A Chinese Multi-Specialty Delphi Consensus to Optimize RAASi Usage and Hyperkalaemia Management in Patients with Chronic Kidney Disease and Heart Failure

Objective Variations are present in common clinical practices regarding best practice in managing hyperkalaemia(HK),there is therefore a need to establish a multi-specialty approach to optimal renin angiotension-aldosterone system inhibitors(RAASi)usage and HK management in patients with chronic kidney disease(CKD)&heart failure(HF).This study aimed to establish a multi-speciality approach to the optimal use of RAASi and the management of HK in patients with CKD and HF.Methods A steering expert group of cardiology and nephrology experts across China were convened to discuss challenges to HK management through a nominal group technique.The group then created a list of 41 statements for a consensus questionnaire,which was distributed for a further survey in extended panel group of cardiologists and nephrologists across China.Consensus was assessed using a modified Delphi technique,with agreement defined as"strong"(≥75%and<90%)and"very strong"(≥90%).The steering group,data collection,and analysis were aided by an independent facilitator.Results A total of 150 responses from 21 provinces across China were recruited in the survey.Respondents were comprised of an even split(n=75,50%)between cardiologists and nephrologists.All 41 statements achieved the 75%consensus agreement threshold,of which 27 statements attained very strong consensus(≥90%agreement)and 14 attained strong consensus(agreement between 75%and 90%).Conclusion Based on the agreement levels from respondents,the steering group agreed a set of recommendations intended to improve patient outcomes in the use of RAASi therapy and HK management in China.

Return to Dialysis after Kidney Graft Failure

Introduction: The transition period from renal transplantation to dialysis is associated with high morbidity and mortality. The aim of this study is to describe the clinical and paraclinical characteristics, therapeutic management and evolutionary profile of patients returning to dialysis after kidney graft failure. Material and Methods: This was a retrospective, descriptive study conducted in the Nephrology-Dialysis-Renal Transplant Department at university hospital IbnSina between January 1998 and December 2021. We included all renal transplant recipients who had experienced kidney graft dysfunction and returned to dialysis. Patients with a follow-up after return to dialysis of less than 1 year were excluded. Results: Among 166 renal transplant recipients, 20 returned to dialysis after a median renal graft life of 85.5 months [42 - 186], corresponding to a prevalence of 12%. The mean age of our patients was 38.7 ± 11.9 years, with a M/F sex ratio of 2.3. Dialysis was initiated urgently in 10 patients (50%). Hemodialysis was the most commonly used modality (75%). Central venous catheterization was used in 35% of cases, including tunneled catheters. General condition is impaired in all patients, with persistent hypertension in 70% of cases. Mean uremia was 2.35 ± 0.8 g/l, mean creatinine 116 ± 48.3 mg/l, giving a mean GFR of 5.1 ± 2.2 ml/min. Mean albuminemia was 32.9 ± 6 g/l and mean hemoglobinemia 8.6 ± 1.9 g/dl. During the first year of follow-up, none of the patients died. However, 13 patients required hospitalization, with a mean length of stay of 15 days. Eight patients were hospitalized for infections and 5 for renal graft intolerance syndrome. After a mean follow-up of 22 months, 6 patients were detransplanted following graft necrosis. Conclusion: Return to dialysis after RT is fraught with a high rate of complications. The management of these patients must be optimized to improve their vital prognosis and quality of life.

Clinical Nursing Intervention of Moxibustion on Abdominal Distension Symptoms in Heart Failure (Heart and Kidney Yang Deficiency and Blood Stasis Blocking Collaterals Syndrome)

Objective:To investigate the clinical nursing intervention effect of moxibustion on abdominal distension symptoms in heart failure(heart and kidney yang deficiency and blood stasis blocking collaterals syndrome).Methods:62 patients with heart failure(heart and kidney yang deficiency and blood stasis blocking collaterals syndrome)admitted to our hospital from February 2023 to February 2024 were selected and divided into the observation group(n=31)and the control group(n=31)by using the random numerical table method.The control group adopted conventional nursing interventions,and the observation group received the nursing program of the control group with the addition of moxibustion nursing interventions.The nursing effectiveness,quality of life scores,and nursing satisfaction were compared between the two groups.Results:The nursing effectiveness of the observation group was significantly higher than the control group(P<0.05);the quality of life score of the observation group was significantly higher than the control group(P<0.05);the nursing satisfaction of the observation group was significantly higher than that of the control group(P<0.05).Conclusion:The use of moxibustion nursing intervention in patients with heart failure(heart and kidney yang deficiency and blood stasis blocking collaterals syndrome)can effectively relieve the symptoms of abdominal distension,improve patients'quality of life,and increase nursing satisfaction,which has promotion and application values.

Gender differences in the etiology of heart failure： A systematic review

Background Heart failure （HF） is an increasing problem for the aging population, specifically among women. The etiology of HF influences both the selection and outcome of the treatment. There are variations between genders in morbidity and mortality in different studies, possibly reflecting etiology. The objective of this study was to examine the strength of evidence available for gender differences in the etiology of chronic heart failure. Methods Computer-assisted searches from 1980-2009 for gender differences in the etiology of heart failure were performed （Medline, EMBASE and PubMed）. From 2347 abstracts reviewed based on inclusion criteria, 35 original articles were chosen for review. Data extraction was based on observational studies （prospective/retrospective cohort or cross sectional） with a mean follow up of 3 months. There was no interrater variability between the 2 reviewers on data-extraction. Results Ventricular systolic dysfunction being more associated with male sex, but female sex was more reported to be associated with preserved left ventricular function. Ischemic etiology and associated coronary heart disease were strongly correlated with male sex. The risk for HF was dramatically more elevated for women with systolic hypertension but the association for diabetes mellitus as the etiology of HF was somewhat equal between males and females. Conclusions One of the limitations in reaching conclusions about gender differences in cardiovascular disease is that many major clinical trials do not include a gender analysis nor they are powered to do so as women are under-represented in most of the HF studies. The need remains for a well designed prospective study of sufficient numbers of male and female patients with and without heart failure and analyzing etiology and risk factors based on the sex differences.

Acute kidney injury in acute-on-chronic liver failure is different from in decompensated cirrhosis

AIM To evaluate the differences in acute kidney injury(AKI) between acute-on-chronic liver failure(ACLF) and decompensated cirrhosis(DC) patients. METHODS During the period from December 2015 to July 2017, 280 patients with hepatitis B virus(HBV)-related ACLF(HBV-ACLF) and 132 patients with HBV-related DC(HBV-DC) who were admitted to our center were recruited consecutively into an observational study. Urine specimens were collected from all subjects and the levels of five urinary tubular injury biomarkers were detected,including neutrophil gelatinase-associated lipocalin(NGAL), interleukin-18(IL-18), liver-type fatty acid binding protein(L-FABP), cystatin C(CysC), and kidney injury molecule-1(KIM-1). Simultaneously, the patient demographics, occurrence and progression of AKI, and response to terlipressin therapy were recorded. All patients were followed up for 3 mo or until death after enrollment. RESULTS AKI occurred in 71 and 28 of HBV-ACLF and HBV-DC patients, respectively(25.4% vs 21.2%, P = 0.358). Among all patients, the levels of four urinary biomarkers(NGAL, CysC, L-FABP, IL-18) were significantly elevated in patients with HBV-ACLF and AKI(ACLF-AKI), compared with that in patients with HBV-DC and AKI(DC-AKI) or those without AKI. There was a higher proportion of patients with AKI progression in ACLF-AKI patients than in DC-AKI patients(49.3% vs 17.9%, P = 0.013). Fortythree patients with ACLF-AKI and 19 patients with DC-AKI were treated with terlipressin. The response rate of ACLFAKI patients was significantly lower than that of patients with DC-AKI(32.6% vs 57.9%, P = 0.018). Furthermore, patients with ACLF-AKI had the lowest 90 d survival rates among all groups(P < 0.001).CONCLUSION AKI in ACLF patients is more likely associated with structural kidney injury, and is more progressive, with a poorer response to terlipressin treatment and a worse prognosis than that in DC patients.

Cystatin C is a biomarker for predicting acute kidney injury in patients with acute-on-chronic liver failure

AIM:To investigate serum cystatin C level as an early biomarker for predicting acute kidney injury(AKI)in patients with acute-on-chronic liver failure(ACLF).METHODS:Fifty-six consecutive patients with hepatitis B virus-related ACLF who had normal serum creatinine(Cr)level(<1.2 mg/dL in men,or<1.1 mg/dL in women)were enrolled in the Liver Failure Treatment and Research Center of Beijing 302 Hospital between August 2011 and October 2012.Thirty patients with chronic hepatitis B(CHB)and 30 healthy controls in the same study period were also included.Measurement of serum cystatin C(CysC)was performed by a particle-enhanced immunonephelometry assay using the BN Prospec nephelometer system.The ACLF patients were followed during their hospitalization period.RESULTS:In the ACLF group,serum level of CysC was 1.1±0.4 mg/L,which was significantly higher(P<0.01)than those in the healthy controls(0.6±0.3mg/L)and CHB patients(0.7±0.2 mg/L).During the hospitalization period,eight ACLF patients developed AKI.Logistic regression analysis indicated that CysC level was an independent risk factor for AKI development(odds ratio=1.8;95%CI:1.4-2.3,P=0.021).The cutoff value of serum CysC for prediction of AKI in ACLF patients was 1.21 mg/L.The baseline CysC-based estimated glomerular filtration rate(eGFR CysC)was significantly lower than the creatinine-based eGFR(eGFR CG and eGFR MDRD)in ACLF patients with AKI,suggesting that baseline eGFR CysC represented early renal function in ACLF patients while the Cr levels were still within the normal ranges.CONCLUSION:Serum CysC provides early prediction of renal dysfunction in ACLF patients with a normal serum Cr level.

Heart failure and left ventricular dysfunction in older patients with chronic kidney disease: the China Hypertension Survey (2012‒2015)

Background Heart failure(HF)is a leading cause of hospitalization and mortality for older chronic kidney disease(CKD)patients.However,the epidemiological data is scarce.We aimed to determine the prevalence of left ventricular(LV)dysfunction and HF,and to explore the risk factors for HF among those patients.Methods This is a cross-sectional analysis of the China Hypertension Survey conducted between October 2012 and December 2015.A total of 5,808 participants aged≥65 years were included in the analysis.Self-reported history of HF and any other cardiovascular diseases was acquired.2-D and Doppler echocardiography were used to assess LV dysfunction.CKD was defined as either estimated glomerular filtration rate(eGFR)<60 mL/min per 1.73 m2 or urinary albumin to creatinine ratio(ACR)≥30 mg/g.Results Among CKD patients aged≥65 years,the weighted prevalence of HF,heart failure with preserved ejection fraction(HFpEF),heart failure with mid-range ejection fraction(HFmrEF),and heart failure with reduced ejection fraction(HFrEF)was 4.8%,2.5%,0.8%,and 1.7%,respectively.The weighted prevalence of HF was 5.0%in patients with eGFR<60 mL/min per 1.73 m2,and was 5.9%in patients with ACR≥30 mg/g.The prevalence of LV systolic dysfunction was 3.1%,and while it was 8.9%for moderate/severe diastolic dysfunction.Multivariate analysis showed that smoking was significantly associated with the risk of HF.Furthermore,age,smoking,and residents in rural areas were significantly associated with a risk of LV diastolic dysfunction.Conclusions The prevalence of HF and LV dysfunction was high in older patients with CKD,suggesting that particular strategies will be required.

Evaluation of chronic kidney disease in chronic heart failure: From biomarkers to arterial renal resistances

Chronic kidney disease and its worsening are recurring conditions in chronic heart failure(CHF) which are independently associated with poor patient outcome.The heart and kidney share many pathophysiological mechanisms which can determine dysfunction in each organ. Cardiorenal syndrome is the condition in which these two organs negatively affect each other, therefore an accurate evaluation of renal function in the clinical setting of CHF is essential. This review aims to revise the parameters currently used to evaluate renal dysfunction in CHF with particular reference to the usefulness and the limitations of biomarkers in evaluating glomerular dysfunction and tubular damage. Moreover, it is reported the possible utility of renal arterial resistance index(a parameter associated with abnormalities in renal vascular bed) for a better assesment of kidney disfunction.

High flux hemodialysis in elderly patients with chronic kidney failure

BACKGROUND Hemodialysis is an advanced blood purification technique to manage kidney failure.However,for conventional hemodialysis,the high prevalence of dyslipidemia may cause cardiovascular diseases and an increase in mortality.Moreover,toxins accumulating in the body over time may induce some complications.High flux hemodialysis can effectively improve disease indexes and clinical symptoms.AIM To investigate the efficacy of high flux hemodialysis in elderly patients with chronic kidney failure(CKF).METHODS A total of 66 elderly patients with CKF who were admitted to our hospital from October 2017 to October 2018 were included in the study.According to the therapies they received,the patients were divided into a study group and a control group with 33 patients in each group.The study group received high flux hemodialysis and the control group received conventional dialysis treatment.Kidney function,toxin levels in serum,and complications were compared in the two groups.RESULTS Before the treatment,there was no significant difference in kidney function,β2-microglobulin,or blood urea nitrogen between the two groups(P>0.05).In contrast,kidney function was better in the study group than in the control group after the treatment(P<0.05).In addition,the study group had significantly lower parathyroid hormone and serum cystatin C than the control group(P<0.05).The incidence of complications was 8.57%in the study group,which was lower than that of the control group(20.00%;P<0.05).CONCLUSION High flux hemodialysis may improve kidney function and reduce toxin levels in serum and the incidence of complications in elderly patients with CKF.

Etiologies of Chronic Renal Failure in Adolescent Patients at the University Hospital of Brazzaville

Objectives:?The aim of our work was to identify the etiologies of chronic renal failure in adolescent patients.?Patients and Methods:?This is a descriptive cross-sectional retrospective study that included adolescents aged 10 to 19 years hospitalized between January 2014 and December 2017 in the department of nephrology at the?University Hospital of Brazzaville. The CRF was defined according to the ANAES criteria by an estimated glomerular filtration rate less than 60 ml/min/?1.73?m2?of body surface.?Results:?Forty cases of CRF were collected, representing a prevalence of 4.9%. The average age was 16.32 ± 3.64 years and the sex ratio was 3. The most common mode of discovery was edematous syndrome 62.5%. The most common etiology was glomerular nephropathies 60%, hereditary nephropathies 25% and malformative uropathies in 15%. Ten patients (25%) had moderate CFR at admission, 62.5% had severe CRF, 12.5% had terminal CFR?and all of them died during the year.?Conclusion:?Epidemiological data CRF in adolescent patients in developing countries are scarce and limit treatment. Glomerular pathologies represent the most frequent etiology. Early treatment of chronic renal failure in childhood and multidisciplinary collaboration between pediatrician and nephrologist are necessary.

Evaluation of Left Ventricular Function by Three-Dimensional Speckle-Tracking Echocardiography in Patients with Chronic Kidney Failure

Objective:To establish a quantitative evaluation of the left ventricle's systolic function in patients with chronic kidney failure(CKF)by three-dimensional speckle-tracking echocardiography.Methods:Two-dimensional and three-dimensional transthoracic echocardiography was performed on 30 patients with CKF.The ejection fraction,mass and global peak longitudinal strain,global circumferential strain,global area strain,and global radial strain of the left ventricle were calculated.Results:The ejection fraction,mass and global peak longitudinal strain(GLS),global circumferential strain(GCS),global area strain(GAS),and global radial strain(GRS)in the CKF group were significantly lower than those in the control group.Simultaneously,the GLS,GCS,GAS and GRS were well correlated with the ejection fraction.For patients with normal ejection fraction in the CKF group,the GLS,GCS,GAS and GRS were lower than those in the control group,while the left ventricular mass was significantly higher in CKF patients than in the control group.For patients with hypertension in the CKF group,ejection fraction,GLS,GCS,GAS and GRS calculated using three-dimensional echocardiography were significantly lower than those in patients with normal blood pressure;however,the myocardial mass was higher.Conclusions:The parameters(GLS,GCS,GAS and GRS)calculated using three-dimensional speckle-tracking software were lower in the CKF group.Simultaneously,the left ventricular mass was higher in CFK patients than in the control group,thus showing that the myocardial contraction function was impaired and that myocardial remodeling had occurred.

Efficacy and safety of Nafamostat mesylate in patients with endstage renal failure

BACKGROUND Recent studies on dialysis anticoagulation therapy in patients with renal failure have shown that Nafamostat mesylate,a broad-spectrum potent serine protease inhibitor,has strong anticoagulation and anti-fiber activity.AIM To evaluate the efficacy and safety of Nafamostat mesylate in patients with end-stage renal failure.METHODS Seventy-five patients with end-stage renal failure who received hemodialysis at our hospital between January 2020 and August 2021 were selected and divided into the observation group(Nafamostat mesylate for injection,n=33)and control group(heparin sodium injection,n=32).General patient data,indicators of clinical efficacy,dialyzer hemocoagulation parameters,coagulation function indices,and hemoglobin concentration and platelet count before and after treatment,and the occurrence of adverse reactions after treatment were compared between the two groups.RESULTS The two groups showed no significant differences in general patient data(P>0.05).The post-treatment effectiveness rate in the control group was lower than that in the observation group(P<0.05).The two groups showed no significant difference in the number of patients in grade I(P>0.05),while the number of patients in grade 0 was lower in the control group,and the number of patients in grades II and III was higher in the control group(P<0.05).The post-treatment prothrombin time,activated partial thromboplastin time,thrombin time,and international normalized ratio values in the control group were higher than those in the observation group,while the fibrinogen level in the control group was lower than that in the observation group(P<0.05).The two groups showed no significant difference in the platelet count and hemoglobin level before and after treatment(P>0.05).The total number of post-treatment adverse reactions in the observation group was lower than that in the control group(P<0.05).CONCLUSION Treatment of patients showing end-stage renal failure with Nafamostat mesylate can significantly improve therapeutic efficacy and has high safety and clinical value.

Prognostic impact of atrial fibrillation on clinical outcomes of acute coronary syndromes,heart failure and chronic kidney disease

Atrial fibrillation(AF) is the most common type of sustained arrhythmia,which is now on course to reach epidemic proportions in the elderly population. AF is a commonly encountered comorbidity in patients with cardiac and major non-cardiac diseases. Morbidity and mortality associated with AF makes it a major healthcare burden. The objective of our article is to determine the prognostic impact of AF on acute coronary syndromes,heart failure and chronic kidney disease. Multiple studies have been conducted to determine if AF has an independent role in the overall mortality of such patients. Our review suggests that AF has an independent adverse prognostic impact on the clinical outcomes of acute coronary syndromes,heart failure and chronic kidney disease.

Clinical trial with traditional Chinese medicine intervention ''tonifying the kidney to promote liver regeneration and repair by affecting stem cells and their microenvironment'' for chronic hepatitis B-associated liver failure

AIM:To study the clinical efficacy of traditional Chinese medicine(TCM)intervention"tonifying the kidney to promote liver regeneration and repair by affecting stem cells and their microenvironment"("TTK")for treating liver failure due to chronic hepatitis B.METHODS:We designed the study as a randomized controlled clinical trial.Registration number of Chinese Clinical Trial Registry is Chi CTR-TRC-12002961.A total of 144 patients with liver failure due to infection with chronic hepatitis B virus were enrolled in this randomized controlled clinical study.Participants were randomly assigned to the following three groups:(1)a modern medicine control group(MMC group,36patients);(2)a"tonifying qi and detoxification"("TQD")group(72 patients);and(3)a"tonifying the kidney to promote liver regeneration and repair by affecting stem cells and their microenvironment"("TTK")group(36patients).Patients in the MMC group received general internal medicine treatment;patients in the"TQD"group were given a TCM formula"tonifying qi and detoxification"and general internal medicine treatment;patients in the"TTK"group were given a TCM formula of"TTK"and general internal medicine treatment.All participants were treated for 8 wk and then followed at 48 wk following their final treatment.The primaryefficacy end point was the patient fatality rate in each group.Measurements of various virological and biochemical indicators served as secondary endpoints.The one-way analysis of variance and the t-test were used to compare patient outcomes in the different treatment groups.RESULTS:At the 48-wk post-treatment time point,the patient fatality rates in the MMC,"TQD",and"TTK"groups were 51.61%,35.38%,and 16.67%,respectively,and the differences between groups were statistically significant(P<0.05).However,there were no significant differences in the levels of hepatitis B virus DNA or prothrombin activity among the three groups(P>0.05).Patients in the"TTK"group had significantly higher levels of serum total bilirubin compared to MMC subjects(339.40μmol/L±270.09μmol/L vs 176.13μmol/L±185.70μmol/L,P=0.014).Serum albumin levels were significantly increased in both the"TQD"group and"TTK"group as compared with the MMC group(31.30 g/L±4.77g/L,30.72 g/L±2.89 g/L vs 28.57 g/L±4.56 g/L,P<0.05).There were no significant differences in levels of alanine transaminase among the three groups(P>0.05).Safety data showed that there was one case of stomachache in the"TQD"group and one case of gastrointestinal side effect in the"TTK"group.CONCLUSION:Treatment with"TTK"improved the survival rates of patients with liver failure due to chronic hepatitis B.Additionally,liver tissue was regenerated and liver function was restored.

Current Status of Stem Cells in the Treatment of Premature Ovarian Failure

Premature ovarian failure (POF) is a prevalent cause of female infertility. POF features include estrogen hypofunction, amenorrhea, infertility, and high gonadotropin levels. The etiology of POF is genetic diseases, autoimmune diseases, enzyme defects and environmental factors. The development of Regenerative medicine has made stem cell and exosome (EXOs) therapy effective for POF. This review discusses POF stem cell research and development.

Thrombotic microangiopathy after kidney transplantation: Expanding etiologic and pathogenetic spectra

Thrombotic microangiopathy(TMA)is an uncommon but serious complication that not only affects native kidneys but also transplanted kidneys.This review is specifically focused on post-transplant TMA(PT-TMA)involving kidney transplant recipients.Its reported prevalence in the latter population varies from 0.8%to 14%with adverse impacts on both graft and patient survival.It has many causes and associations,and the list of etiologic agents and associations is growing constantly.The pathogenesis is equally varied and a variety of pathogenetic pathways lead to the development of microvascular injury as the final common pathway.PT-TMA is categorized in many ways in order to facilitate its management.Ironically,more than one causes are contributory in PT-TMA and it is often difficult to pinpoint one particular cause in an individual case.Pathologically,the hallmark lesions are endothelial cell injury and intravascular thrombi affecting the microvasculature.Early diagnosis and classification of PT-TMA are imperative for optimal outcomes but are challenging for both clinicians and pathologists.The Banff classification has addressed this issue and has developed minimum diagnostic criteria for pathologic diagnosis of PT-TMA in the first phase.Management of the condition is also challenging and still largely empirical.It varies from simple maneuvers,such as plasmapheresis,drug withdrawal or modification,or dose reduction,to lifelong complement blockade,which is very expensive.A thorough understanding of the condition is imperative for an early diagnosis and quick treatment when the treatment is potentially effective.This review aims to increase the awareness of relevant stakeholders regarding this important,potentially treatable but under-recognized cause of kidney allograft dysfunction.

Heart failure in subjects with chronic kidney disease:Best management practices

Renal dysfunction is common in patients with heart failure(HF) and can complicate HF therapy.Treating patients with HF and kidney disease is difficult and requires careful assessment,monitoring and balancing of risk between potential benefits of treatment and adverse impact on renal function.In this review,we address the pathophysiological contexts and management options in this adversarial relation between the heart and the kidney,which exists in a substantial proportion of HF patients.Angiotensin converting enzyme inhibitors and β-blockers are associated with similar reductions in mortality in patients with and without renal insufficiency but usually are less often prescribed in patients with renal insufficiency.Careful monitoring of side effects and renal function should be done in all patients with renal insufficiency and prompt measures should be adopted to prevent further complications.

OFD1 mutation induced renal failure and polycystic kidney disease in a pair of childhood male twins in China

BACKGROUND Oral-facial-digital syndrome type 1(OFD1) is a rare ciliopathy mainly with an Xlinked dominant pattern of inheritance, which is caused by mutations in the OFD1 gene. The OFD1 protein is located within the centrosomes and basal bodies of the primary cilia. It is reported that approximately 15%–50% cases of OFD1 progress to end-stage renal disease(ESRD) following development of polycystic kidney diseases(PKD). Here we report a pair of childhood male twins who presented only renal failure and PKD caused by an OFD1 mutation in China.CASE SUMMARY A pair of 14-year male twins were hospitalized with a complaint of abnormal renal function for nine days. They both complained of ankle pain for 3 mo vs 2 wk, respectively. They denied fever, abdominal pain, daytime or nighttime enuresis, urgency, dysuria, or gross hematuria. Laboratory tests at a local hospital showed renal failure(serum creatinine 485 μmol/L vs 442 μmol/L, blood urea nitrogen 14.7 mol/L vs 14.5 mol/L) and anemia(hemoglobin 88 g/L vs 98 g/L).The twins are monozygotic. There was no abnormal birth, past medical, or family history. Clinical data were analyzed and genetic analysis on PKD was carried out in the twins by next-generation sequencing. The results showed that the twins presented low-molecular-weight proteinuria, hyposthenuria, anemia, renal failure, and renal polycystic changes. Genetic tests showed that the twins both carried a hemizygous mutation in exon 19 c.2524 G>A(p. G842 R) of the OFD1 gene. Their mother heterozygously carried the same mutation as the twins but was without any phenotypes while their father was normal.CONCLUSION We have reported a pair of childhood male twins with an OFD1 mutation who presented ESRD and PKD but without any other phenotypes of OFD1 in China.

Fetal kidney stem cells ameliorate cisplatin induced acute renal failure and promote renal angiogenesis

AIM: To investigate whether fetal kidney stem cells(f KSC) ameliorate cisplatin induced acute renal failure(ARF) in rats and promote renal angiogenesis.METHODS: The f KSC were isolated from rat fetuses of gestation day 16 and expanded in vitro up to 3rd passage. They were characterized for the expression of mesenchymal and renal progenitor markers by flow cytometry and immunocytochemistry, respectively. The in vitro differentiation of f KSC towards epithelial lineage was evaluated by the treatment with specific induction medium and their angiogenic potential by matrigel induced tube formation assay. To study the effect of f KSC in ARF, f KSC labeled with PKH26 were infused in rats with cisplatin induced ARF and, the blood and renal tissues of the rats were collected at different time points. Blood biochemical parameters were studied to evaluate renal function. Renal tissues were evaluated for renal architecture, renal cell proliferation and angiogenesis by immunohistochemistry, renal cell apoptosis by terminal deoxynucleotidyl transferase nickend labeling assay and early expression of angiogenic molecules viz. vascular endothelial growth factor(VEGF), hypoxia-inducible factor(HIF)-1α and endothelial nitric oxide synthase(eN OS) by western blot.RESULTS: The fK SC expressed mesenchymal markers viz. CD29, CD44, CD73, CD90 and CD105 as well asrenal progenitor markers viz. Wt1, Pax2 and Six2. They exhibited a potential to form CD31 and Von Willebrand factor expressing capillary-like structures and could be differentiated into cytokeratin(CK)18 and CK19 positive epithelial cells. Administration of fK SC in rats with ARF as compared to administration of saline alone, resulted in a significant improvement in renal function and histology on day 3(2.33 ± 0.33 vs 3.50 ± 0.34, P < 0.05) and on day 7(0.83 ± 0.16 vs 2.00 ± 0.25, P < 0.05). The infused PKH26 labeled fK SC were observed to engraft in damaged renal tubules and showed increased proliferation and reduced apoptosis(P < 0.05) of renal cells. The kidneys of fK SC as compared to saline treated rats had a higher capillary density on day 3 [13.30 ± 1.54 vs 7.10 ± 1.29, capillaries/high-power fields(HPF), P < 0.05], and on day 7(21.10 ± 1.46 vs 15.00 ± 1.30, capillaries/HPF, P < 0.05). In addition, kidneys of fK SC treated rats had an upregulation of angiogenic proteins hypoxia-inducible factor-1α, VEGF and eN OS on day 3(P < 0.05).CONCLUSION: Our study shows that fK SC ameliorate cisplatin induced ARF in rats and promote renal angiogenesis, which may be an important therapeutic mechanism of these stem cells in the disease.

Cardiac evaluation of renal transplant candidates with heart failure

Patients with advanced kidney disease are at elevated risk of developing heart failure and appropriate risk stratification is important to permit them to receive kidney transplantation.The American Heart Association and American College of Cardiology joint statement provides guidance on risk stratification for the major cause of heart failure for these patients in its recommendations for coronary heart disease.Herein we provide an overview of the available literature on risk strati-fication for nonischemic heart failure and functional heart disease states such as pulmonary hypertension.Many of these options for optimizing these patients be-fore transplant include optimizing their volume status,often with more agg-ressive ultrafiltration.Kidney transplantation remains the treatment of choice for patients with advanced kidney disease and cardiac disease,the correction of the azotemic substances with kidney transplantation has been associated with imp-roved survival than remaining on dialysis long-term.The findings in the studies reviewed here are expected to help clinicians refine current strategies for evalua-ting potential kidney transplant recipients.