The effects of the dialysis on the white matter tracts in patients with end-stage renal disease using differential tractography study.

This study aimed to determine whether white matter tracts correlate with kidney function using correlation tractography, and to investigate the effects of dialysis on white matter tracts in patients with end-stage renal disease (ESRD) using differential tractography. Ten patients with ESRD, who had a glomerular filtration rate of < 15 mL/min/1.73 m2, were enrolled in this prospective study. Diffusion tensor imaging (DTI) was performed both before and after dialysis. We discovered that white matter tracts correlated with the estimated glomerular filtration rate based on pre- and post-dialysis DTI using correlation tractography and investigated the differences in the white matter tracts between pre- and post-dialysis DTI in patients with ESRD using differential tractography. Correlation tractography revealed no quantitative anisotropy of the white matter tracts that correlated with the estimated glomerular filtration rate in pre- and post-dialysis patients with ESRD. Differential tractography revealed significant differences in several white matter tracts, particularly the cingulum, thalamic radiation, corpus callosum, and superior longitudinal fasciculus, between pre- and post-dialysis DTI, which revealed increased diffusion density after dialysis. We demonstrated the significant effects of dialysis on several white matter tracts in patients with ESRD using differential tractography, which showed increased diffusion density after dialysis. In this study, we confirmed the effects of dialysis on brain structure, especially white matter tracts.

Difference in functional connectivity between end-stage renal disease patients with and without restless legs syndrome: A prospective study.

PURPOSE: The purpose of this study was to examine differences in functional connectivity between patients with end-stage renal disease (ESRD) with and without restless legs syndrome (RLS). In addition, the study aimed to identify any potential associations between RLS severity and functional connectivity. METHODS: We enrolled patients with ESRD who had been undergoing hemodialysis. Patients with and without RLS were separated into two groups. Using functional near-infrared spectroscopy (fNIRS) and a graph theory approach, we determined the functional connectivity of patients with ESRD. The data were collected during a 300-s resting state evaluation performed in the dialysis room prior to dialysis. RESULTS: Eighteen of 48 patients with ESRD were diagnosed with RLS, whereas 30 patients did not exhibit RLS symptoms. Notably, functional connectivity metrics differed significantly between patients with and without RLS. Specifically, patients with ESRD and RLS displayed higher values for mean clustering coefficient (0.474 vs. 0.352, p = 0.001), global efficiency (0.520 vs. 0.414, p = 0.001), strength (6.538 vs. 4.783, p = 0.001), and transitivity (0.714 vs. 0.521, p = 0.001), while values for diameter (5.451 vs. 7.338, p = 0.002), eccentricity (4.598 vs. 5.985, p = 0.004), and characteristic path length (2.520 vs. 3.271, p = 0.002) were lower in patients with ESRD and RLS compared to those without RLS. In addition, there were correlations between the RLS severity score and the assortative coefficient (r = 0.479, p = 0.044), the small-worldness index (r = -0.475, p = 0.046), and transitivity (r = 0.500, p = 0.034). CONCLUSIONS: We demonstrated differences in functional connectivity between patients with ESRD with and without RLS, which may shed light on the pathophysiology of RLS. Notably, a number of functional connectivity metrics demonstrated strong associations with RLS severity. Our study also confirmed the applicability of fNIRS as a tool for investigating functional connectivity in patients with RLS.

Changes in the glymphatic system before and after dialysis initiation in patients with end-stage kidney disease.

INTRODUCTION: The aims of this study were to evaluate 1) glymphatic system function in patients with end-stage kidney disease (ESKD) before initiating dialysis compared to healthy controls, and 2) changes in the glymphatic system function after kidney replacement therapy including dialysis in patients with ESKD using the diffusion tensor image analysis along the perivascular space (DTI-ALPS) method. MATERIALS AND METHODS: This study was prospectively conducted at a single hospital. We enrolled 14 neurologically asymptomatic patients who first initiated hemodialysis or peritoneal dialysis for ESKD and 17 healthy controls. Patients had magnetic resonance imaging scans before initiating dialysis and again 3 months after initiating dialysis and the DTI-ALPS index was calculated. We compared the DTI-ALPS index before and after the initiation of dialysis and compared the DTI-ALPS index between the patients with ESKD and healthy control. RESULTS: There were differences in the DTI-ALPS index between ESKD patients before initiating dialysis and healthy controls (1.342 vs. 1.633, p = 0.003). DTI-ALPS index between ESKD patients before initiating dialysis and those after dialysis were not different (1.342 vs. 1.262, p = 0.386). There was a positive correlation between DTI-ALPS index and phosphate (r = 0.610, p = 0.020) in patients with ESKD. CONCLUSION: We confirmed the presence of glymphatic dysfunction in patients with ESKD. However, there was no difference in the glymphatic system before and after dialysis initiation. This finding may be related to uremic toxins that are not removed by dialysis in patients with ESKD. This study can be used for the development of pathophysiology of patients with ESKD.

Intrinsic prefrontal functional connectivity according to cognitive impairment in patients with end-stage renal disease.

Background: This study aimed to investigate differences in intrinsic prefrontal functional connectivity according to the presence of cognitive impairment in patients with end-stage renal disease (ESRD) using functional near-infrared spectroscopy (fNIRS). Methods: We prospectively enrolled 37 patients with ESRD who had been undergoing hemodialysis for more than 6 months and had no history of neurological or psychiatric disorders. All patients with ESRD underwent the Korean version of the Montreal Cognitive Assessment (MoCA-K) to assess cognitive function. The NIRSIT Lite device (OBELAB Inc.) was used to acquire fNIRS data, and the NIRSIT Lite Analysis Tool program was used to process the data and generate a functional connectivity matrix. We obtained functional connectivity measures by applying graph theory to the connectivity matrix using the BRAPH (brain analysis using graph theory) program. Results: Of the 37 patients with ESRD, 23 had cognitive impairment, whereas 14 patients showed no cognitive impairment. Intrinsic prefrontal functional connectivity was significantly different between groups. Network measures of strength, global efficiency, and mean clustering coefficient were lower in ESRD patients with cognitive impairment than in those without cognitive impairment (4.458 vs. 5.129, p = 0.02; 0.397 vs. 0.437, p = 0.03; and 0.316 vs. 0.421, p = 0.003; respectively). There were no significant correlations between MoCA-K scores and clinical characteristics. Conclusion: We demonstrated a significant association between cognitive function and intrinsic prefrontal functional connectivity in patients with ESRD. ESRD patients with cognitive impairment have reduced connectivity and segregation in the prefrontal brain network compared to those without cognitive impairment.

The effects of hemodialysis on the functional brain connectivity in patients with end-stage renal disease with functional near-infrared spectroscopy.

This study aimed to investigate functional brain connectivity in patients with end-stage renal disease (ESRD) undergoing hemodialysis using functional near-infrared spectroscopy (fNIRS) and to analyze the effect of hemodialysis on functional brain connectivity. We prospectively enrolled patients with ESRD undergoing hemodialysis for > 6 months without any history of neurological or psychiatric disorders. fNIRS data were acquired using a NIRSIT Lite device. Measurements were performed thrice in the resting state for each patient: before the start of hemodialysis (pre-HD), 1 h after the start of hemodialysis (mid-HD), and after the end of hemodialysis (post-HD). We processed and exported all data, and created a weighted connectivity matrix using Pearson correlation analysis. We obtained functional connectivity measures from the connectivity matrix by applying a graph theoretical analysis. We then compared differences in functional connectivity measures according to hemodialysis status in patients with ESRD. We included 34 patients with ESRD. There were significant changes in the mean clustering coefficient, transitivity, and assortative coefficient between the pre- and post-HD periods (0.353 vs. 0.399, p = 0.047; 0.523 vs. 0.600, p = 0.042; and 0.043 vs. - 0.012, p = 0.044, respectively). However, there were no changes in the mean clustering coefficient, transitivity, and assortative coefficient between the pre- and mid-HD periods, or between the mid- and post-HD periods. In addition, there were no significant differences in the average strength, global efficiency, and local efficiency among the pre-, mid-, and post-HD periods. We demonstrated a significant effect of hemodialysis on functional brain connectivity in patients with ESRD. Functional brain connectivity changes more efficiently during hemodialysis.

Humoral response to vaccination against SARS-CoV-2 in patients undergoing dialysis.

Patients undergoing maintenance dialysis have a higher mortality rate associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, and response rates to vaccination against SARS-CoV-2 vary from 29.6% to 96.4% in such patients. This study aimed to assess the immunogenicity of SARS-CoV-2 vaccination in Korean patients undergoing dialysis. We enrolled 70 SARS-CoV-2-vaccinated patients undergoing dialysis, with 11 healthcare workers serving as healthy control subjects. Thirty-two patients had received a third vaccination, whereas 38 had received 2 vaccinations. The healthy control subjects completed the second vaccination. Immunoglobulin G (IgG) antibodies targeting the receptor-binding domain of the S1 subunit of the SARS-CoV-2 spike protein were measured The vaccination responder rates were 86% (37/43), 96% (26/27), and 91% (10/11) in the patients undergoing hemodialysis and peritoneal dialysis and healthy controls, respectively. IgG antibody levels were significantly higher when a third dose was administered, independent of the type of vaccine or the time interval between vaccination and the subsequent blood sampling date. When a third dose of vaccine was administered, there was no difference in IgG antibody levels between those receiving cross-vaccination or a single vaccine. There was no significant difference in IgG antibodies between healthy controls and patients undergoing dialysis. Patients on dialysis exhibited a sufficient antibody-related response to vaccination against SARS-CoV-2, even in those receiving cross-vaccination, and the antibody titer was higher after a third vaccination. Therefore, it is necessary to administer a third vaccine dose to Korean patients undergoing dialysis.

Glymphatic system dysfunction in patients with early chronic kidney disease.

Introduction: It is a recent finding that glymphatic system dysfunction contributes to various neurological problems. The purpose of this research was to assess the function of the glymphatic system in neurologically asymptomatic early chronic kidney disease (CKD) patients and healthy controls, using diffusion tensor image analysis along perivascular space (DTI-ALPS) index. Methods: In a prospective study, we included patients with early CKD who were asymptomatic for neurological issues and obtained clinical and laboratory data. In all participants, brain magnetic resonance imaging (MRI) with diffusion tensor imaging (DTI) was conducted. We used DSI program for DTI preprocessing and DTI-ALPS index estimation. The DTI-ALPS index was compared between patients with early CKD and healthy controls, and the association between clinical characteristics and the DTI-ALPS index was investigated. Results: Eighteen patients with early CKD and 18 healthy controls were included in this study. Patients with early CKD had lower DTI-ALPS index than healthy controls (1.259 ± 0.199 vs. 1.477 ± 0.232, p = 0.004). In the correlation analysis, the DTI-ALPS index had no significant relationship with other clinical factors. Conclusion: We suggest dysfunction of glymphatic system in patients with early chronic kidney disease using the DTI-ALPS index. This may be related to the pathophysiology of neurological problems including impairment of cognition in patients with early CKD.

Top 100 cited articles on hemodialysis: A bibliometric analysis.

INTRODUCTION: This study was conducted to better understand hemodialysis by reviewing the most-cited articles related to it. METHODS: We searched articles on the Web of Science and selected the 100 most frequently cited articles. Subsequently, we reviewed these articles and identified their characteristics. RESULTS: The 100 most frequently cited articles were published in 21 journals. The majority of these papers were published in the following journals: Kidney International (26 articles), New England Journal of Medicine (18 articles), Journal of the American Society of Nephrology (14 articles), and the American Journal of Kidney Disease (13 articles). The 100 most-cited articles were published in 25 countries. The United States of America was the country with the highest number of publications (65 articles). The University of Michigan was the institution with the highest number of articles (14 articles). FK Port was the author with the largest number of publications (13 articles). CONCLUSIONS: This is the first study in the field of nephrology that provides a list of the 100 most-cited articles on hemodialysis. Through this study, clinicians will be able to recognize major academic interests and research trends in hemodialysis.

Artificial intelligence with kidney disease: A scoping review with bibliometric analysis, PRISMA-ScR.

BACKGROUND: Artificial intelligence (AI) has had a significant impact on our lives and plays many roles in various fields. By analyzing the past 30 years of AI trends in the field of nephrology, using a bibliography, we wanted to know the areas of interest and future direction of AI in research related to the kidney. METHODS: Using the Institute for Scientific Information Web of Knowledge database, we searched for articles published from 1990 to 2019 in January 2020 using the keywords AI; deep learning; machine learning; and kidney (or renal). The selected articles were reviewed manually at the points of citation analysis. RESULTS: From 218 related articles, we selected the top fifty with 1188 citations in total. The most-cited article was cited 84 times and the least-cited one was cited 12 times. These articles were published in 40 journals. Expert Systems with Applications (three articles) and Kidney International (three articles) were the most cited journals. Forty articles were published in the 2010s, and seven articles were published in the 2000s. The top-fifty most cited articles originated from 17 countries; the USA contributed 16 articles, followed by Turkey with four articles. The main topics in the top fifty consisted of tumors (11), acute kidney injury (10), dialysis-related (5), kidney-transplant related (4), nephrotoxicity (4), glomerular disease (4), chronic kidney disease (3), polycystic kidney disease (2), kidney stone (2), kidney image (2), renal pathology (2), and glomerular filtration rate measure (1). CONCLUSIONS: After 2010, the interest in AI and its achievements increased enormously. To date, AIs have been investigated using data that are relatively easy to access, for example, radiologic images and laboratory results in the fields of tumor and acute kidney injury. In the near future, a deeper and wider range of information, such as genetic and personalized database, will help enrich nephrology fields with AI technology.

Alteration of brain connectivity in neurologically asymptomatic patients with chronic kidney disease.

ABSTRACT: Our previous study demonstrated that patients with end-stage renal disease had decreased structural and functional brain connectivity, and there was a significant association between brain connectivity and cognitive function. The aim of this study was to evaluate the alterations of structural and functional connectivity using graph theoretical analysis in neurologically asymptomatic patients with relatively early-stage chronic kidney disease (CKD).We enrolled 18 neurologically asymptomatic patients with early CKD and 28 healthy controls. All the subjects underwent diffusion-tension imaging and resting functional magnetic resonance imaging. We calculated structural and functional connectivity based on diffusion-tension imaging and resting functional magnetic resonance imaging using a graph theoretical analysis. Then, we investigated differences of structural and functional connectivity between the CKD patients and the healthy controls.All the measures of structural connectivity were significantly different between the patients with CKD and healthy controls. The global efficiency, local efficiency, mean clustering coefficient, and small-worldness index were decreased, whereas the characteristic path length was increased in the patients with CKD compared with healthy controls. The structural betweenness centrality of the left calcarine and right posterior cingulum was also significantly different from that in healthy participants. However, all the measures of global functional connectivity in patients with CKD were not different from those in healthy controls. In patients with CKD, the functional betweenness centrality of the right insular cortex, right occipital pole, and right thalamus was significantly different from that in healthy participants.There are significant alterations of the global structural connectivity between the patients with CKD and the healthy subjects, whereas the global functional connectivity of the brain network is preserved. We find that the efficiency of the structural brain network is decreased in the patients with CKD.

Comparison of Different Types of Oral Adsorbent Therapy in Patients with Chronic Kidney Disease: A Multicenter, Randomized, Phase IV Clinical Trial.

PURPOSE: Oral adsorbents delay disease progression and improve uremic symptoms in patients with chronic kidney disease (CKD). DW-7202 is a newly developed oral adsorbent with high adsorptive selectivity for uremic toxins. We evaluated patient preference for and adherence to DW-7202 versus AST-120 therapy and compared treatment efficacy and safety in patients with pre-dialysis CKD. MATERIALS AND METHODS: A seven-center, randomized, open-label, two-way crossover, active-controlled, phase IV clinical trial was conducted. Patients with stable CKD were randomly assigned to receive DW-7202 (capsule type) or AST-120 (granule type) for 12 weeks. The groups then switched to the other adsorbent and took it for the next 12 weeks. Patient preference was the primary outcome. Secondary outcomes included changes in estimated glomerular filtration rate (eGFR) and serum creatinine, cystatin C, and indoxyl sulfate (IS) levels. RESULTS: Significantly more patients preferred DW-7202 than AST-120 (p<0.001). Patient adherence improved after switching from AST-120 to DW-7202; there was no apparent change in adherence after switching from DW-7202 to AST-120. Changes in eGFR and serum creatinine, cystatin C, and IS levels were not significantly different according to adsorbent type. There was also no significant difference in the incidences of adverse events during treatment with DW-7202 and AST-120. CONCLUSION: DW-7202 can be considered as an alternative to AST-120 in patients who cannot tolerate or show poor adherence to granule type adsorbents. Further studies to evaluate factors affecting patient preferences and improved adherence are warranted (Clinical trial registration No. NCT02681952).

Glymphatic Dysfunction in Patients With End-Stage Renal Disease.

BACKGROUND: We aimed to compare glymphatic dysfunction between patients with end-stage renal disease (ESRD) and healthy controls and analyze the correlation between the glymphatic function and clinical characteristics using the diffusion tensor image analysis along with the perivascular space (DTI-ALPS) index. METHODS: We prospectively enrolled neurologically asymptomatic 49 patients with ESRD undergoing dialysis and 38 healthy controls. Diffusion tensor image was conducted using the same 3T scanner, and the DTI-ALPS index was calculated. We compared the DTI-ALPS index between the patients with ESRD and healthy controls. In addition, we conducted a correlation analysis between the clinical characteristics and DTI-ALPS index in patients with ESRD. RESULTS: There were significant differences in the DTI-ALPS index between patients with ESRD and healthy controls. The DTI-ALPS index in patients with ESRD was lower than that in healthy controls (1.460 vs. 1.632, p = 0.003). In addition, there was a significant positive correlation between the DTI-ALPS index and serum parathyroid hormone levels (r = 0.357, p = 0.011). CONCLUSION: We demonstrated glymphatic dysfunction in patients with ESRD, as revealed by the DTI-ALPS index. This study also reveals the feasibility of the DTI-ALPS method to determine glymphatic function in patients with ESRD, which could be used in future research studies.

Analysis of the risk factors of acute kidney injury after total hip or knee replacement surgery.

BACKGRUOUND: Postoperative acute kidney injury (AKI), which increases the risk of postoperative morbidity and mortality, poses a major concern to surgeons. We conducted this study to analyze the risk factors associated with the occurrence of AKI after orthopedic surgery. METHODS: This was a retrospective study that included 351 patients who underwent total hip or knee replacement surgery at Inje University Haeundae Paik Hospital between January 2012 and December 2016. RESULTS: AKI occurred in 13 (3.7%) of the 351 patients. The patients' preoperative estimated glomerular filtration rate (eGFR) was 66.66±34.02 mL/min/1.73 m2 in the AKI group and 78.07±21.23 mL/min/1.73 m2 in the non-AKI group. The hemoglobin levels were 11.21±1.65 g/dL in the AKI group and 12.39±1.52 g/dL in the non-AKI group. Hemoglobin level was related to increased risk of AKI (odds ratio [OR], 0.13; 95% confidence interval [CI], 0.02-0.68; p=0.016). Administration of crystalloid or colloid fluid alone and the perioperative amount of fluid did not show any significant relationship with AKI. Further analysis of the changes in eGFR was performed using a cutoff value of 7.54. The changes in eGFR were significantly related to decreased risk of AKI (OR, 0.74; 95% CI, 0.61-0.89; p=0.002). CONCLUSION: Renal function should be monitored closely after orthopedic surgery if patients have chronic kidney disease and low hemoglobin level. Predicting the likelihood of AKI occurrence, early treatment of high-risk patients, and monitoring perioperative laboratory test results, including eGFR, will help improve patient prognosis.

Clinical efficacy of biomarkers for evaluation of volume status in dialysis patients.

Volume status is a key parameter for cardiovascular-related mortality in dialysis patients. Although N-terminal pro-B-type natriuretic peptide (NT-proBNP), myeloperoxidase, copeptin, and pro-adrenomedullin have been reported as volume markers, the relationship between body fluid status and volume markers in dialysis patients is uncertain. Therefore, we investigated the utility of volume status biomarkers based on body composition monitor (BCM) analyses.We enrolled pre-dialysis, hemodialysis (HD), and peritoneal dialysis (PD) patients and age- and gender-matched healthy Korean individuals (N = 80). BCM and transthoracic echocardiography were performed and NT-proBNP, myeloperoxidase, copeptin, and pro-adrenomedullin concentrations were measured. Relative hydration status (ΔHS, %) was defined in terms of the hydration status-to-extracellular water ratio with a cutoff of 15%, and hyperhydrated status was defined as ΔHS > 15%.Although there were no significant differences in total body water, extracellular water, or intracellular water among groups, mean amount of volume overload and hyperhydrated status were significantly higher in HD and PD patients compared with control and pre-dialysis patients. Mean amount of volume overload and hyperhydrated status were also significantly associated with higher NT-proBNP and pro-adrenomedullin levels in HD and PD patients, although not with myeloperoxidase or copeptin levels. Furthermore, they were significantly associated with cardiac markers (left ventricular mass index, ejection fraction, and left atrial diameter) in HD and PD patients compared with those in the control and pre-dialysis groups.On the basis of increased plasma NT-proBNP and pro-adrenomedullin concentrations, we might be able to make predictions regarding the volume overload status of dialysis patients, and thereby reduce cardiovascular-related mortality through appropriate early volume control.

Alterations in Structural and Functional Connectivities in Patients with End-Stage Renal Disease.

BACKGROUND AND PURPOSE: The aim of this study was to evaluate the structural and functional connectivities of brain network using graph theoretical analysis in neurologically asymptomatic patients with end-stage renal disease (ESRD). We further investigated the prevalence of cognitive impairment (CI) in ESRD patients and analyzed the association between network measures of brain connectivity and cognitive function. METHODS: We prospectively enrolled 40 neurologically asymptomatic ESRD patients, 40 healthy controls, and 20 disease controls. All of the subjects underwent diffusion-tensor imaging (DTI) and resting-state functional magnetic resonance imaging (rs-fMRI). We calculated measures of structural and functional connectivities based on DTI and rs-fMRI, respectively, and investigated differences therein between the ESRD patients and the healthy controls. We assessed cognitive function in the ESRD patients using the Korean version of the Consortium to Establish a Registry for Alzheimer's Disease neuropsychological battery. RESULTS: The ESRD patients exhibited decreased global structural and functional brain connectivities, as well as alterations of network hubs compared to the healthy controls and disease controls. About 70% of the ESRD patients had CI. Moreover, ESRD patients without CI exhibited decreased global connectivity and alterations of network hubs. Furthermore, there was a significant positive association between measures of brain connectivity and cognitive function. CONCLUSIONS: We found that ESRD patients exhibited decreased structural and functional brain connectivities, and that there was a significant association between brain connectivity and cognitive function. These alterations in the brain network may contribute to the pathophysiological mechanism of CI in ESRD patients.

Differences of connectivity between ESRD patients with PD and HD.

OBJECTIVES: The aim of this study was to investigate alterations in structural and functional brain connectivity between patients with end-stage renal disease (ESRD) who were undergoing peritoneal dialysis (PD) and hemodialysis (HD). METHODS: We enrolled 40 patients with ESRD who were undergoing PD (20 patients) and HD (20 patients). We also enrolled healthy participants as a control group. All of the subjects underwent diffusion tensor imaging (DTI) and resting-state functional magnetic resonance imaging (rs-fMRI). Using data from the structural and functional connectivity matrix based on DTI and rs-fMRI, we calculated several network measures using graph theoretical analysis. RESULTS: The measures of global structural connectivity were significantly different between the patients with ESRD who were undergoing PD and healthy subjects. The global efficiency and local efficiency in the patients with PD were significantly decreased compared with those in healthy participants. However, all of the measures of global structural connectivity in the patients with HD were not different from those in healthy participants. Conversely, in the global functional connectivity, the characteristic path length was significantly increased and the small-worldness index was decreased in patients with HD. However, the measures of the global functional connectivity in the patients with PD were not different from those in healthy subjects. CONCLUSION: This study revealed that alterations in structural and functional connectivity in patients who were undergoing PD and HD were different than those in healthy controls. These findings suggest that brain networks may be affected by different types of renal replacement therapy.

Atypical Hemolytic Uremic Syndrome (p.Gly1110Ala) with Autoimmune Disease.

BACKGROUND Hemolytic uremic syndrome (HUS) can be categorized as primary (typical or atypical) or secondary (with a coexisting diseases). Typical HUS usually means shiga-toxin-medicated and thrombotic thrombocytopenic purpura. Secondary HUS is often initiated by coexisting diseases or conditions such as infections, transplantation, cancer, and autoimmune disease. Atypical HUS (aHUS) is usually induced by genetic mutations of one or several complement-regulating genes and associated with dysregulated complement activation. In the era of compliment-inhibiting therapy, early recognition of aHUS is important for patient prognosis. However, compliment-inhibiting therapy is not always beneficial in patients with secondary HUS. CASE REPORT We present a case of a 49-year-old woman with aHUS, which was caused by a novel genetic point mutation of complement factor H gene (p.Gly1110Ala) mimicking secondary HUS with scleroderma. Instead of administering eculizumab treatment for C5 polymorphism, the patient was successfully treated with mycophenolate mofetil. CONCLUSIONS HUS has complex and mixed etiologies and requires genetic testing. Attention should be paid to new point mutations in aHUS.

Serum S100B represents a biomarker for cognitive impairment in patients with end-stage renal disease.

OBJECTIVES: Cognitive impairment (CI) has been recognized as a complication of end-stage renal disease (ESRD) and its treatment. Neuron-specific enolase (NSE) and S100B protein are known neuro-biochemical markers of brain damage. The aim of this study was to investigate the potential role of serum NSE and S100B levels in predicting CI in patients with ESRD. PATIENTS AND METHODS: Thirty patients with ESRD were prospectively enrolled. All of them were receiving maintenance hemodialysis three times weekly for 180 days. We analyzed the potential value of serum NSE and S100B levels for distinguishing patients with CI from those without CI. The Mini-Mental State Examination was used for neuropsychological assessment. The differences between the groups were analyzed using demographic and laboratory profiles as independent variables. RESULTS: Of the 30 patients with ESRD, 13 had CI, whereas the other 17 did not. The demographic profiles, including age, and laboratory profiles, including S100B level, were significantly different between the patients with and without CI. The patients with CI were older than those without CI. Additionally, serum S100B levels in patients with CI were significantly higher than those in patients without CI. However, serum NSE levels did not differ between the groups. The best cut-off values for predicting CI were 17.7 mg/mL for NSE and 36.1 pg/mL for S100B, respectively, based on receiver operating characteristic analysis. Multiple logistic regression analyses showed that serum S100B level was a statistically significant independent predictor of CI. CONCLUSIONS: We found that approximately 40% of patients with ESRD had CI. Serum S100B levels but not serum NSE levels are significantly increased in patients with ESRD. These findings suggest that CI in patients with ESRD is associated with glial cell dysfunction in the brain.

Efficacy and safety of CKD-11101 (darbepoetin-alfa proposed biosimilar) compared with NESP in anaemic chronic kidney disease patients not on dialysis.

OBJECTIVE: To evaluate the efficacy and safety of CKD-11101 (biosimilar darbepoetin-alfa, Chong Kun Dang Pharm.) compared with NESP® in treatment of anaemia in patients with chronic kidney disease not on dialysis. CLINICAL TRIAL REGISTRATION: NCT03431623. METHOD: In this multi-centre, randomized, double-blind study, patients were treated with CKD-11101 and NESP. The efficacy evaluation period (EEP) was 24 weeks, during which patients were treated every 2 weeks. All patients who completed the EEP were treated with CKD-11101 every 2 weeks for the first 4 weeks and every 4 weeks for the safety evaluation period (SEP), which was from 24 weeks to 52 weeks. The primary efficacy endpoint was the change in mean haemoglobin (Hb) level from baseline to end of EEP and mean dose needed to achieve the target Hb. RESULTS: The mean Hb level was increased in both groups during the EEP (both p < 0.001). The difference in mean Hb level change between the two groups was 0.01 g/dL (95% CI = -0.213-0.242), indicating that CKD-11101 was equivalent to NESP. The difference in mean administration dose between groups was -1.40 mcg (95% CI = -6.859-4.059) included in the equivalent range. The incidence of AEs and ADRs was not different between the two groups, and the frequency of ADRs was favourable in both groups (1.2% in CKD-11101 vs 7.7% in the NESP to CKD-11101 conversion group). CONCLUSION: CKD-11101 has an equivalent therapeutic effect as NESP in chronic kidney disease patients with renal anaemia. CKD-11101 can be safely used for long-term treatment and in patients converted from NESP.

A Case of Seizure Revealing Fahr's Syndrome with Primary Hypoparathyroidism.

BACKGROUND Idiopathic basal ganglia calcification, also known as Fahr's disease or Fahr's syndrome, is a rare neurological disorder characterized by abnormal calcified deposits in the basal ganglia. Here, we report a case of Fahr's syndrome with calcification of the basal ganglia due to hypoparathyroidism in a patient with seizures. CASE REPORT A 52-year-old male patient visited our clinic with seizures. Brain computed tomography (CT) showed bilateral symmetrical calcifications in cerebellar white matter, the corpus striatum, the posterior thalami, and the centrum semiovale of both cerebral hemispheres. He had symptoms of hypocalcemia and low parathyroid hormone levels. The patient was diagnosed with Fahr's syndrome due to primary hypoparathyroidism. He underwent calcium supplementation and calcifediol treatment. His symptoms improved, and he was discharged from the hospital. CONCLUSIONS In patients with hypocalcemia accompanied by parathyroid dysfunction, neurological examination and CT should be performed to confirm abnormal intracranial calcification.