Introduction The incidence of kidney stones is increasing in Australia and Aotearoa New Zealand, increasing pressure on emergency departments for acute pain management and stretching nephrology and dietetics services tasked with preventing recurrence. To address this, the Caring for Australians and New ZealandeRs with kidney Impairment (CARI) guidelines provide evidence-based recommendations that complement urological and surgical care, contextualized for our region and developed with input from people with lived experience. Methods Guidelines were developed through a 5-stage process aligned with the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. A multidisciplinary working group formulated Population Intervention/Exposure Comparator Outcome Methodology questions, and a group of consumers in a dedicated workshop scoped guideline topics. A systematic literature review was conducted, prioritizing high-quality evidence that was critically appraised and synthesized. Recommendations were drafted using the GRADE Evidence-to-Decision framework, incorporating perspectives of lived experience throughout. Results These guidelines provide recommendations on the diagnosis, metabolic evaluation, and management to prevent recurrent kidney stones. Nutrition therapy provided by dietitians with expertise is a fundamental and cost-effective intervention for preventing recurrent kidney stones. Although nutrition therapy should precede pharmacological interventions in most cases, the use of potassium citrate and thiazide diuretics may be considered when nutrition therapy alone is insufficient. Conclusion These CARI guidelines address a critical gap in the management of kidney stones in Australia and New Zealand by providing evidence-based, contextualized recommendations to support nephrologists, dietitians with expertise in kidney stones, and other health professionals in delivering consistent, high-quality care that reduces kidney stone recurrence and improves outcomes.

BACKGROUND:The prevalence and impact of early-stage chronic kidney disease (CKD) on pregnancy is poorly defined. We aimed to identify women with suspected early CKD pre-conception and assess the impact on maternofoetal outcomes. METHODS:We linked statewide perinatal datasets to pathology, renal registry and administrative datasets between 2005-23. We included women ≥18 years with one or more maternal serum creatinine measure within 5 years pre-conception or <30 days post-conception and examined maternofetal outcomes. RESULTS:From 96 721 pregnancies (56 587 women), we included 42 212 pregnancies (33 116 women). Of those included, 644 had proteinuria or diagnosed kidney disease [and an estimated glomerular filtration rate (eGFR) >90 mL/min/1.73 m2], 2838 with an eGFR 60-90 mL/min/1.73 m2, and 30 with an eGFR 15-60 mL/min/1.73 m2. Only 6% of women with an eGFR <90 mL/min/1.73 m2 had formal quantitative measurement of albuminuria or proteinuria pre-conception. On multivariable analysis, women with suspected early CKD had an increased risk of gestation hypertension [odds ratio (OR) 1.21; confidence interval (CI) 1.04, 1.41] preeclampsia (OR 1.26; CI 1.03, 1.52), low birth weight (OR 1.15; CI 1.02, 1.31) and preterm delivery (OR 1.27; CI 1.03, 1.56) compared with those with normal kidney function. CONCLUSION:We identified that 1 in 10 pregnancies had an eGFR <90 mL/min/1.73 m2, proteinuria or diagnosed kidney disease, warranting further research focussing on pre-conception kidney function assessments. While these women were at an increased risk of some maternofetal outcomes, the absolute risk remains small.

Genetic kidney disease (GKD) significantly affects the community and is responsible for a notable portion of adult kidney disease cases and about half of cases in paediatric patients. It substantially impacts the quality of life and life expectancy for affected children and adults across all stages of kidney disease. Precise genetic diagnosis in GKD promises to improve patient outcomes, provide access to targeted treatments, and reduce the disease burden for individuals, families, and healthcare systems. Genetic investigations are increasingly used in nephrology practice; however, many patients who undergo testing still lack a definitive diagnosis. The KidGen National Kidney Genomics Study aims to increase diagnostic yield for those with suspected monogenic kidney disease without a diagnosis after standard diagnostic genetic testing. The program will seek to enrol up to 200 families from KidGen Collaborative kidney genetics clinics across Australia who have yet to receive conclusive diagnoses despite prior testing. Participants will undergo a personalised pathway of research genomic investigations. These include re-analysing existing data and/or undergoing advanced genomic testing methods, including short and long-read whole-genome sequencing, RNA sequencing, and functional genomics strategies using mouse modelling or kidney organoids. The KidGen National Kidney Genomics Study is a coordinated, multidisciplinary extension of previous research projects that aims to assess the diagnostic yield of advanced genomic approaches. The study's evidence will drive changes to current diagnostic pathways, including identifying which chronic kidney disease patients are most likely to benefit from a more comprehensive genomic approach to diagnosis.

BACKGROUND:Incidence estimates of acute kidney injury (AKI) in Australia are frequently based on episodes of admitted care. Jurisdiction-wide patient-level linked analysis can better define the epidemiology of AKI. AIMS:Outline the incidence and prevalence of AKI and the outcomes associated with an admission complicated by AKI. METHODS:Retrospective data-linkage analysis using the Victorian Death Index, Cancer Registry, Integrated Non-Admitted Health and Admitted Episode datasets, examining adult, acute, overnight inpatient admissions in Victoria, Australia from July 2016 to June 2017. AKI episodes were identified using ICD-10-AM codes. The initial AKI episode marked the index admission, with a 36-month look-back and 36-month follow-up for comorbidity and outcomes. Primary outcomes were the incidence and prevalence of AKI. Secondary analyses examined readmission rates, the development of new morbidity and mortality, including causes of death. RESULTS:The incidence of AKI was 10.8% (95% confidence interval (CI): 10.7%-10.8%), with higher rates in the elderly, overseas-born and comorbid patients. The prevalence of AKI was 1.1 cases per 100 annually. The inpatient mortality rate was 5.2% and was associated with Indigenous status (odds ration (OR) 1.81, 95% CI: 1.31-2.50), higher comorbidity burden (OR 3.97, 95% CI: 3.46-4.55), age ≥ 65 (OR 1.24, 95% CI: 1.07-1.43) and aged care facility residence (OR 2.32, 95% CI: 1.81-2.98). Among survivors of an index admission, 70.4% were readmitted within 12 months and 43.3% experienced recurrent AKI. Mortality was 23.2% at 12 months and 39.8% at 36 months, primarily attributed to cancer (26.0%) and cardiovascular disease (20.8%). CONCLUSIONS:This jurisdiction-wide, patient-level study demonstrates a high prevalence of AKI, incidence and mortality, highlighting it as a public health issue.

AIM:The prevention and management of recurrent kidney stones can be challenging and requires patients to modify their diet and daily rountines that impact their quality of life. Our study aims to describe the process of integrating consumer-prioritised topics and outcomes in guidelines on kidney stones to ensure patient relevance. METHODS:Two workshops were convened in Aotearoa New Zealand with people with kidney stones invited to identify topics and outcomes for inclusion in the guidelines. Flipcharts and transcripts were analysed thematically to identify the reasons for participants' choices. RESULTS:The topics identified by the twenty-eight participants included education on nutrition, better diagnosis, and individualised nutritional and pharmacological management. Pain, equity of access, anxiety about recurrence, and life participation were identified as important outcomes to be included. Four themes (and subthemes) underpinning priorities were: unresolvable debilitating pain (complexity of exctruciating acute episodic pain, inadequacy of pain relief medication, frustrated by stigma associated with opioids), dissatisfied at delayed access to care (prolonged difficulties in diagnosis, struggling to obtain individualised care), inadequate knowledge to enable self-management (insufficient information on kidney stones, conflicting nutrition advice, cultural deficit), and limiting life participation (restricting life choices, psychological burden of kidney stones). CONCLUSIONS:Participants identified topics that would support symptom management to improve quality of life and reduce the burden on families. Guidelines should provide essential, consistent and clear guidance, particularly on nutrition, to support self-management. Incoporating consumer priorities in guidelines can help to support decision-making and patient-centred care in kidney stones.

Recently, a double-blind randomised controlled trial (RCT) NOSTONE compared hydrochlorothiazide (12.5 mg/d, 25 mg/d, 50 mg/d) to placebo in adults with at least two episodes of kidney stones (at least 50% calcium oxalate, calcium phosphate or a mixture of both) within ten years from 12 centres in Switzerland. The participants in the trial were mostly male (80%) and white ethnicity (99%) with a median age of 49 (interquartile range 39-55) years old, and 63% had hypercalciuria. The study found no difference in kidney stone recurrence compared to placebo and no hydrochlorothiazide dose-response relationship over the mean 34 months of follow-up.

AIM:The aim of this study was to describe long-term outcomes of kidney replacement therapy (KRT) in Australians with prune belly syndrome in comparison to a control group of congenital kidney disease. METHODS:We identified all Australians treated with KRT between 1977 and 2021 with a diagnosis of PBS from the Australia and New Zealand Dialysis and Transplant Registry. RESULTS:We identified 37 males (no females) who commenced KRT at a median age of 17 years (range 1-45). At initiation of KRT treatment, 54% of patients were on haemodialysis, 30% on peritoneal dialysis and 16% received a pre-emptive kidney transplant. Forty-eight kidney transplants (35 first, 11 second and 2 third grafts) occurred, of which 48% were from deceased donors. Median age at first transplant was 21 years (range 2-47). Graft survival at 1, 5 and 10 years for first grafts was 91%, 71% and 51%, respectively (range 6 days to 36 years). Three men reported parenthood at median age 35 years. There were 10 deaths reported at a median age of 37 years (range 17-49). Reported aetiology was cardiac death (50%), malignancy (20%), dialysis cessation (10%) and uncertain cause (20%). Compared to an age and gender-matched control group of people with congenital kidney dysplasia, Australians with PBS had equivalent peritoneal dialysis technique survival, but slightly better transplant graft and overall survival. CONCLUSION:Prune belly syndrome has marked variation in outcomes from KRT, but overall, these were equivalent or better than a matched control group with congenital kidney disease, including use of peritoneal dialysis (despite lack of abdominal wall musculature).

BACKGROUND:Haemodialysis (HD) requires safe and effective anticoagulation to prevent clot formation within the extracorporeal circuit during dialysis treatments to enable adequate dialysis and minimise adverse events, including major bleeding. Low molecular weight heparin (LMWH) may provide a more predictable dose, reliable anticoagulant effects and be simpler to administer than unfractionated heparin (UFH) for HD anticoagulation, but may accumulate in the kidneys and lead to bleeding. OBJECTIVES:To assess the efficacy and safety of anticoagulation strategies (including both heparin and non-heparin drugs) for long-term HD in people with kidney failure. Any intervention preventing clotting within the extracorporeal circuit without establishing anticoagulation within the patient, such as regional citrate, citrate enriched dialysate, heparin-coated dialysers, pre-dilution haemodiafiltration (HDF), and saline flushes were also included. SEARCH METHODS:We searched the Cochrane Kidney and Transplant Register of Studies up to November 2023 through contact with the Information Specialist using search terms relevant to this review. Studies in the Register are identified through searches of CENTRAL, MEDLINE, and EMBASE, conference proceedings, the International Clinical Trials Registry Platform (ICTRP) Search Portal and ClinicalTrials.gov. SELECTION CRITERIA:Randomised controlled trials (RCTs) and quasi-randomised controlled studies (quasi-RCTs) evaluating anticoagulant agents administered during HD treatment in adults and children with kidney failure. DATA COLLECTION AND ANALYSIS:Two authors independently assessed the risk of bias using the Cochrane tool and extracted data. Treatment effects were estimated using random effects meta-analysis and expressed as relative risk (RR) or mean difference (MD) with 95% confidence intervals (CI). Evidence certainty was assessed using the Grading of Recommendation, Assessment, Development and Evaluation approach (GRADE). MAIN RESULTS:We included 113 studies randomising 4535 participants. The risk of bias in each study was adjudicated as high or unclear for most risk domains. Compared to UFH, LMWH had uncertain effects on extracorporeal circuit thrombosis (3 studies, 91 participants: RR 1.58, 95% CI 0.46 to 5.42; I2 = 8%; low certainty evidence), while major bleeding and minor bleeding were not adequately reported. Regional citrate anticoagulation may lower the risk of minor bleeding compared to UFH (2 studies, 82 participants: RR 0.34, 95% CI 0.14 to 0.85; I2 = 0%; low certainty evidence). No studies reported data comparing regional citrate to UFH on risks of extracorporeal circuit thrombosis and major bleeding. The effects of very LMWH, danaparoid, prostacyclin, direct thrombin inhibitors, factor XI inhibitors or heparin-grafted membranes were uncertain due to insufficient data. The effects of different LMWH, different doses of LMWH, and the administration of LMWH anticoagulants using inlet versus outlet bloodline or bolus versus infusion were uncertain. Evidence to compare citrate to another citrate or control was scant. The effects of UFH compared to no anticoagulant therapy or different doses of UFH were uncertain. Death, dialysis vascular access outcomes, blood transfusions, measures of anticoagulation effect, and costs of interventions were rarely reported. No studies evaluated the effects of treatment on non-fatal myocardial infarction, non-fatal stroke and hospital admissions. Adverse events were inconsistently and rarely reported. AUTHORS' CONCLUSIONS:Anticoagulant strategies, including UFH and LMWH, have uncertain comparative risks on extracorporeal circuit thrombosis, while major bleeding and minor bleeding were not adequately reported. Regional citrate may decrease minor bleeding, but the effects on major bleeding and extracorporeal circuit thrombosis were not reported. Evidence supporting clinical decision-making for different forms of anticoagulant strategies for HD is of low and very low certainty, as available studies have not been designed to measure treatment effects on important clinical outcomes.