The KidGen Collaborative\'s Policy Implementation Workshop 2023 celebrated the 10th anniversary of Australia\'s first kidney genetics clinic in Brisbane. This event marked the establishment of a national network now comprising 19 kidney genetics clinics across Australia, all dedicated to providing equitable access to genomic testing for families affected by genetic kidney diseases. The workshop reflected on past progress and outlined future objectives for kidney genetics in Australia, recognising the collaborative efforts of clinical teams, researchers, and patients. Key insights from the workshop are documented in the proceedings.

BACKGROUND The tip-flexible suctioning ureteral access sheath (TFS-UAS) can be bent under flexible ureteroscopes, which facilitates removal of renal stone segments by irrigation and suctioning effects. Small-scale comparative studies found it safer and more efficacious than traditional UAS. However, complications such as renal abscess were not documented after TFS-UAS combined with digital FURS. CASE REPORT A 57-year-old woman had right lumbar pain that persisted for 1 year. A plain computed tomography (CT) scan revealed multiple renal pelvicalyceal stones (maximum diameter 20×9 mm). She was admitted to undergo elective surgery with a TFS-UAS combined with digital flexible ureteroscopic lithotripsy. The operation was deemed successful and she was given postoperative antibiotics for 2 days before discharge. Eight postoperative days later, she was admitted to the emergency department due to high fever (39.6°C). Plain CT revealed intact double-J stents and no abnormalities. She was readmitted to the urological department to receive antibiotic therapy, which progressed to septic shock (blood pressure 80/50 mmHg) and required immediate transfer to the intensive care unit. Contrast-enhanced CT revealed a right renal abscess. She was promptly resuscitated and given stronger antibiotics. She recovered well and was discharged with 2-week oral levofloxacin treatment. Follow-up ultrasound found no renal abscess. CONCLUSIONS While TFS-UAS with digital FURs is an effective approach for multiple renal stones, there is a risk of postoperative renal abscess, possibly due to altered intrarenal pressure.

The KDIGO Update 2024 was supplemented by new \"Clinical Practice Points\", which were derived from the current evidence but are not necessarily comprehensively proven by prospective controlled studies. The most significant change in the Update 2024 for Lupus nephritis concerns the recommendations for induction therapy for lupus nephritis classes III and IV. The basis is still high-dose glucocorticoid treatment and the use of hydroxychloroquine. The 2 new developments in the 2024 Update concerning ANCA-associated nephritis are based on the studies on the use of the C5a receptor inhibitor Avacopan and the increasing data on induction protocols with reduced glucocorticoid dosage. Due to the inconsistency and variability of the conditions under which blood pressure measurements are carried out in practice, an international consensus statement was issued which defines 4 steps to achieve sufficient validity of the measurement results. CKD-MBD Controversies Conference 2023: The update for CKD-MBD, which was discussed in the Controversies Conference 2023, is in progress and has not been released yet. However, there were no serious contradictions between the 2023 data and the 2017 guidelines - the risk assessment regarding calcium-containing phosphate binders may have been put into perspective.
UNASSIGNED: Das Update 2024 betrifft die Empfehlungen zur Einschätzung und zum generellen Management chronischer Nierenkrankheiten. Das Update wurde um sog. „Clinical Practice Points“ ergänzt, die zwar von der aktuellen Evidenzlage abgeleitet wurden, aber nicht zwingend durch prospektive kontrollierte Studien umfassend belegt sind.
UNASSIGNED: Die wesentliche Änderung des Updates 2024 betrifft die Empfehlungen zur Induktionstherapie (inklusive Belimumab, Calcineurin-Inhibitor) der Lupus-Nephritis-Klassen III und IV. Nach wie vor bestehen hier als Basis die hochdosierte Glukokortikoid-Behandlung sowie der Einsatz von Hydroxychloroquin.
UNASSIGNED: Die beiden neuen Entwicklungen im Update 2024 waren die Studien zum Einsatz des C5a-Rezeptor-Inhibitors Avacopan und die zunehmende Datenlage zu Induktionsprotokollen mit reduzierter Glukokortikoid-Dosierung.
UNASSIGNED: Aufgrund der Uneinheitlichkeit und Variabilität der Bedingungen, unter denen Blutdruckmessungen in der Praxis erfolgen, erfolgte ein Internationales Konsensus-Statement, welches 4 Schritte festlegt, um eine ausreichende Validität der Messergebnisse zu erreichen. In der Quintessenz wird die automatisierte Oberarmmessung unter entspannten Ruhebedingungen empfohlen. CKD-MBD CONTROVERSIES CONFERENCE 2023: Das Update befindet sich in Bearbeitung und wurde noch nicht veröffentlicht. Vorerst ergaben sich aber keine gravierenden Widersprüche zwischen der Datenlage 2023 und den Leitlinien von 2017 – möglicherweise hat sich die Risikoeinschätzung bezüglich kalziumhaltiger Phosphatbinder relativiert.

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OBJECTIVE: This paper presents an analysis of the pregnancy trajectory and therapeutic regimen documentation of a primigravida with APSN. It aims at communicating the therapeutic approach and preventive measures for APSN in pregnancy.
METHODS: This paper reports the trajectory and therapeutic regimen documentation of a primigravida with APSN. The APSN was discovered in a primigravida woman aged 26 years at 11 weeks of gestation. The initial therapy regimen consists of daily administration of prednisone 10 mg, hydroxychloroquine 200 mg, dapparin 5000 IU, and aspirin 50 mg. At a gestational age of 20 + 3 weeks, the dosage of dapparin was modified to 5000 IU/other day, along with a significant rise in urinary protein level seen at 30 + 3 weeks of gestational age. The initial dosage of dapanin sodium was renewed. The patient delivered at 38 + 3 weeks of gestation without other complications.
CONCLUSIONS: It is imperative to acknowledge that altering the dosage and administration of medication should not be done haphazardly during pregnancy.

Many kidney diseases are associated with proteinuria. Since proteinuria is independently associated with kidney function loss, anti-proteinuric medication, often in combination with dietary salt restriction, comprises a major cornerstone in the prevention of progressive kidney failure. Nevertheless, complete remission of proteinuria is very difficult to achieve, and most patients with persistent proteinuria slowly progress toward kidney failure. It is well-recognized that proteinuria leads to kidney inflammation and fibrosis via various mechanisms. Among others, complement activation at the apical side of the proximal tubular epithelial cells is suggested to play a crucial role as a cause of progressive loss of kidney function. However, hitherto limited attention is given to the pathophysiological role of tubular complement activation relative to glomerular complement activation. This review aims to summarize the evidence for tubular epithelial complement activation in proteinuric kidney diseases in relation to loss of kidney function.

Renal fibrosis (RF) is one of the underlying pathological conditions leading to progressive loss of renal function and end-stage renal disease (ESRD). Over the years, various therapeutic approaches have been explored to combat RF and prevent ESRD. Despite significant advances in understanding the underlying molecular mechanism(s), effective therapeutic interventions for RF are limited. Current therapeutic strategies primarily target these underlying mechanisms to halt or reverse fibrotic progression. Inhibition of transforming growth factor-β (TGF-β) signaling, a pivotal mediator of RF has emerged as a central strategy to manage RF. Small molecules, peptides, and monoclonal antibodies that target TGF-β receptors or downstream effectors have demonstrated potential in preclinical models. Modulating the renin-angiotensin system and targeting the endothelin system also provide established approaches for controlling fibrosis-related hemodynamic changes. Complementary to pharmacological strategies, lifestyle modifications, and dietary interventions contribute to holistic management. This comprehensive review aims to summarize the underlying mechanisms of RF and provide an overview of the therapeutic strategies and novel antifibrotic agents that hold promise in its treatment.

Intravascular hemolysis is a central feature of congenital and acquired hemolytic anemias, complement disorders, infectious diseases, and toxemias. Massive and/or chronic hemolysis is followed by the induction of inflammation, very often with severe damage of organs, which enhances the morbidity and mortality of hemolytic diseases. Galectin-3 (Gal-3) is a β-galactoside-binding lectin that modulates the functions of many immune cells, thus affecting inflammatory processes. Gal-3 is also one of the main regulators of fibrosis. The role of Gal-3 in the development of different kidney and liver diseases and the potential of therapeutic Gal-3 inhibition have been demonstrated. Therefore, the objective of this review is to discuss the possible effects of Gal-3 on the process of kidney and liver damage induced by intravascular hemolysis, as well as to shed light on the potential therapeutic targeting of Gal-3 in intravascular hemolysis.

BACKGROUND: Despite accumulating evidence of an association between air pollution and renal disease, studies on the association between long-term exposure to air pollution and renal function are still contradictory. This study aimed to investigate this association in a large population with relatively low exposure and with improved estimation of renal function as well as renal injury biomarkers.
METHODS: We performed a cross-sectional analysis in the middle-aged general population participating in the Swedish CardioPulmonary bioImaging Study (SCAPIS; n = 30 154). Individual 10-year exposure to total and locally emitted fine particulate matter (PM2.5), inhalable particulate matter (PM10), and nitrogen oxides (NOx) were modelled using high-resolution dispersion models. Linear regression models were used to estimate associations between exposures and estimated glomerular filtration rate (eGFR, combined creatinine and cystatin C) and serum levels of renal injury biomarkers (KIM-1, MCP-1, IL-6, IL-18, MMP-2, MMP-7, MMP-9, FGF-23, and uric acid), with consideration of potential confounders.
RESULTS: Median long-term PM2.5 exposure was 6.2 µg/m3. Almost all participants had a normal renal function and median eGFR was 99.2 mL/min/1.73 m2. PM2.5 exposure was associated with 1.3% (95% CI 0.6, 2.0) higher eGFR per 2.03 µg/m3 (interquartile range, IQR). PM2.5 exposure was also associated with elevated serum matrix metalloproteinase 2 (MMP-2) concentration, with 7.2% (95% CI 1.9, 12.8) higher MMP-2 per 2.03 µg/m3. There was a tendency towards an association between PM10 and higher levels of uric acid, but no associations were found with the other biomarkers. Associations with other air pollutants were null or inconsistent.
CONCLUSIONS: In this large general population sample at low exposure levels, we found a surprising association between PM2.5 exposure and a higher renal filtration. It seems unlikely that particle function would improve renal function. However, increased filtration is an early sign of renal injury and may be related to the relatively healthy population at comparatively low exposure levels. Furthermore, PM2.5 exposure was associated with higher serum concentrations of MMP-2, an early indicator of renal and cardiovascular pathology.

Copper is a trace element essential for numerous biological activities, whereas the mitochondria serve as both major sites of intracellular copper utilization and copper reservoir. Here, we investigated the impact of mitochondrial copper overload on the tricarboxylic acid cycle, renal senescence and fibrosis. We found that copper ion levels are significantly elevated in the mitochondria in fibrotic kidney tissues, which are accompanied by reduced pyruvate dehydrogenase (PDH) activity, mitochondrial dysfunction, cellular senescence and renal fibrosis. Conversely, lowering mitochondrial copper levels effectively restore PDH enzyme activity, improve mitochondrial function, mitigate cellular senescence and renal fibrosis. Mechanically, we found that mitochondrial copper could bind directly to lipoylated dihydrolipoamide acetyltransferase (DLAT), the E2 component of the PDH complex, thereby changing the interaction between the subunits of lipoylated DLAT, inducing lipoylated DLAT protein dimerization, and ultimately inhibiting PDH enzyme activity. Collectively, our study indicates that mitochondrial copper overload could inhibit PDH activity, subsequently leading to mitochondrial dysfunction, cellular senescence and renal fibrosis. Reducing mitochondrial copper overload might therefore serve as a strategy to rescue renal fibrosis.