Modeling of Fabry disease nephropathy using patient derived human induced pluripotent stem cells and kidney organoid system.

OBJECTIVES: To explore the possibility of kidney organoids generated using patient derived human induced pluripotent stem cells (hiPSC) for modeling of Fabry disease nephropathy (FDN). METHODS: First, we generated hiPSC line using peripheral blood mononuclear cells (PBMCs) from two male FD-patients with different types of GLA mutation: a classic type mutation (CMC-Fb-001) and a non-classic type (CMC-Fb-003) mutation. Second, we generated kidney organoids using wild-type (WT) hiPSC (WTC-11) and mutant hiPSCs (CMC-Fb-001 and CMC-Fb-003). We then compared alpha-galactosidase A (α-GalA) activity, deposition of globotriaosylceremide (Gb-3), and zebra body formation under electromicroscopy (EM). RESULTS: Both FD patients derived hiPSCs had the same mutations as those detected in PBMCs of patients, showing typical pluripotency markers, normal karyotyping, and successful tri-lineage differentiation. Kidney organoids generated using WT-hiPSC and both FD patients derived hiPSCs expressed typical nephron markers without structural deformity. Activity of α-GalA was decreased and deposition of Gb-3 was increased in FD patients derived hiPSCs and kidney organoids in comparison with WT, with such changes being far more significant in CMC-Fb-001 than in CMC-Fb-003. In EM finding, multi-lammelated inclusion body was detected in both CMC-Fb-001 and CMC-Fb-003 kidney organoids, but not in WT. CONCLUSIONS: Kidney organoids generated using hiPSCs from male FD patients might recapitulate the disease phenotype and represent the severity of FD according to the GLA mutation type.

Prediction of intradialytic hypotension using pre-dialysis features-a deep learning-based artificial intelligence model.

BACKGROUND: Intradialytic hypotension (IDH) is a serious complication of hemodialysis (HD) that is associated with increased risks of cardiovascular morbidity and mortality. However, its accurate prediction remains a clinical challenge. The aim of this study was to develop a deep learning-based artificial intelligence (AI) model to predict IDH using pre-dialysis features. METHODS: Data from 2007 patients with 943 220 HD sessions at seven university hospitals were used. The performance of the deep learning model was compared with three machine learning models (logistic regression, random forest and XGBoost). RESULTS: IDH occurred in 5.39% of all studied HD sessions. A lower pre-dialysis blood pressure (BP), and a higher ultrafiltration (UF) target rate and interdialytic weight gain in IDH sessions compared with non-IDH sessions, and the occurrence of IDH in previous sessions was more frequent among IDH sessions compared with non-IDH sessions. Matthews correlation coefficient and macro-averaged F1 score were used to evaluate both positive and negative prediction performances. Both values were similar in logistic regression, random forest, XGBoost and deep learning models, developed with data from a single session. When combining data from the previous three sessions, the prediction performance of the deep learning model improved and became superior to that of other models. The common top-ranked features for IDH prediction were mean systolic BP (SBP) during the previous session, UF target rate, pre-dialysis SBP, and IDH experience during the previous session. CONCLUSIONS: Our AI model predicts IDH accurately, suggesting it as a reliable tool for HD treatment.

Resveratrol increases AdipoR1 and AdipoR2 expression in type 2 diabetic nephropathy.

BACKGROUND: Adiponectin has multiple functions including insulin sensitization, anti-inflammation and antiatherogenesis in various organs. Adiponectin activates 5'-adenosine monophosphate-activated protein kinase (AMPK) and peroxisome proliferator-activated receptor (PPAR)α via the adiponectin receptor (AdipoR) 1 and 2, which are critical for regulating lipids and glucose homeostasis and for controlling oxidative stress. We investigated whether resveratrol can inhibit renal damage in type 2 diabetic db/db mice and the underlying mechanisms of its effects. METHODS: Four groups of male C57 BLKS/J db/m and db/db mice and human glomerular endothelial cells (HGECs) were used. Resveratrol was administered to diabetic and nondiabetic mice by oral gavage for 12 weeks starting at 8 weeks of age. RESULTS: In db/db mice, resveratrol increased serum adiponectin levels and decreased albuminuria, glomerular matrix expansion, inflammation and apoptosis in the glomerulus. Resveratrol increased the phosphorylation of AMPK and silent information regulator T1 (SIRT1), and decreased phosphorylation of downstream effectors class O forkhead box (FoxO)1 and FoxO3a via increasing AdipoR1 and AdipoR2 in the renal cortex. Furthermore, resveratrol increased expression of PPARγ coactivator (PGC)-1α, estrogen-related receptor-1α, and phosphorylated acetyl-CoA carboxylase and decreased sterol regulatory element-binding protein 1. This effect lowered the content of nonesterified fatty acid and triacylglycerol in the kidneys, decreasing apoptosis, oxidative stress and activating endothelial nitric oxide synthase. Resveratrol prevented cultured HGECs from undergoing high-glucose-induced oxidative stress and apoptosis by activating the AMPK-SIRT1-PGC-1α axis and PPARα through increases in AdipoR1 and AdipoR2 expression. CONCLUSIONS: These results suggest that resveratrol prevents diabetic nephropathy by ameliorating lipotoxicity, oxidative stress, apoptosis and endothelial dysfunction via increasing AdipoR1 and AdipoR2 expression.

Comparison of Outcomes with Arteriovenous Fistula and Arteriovenous Graft for Vascular Access in Hemodialysis: A Prospective Cohort Study.

BACKGROUND: Poor vessel quality and limited life expectancy in the elderly may make arteriovenous fistula (AVF) less ideal than arteriovenous graft (AVG) or catheter for vascular access (VA) in hemodialysis (HD). METHODS: A total of 946 adult incident HD patients from clinical research center registry for end-stage renal disease prospective cohort in South Korea were analyzed for outcomes with AVF and AVG. RESULTS: Overall, AVF was associated with better patient survival only in male (p < 0.001) and diabetic (p = 0.004) patients, although it was superior to AVG in access patency, regardless of diabetes mellitus status and gender. AVG (vs. AVF; hazard ratio (HR) 2.282; 95% CI 1.071-4.861; p = 0.032) was associated with poor patient survival. In elderly patients (≥65 years), AVF was associated with survival benefit only in male (p < 0.001) and diabetic (p = 0.04) patients, and with better access patency only in female (p = 0.05) and diabetic (p = 0.04) patients. AVG (vs. AVF; HR 3.158; 95% CI 1.080-9.238; p = 0.036) was associated with poor patient survival. In septuagenarian patients, AVF was associated only with survival benefit (p = 0.01) and there was no advantage in access patency (p = 0.12). However, AVF was superior to AVG in both access patency (p = 0.001) and patient survival (p = 0.03) even with propensity matching. CONCLUSION: AVF is the more desirable VA and its survival benefits warrant its consideration in septuagenarian patients although a prolonged life expectancy is essential to realize the potential benefits of AVF.

Plasma exchange successfully treats central pontine myelinolysis after acute hypernatremia from intravenous sodium bicarbonate therapy.

BACKGROUND: Osmotic demyelination syndrome (ODS) primarily occurs after rapid correction of severe hyponatremia. There are no proven effective therapies for ODS, but we describe the first case showing the successful treatment of central pontine myelinolysis (CPM) by plasma exchange, which occurred after rapid development of hypernatremia from intravenous sodium bicarbonate therapy. CASE PRESENTATION: A 40-year-old woman presented with general weakness, hypokalemia, and metabolic acidosis. The patient was treated with oral and intravenous potassium chloride, along with intravenous sodium bicarbonate. Although her bicarbonate deficit was 365 mEq, we treated her with an overdose of intravenous sodium bicarbonate, 480 mEq for 24 hours, due to the severity of her acidemia and her altered mental status. The next day, she developed hypernatremia with serum sodium levels rising from 142.8 mEq/L to 172.8 mEq/L. Six days after developing hypernatremia, she exhibited tetraparesis, drooling, difficulty swallowing, and dysarthria, and a brain MRI revealed high signal intensity in the central pons with sparing of the peripheral portion, suggesting CPM. We diagnosed her with CPM associated with the rapid development of hypernatremia after intravenous sodium bicarbonate therapy and treated her with plasma exchange. After two consecutive plasma exchange sessions, her neurologic symptoms were markedly improved except for mild diplopia. After the plasma exchange sessions, we examined the patient to determine the reason for her symptoms upon presentation to the hospital. She had normal anion gap metabolic acidosis, low blood bicarbonate levels, a urine pH of 6.5, and a calyceal stone in her left kidney. We performed a sodium bicarbonate loading test and diagnosed distal renal tubular acidosis (RTA). We also found that she had Sjögren's syndrome after a positive screen for anti-Lo, anti-Ra, and after the results of Schirmer's test and a lower lip biopsy. She was discharged and treated as an outpatient with oral sodium bicarbonate and potassium chloride. CONCLUSION: This case indicates that serum sodium concentrations should be carefully monitored in patients with distal RTA receiving intravenous sodium bicarbonate therapy. We should keep in mind that acute hypernatremia and CPM can be associated with intravenous sodium bicarbonate therapy, and that CPM due to acute hypernatremia may be effectively treated with plasma exchange.

Predictors of end-stage renal disease and recurrence of lupus activity after initiation of dialysis in patients with lupus nephritis.

OBJECTIVES: The present paper aims at identifying the predictors of end-stage renal disease (ESRD) and at determining the long-term outcome of ESRD patients according to renal replacement modality in Korean patients with lupus nephritis (LN). METHODS: Between 1985 and 2010, 321 Korean patients with LN were enrolled in this study. We analysed the clinical and laboratory indices, the treatment responses and the biopsy findings. The events of interest were estimated by the Kaplan-Meier method and the risk factors were assessed by univariate and multivariate Cox proportional hazards regression analyses. RESULTS: The median follow-up time after the diagnosis of LN was 84 months. During follow-up, twenty-nine patients evolved to ESRD. Renal survival rate at 5 and 10 years after LN onset was 95.9% and 91.1%, respectively. Deteriorated renal function (estimated glomerular filtration rate <60 ml/min/1.73m2) at LN onset (hazard ratio: 9.223) was found to be an independent risk factor for the development of ESRD. Recurrence of lupus nephritis in renal allograft and flare-ups of lupus activity were not observed among the patients undergoing kidney transplantation (KT) (n=11). In contrast, those with maintenance dialysis (n=18) developed 13 episodes of lupus flare in 10 patients and 5 died of either infection (n=2) or lupus flare (n=3). CONCLUSIONS: The impaired renal function at baseline is an independent predictor of ESRD in Korean patients with LN. The benefits of KT on the control of lupus activity and survival should be emphasised.

Type III membranoproliferative glomerulonephritis in a patient with primary Sjögren's syndrome.

Renal involvement in the form of glomerulonephritis in Sjögren's syndrome (SS) is less common and usually a latent sequel in the course of the disease. We report a patient with Type III membarnoproliferative glomerulonephritis (MPGN) with hypothyroidism, which precedes the onset of the clinical manifestation of SS. She received immunosuppressions consisting of i.v. cyclophosphamide and high-dose corticosteroid and subsequently oral corticosteroid resulting in complete remission of nephrotic syndrome. To our knowledge, this is the first report of successfully treated Type III MPGN associated with SS.

Clinical characteristics and treatment of patients with IgA nephropathy and hepatitis B surface antigen.

BACKGROUND: The aim of this study is to investigate the clinical characteristics and our experience of treating patients with IgA nephropathy (IgAN) and IgA nephropathy with hepatitis B surface antigen (HBs-IgAN). METHODS: From 1996 to 2011, biopsy-proven IgAN was diagnosed in 477 patients and 22 (4.6%) had hepatitis B surface antigen (HBsAg). Of these, we included 360 patients who had more than 6-month follow-up period, and compared clinical characteristics and renal function decline between the patients with IgAN and HBs-IgAN. RESULTS: Of 360 patients, 22 were classified as HBs-IgAN. There were no differences in the clinical characteristics and renal function decline between idiopathic IgAN and HBs-IgAN (-0.01 vs. -0.17 mL/min per 1.73 m(2)/month, p = 0.319). Of 22 patients with HBs-IgAN, nine had hepatitis B virus (HBV) replication marker (RM), of which six were treated with anti-viral agents. However, there were no differences in renal function decline and urinary protein excretion between patients who did or did not receive anti-viral therapy. Five patients with HBs-IgAN received corticosteroid therapy. Of these, three without HBV RM and one with HBV RM who received entecavir did not exhibit active viral replication, whereas the other patients with HBV RM experienced viral replication after lamivudine was discontinued. CONCLUSION: There were no differences in the clinical characteristics and prognosis between the patients with IgAN and HBs-IgAN. Further, there were no differences in renal function decline and urinary protein excretion between patients with and without anti-viral therapy. Anti-viral therapy may be considered for treating patients with HBs-IgAN receiving immunosuppressants according to HBV RM.

Evaluation of outcomes with permanent vascular access in an elderly Korean population based on the National Health Insurance Service database.

INTRODUCTION: As nearly half of patients with end-stage kidney disease (ESKD) who initiate hemodialysis (HD) are over 65 years old (commonly defined as elderly), the fistula first strategy is controversial even in HD patients ≥65 years. METHODS: In Korea's National Health Insurance Service database from 2008 to 2019, 41,989 elderly (≥ 65 years) HD patients were retrospectively reviewed to identify their clinical characteristics and outcomes. Vascular access (VA) patencies, risk factors associated with patencies and patient survival between arteriovenous fistula (AVF) and arteriovenous graft (AVG) were compared. RESULTS: Elderly AVF group (n = 28,467) had superior primary, primary assisted, and secondary patencies than elderly AVG group (n = 13,522) (all p values are <0.001). Patient survival was also better in the elderly AVF group than in the elderly AVG (p < 0.001). In multivariate Cox regression analyses for diverse outcomes, AVG (vs. AVF) was identified as a risk factor for all-cause mortality (adjusted hazard ratio [HR]: 1.307; 95% confidence interval [CI]: 1.272-1.343; p < 0.001), primary patency (adjusted HR: 1.745; 95% CI: 1.701-1.790; p < 0.001), primary-assisted patency (adjusted HR: 2.163; 95% CI: 2.095-2.233; p < 0.001), and secondary patency (adjusted HR: 3.718; 95% CI: 3.533-3.913; p < 0.001). CONCLUSION: Our study demonstrated that as a permanent VA for HD, AVF should be strongly considered in elderly (≥ 65 years) ESKD Korean patients. The age limit for AVF creation in ESKD patients should be adjusted more upward.

Development of in-vitro pulsatile flow generator for evaluating the performance of hemodialysis catheters.

Hemodialysis (HD) using an HD catheter is performed widely on renal failure patients. The catheter was evaluated using the recirculation ratio in pre-clinical status, which is a crucial index indicating its performance. However, pre-clinical in-vivo experiments have limitations: high cost, and ethical issues. Hence, computational and in-vitro methods have been developed as alternatives. However, computational methods require fluid dynamic knowledge, whereas in-vitro experiments are complicated and expensive. In this study, we developed a pulsatile flow generator to mimic blood flow achieving cost effectiveness and user convenience. The device used iterative learning control, achieving blood flow in the superior and inferior vena cava within a 3.3% error. Furthermore, the recirculation ratios were measured based on two insertion directions and two different external pipe materials to evaluate the catheter regarding patients' posture and blood vessel stiffness. The results provide a better understanding of cardiovascular device performance without complicated and costly pre-clinical tests.

Safety and durable patency of tunneled hemodialysis catheter inserted without fluoroscopy.

BACKGROUND: A tunneled hemodialysis (HD) catheter is preferred due to its lower incidence of infection and malfunction than non-tunneled ones. For safer insertion, fluoroscopic guidance is desirable. However, if the patient is unstable, transfer to the fluoroscopy may be impossible or inappropriate. METHODS: From June 2019 to September 2022, 81 tunneled HD catheter insertion cases performed under ultrasound guidance without fluoroscopy and 474 cases with fluoroscopy in our institutional HD catheter cohort were retrospectively compared. RESULTS: Immediate complications, later catheter-associated problems, including infections and catheter dysfunction, were comparable between the two groups (p = 0.20 and p = 0.37, respectively). The patency of tunneled catheters inserted without fluoroscopy was comparable to the patency of tunneled catheters inserted with fluoroscopic guidance (p = 0.90). CONCLUSION: Tunneled HD catheter insertion without fluoroscopy can be performed safely and has durable patency compared to the insertion with fluoroscopy. Therefore, this method can be considered for the selected unstable patients (e.g., ventilator care) in the intensive care unit.

Salvage treatment of forearm arteriovenous fistula with small caliber inflow distal artery by percutaneous transluminal angioplasty.

BACKGROUND: Dysfunctional distal arteriovenous fistulas (AVFs) with small caliber distal inflow arteries theoretically require percutaneous transluminal angioplasty (PTA) throughout the entire arterial length. However, in clinical practice, whole distal inflow arterial PTA is not frequently performed due to concerns about possible arterial rupture. Therefore, we investigated the safety and efficacy of this procedure at our center, comparing it with the standard venous PTA. METHODS: From March 2017 to December 2022, 48 cases of distal AVF salvaged by whole distal inflow arterial PTA were assigned into a treatment group and 121 cases of distal AVF salvaged by venous standard PTA not involving the whole inflow artery were assigned into a control group. These two groups were then compared. RESULTS: Those in the treatment group (who received whole distal inflow arterial PTA) were older than those in the control group (mean age, 69 vs 59 years, p < 0.001). Otherwise, differences between the two groups were unremarkable. After the salvage treatment, primary patency seemed to decrease in the treatment group with whole distal inflow arterial PTA compared to the control group with conventional PTA, although such decrease was not significant (p = 0.072). However, primary assisted patency and secondary patency were comparable between the two groups (p = 0.350 and p = 0.590, respectively). And in the treatment group, only one arterial dissection occurred, which was successfully managed with balloon tamponade so that no distal AVF was abandoned due to complications following whole distal inflow arterial PTA. CONCLUSION: Whole distal inflow arterial PTA is an effective and safe option for distal AVF salvage with a narrowed inflow artery, frequently refractory to conventional venous PTA.

The Oxford classification as a predictor of prognosis in patients with IgA nephropathy.

BACKGROUND: In 2009, the Oxford classification was developed as a pathological classification system for immunoglobulin A nephropathy (IgAN) to predict the risk of disease progression. The aim of this retrospective study was to evaluate the clinical and pathologic relevance of the Oxford classification in Korean patients with a pathologic diagnosis of IgAN. PATIENTS AND METHODS: We reviewed the renal pathology archives from January 2000 to December 2006 at Seoul St Mary's Hospital in Korea and identified 273 patients, who were diagnosed as having IgAN. We enrolled 197 patients who were available for further clinicopathologic analysis. All cases of IgAN were categorized according to the WHO classification, the semiquantitative classification and the Oxford classification. These pathologic classifications were compared. The clinical and laboratory findings at the time of biopsy were compared with those at the end of the follow-up according to the Oxford classification. RESULTS: When three pathologic classifications were compared, M1, S1, E1, T1 or T2 were associated with a higher score in the activity index. S1, T1 or T2 were associated with a higher score in the chronicity index and a higher grade in the WHO classification. The clinical and laboratory findings were compared according to the Oxford classification. At the time of biopsy, the proteinuria in patients with M1 was more than that of M0 (P = 0.035). At the end of follow-up, the number of antihypertensive drugs taken among patients with M1 was greater than that of patients with M0 (P = 0.001). At the time of biopsy, the proteinuria of patients with S1 was greater than that of S0 patients (P = 0.009). At the end of follow-up, the number of patients who received immunosuppressants was increased as the grade of T increased (P = 0.000). At the end point of the follow-up, the estimated glomerular filtration rate (eGFR) decreased as the grade of T increased (P = 0.008). The time-average proteinuria after adjusting the initial proteinuria increased significantly with increasing degree of T (P = 0.000). Levels of tubular atrophy/interstitial fibrosis were predictive for survival from end-stage renal disease or of having a 50% reduction of eGFR. CONCLUSION: The pathologic variables of the Oxford classification correlated significantly with other classifications (the WHO classification and the semiquantitative classification). The Oxford classification is a simple method for predicting renal outcome and differentiating between active and chronic lesions. We suggest that the Oxford classification offers an advantage for determining treatment policy for patients with IgAN.

Clinical usefulness of BK virus plasma quantitative PCR to prevent BK virus associated nephropathy.

The present study investigated the clinical usefulness of plasma real-time polymerase chain reaction (PCR) (plasma-PCR) in the prevention of BK virus-associated nephropathy (BKVAN). First, we investigated the diagnostic value of plasma BK-PCR, urine BK-PCR, and urine cytology for the prediction of BKVAN retrospectively. Then we designed a prospective study of regular plasma-PCR monitoring and pre-emptive immunosuppression (IS) reduction based on the result. In the retrospective cohort, the prevalence of BKVAN was 3.7% (14/379) and the positive rate of decoy cells, urine-PCR (>1 × 10(10) copies/ml), and plasma-PCR (>1 × 10(4) copies/ml) was 18.6%, 11.1%, and 5.5%, respectively. Plasma-PCR was superior to urine-PCR or urine cytology in specificity and positive predictive value for detection of BKVAN. In prospective study, regular monitoring of plasma-PCR detected significant BKV viremia in 8.3% (12/145) and BKVAN in 1 patient (0.6%). After IS reduction, BKV viremia was eliminated in 91.6% (11/12) within 103 days (25-254). In patients with viremia, the frequency of acute rejection did not increase and allograft function did not differ significantly compared with those in patients without viremia during the first year post-transplant (P > 0.05, in both). Plasma-PCR is useful to predict an increased risk for BKVAN, and regular monitoring is effective to prevent the development of BKVAN.

Comparison of the palindrome vs. step-tip tunneled hemodialysis catheter: a prospective randomized trial.

Numerous designs for tunneled hemodialysis catheter have been developed in an effort to improve catheter function and survival. In this prospective randomized controlled study, 97 patients were randomized into the palindrome catheter group (PC, n = 47) and step-tip catheter group (SC, n = 50). Demographic characteristics were not different between the two groups. The effective blood flow rates at different pump speeds were comparable between the two groups. The recirculation was low within acceptable range in both types of catheter, and hemodialysis adequacy was not different between the two groups. However, when arterial and venous blood lines were reversed, while the recirculation was significantly increased in SC, it was not increased at all in PC. The catheter dysfunction-free survival rate was significantly higher in PC than in SC (78.9% vs. 54.4% at 2 months, p = 0.008). The overall catheter survival rate was also higher in PC than in SC (90.6% vs. 68.8% at 2 months, p = 0.015). We conclude that both catheters are equally effective on the adequate hemodialysis and low recirculation. However, the PCs have advantages over the SCs in terms of lower catheter dysfunction rate, lower recirculation with reversed blood lines, higher short-term catheter survival rate.

Changes in renal function in long-term survivors of allogeneic hematopoietic stem-cell transplantation: single-center experience.

BACKGROUND: Renal dysfunction after allogeneic hematopoietic stem cell transplantation (HSCT) has been increasingly reported. However, there are few reports on the changes of the estimated glomerular filtration rate (eGFR) in long-term survivors after allogeneic HSCT. PATIENTS AND METHODS: The medical records at Seoul St. Mary's Hospital in Korea were reviewed to identify all adult (> 18-years-of-age) patients who had undergone high-dose chemotherapy and allogeneic HSCT between January 2001 and December 2005. Among these patients, those with < 5 years of follow-up and relapse within 5 years after HSCT were excluded. 85 patients were enrolled. RESULTS: The mean follow-up was 76.0 ± 13.5 months. The eGFR recorded 3 months after HSCT was significantly decreased compared with the eGFR recorded before HSCT. Subsequently, early decreased eGFR was maintained during the 60 months after HSCT. Multivariate analysis showed that acute kidney injury (AKI) during HSCT, hypertension (HTN) and eGFR before HSCT was differently associated with changes in eGFR. The eGFR in patients who had AKI decreased significantly at 3 months after HSCT. After 3 months, the eGFR recovered to reach a lower level than in patients without AKI. The level was maintained during the 60 months after HSCT. The eGFR in patients who had low eGFR before HSCT (< 90 ml/min) decreased significantly at 3 months after HSCT, which was also maintained during the 60 months after HSCT. The eGFR in patients who had HTN also decreased significantly at 3 months after HSCT. By contrast, the eGFR decreased consistently and slowly from 3 to 60 months. CONCLUSION: AKI and low baseline eGFR are associated with early renal dysfunction in patients after HSCT, but are not closely associated with long-term decline in eGFR. In contrast, eGFR in patients with HTN continuously decrease after 3 months of HSCT. Therefore, HTN seems to play a major role in the long-term decline in eGFR. These findings suggest that eGFR at 3 months after HSCT should be monitored closely for all patients who have undergone HSCT. Additionally, long-term follow-up of renal function is needed to prevent further renal damage for patients with HTN.

Blood Pressure Control in Patients with Diabetic Kidney Disease.

Diabetic kidney disease (DKD) is the most common cause of end-stage kidney disease. Blood pressure (BP) control can reduce the risks of cardiovascular (CV) morbidity, mortality, and kidney disease progression. Recently, the Kidney Disease: Improving Global Outcomes (KDIGO) guidelines have suggested the implementation of a more intensive BP control with a target systolic BP (SBP) of <120 mmHg based on the evidence that the CV benefits obtained is outweighed by the kidney injury risk associated with a lower BP target. However, an extremely low BP level may paradoxically aggravate renal function and CV outcomes. Herein, we aimed to review the existing literature regarding optimal BP control using medications for DKD.

Hyperglycemic hyperosmolar syndrome caused by steroid therapy in a patient with lupus nephritis.

A 51-yr-old female was referred to our outpatient clinic for the evaluation of generalized edema. She had been diagnosed with idiopathic thrombocytopenic purpura (ITP). She had taken no medicine. Except for the ITP, she had no history of systemic disease. She was diagnosed with systemic lupus erythematosus. Immunosuppressions consisting of high-dose steroid were started. When preparing the patient for discharge, a generalized myoclonic seizure occurred at the 47th day of admission. At that time, the laboratory and neurology studies showed hyperglycemic hyperosmolar syndrome. Brain MRI and EEG showed brain atrophy without other lesion. The seizure stopped after the blood sugar and serum osmolarity declined below the upper normal limit. The patient became asymptomatic and she was discharged 10 weeks after admission under maintenance therapy with prednisolone, insulin glargine and nateglinide. The patient remained asymptomatic under maintenance therapy with deflazacort and without insulin or medication for blood sugar control.