Search

BACKGROUND: Hyperphosphatemia is associated with increased morbidity and mortality in patients with end-stage kidney disease (ESKD). Whereas clinical and observational studies have demonstrated the effectiveness of sucroferric oxyhydroxide (SO) in controlling serum phosphorus (sP) in ESKD, data on the real-world impact of switching to SO in patients on peritoneal dialysis (PD) are limited. In this retrospective database analysis, we examine the impact of SO on sP management over a 1-year period among PD patients prescribed SO as part of routine clinical care. METHODS: We analyzed de-identified data from adults on PD in Fresenius Kidney Care clinics who were prescribed SO monotherapy between May 2018 and December 2019 as part of routine clinical management. Changes from baseline in sP levels, phosphate binder (PB) pill burden, and laboratory parameters were evaluated during the four consecutive 91-day intervals of SO treatment. RESULTS: The mean age of the 402 patients who completed 1 year of SO was 55.2 years at baseline, and they had been on PD for an average of 19.9 months. SO was initiated with no baseline PB recorded in 36.1% of patients, whereas the remaining 257 patients were switched to SO from sevelamer (39.7%), calcium acetate (30.4%), lanthanum (1.2%), ferric citrate (14.0%), or more than one PB (14.8%). Mean sP at baseline was 6.26 mg/dL. After being prescribed SO, the percentage of patients achieving sP ≤ 5.5 mg/dL increased from 32.1% (baseline) to 46.5-54.0% during the 1-year follow-up, whereas the mean number of PB pills taken per day decreased from 7.7 at baseline (among patients on a baseline PB) to 4.6 to 5.4. Serum phosphorus and PB pill burden decreased regardless of changes in residual kidney function over the 12-month period. Similar results were observed for the full cohort (976 patients who either completed or discontinued SO during the 1-year follow-up). CONCLUSIONS: Patients on PD who were prescribed SO as part of routine care for phosphorus management experienced significant reductions in SP and PB pills per day and improvements in sP target achievement, suggesting the effectiveness of SO on SP management with a concurrent reduction in pill burden.

Hemodialysis is a conservative treatment for end-stage renal disease. It has various complications which negatively affect quality of life (QOL). This study aimed to examine the relationship between fatigue, pruritus, and thirst distress (TD) with QOL of patients receiving hemodialysis, while also considering the mediating role of treatment adherence (TA). This cross-sectional study was carried out in 2023 on 411 patients receiving hemodialysis. Participants were consecutively recruited from several dialysis centers in Iran. Data were collected using a demographic information form, the Fatigue Assessment Scale, the Thirst Distress Scale, the Pruritus Severity Scale, the 12-Item Short Form Health Survey, and the modified version of the Greek Simplified Medication Adherence Questionnaire for Hemodialysis Patients. Covariance-based structural equation modeling was used for data analysis. The structural model and hypothesis testing results showed that all hypotheses were supported in this study. QOL had a significant inverse association with fatigue, pruritus, and TD and a significant positive association with TA. TA partially mediated the association of QOL with fatigue, pruritus, and TD, denoting that it helped counteract the negative association of these complications on QOL. This model explained 68.5% of the total variance of QOL. Fatigue, pruritus, and TD have a negative association with QOL among patients receiving hemodialysis, while TA reduces these negative associations. Therefore, TA is greatly important to manage the associations of these complications and improve patient outcomes. Healthcare providers need to assign high priority to TA improvement among these patients to reduce their fatigue, pruritus, and TD and improve their QOL. Further studies are necessary to determine the most effective strategies for improving TA and reducing the burden of complications in this patient population.

KEY POINTS: In one hospital-based safety-net dialysis unit, only one half of patients with ESKD knew their cause of kidney failure, which did not differ by English proficiency status. Patients with limited English proficiency (versus English-proficient patients) reported poorer communication with the dialysis care team (less listening, fewer clear explanations, less time spent). We highlight the need for tailored, patient-centered communication between limited English-proficient patients and dialysis care team members. BACKGROUND: ESKD is a chronic health condition for which communication between health care teams and patients is important to guide patient self-management activities. Yet, little is known about the quality of communication among patients with ESKD and their care team members. We examined the influence of patient's limited English proficiency (LEP) status on communication experiences at one dialysis center. METHODS: A survey was administered to adults receiving ESKD care at a dialysis unit within a public health care delivery system between July 2022 and February 2023, to ascertain kidney disease knowledge and perceptions of communication quality with the dialysis care team. Multivariable logistic and ordinal logistic regression models adjusted for age and sex were used to determine associations between LEP status and CKD knowledge. RESULTS: Among 93 eligible patients, 88.2% (n=82) completed the survey. Approximately 37.8% (n=31) had LEP, mean age was 58.8 years, 68.3% were men, mean dialysis vintage was 3.9 years, and 25% had a positive depression screen (LEP 30%; English-proficient 22%). A higher proportion of English-proficient patients screened positive for limited health literacy compared to those with LEP (74.5% versus 38.7%, P = 0.002). Overall, knowledge of assigned cause of ESKD (53.4%) and CKD/transplant knowledge (57.3%) was suboptimal. After adjustment, LEP status was not significantly associated with knowing the correct cause of kidney failure (odds ratio, 0.49; 95% confidence interval, 0.19 to 1.27) but was significantly associated with having a higher score on a CKD/transplant knowledge scale (odds ratio, 3.99; 95% confidence interval, 1.66 to 9.58). Patients with LEP reported poorer communication quality with dialysis providers and staff (less listening, fewer clear explanations, less time spent with patients) compared with English-proficient patients, although differences were not statistically significant. CONCLUSIONS: Overall communication between patients with ESKD and members of the dialysis care team was suboptimal, regardless of English proficiency. Interventions to enhance communication for ESKD patients are needed.

The South Asia region is facing a high burden of chronic kidney disease (CKD) with limited health resources and low expenditure on health care. In addition to the burden of CKD and kidney failure from traditional risk factors, CKD of unknown etiologies from India and Sri Lanka compounds the challenges of optimal management of CKD in the region. From the third edition of the International Society of Nephrology Global Kidney Health Atlas (ISN-GKHA), we present the status of CKD burden, infrastructure, funding, resources, and health care personnel using the World Health Organizations building blocks for health systems in the ISN South Asia region. The poor status of the public health care system and low health care expenditure resulted in high out-of-pocket expenditures for people with kidney disease, which further compounded the situation. There is insufficient country capacity across the region to provide kidney replacement therapies to cover the burden. The infrastructure was also not uniformly distributed among the countries in the region. There were no chronic hemodialysis centers in Afghanistan, and peritoneal dialysis services were only available in Bangladesh, India, Nepal, Pakistan, and Sri Lanka. Kidney transplantation was not available in Afghanistan, Bhutan, and Maldives. Conservative kidney management was reported as available in 63% (n = 5) of the countries, yet no country reported availability of the core CKM care components. There was a high hospitalization rate and early mortality because of inadequate kidney care. The lack of national registries and actual disease burden estimates reported in the region prevent policymakers attention to CKD as an important cause of morbidity and mortality. Data from the 2023 ISN-GKHA, although with some limitations, may be used for advocacy and improving CKD care in the region.

BACKGROUND: Residence in rural areas is often a barrier to health care access. To date, differences in access to kidney transplantation among children who reside in rural and micropolitan areas of the US have not been explored. METHODS: A retrospective cohort study of children

OBJECTIVE: For the majority of patients with lupus nephritis-related end-stage kidney disease (LN-ESKD), kidney transplant is associated with better outcomes than dialysis. Access to kidney transplant requires an initial referral to a transplant center and medical evaluation prior to waitlisting. The studys objective was to examine access to these early steps in the kidney transplant process among patients with LN-ESKD. METHODS: Adults who began treatment for ESKD in the Southeast, Northeast, New York, or Ohio River Valley U.S. regions from 1/1/2012 to 12/31/2019, followed through 6/30/2021, were identified from the United States Renal Data System. Referral and evaluation start data were collected from 28 of 48 transplant centers across these regions. The exposure was primary cause of ESKD (LN-ESKD vs other-ESKD). The outcomes were referral and evaluation start at a transplant center. Cox models quantified the association between LN-ESKD (vs other-ESKD) and referral and evaluation start. RESULTS: Among 192,318 patients initiating treatment for ESKD, 0.4% had LN-ESKD. Over half (58%) of LN-ESKD patients were referred before study end, and among those referred, 66% started the evaluation. In adjusted analyses, patients with LN-ESKD were referred (HR: 1.09, 95% CI: 0.99, 1.19) and started the transplant evaluation (HR: 1.13, 95% CI: 1.00, 1.28) at a higher rate than patients with other-ESKD. Among referred patients with LN-ESKD, the median time from ESKD start to referral was 2.9 months (IQR:

Aims Methods Results Conclusion Across its operational span of more than 25 years, the observational, nationwide, multicentre Finnish Diabetic Nephropathy (FinnDiane) Study has aimed to unravel mechanisms underlying diabetic kidney disease, with a special focus on its metabolic risk factors. We sought to compile key findings relating to this topic and to offer a current perspective on the natural course of diabetic kidney disease among individuals with type 1 diabetes.In this narrative review, articles relevant to the subject published by the FinnDiane Study were identified and summarized together with work published by others, when relevant.The FinnDiane Study has underscored the significance of dysglycaemia and insulin resistance, increased visceral fat mass, hypertension and dyslipidaemia—particularly high triglycerides and remnant cholesterol—as risk factors for diabetic kidney disease. Factors like abdominal obesity seem to influence the early stages of the disease, while the presence of the metabolic syndrome becomes implicated at later stages. Epidemiological reports have revealed that after an initial decline, the cumulative incidence of albuminuria plateaued post‐1980s, with the progression rate to kidney failure remaining high. Fortunately, 23% of the FinnDiane cohort regressed to less advanced stages of albuminuria, improving their overall prognosis.A substantial burden of albuminuria associated with type 1 diabetes persists, and therefore, novel kidney‐protecting therapies are highly awaited. In addition, given that metabolic factors influence the progression of diabetic kidney disease both in its early and advanced stages, emphasis should be placed on ensuring that their treatment targets are met. [ABSTRACT FROM AUTHOR]

Background Kidney transplantation (KT) is the preferred modality of kidney replacement therapy with better patient outcomes and quality of life compared with dialytic therapies. This study aims to evaluate the epidemiology, accessibility and availability of KT services in countries and regions around the world. Methods This study relied on data from an international survey of relevant stakeholders (clinicians, policymakers and patient advocates) from countries affiliated with the International Society of Nephrology that was conducted from July to September 2022. Survey questions related to the availability, access, donor type and cost of KT. Results In total, 167 countries responded to the survey. KT services were available in 70% of all countries, including 86% of high-income countries, but only 21% of low-income countries. In 80% of countries, access to KT was greater in adults than in children. The median global prevalence of KT was 279.0 [interquartile range (IQR) 58.0–492.0] per million people (pmp) and the median global incidence was 12.2 (IQR 3.0–27.8) pmp. Pre-emptive KT remained exclusive to high- and upper-middle-income countries, and living donor KT was the only available modality for KT in low-income countries. The median cost of the first year of KT was $26 903 USD and varied 1000-fold between the most and least expensive countries. Conclusion The availability, access and affordability of KT services, especially in low-income countries, remain limited. There is an exigent need to identify strategies to ensure equitable access to KT services for people with kidney failure worldwide, especially in the low-income countries. [ABSTRACT FROM AUTHOR]

Background Hemodialysis (HD) is the most commonly utilized modality for kidney replacement therapy worldwide. This study assesses the organizational structures, availability, accessibility, affordability and quality of HD care worldwide. Methods This cross-sectional study relied on desk research data as well as survey data from stakeholders (clinicians, policymakers and patient advocates) from countries affiliated with the International Society of Nephrology from July to September 2022. Results Overall, 167 countries or jurisdictions participated in the survey. In-center HD was available in 98% of countries with a median global prevalence of 322.7 [interquartile range (IQR) 76.3–648.8] per million population (pmp), ranging from 12.2 (IQR 3.9–103.0) pmp in Africa to 1575 (IQR 282.2–2106.8) pmp in North and East Asia. Overall, home HD was available in 30% of countries, mostly in countries of Western Europe (82%). In 74% of countries, more than half of people with kidney failure were able to access HD. HD centers increased with increasing country income levels from 0.31 pmp in low-income countries to 9.31 pmp in high-income countries. Overall, the annual cost of in-center HD was US$19 380.3 (IQR 11 817.6–38 005.4), and was highest in North America and the Caribbean (US$39 825.9) and lowest in South Asia (US$4310.2). In 19% of countries, HD services could not be accessed by children. Conclusions This study shows significant variations that have remained consistent over the years in availability, access and affordability of HD across countries with severe limitations in lower-resourced countries. [ABSTRACT FROM AUTHOR]

Background Conservative kidney management (CKM) describes supportive care for people living with kidney failure who choose not to receive or are unable to access kidney replacement therapy (KRT). This study captured the global availability of CKM services and funding. Methods Data came from the International Society of Nephrology Global Kidney Health survey conducted between June and September 2022. Availability of CKM, infrastructure, guidelines, medications and training were evaluated. Results CKM was available in some form in 61% of the 165 responding countries. CKM chosen through shared decision-making was available in 53%. Choice-restricted CKM—for those unable to access KRT—was available in 39%. Infrastructure to provide CKM chosen through shared decision-making was associated with national income level, reported as being "generally available" in most healthcare settings for 71% of high-income countries, 50% of upper-middle-income countries, 33% of lower-middle-income countries and 42% of low-income countries. For choice-restricted CKM, these figures were 29%, 50%, 67% and 58%, respectively. Essential medications for pain and palliative care were available in just over half of the countries, highly dependent upon income setting. Training for caregivers in symptom management in CKM was available in approximately a third of countries. Conclusions Most countries report some capacity for CKM. However, there is considerable variability in terms of how CKM is defined, as well as what and how much care is provided. Poor access to CKM perpetuates unmet palliative care needs, and must be addressed, particularly in low-resource settings where death from untreated kidney failure is common. [ABSTRACT FROM AUTHOR]

Background Methods Results Conclusions BK polyomavirus‐associated nephropathy (BKPyVAN) is an important cause of allograft dysfunction and failure in kidney transplant recipients (KTRs) and there are no proven effective treatments. Case reports and in vitro data support the potential activity of cidofovir against BK polyomavirus (BKPyV).We report the results of a phase I/II, double‐blind, placebo‐controlled randomized dose‐escalation trial of cidofovir in KTRs with biopsy‐confirmed BKPyVAN and estimated glomerular filtration rate ≥30 mL/min. Intravenous cidofovir (0.25 mg/kg/dose or 0.5 mg/kg/dose) or placebo was administered on days 0, 7, 21, and 35, with final follow‐up through day 49.The trial was prematurely discontinued due to slow accrual after 22 KTRs had completed the study. Cidofovir was safe and tolerated at the doses and duration studied. The proportion of subjects with any adverse event (AE) was similar between groups (9/14 [64%] in the combined cidofovir dose groups and 6/8 [75%] in the placebo group); 84% of AEs were mild. BKPyV DNAemia reduction by day 49 was similar between groups (>1 log10 reduction in (2/9 [22.2%] of 0.25 mg/kg group, 1/5 [20%] of 0.5 mg/kg group, and 2/8 [25%] of placebo group).These preliminary results indicate that low‐dose cidofovir was safe and tolerated but had no significant BKPyV‐specific antiviral effect in KTRs with BKPyVAN. [ABSTRACT FROM AUTHOR]

Introduction The relationship between mortality and troponin in non-ST-elevation myocardial infarction (NSTEMI) patients with a history of renal failure is quite limited. This study investigated the relationship between blood delta troponin T levels and 30-day mortality in patients with chronic renal dysfunction and NSTEMI. Materials and Methods This study was conducted prospectively by including patients with chronic renal dysfunction and clinical findings of NSTEMI between February 1, 2021, and August 1, 2022. Demographics, medical history, laboratory parameters, and mortality data were noted. Thirty-day morbidity data was used for mortality. Delta troponin T was calculated using initial and first-hour troponin T values. Patients were grouped as healthy and deceased. Data were evaluated using univariant analysis and receiver operating characteristics analysis. Results Of the 73 patients included in the study, 29 were female. The mean age of the patients was 67.3 years. The 30-day mortality rate was 9.5%. The sensitivity of the initial troponin T value was 85.7% (42.1–99.6), the specificity was 68.2% (55.6–79.1), and the accuracy was 69.9% (58–80.1), and the sensitivity of the first-hour troponin T value was 85.7% (42.1–99.6), specificity was 75.8% (63.6–85.5), and accuracy was 76.7% (65.4–85.8). The delta troponin T median of the mortality group was 56 (24.2–286.4), and the delta troponin median of the surviving patients was 29.4 (10.7–79.6). The difference was not statistically significant (p = 0.072). Conclusion The current study's results show that delta troponin T (initial and first hour) is not associated with short-term mortality in patients with chronic renal dysfunction and NSTEMI. [ABSTRACT FROM AUTHOR]

Objectives Increasing studies demonstrated the importance of C5a and anti-neutrophil cytoplasmic antibody (ANCA)-induced neutrophil activation in the pathogenesis of ANCA-associated vasculitis (AAV). Sphingosine-1-phosphate (S1P) acts as a downstream effector molecule of C5a and enhances neutrophil activation induced by C5a and ANCA. The current study investigated the role of a S1P receptor modulator, FTY720, in experimental autoimmune vasculitis (EAV) and explored the immunometabolism-related mechanisms of FTY720 in modulating ANCA-induced neutrophil activation. Methods The effects of FTY720 in EAV were evaluated by quantifying haematuria, proteinuria, crescent formation, tubulointerstitial injury and pulmonary haemorrhage. RNA sequencing of renal cortex and gene enrichment analysis were performed. The proteins of key identified pathways were analysed in neutrophils isolated from peripheral blood of patients with active AAV and normal controls. We assessed the effects of FTY720 on ANCA-induced neutrophil respiratory burst and neutrophil extracellular traps formation (NETosis). Results FTY720 treatment significantly attenuated renal injury and pulmonary haemorrhage in EAV. RNA sequencing analyses of renal cortex demonstrated enhanced fatty acid oxidation (FAO) and peroxisome proliferator-activated receptor (PPAR) signalling in FTY720-treated rats. Compared with normal controls, patients with active AAV showed decreased FAO in neutrophils. FTY720-treated differentiated HL-60 cells showed increased expression of carnitine palmitoyltransferase 1a (CPT1a) and PPARα. Blocking or knockdown of CPT1a or PPARα in isolated human neutrophils and HL-60 cells reversed the inhibitory effects of FTY720 on ANCA-induced neutrophil respiratory burst and NETosis. Conclusion FTY720 attenuated renal injury in EAV through upregulating FAO via the PPARα–CPT1a pathway in neutrophils, offering potential immunometabolic targets in AAV treatment. [ABSTRACT FROM AUTHOR]

Purpose: Recent advances have led to greater recognition of the role of mitochondrial dysfunction in the pathogenesis of chronic kidney disease (CKD). There has been evidence that CKD is also associated with dysbiosis. Here, we aimed to evaluate whether probiotic supplements can have protective effects against kidney injury via improving mitochondrial function. Methods: An animal model of CKD was induced by feeding C57BL/6 mice a diet containing 0.2% adenine. KBL409, a strain of Lactobacillus acidophilus, was administered via oral gavage at a dose of 1 × 109 CFU daily. To clarify the underlying mechanisms by which probiotics exert protective effects on mitochondria in CKD, primary mouse tubular epithelial cells stimulated with TGF-β and p-cresyl sulfate were administered with butyrate. Results: In CKD mice, PGC-1α and AMPK, key mitochondrial energy metabolism regulators, were down-regulated. In addition, mitochondrial dynamics shifted toward fission, the number of fragmented cristae increased, and mitochondrial mass decreased. These alterations were restored by KBL409 administration. KBL409 supplementation also improved defects in fatty acid oxidation and glycolysis and restored the suppressed enzyme levels involved in TCA cycle. Accordingly, there was a concomitant improvement in mitochondrial respiration and ATP production assessed by mitochondrial function assay. These favorable effects of KBL409 on mitochondria ultimately decreased kidney fibrosis in CKD mice. In vitro analyses with butyrate recapitulated the findings of animal study. Conclusions: This study demonstrates that administration of the probiotic Lactobacillus acidophilus KBL409 protects against kidney injury via improving mitochondrial function. [ABSTRACT FROM AUTHOR]

Objectives Temocillin, a carbapenem-sparing β-lactam antibiotic, is commonly used at the standard 4 g/day dosage for treating complicated urinary tract infections (cUTIs). However, pharmacokinetic/pharmacodynamic (PK/PD) data supporting this regimen is limited. This study evaluated the plasma pharmacokinetics (PK) and PTA of temocillin in non-critically ill cUTI patients with varying degrees of renal insufficiency (RI). Methods In this single-centre clinical study, 22 cUTI patients received a fixed 4 g/day (2 g q12h, intravenously) temocillin dose, irrespective of renal function (no RI: n  = 5, mild RI: n  = 8, moderate RI: n  = 9). Plasma samples were collected post-dosing for LC-MS analysis of total and unbound temocillin levels. Monte Carlo simulations were performed based on the established PK/PD target of ≥35% f T > MIC (minimal inhibitory concentration). Results Among patients, the highest plasma drug exposure and PK/PD target attainment were observed in those with moderate RI (median AUC0–12h = 1143 h.mg/L and % f T > MIC = 68%), followed by mild RI patients (median AUC0–12h = 918 h.mg/L and % f T > MIC = 34%), and the lowest in those with healthy kidney function (median AUC0–12h = 692 h.mg/L and % f T > MIC = 26%). Simulations indicated that the 4 g/day temocillin dose achieves 90% PTA only for glomerular filtration rate < 60 mL/min and MIC ≤ 8 mg/L. Conclusion The standard temocillin dose may need to be increased from 4 to 6 g/day to treat non-critically ill cUTI patients, in line with recent EUCAST recommendations. For patients with moderate RI, who experience higher exposure due to reduced renal drug clearance, 4 g/day temocillin remains appropriate. [ABSTRACT FROM AUTHOR]

Purpose: To summarize the clinical and biochemical characteristics of patients with ceftriaxone-induced liver injury and guide the selection of safe medication. Methods: Retrieved domestic and foreign databases from inception to October 2023, collected case data conforming to ceftriaxone-induced liver injury, and statistically analyzed the data. Results: A total of 617 articles were retrieved, and 16 articles with 33 cases (10 children, 23 adults) were included. Males represented 60% (18/30), with a male-to-female ratio of 1.5:1. The age of onset ranged from 2 days to 96 years, with 15 of 23 adults (65%) over 55 years old. The time from ceftriaxone use to liver injury fluctuated between 0.5 and 47 days. Only 9 patients (27.3%, 9/33) had clinical symptoms, and the clinical classification was dominated by cholestatic injury (46.2%, 12/26). There was a significant difference in the clinical classification of ceftriaxone-induced liver injury between children and adults (P = 0.0126), with hepatocellular injury predominating in children and cholestatic injury predominating in adults. The severity of liver injury was mainly mild (66.7%, 12/18). Peak values of alanine aminotransferase ranging from 228.5 to 8098 U/L, aspartate aminotransferase ranging from 86.7 to 21575 U/L, alkaline phosphatase ranging from 143 to 2434 U/L, and total bilirubin ranging from 3.35 to 66.1 mg/dL. There was a significant difference in peak values of alkaline phosphatase between children and adults (P = 0.027), with a higher peak value of alkaline phosphatase in adults (1039 ± 716.4 U/L vs. 257 ± 134.9 U/L). Patients with normal imaging examinations accounted for the majority (61.5%, 7/13). The prognosis of 32 patients (97%, 32/33) was good, and one child with sickle cell anemia who developed immune hemolysis, progressive renal failure, and acute liver injury after using ceftriaxone died in the end. Conclusion: Ceftriaxone-induced liver injury can occur at any age, with a higher risk in the elderly, and age may be related to the clinical classification. Although the clinical manifestations are not specific, close monitoring of liver biochemical indicators during the use can detect liver injury early. Most cases have a good prognosis, but for people with concomitant sickle cell anemia, it is necessary to be vigilant about the occurrence of severe hemolytic anemia. [ABSTRACT FROM AUTHOR]

Nuclear factor kappa B (NF-κB) family plays a central role in the human immune system. Heterozygous variants in NFKB2 typically cause immunodeficiency with various degrees of central adrenal insufficiency, autoimmunity, and ectodermal dysplasia. No reported case has presented kidney failure as an initial symptom. Moreover, documentation of kidney involvement of this disease is limited. Case diagnosis: A 2-year-old female who presented with dyspnea and hypertensive emergency in the setting of new-onset nephrotic syndrome with acute-on chronic kidney injury with resultant chronic kidney disease (CKD) was found to have a novel heterozygous N-terminal variant in NFKB2 (c.880del: p. Tyr294Ilefs*4) with mild hypogammaglobulinemia, but no adrenal insufficiency or ectodermal dysplasia. She became dialysis-dependent during her initial hospitalization and developed CKD stage 5D, requiring continued peritoneal dialysis. She is currently awaiting kidney transplantation. Conclusions: Whether nephrotic syndrome or kidney injury or failure is the primary symptom of this variant or secondary to some event remains unknown. Further case accumulation is warranted. [ABSTRACT FROM AUTHOR]

The incidence of rare diseases is expected to be comparatively higher in the Middle East and North Africa (MENA) region than in other parts of the world, attributed to the high prevalence of consanguinity. Most MENA countries share social and economic statuses, cultural relativism, religious beliefs, and healthcare policies. Polycystic kidney diseases (PKDs) are the most common genetic causes of kidney failure, accounting for nearly 8.0% of dialysis cases. The development of PKDs is linked to variants in several genes, including PKD1, PKD2, PKHD1, DZIP1L, and CYS1. Autosomal recessive PKD (ARPKD) is the less common yet aggressive form of PKD. ARPKD has an estimated incidence between 1:10,000 and 1:40,000. Most patients with ARPKD require kidney replacement therapy earlier than patients with autosomal dominant polycystic kidney disease (ADPKD), often in their early years of life. This review gathered data from published research studies and reviews of ARPKD, highlighting the epidemiology, phenotypic presentation, investigations, genetic analysis, outcomes, and management. Although limited data are available, the published literature suggests that the incidence of ARPKD may be higher in the MENA region due to consanguineous marriages. Patients with ARPKD from the MENA region usually present at a later disease stage and have a relatively short time to progress to kidney failure. Limited data are available regarding the management practice in the region, which warrants further investigations. [ABSTRACT FROM AUTHOR]

Aim: The aim of this study was to examine the clinical and pathological characteristics as well as the prognosis of immunoglobulin A nephropathy (IgAN) accompanied by renal thrombotic microangiopathy (rTMA) in paediatric patients. Methods: After balancing epidemiological characteristics and pathological types between groups, 427 patients (rTMA group: 23, non‐rTMA group: 46) were included. The clinical and pathological features, prognosis and clinical risk factors of the two groups were analysed. Results: IgAN‐rTMA children showed more severe clinical and pathological manifestations. The findings from the logistic regression analysis indicated that hypercellularity 1 (E1) (HR: 0.805, 95% CI: 0.763 ~ 1.452, P =.016), endocapillary proliferation (HR: 1.214, 95% CI: 0.093 ~ 4.815, P =.025) and C3 staining (HR: 7.554, 95% CI: 2.563 ~ 15.729, P =.037) were the risk factors for rTMA in children with IgAN. The renal survival in rTMA group was lower than non‐rTMA group (χ2 = 18.467, P =.000). Cox regression analysis showed that E1 (HR: 7.441, 95% CI: 1.095 ~ 10.768, P =.037), C3 disposition (HR: 3.414, 95% CI: 0.834 ~ 11.578, P =.027) and rTMA (HR: 8.918, 95% CI: 1.032 ~ 16.754, P =.041) were identified as independent risk factors for the development of end‐stage renal disease (ESRD). Conclusion: The presence of rTMA had a significant impact on the severity and prognosis of IgAN. And rTMA has been identified as an independent risk factor for the development of renal failure in children diagnosed with IgAN. [ABSTRACT FROM AUTHOR]

Primary coenzyme Q10 deficiency‐1, caused by COQ2 disease‐causing variants, is an autosomal recessive disorder, and genetic testing is the gold standard for diagnosing this condition. A Chinese boy with steroid‐resistant nephrotic syndrome, focal segmental glomerulosclerosis, and progressive kidney insufficiency was included in the study. Electron microscopy revealed the glomerular basement membrane with irregular thickness and lamellation with diffuse effacement of foot processes in the podocytes, and swollen mitochondria with abnormal cristae in the podocytes. Coenzyme Q10 supplementation started about 3 weeks after the onset of mild kidney dysfunction did not improve the proband's kidney outcome. Proband‐only whole‐exome sequencing and Sanger sequencing revealed two heteroallelic COQ2 variants: a maternally inherited novel variant c.1013G > A[p.(Gly338Glu)] in exon 6 and a variant of unknown origin c.1159C > T[p.(Arg387*)] in exon 7. Subsequent long‐read sequencing demonstrated these two variants were located on different alleles. Our report extends the phenotypic and genotypic spectrum of COQ2 glomerulopathy. [ABSTRACT FROM AUTHOR]

The management of acute kidney injury (AKI) in cirrhosis is challenging. The EASL guidelines proposed an algorithm for the management of AKI, but this has never been validated. We aimed to prospectively evaluate this algorithm in clinical practice. We performed a prospective cohort study in consecutive hospitalized patients with cirrhosis and AKI. The EASL management algorithm includes identification/treatment of precipitating factors, 2-day albumin infusion in patients with AKI ≥stage 1B, and treatment with terlipressin in patients with hepatorenal syndrome (HRS-AKI). The primary outcome was treatment response, which included both full and partial response. Secondary outcomes were survival and adverse events associated with terlipressin therapy. A total of 202 AKI episodes in 139 patients were included. Overall treatment response was 80%, while renal replacement therapy was required in only 8%. Response to albumin infusion was achieved in one-third of episodes. Of patients not responding to albumin, most (74%) did not meet the diagnostic criteria of HRS-AKI, with acute tubular necrosis (ATN) being the most common phenotype. The response rate in patients not meeting the criteria for HRS-AKI was 70%. Only 30 patients met the diagnostic criteria for HRS-AKI, and their response rate to terlipressin was 61%. Median time from AKI diagnosis to terlipressin initiation was only 2.5 days. While uNGAL (urinary neutrophil gelatinase-associated lipocalin) could differentiate ATN from other phenotypes (AUROC 0.78), it did not predict response to therapy in HRS-AKI. Ninety-day transplant-free survival was negatively associated with MELD-Na, ATN and HRS-AKI as well as uNGAL. Three patients treated with terlipressin developed pulmonary edema. The application of the EASL AKI algorithm is associated with very good response rates and does not significantly delay initiation of terlipressin therapy. The occurrence of acute kidney injury (AKI) in patients with cirrhosis is associated with poor short-term mortality. Improving its rapid identification and prompt management was the focus of the recently proposed EASL AKI algorithm. This is the first prospective study demonstrating that high AKI response rates are achieved with the use of this algorithm, which includes identification of AKI, treatment of precipitating factors, a 2-day albumin challenge in patients with AKI ≥1B, and supportive therapy in patients with persistent AKI not meeting HRS-AKI criteria or terlipressin with albumin in those with HRS-AKI. These findings support the use of this algorithm in clinical practice. [Display omitted] • In 2018, EASL published an algorithm for the diagnosis/management of acute kidney injury in patients with cirrhosis. • This algorithm had not been validated in real-world practice. • In this prospective study, this algorithm was associated with high renal response rates, both overall and in different phenotypes. • The use of the algorithm resulted in the swift diagnosis and treatment of hepatorenal syndrome. • These results support the use of this algorithm in clinical practice. [ABSTRACT FROM AUTHOR]

This article examines the relationship between microscopic hematuria (blood in the urine) and kidney outcomes in patients with C3 glomerulonephritis (C3GN) and immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN). The study found that patients who achieved remission of hematuria had better kidney survival compared to those who did not achieve remission. Conversely, patients with persistent microscopic hematuria had worse kidney outcomes. The study suggests that routine assessment of microscopic hematuria could be used as a biomarker of disease activity in clinical practice, but further research is needed to confirm this. [Extracted from the article]