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Objectives: Currently, there is limited data regarding the outcomes of lupus nephritis (LN) with moderate to severe renal failure at presentation (defined by low glomerular filtration rate; GFR <30 mL/min). Methods: Sixty‐four patients with biopsy‐proven LN and estimated GFR (eGFR) <30 mL/min were prospectively analyzed. Outcome measure of persistently low eGFR, end‐stage renal disease (ESRD) or death at 365 days were grouped as Major Adverse Kidney Events (MAKE365). Results: Diagnosis of lupus was simultaneous with onset of renal disease in 60% of cases. Histologically, 82.3% (n = 51) were class IV, the median serum creatinine was 4 mg/dL (interquartile range [IQR] 3.1‐5.9 mg/dL), median eGFR was 13.75 mL/min (IQR 9.25‐19 mL/min) and 42.2% (n = 27) required dialysis at presentation. Induction regimens included National Institute of Health (68.2%), Eurolupus protocol (10.9%) and mycophenolate mofetil (8%). Over 365 days, 23 (37.5%) subjects died, while 41 (62.5%) survived. The majority of deaths were due to infection and sepsis (14/23). Among the survivors, 70.7% had good renal outcome, 12.1% had persistently low GFR (<30 mL/min), while 17% developed ESRD. In this group, treatment response rate was 84.6% (complete response 25.6%, partial response 59%). Those with a better renal function at presentation had a good treatment response (100% vs. 40%). Altogether, n = 35 (54.6%) were included in the MAKE365 category. Between the renal survival group (n = 29) versus the MAKE365 group (n = 35) there was no difference in clinical or histological parameters. Conclusion: The current treatment protocols had a good response rate in patients with LN even with severe kidney injury at presentation. However, the risk of serious infections and subsequent mortality was high. [ABSTRACT FROM AUTHOR]

Alport syndrome (AS) is an inherited disorder of basement membranes caused by mutations affecting specific proteins of the type IV collagen family, presenting with nephropathy and extrarenal manifestations such as sensorineural deafness and ocular anomalies. Ten percentage to 15% of the patients with AS have autosomal recessive (ARAS) due to mutation in either COL4A3 or COL4A4 gene. We report a novel mutation in the COL4A3 gene in an Indian family with ARAS. The above‐mentioned genetic anomaly was a missense variation in exon 26 of the COL4A3 gene (chr2:228137797G>A; c.1891G>A) that resulted in the amino acid substitution of Arginine for Glycine at codon 631 (p.Gly631Arg) that was present in the heterozygous state in the asymptomatic parents and homozygous state in the male offspring who presented with early‐onset end‐stage renal disease, lenticonus and hearing loss. The patient (male offspring) underwent successful renal transplantation with his mother as a donor. SUMMARY AT A GLANCE: A novel mutation in the COL4A3 gene in an Indian family with autosomal recessive Alport syndrome is described. The index case underwent a successful renal transplantation with his mother (who possessed the associated missense mutation) as the donor. [ABSTRACT FROM AUTHOR]

Objective: End points currently used in lupus nephritis (LN) clinical trials lack uniformity and questionably reflect long‐term kidney survival. This study was undertaken to identify short‐term end points that predict long‐term kidney outcomes for use in clinical trials. Methods: A database of 944 patients with LN was assembled from 3 clinical trials and 12 longitudinal cohorts. Variables from the first 12 months of treatment after diagnosis of active LN (prediction period) were assessed as potential predictors of long‐term outcomes in a 36‐month follow‐up period. The long‐term outcomes examined were new or progressive chronic kidney disease (CKD), severe kidney injury (SKI), and the need for permanent renal replacement therapy (RRT). To predict the risk for each outcome, hazard index tools (HITs) were derived using multivariable analysis with Cox proportional hazards regression. Results: Among 550 eligible subjects, 54 CKD, 55 SKI, and 22 RRT events occurred. Variables in the final CKD HIT were prediction‐period CKD status, 12‐month proteinuria, and 12‐month serum creatinine level. The SKI HIT variables included prediction‐period CKD status, International Society of Nephrology (ISN)/Renal Pathology Society (RPS) class, 12‐month proteinuria, 12‐month serum creatinine level, race, and an interaction between ISN/RPS class and 12‐month proteinuria. The RRT HIT included age at diagnosis, 12‐month proteinuria, and 12‐month serum creatinine level. Each HIT validated well internally (c‐indices 0.84–0.92) and in an independent LN cohort (c‐indices 0.89–0.92). Conclusion: HITs, derived from short‐term kidney responses to treatment, correlate with long‐term kidney outcomes, and now must be validated as surrogate end points for LN clinical trials. [ABSTRACT FROM AUTHOR]

Whether or not completing the hepatitis B vaccination in patients who have undergone kidney transplantation in the middle of incomplete vaccination schedule leads to development of protective antibody titres is not known. This study was designed to determine whether the strategy of completing hepatitis B virus (HBV) vaccination after transplantation is efficacious. Sixty-four end-stage renal disease (ESRD) patients were screened for hepatitis B surface antigen (HBsAg), antibodies to hepatitis B surface antigen (anti-HBs), hepatitis B e-antigen (HBeAg) and HBV DNA. HBsAg negative patients received four doses of 40 µg recombinant HBV vaccine. Schedule was continued in after transplantation period if it was incomplete before transplant. Anti-Hbs titres were evaluated at 1, 3, 6, 9 and 12 months. Past HBV infection was noted in 12 patients: 10 by serology plus viraemia and two by viraemia alone. Of the 46 patients without current or past HBV infection who had received at least two doses of the vaccine before transplant, 17 each had received two and three doses and 12 had completed the schedule. Seventeen (37%) exhibited protective titres. Patients who had completed vaccination were more likely to have protective titres than those incompletely vaccinated ( P = 0.02). Five patients responded to post-transplant vaccination. Partially vaccinated patients do not mount an adequate antibody response despite continued vaccination in the post-transplant period, whereas complete vaccination provides protection in 60%. The present study data highlights the need of administration of a full schedule of HBV vaccination before kidney transplantation. Nucleic acid-based tests can identify occult HBV infection. Patients partially vaccinated against HBV do not mount an adequate antibody response despite continued vaccination in the post-transplant period. The data presented here highlights the need for administration of a full schedule of vaccination before kidney transplantation for maximal protection against HBV infection. [ABSTRACT FROM AUTHOR]