Beyond.

Acute kidney injury (AKI) is a common clinical syndrome characterized by rapid impairment of kidney function. The incidence of AKI and its severe form AKI requiring dialysis (AKI-D) has been increasing over the years. AKI etiology may be multifactorial and is substantially associated with increased morbidity and mortality. The outcome of AKI-D can vary from partial or complete recovery to transitioning to chronic kidney disease, end stage kidney disease, or even death. Predicting outcomes of patients with AKI is crucial as it may allow clinicians to guide policy regarding adequate management of this problem and offer the best long-term options to their patients in advance. In this manuscript, we will review the current evidence regarding the determinants of AKI outcomes, focusing on AKI-D.

Association of serum lncRNA H19 expression with inflammatory and oxidative stress markers and routine biochemical parameters in chronic kidney disease.

BACKGROUND: Chronic kidney disease (CKD) is a disorder that affects millions worldwide, and current treatment options aiming at inhibiting the progression of kidney damage are limited. Long noncoding RNA (lncRNA) H19 is one of the first explored lncRNAs and its deregulation is associated with renal pathologies, such as renal cell injury and nephrotic syndrome. However, there is still no research investigating the connection between serum lncRNA H19 expressions and clinical outcomes in CKD patients. Therefore, we investigated the relation of serum lncRNA H19 expressions with routine biochemical parameters, inflammatory cytokines, oxidative stress and mineralization markers in advanced CKD patients. METHODS: lncRNA H19 serum levels from 56 CKD patients and 20 healthy controls were analyzed with reverse-transcription quantitative polymerase chain reaction method. Serum tumor necrosis factor-alpha (TNF-α), interleukin 6 (IL-6), and osteocalcin (OC) levels were measured with enzyme linked-immunosorbent assay. Total antioxidant status (TAS) and total oxidative status (TOS) levels were evaluated by the routine measurement method. RESULTS: We found that lncRNA H19 expressions were upregulated in patients with CKD compared to the controls. Furthermore, lncRNA H19 relative expression levels showed a negative relationship with glomerular filtration rate (GFR) while it was positively correlated with ferritin, phosphorus, parathyroid hormone, TNF-α, IL-6, OC, TAS and TOS levels. CONCLUSION: lncRNA H19 expressions were increased in CKD stage 3-5 and HD patients, and elevated lncRNA H19 expressions were associated with decreased glomerular filtration rate, inflammation, and mineralization markers in these patients.

Determination of Serum Perfluorooctanoic Acid and Perfluorooctanesulfonic Acid Levels with Different Stages of Chronic Kidney Disease.

The aim of this study is to investigate the perfluorooctanoic acid (PFOA) and perfluorooctanesulfonic acid (PFOS) levels in patients with different stages of chronic kidney disease (CKD). Sixty-one CKD stage 1-4 patients who applied to the nephrology outpatient clinic were recruited. A control group consisting of 26 age- and sex-matched healthy controls were also included in the study. Concentrations of PFOA and PFOS were determined by comparing their peak areas with their standard curves. All samples were analyzed three times. The average values of blank samples were subtracted from the detected PFOA and PFOS values. PFOA and PFOS levels were significantly higher in CKD group than the controls (11.4 ± 7.47, 0.45 ± 0.55; 0.13 ± 0. 17, 0.19 ± 0.4 ng/mL, respectively) (P = 0.001). Hemoglobin, serum albumin, and estimated glomerular filtration rate (eGFR) levels were significantly lower and potassium and uric acid levels were higher in the CKD group than the controls. PFOA and PFOS levels were significantly higher in all stages of CKD patients than healthy controls. However, there was no correlation between eGFR, and PFOS and PFOA. We have demonstrated significantly increased PFOA and PFOS concentrations in different stages of CKD patients. We could not find an association between eGFR, age, and serum PFOS and PFOA concentrations.

Optical coherence tomography angiography analysis of fabry disease.

PURPOSE: Fabry disease (FD) is characterized by a deficiency in α-galactosidase A activity that leads to the cumulative deposition of unmetabolized glycosphingolipids within organs, including the vascular endothelium and the eyes. The purpose of this study was to assess the effects of FD on the retinal microvasculature, foveal avascular zone (FAZ), macular thickness and retinal nerve fiber layer (RNFL) using optical coherence tomography angiography (OCT-A). METHODS: Twenty-five patients (14 female and 11 male; mean age 33.16 ± 11.44) with genetically verified FD were compared with 37 age- and sex-matched healthy controls (mean age 32.36 ± 15.54). The vessel density (VD) values of the superficial and deep capillary plexuses (SCP and DCP), the area of the FAZ, the density of radial peripapillary capillaries (RPC), the macular thickness and the retinal nerve fiber layer thickness were measured by OCT-A examination. RESULTS: The patients showed significantly lower VD values than controls in the foveal regions of both SCP and the DCP (21.15 ± 5.56 vs. 23.79 ± 4.64 (p = 0.048), 37.92 ± 6.78 vs. 41.11 ± 5.59 (p = 0.048), respectively). The FAZ was significantly larger in the FD group than in the control group (0.3 ± 0.1 vs. 0.24 ± 0.08 (p = 0.011)). No significant difference was identified in measurements of RPC density, peripapillary RNFL thickness or macular thickness between the two groups (p > 0.05 for all). CONCLUSION: Decreased VD and an enlarged foveal avascular area suggest possible changes in the retinal microvasculature of patients with FD. OCT-A can serve as a useful, noninvasive, quantitative tool for diagnosing FD and monitoring its progression.

Assessment of corneal topographic, tomographic, densitometric, and biomechanical properties of Fabry patients with ocular manifestations.

PURPOSE: To investigate whether cornea verticillata affects corneal topography, tomography, densitometry, or biomechanics of Fabry patients with ocular manifestations and to compare these results with those obtained from healthy subjects. METHODS: This prospective, cross-sectional study included 23 Fabry patients (Fabry group) with cornea verticillata and the 37 age- and sex-matched healthy subjects (control group). After comprehensive ophthalmological examinations, corneal topography, tomography, and densitometry measurements were taken using Pentacam HR and corneal biomechanics were captured via Corvis ST for all participants. RESULTS: All the investigated topographic and tomographic values were similar in the eyes with Fabry disease (FD) and the controls (P > 0.05). The corneal densitometry values of patients with FD were statistically significantly higher in all the concentric zones and layers, except posterior 0-2 mm and posterior 2-6 mm zones, compared to the controls (P < 0.05). The mean values of A1 velocity, A2 velocity, deformation amplitude ratio, Corvis biomechanical index, tomographic and biomechanical index, and Stiffness parameter at the first applanation in the Fabry group were statistically significantly different compared to control group (P < 0.05). However, the mean values of A1 length, A2 length, and the biomechanically corrected intraocular pressure were similar between the groups (P = 0.317, P = 0.819, and P = 0.468; respectively). CONCLUSION: Although cornea verticillata associated with FD is not considered to affect vision, it is associated with increased light backscattering and reduced corneal transparency as well as altered corneal biomechanical properties.

Retinal Microcirculation in Predicting Diabetic Nephropathy in Type 2 Diabetic Patients without Retinopathy.

PURPOSE: To evaluate retinal thicknesses and retinal microcirculation in healthy controls and in diabetic patients with or without microalbuminuria. METHODS: Eighty-six diabetic patients without diabetic retinopathy (DR) (44 normoalbuminuric, 42 microalbuminuric) and 51 healthy controls were enrolled in this cross-sectional, prospective study. Optical coherence tomography (OCT) and OCT angiography (OCTA) were performed. Correlations between OCTA parameters with mean urinary albumin levels were evaluated. RESULTS: The mean vessel densities of superficial capillary plexus (SCP), whole disc, and peripapillary area were significantly decreased in patients with microalbuminuria compared to patients with normoalbuminuria and controls (p < 0.05 for all). The mean vessel density of deep capillary plexus was significantly reduced in patients with microalbuminuria compared to controls (p < 0.05 for all). There were no significant differences in retinal thickness between groups (p > 0.05). Both duration of diabetes and urinary albumin levels were significantly and moderately correlated with mean vessel density of whole SCP in diabetic patients (r = 0.330, p = 0.021; r = 0.356, p = 0.017, respectively). CONCLUSION: Diabetic eyes without clinically detectable DR show impaired retinal microcirculation. Microalbuminuria is associated with alterations of retinal microcirculation in diabetic patients without DR. Evaluation of retinal microcirculation is likely useful for detecting early changes related to microvascular complications in type 2 diabetic patients.

Upper Zone of Growth Plate and Cartilage Matrix (UCMA) Levels in Patients with Chronic Kidney Disease

Abstract Chronic kidney disease (CKD) is an important health problem across the world affecting the adult population with an enormous social and economic burden. Calcium regulation is also affected in patients with CKD, and related to several disorders including vascular calcifications, mineral bone disorders, and cardiovascular diseases (CVD). Upper zone of growth plate and cartilage matrix (UCMA) is vitamin K-dependent protein (VKDP) and acts as a calcification inhibitor in the cardiovascular system. The molecular mechanism of UCMA action remains unclear in CKD. In the current study, we aimed to investigate serum total UCMA levels and its association with calcium metabolism parameters in CKD patients including hemodialysis (HD) patients. Thirty-seven patients with CKD stage 3-5, 41 HD patients, and 34 healthy individuals were enrolled in this cross-sectional study. Serum UCMA and calcification related protein levels (Matrix Gla Protein (MGP), Osteocalcin (OC), and Fetuin-A) were analyzed with enzyme-linked immunosorbent assay (ELISA). Calcium mineral disorder parameters (Serum Ca, P, iPTH) were quantified with routine techniques. We, for the first time, report the potential biomarker role of UCMA in CKD including HD. Serum total UCMA levels were significantly higher in patients with CKD including HD patients than the healthy controls. Also, serum UCMA levels showed negative correlations with serum calcium, and eGFR, while showed positive relationships with P, iPTH, MGP, OC. Increased total UCMA levels may have a role in the Ca metabolism disorder and related to the pathogenesis of Vascular Calcification in patients with CKD.

Serum Matrix Metalloproteinase-9, Tissue Inhibitor of Metalloproteinase-1 and Matrix Metalloproteinase-9/ Neutrophil Gelatinase-associated Lipocalin Complex Levels in Patients With Early-stage Diabetic Nephropathy.

INTRODUCTION: This study was aimed to investigate the efficacy of matrix metalloproteinase-9 (MMP-9), tissue inhibitor of metalloproteinase-1 (TIMP-1), and the neutrophil gelatinase-associated lipocalin (NGAL)-bound form of MMP-9 (MMP-9/NGAL complex) markers in the determination of early nephropathy in patients with type 2 diabetes mellitus. MATERIALS AND METHODS: Twenty-five type 2 diabetic patients with normoalbuminuria, 27 type 2 diabetic patients with microalbuminuria, and 23 healthy controls were recruited. Serum levels of MMP-9, TIMP-1, and MMP9/NGAL complex were measured in all participants. RESULTS: The MMP-9 level and MMP-9/TIMP-1 ratio were higher in patients with microalbuminuria when compared to the controls, while TIMP-1 level was lower (P = .005, P = .006, and P = .02, respectively). The MMP-9 level and MMP-9/TIMP-1 ratio were significantly higher in the patients with normoalbuminuria when compared to the controls (P = .005 and P = .02, respectively). In the normoalbuminuric patients, MMP-9 levels were negatively correlated with estimated GFR (r = -0.553, P = .008) and positively correlated with glucose (r = 0.449, P = .04), creatinine (r = 0.454, P = .03), and MMP9/NGAL complex (r = 0.575, P = .005). In the microalbuminuric patients, MMP-9 levels were positively correlated with total cholesterol (r = 0.430, P = .03), and MMP9/NGAL complex (r = 0.650, P = .001). CONCLUSIONS: The levels of MMP-9, TIMP-1, and MMP9/NGAL complex were similar in microalbuminuric and normoalbuminuric patients with type 2 diabetes mellitus. However, there was a significant MMP-9/TIMP-1 imbalance in both groups which may reflect impaired extracellular matrix homeostasis.

The association between methylation levels of targeted genes and albuminuria in patients with early diabetic kidney disease.

OBJECTIVE: The incidence of diabetes and its complications are greatly increasing world-wide. Diabeticnephropathy (DN) is the main cause of end-stage renal disease and is associated with high morbidity and mortality. It is important to predict patients with high risk for DN in the early stage. We selected the genes which have an important role on diabetic kidney disease. We aimed to investigate the association between DNA methylation levels of targeted genes and albuminuria in patients with early DN. METHODS: We collected the clinical data of patients with type 2 diabetes mellitus. We measured spot urine albumin creatinine ratio to calculate albuminuria level. We divided patients into two groups based on albumin excretion as patients with (n = 69) and without DN (n = 27). We performed methylation profiling after bisulfite conversion by pyrosequencing method. The mean value of percent methylation level of each gene was calculated. RESULTS: We compared targeted genes (TIMP-2, AKR1B1, MMP-2, MMP-9, MYL9, SCL2A4, SCL2A1, SCL4A3) methylation levels and albuminuria. We found significant negative correlation between TIMP-2 and AKR1B1 gene methylation levels and albuminuria levels. CONCLUSIONS: The present study provided evidence that hypomethylation of TIMP-2 and AKR1B1 genes can be associated with albuminuria in patients with early DN. We may speculate that the hypomethylation of TIMP-2 and AKR1B1 genes may be an early surrogate marker of DN.

Challenges Associated with Managing End-Stage Renal Disease in Extremely Morbid Obese Patients: Case Series and Literature Review.

Obesity has grown into an epidemic across the globe. Mirroring this growth in the number of obese individuals in the general population, the prevalence of obesity and extremely morbid obesity (body mass index >50 kg/m2) is similarly rising in patients with end-stage renal disease (ESRD). Obesity, and more so, extremely morbid obesity, creates additional challenges for clinicians aiming at providing the best treatment options to patients with ESRD. Although literature is available regarding renal replacement therapy (RRT) for obese patients, literature about challenges facing clinicians managing ESRD in the extremely obese patients is lacking. Furthermore, the optimal strategies and goals for managing these patients with ESRD are not well defined. To our knowledge, no study has been done evaluating RRT options available to extremely morbid obese patients and the challenges associated with these options. Here, we report a case series and discuss the potential challenges faced by extremely obese patients with ESRD requiring RRT.

Dialysis Requirement and Long-Term Major Adverse Cardiovascular Events in Patients with Chronic Kidney Disease and Superimposed Acute Kidney Injury.

BACKGROUND: Chronic kidney disease (CKD) patients who experience superimposed acute kidney injury (AKI) have been shown to be at higher risk of long-term sequelae of AKI when compared to those who do not experience AKI. It remains unclear whether the need for temporary dialysis intervention following superimposed AKI in patients with CKD has any effect on the long-term major adverse cardiovascular events (MACE). This study examines the relationship between temporary dialysis therapy following AKI and long-term major cardiovascular events in patients with background CKD. METHODS: The study population consists of adults who developed AKI while on admission at the University of Virginia Medical Center between January 1, 2002 and December 31, 2012, and who had preadmission estimated glomerular filtration rate (eGFR) between 20 and 60 mL/min/1.73 m2 and survived beyond 30 days of AKI. Demographic and baseline clinical variables were used to generate propensity score. Survivors who had temporary dialysis were matched to those managed conservatively according to the propensity score in a ratio of 1:3. RESULTS: Overall, 6,634 (n = 381 and 6,253 in the temporary dialysis-requiring AKI and non-dialysis AKI groups respectively) met entry criteria for the full cohort. Of these, 381 (5.7%) received temporary dialysis. There were 3,147 (47.4% of all patients) MACE events during the study period. The crude incidence for MACE after 30 days of AKI was similar in both dialyzed and non-dialyzed patients. After the propensity score matching, the adjusted hazard ratio for MACE in dialyzed versus non dialyzed patients was 1.162 (95% CI 0.978-1.381). CONCLUSIONS: Treatment of AKI with temporary dialysis in hospitalized patients with baseline eGFR between 20 and 60 mL/min/1.73 m2 was not associated with an increased risk for subsequent admission for MACE. If confirmed by prospective studies, clinicians may not need to worry that the dialysis procedure may contribute to additional risk for long-term MACE in CKD patients with superimposed AKI.

Role of ultrasonographic chronic kidney disease score in the assessment of chronic kidney disease.

PURPOSE: Ultrasonography (US) is an inexpensive, noninvasive and easy imaging procedure to comment on the kidney disease. Data are limited about the relation between estimated glomerular filtration rate (e-GFR) and all 3 renal US parameters, including kidney length, parenchymal thickness and parenchymal echogenicity, in chronic kidney disease (CKD). In this study, we aimed to investigate the association between e-GFR and ultrasonographic CKD score calculated via these ultrasonographic parameters. METHODS: One hundred and twenty patients with stage 1-5 CKD were enrolled in this study. The glomerular filtration rate was estimated by the Chronic Kidney Disease Epidemiology Collaboration equation. US was performed by the same radiologist who was blinded to patients' histories and laboratory results. US parameters including kidney length, parenchymal thickness and parenchymal echogenicity were obtained from both kidneys. All 3 parameters were scored for each kidney, separately. The sum of the average scores of these parameters was used to calculate ultrasonographic CKD score. RESULTS: The mean age of patients was 63.34 ± 14.19 years. Mean kidney length, parenchymal thickness, ultrasonographic CKD score and median parenchymal echogenicity were found as 96.2 ± 12.3, 10.97 ± 2.59 mm, 6.28 ± 2.52 and 1.0 (0-3.5), respectively. e-GFR was positively correlated with kidney length (r = 0.343, p < 0.001), parenchymal thickness (r = 0.37, p < 0.001) and negatively correlated with CKD score (r = -0.587, p < 0.001) and parenchymal echogenicity (r = -0.683, p < 0.001). Receiver operating characteristic curve analysis for distinction of e-GFR lower than 60 mL/min showed that the ultrasonographic CKD score higher than 4.75 was the best parameter with the sensitivity of 81% and positive predictivity of 92% (AUC, 0.829; 95% CI, 0.74-0.92; p < 0.001). CONCLUSION: We found correlation between e-GFR and ultrasonographic CKD score via using all ultrasonographic parameters. Also, our study showed that ultrasonographic CKD score can be useful for distinction of CKD stage 3-5 from stage 1 and 2. We suggested that the ultrasonographic CKD score provided more objective data in the assessment of CKD.

The Prevalence of Fabry Disease in Patients with Chronic Kidney Disease in Turkey: The TURKFAB Study.

BACKGROUND/AIMS: Fabry disease is a treatable cause of chronic kidney disease (CKD) characterized by a genetic deficiency of α-galactosidase A. European Renal Best Practice (ERBP) recommends screening for Fabry disease in CKD patients. However, this is based on expert opinion and there are no reports of the prevalence of Fabry disease in stage 1-5 CKD. Hence, we investigated the prevalence of Fabry disease in CKD patients not receiving renal replacement therapy. METHODS: This prospective study assessed α-galactosidase activity in dried blood spots in 313 stage 1-5 CKD patients, 167 males, between ages of 18-70 years whose etiology of CKD was unknown and were not receiving renal replacement therapy. The diagnosis was confirmed by GLA gene mutation analysis. RESULTS: Three (all males) of 313 CKD patients (0.95%) were diagnosed of Fabry disease, for a prevalence in males of 1.80%. Family screening identified 8 aditional Fabry patients with CKD. Of a total of 11 Fabry patients, 7 were male and started enzyme replacement therapy and 4 were female. The most frequent manifestations in male patients were fatigue (100%), tinnitus, vertigo, acroparesthesia, hypohidrosis, cornea verticillata and angiokeratoma (all 85%), heat intolerance (71%), and abdominal pain (57%). The most frequent manifestations in female patients were fatigue and cornea verticillata (50%), and tinnitus, vertigo and angiokeratoma (25%). Three patients had severe episodic abdominal pain attacks and proteinuria, and were misdiagnosed as familial Mediterranean fever. CONCLUSIONS: The prevalence of Fabry disease in selected CKD patients is in the range found among renal replacement therapy patients, but the disease is diagnosed at an earlier, treatable stage. These data support the ERBP recommendation to screen for Fabry disease in patients with CKD of unknown origin.

Higher Serum Bisphenol A Levels in Diabetic Hemodialysis Patients.

BACKGROUND: Bisphenol A (BPA) has been implicated as an 'endocrine disruptor'. We aimed at exploring the association between serum BPA levels and patient characteristics, particularly the presence of diabetes mellitus, and laboratory parameters in hemodialysis patients. METHODS: This study included 47 chronic hemodialysis patients. Patient characteristics were recorded. Blood was drawn before and after hemodialysis session. Serum BPA levels were measured by the high-performance-liquid-chromatography and laboratory parameters were measured by using standard methods. RESULTS: In hemodialysis patients, postdialysis serum BPA levels were significantly higher than predialysis after a single hemodialysis session (5.57 ± 1.2 vs. 4.06 ± 0.73, p < 0.0001). Predialysis serum BPA levels were significantly higher in patients with diabetes than non-diabetics (4.4 ± 0.6 vs. 3.9 ± 0.7, p = 0.025). No association was found between serum BPA levels and patient characteristics, and particularly laboratory parameters. CONCLUSION: Serum BPA levels were rising significantly after a single dialysis session. Diabetic hemodialysis patients had higher predialysis serum BPA levels.

Platelet-to-lymphocyte ratio predicts mortality better than neutrophil-to-lymphocyte ratio in hemodialysis patients.

PURPOSE: Neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) were established showing the poor prognosis in some diseases, such as cardiovascular diseases and malignancies. The risk of mortality in patients with end-stage renal disease (ESRD) was higher than normal population. In this study, we aimed to investigate the relationship between NLR, PLR, and all-cause mortality in prevalent hemodialysis (HD) patients. METHODS: Eighty patients were enrolled in study. NLR and PLR obtained by dividing absolute neutrophil to absolute lymphocyte count and absolute platelet count to absolute lymphocyte count, respectively. The patients were followed prospectively for 24 months. The primary end point was all-cause mortality. RESULTS: Mean levels of neutrophil, lymphocyte, and platelet were 3904 ± 1543/mm(3), 1442 ± 494/mm(3), 174 ± 56 × 10(3)/mm(3), respectively. Twenty-one patients died before the follow-up at 24 months. Median NLR and PLR were 2.52 and 130.4, respectively. All-cause mortality was higher in patients with high NLR group compared to the patients with low NLR group (18.8 vs. 7.5 %, p = 0.031) and in patients with higher PLR group compared to patients with lower PLR group (18.8 vs. 7.5 %, p = 0.022). Following adjusted Cox regression analysis, the association of mortality and high NLR was lost (p = 0.54), but the significance of the association of high PLR and mortality increased (p = 0.013). CONCLUSION: Although both NLR and PLR were associated with all-cause mortality in prevalent HD patients, only PLR could independently predict all-cause mortality in these populations.