Kimura’s disease (KD) is a rare chronic inflammatory disorder with a high incidence of renal involvement. In this report, we present a case study of KD-associated nephrotic syndrome combined with minimal change disease (MCD) and acute renal tubular injury. Meanwhile, the clinical and histopathological characteristics of 26 patients with KD presenting with renal involvement were retrospectively evaluated. Here, we report a case study of a 59-year-old male patient with KD confirmed by a lymph node biopsy. He developed widespread edema and decreased urine output. A palpable swollen mobile and non-tender lymph node behind the left ear was observed upon admission. A renal biopsy revealed minimal-change lesions and acute renal tubular injury. The patient received hemodialysis because of the oliguria and renal insufficiency, and an initial dose of 40 mg/d methylprednisolone and then continued treatment with 40 mg/d prednisolone. He exhibited a good clinical response to the steroid after 6 weeks of treatment. Of the other 26 patients included in the review, 13 patients presented with mesangial proliferative glomerulonephritis, 4 with membranous nephropathy, 3 with MCD, 3 with focal segmental glomerulosclerosis, 2 with IgA nephropathy and 1 with acute tubular injury. With the exception of 2 patients who progressed to end-stage renal disease and received hemodialysis, the majority of patients responded well to treatment with corticosteroids alone. MCD combined with acute renal tubular injury is rare in patients with KD presenting with renal involvement. Corticosteroids may be a beneficial treatment for renal injury in patients with KD.

Background: Decreased Core I β3-Gal-T-specific molecular chaperone (Cosmc) expression induced IgA1 aberrant glycosylation is the main characteristic of IgA nephropathy (IgAN). This study tried to elucidate the effect of Astragalus membranaceus on Cosmc expression and IgA O-glycosylation of peripheral B lymphocytes in IgAN patients. Methods: Peripheral B lymphocytes of 21 IgAN patients and 10 normal controls were isolated and cultured with or without lipopolysaccharide (LPS) and Astragalus membranaceus injection (AMI). Cosmc mRNA and protein expression levels were measured by real-time RT-PCR and Western blot. IgA1 and glycosylation level were determined by enzyme-linked immunosorbent assay (ELISA) and VV lectin-binding method. Results: Cosmc mRNA expression and IgA1 O-glycosylation level in IgAN patients was significantly lower than normal controls at baseline. Treatment of LPS could obviously inhibit Cosmc expression and increase the IgA1 secretion in peripheral B lymphocytes of IgAN patients, which resulted in a significantly increase in IgA1 aberrant glycosylation level. Addition of AMI could remarkably up regulated Cosmc expression, decrease IgA1 secretion, and reverse glycosylation level in a dose related manner. Conclusion: AMI can up-regulate Cosmc expression of peripheral B lymphocytes and reverse IgA1 aberrant O-glycosylation level, which might be the underlying mechanism of AMI therapy in treating IgAN. Trial registration: TCTR20140515001 (Registration Date: 2014-05-15)

Background: This study aimed to evaluate the efficacy and safety of telitacicept versus mycophenolate mofetil (MMF) in high-risk progressive immunoglobulin A nephropathy (IgAN). Methods: This retrospective, multicentre cohort study included patients with high-risk progressive IgAN who received telitacicept or MMF therapy, both combined with low-dose steroids. Clinical data were collected from treatment initiation to 12 months. Results: A total of 104 patients were included, with 56 receiving MMF and 48 receiving telitacicept. The average age was 36.9 ± 11.8 years. Baseline characteristics were well balanced between groups, except for serum albumin, uric acid and tubular pathology based on the Oxford classification, which showed significant differences. At 12 months, telitacicept plus low-dose steroids demonstrated superior proteinuria reduction (−62.5% versus −52.9%, P = .041) and stabilized renal function (4.1% improvement in estimated glomerular filtration rate versus 5.3% decline with MMF, P = .085). Telitacicept plus low-dose steroids achieved higher complete remission rates (33.3% versus 16.1%; P = .04) and significantly lower non-response rates (29.2% versus 48.2%, P = .048) compared with MMF plus low-dose steroids. Cumulative remission rates (complete + partial) favoured telitacicept at all time points, with the largest difference at 12 months. Notably, telitacicept required substantially lower cumulative glucocorticoid doses (P < .001) and exhibited a superior safety profile, with significantly fewer adverse events (22.9% versus 42.9%, P = .032) and no serious complications reported. Multivariable analysis indicated telitacicept was associated with a higher likelihood of achieving 12-month complete remission [adjusted hazard ratio 6 (95% confidence interval 1.41–25.62). Conclusions: Telitacicept may offer better efficacy compared with MMF for proteinuria reduction in high-risk IgAN patients, while reducing combined glucocorticoid requirements and demonstrating a more favourable safety profile. These advantages position it as a promising therapeutic option, warranting further randomized validation.

Autosomal recessive polycystic kidney disease (ARPKD) is a hereditary fibrocystic disease that primarily involves the kidneys and hepatobiliary tract. The polycystic kidney and hepatic disease 1 (PKHD1) gene is the only gene implicated in ARPKD. The present study aimed to identify PKHD1 mutations causing ARPKD in a Chinese family. A couple that underwent prenatal genetic diagnosis for ARPKD and their families were recruited for the present study. Genomic DNA was collected from the amniotic fluid of the fetus (proband) and from peripheral blood of all other available family members. Targeted exome sequencing was performed on the couple and the proband, followed by direct Sanger sequencing on other family members and normal controls to confirm candidate pathogenic variants. Two novel compound heterozygous mutations in the PKHD1 gene were identified as causative in the proband, including maternally inherited c.2876C>T (p.Ser959Phe) and paternally inherited c.5772C>A (p.Phe1924Leu). Each mutation was found to co‑segregate with the ARPKD phenotype in the family. Other family members either carried one of the two mutations or lacked both mutations, while the mutations were not found in 576 ethnically matched normal controls. Therefore, two novel compound heterozygous PKHD1 mutations were implicated in causing ARPKD in a Han Chinese family. The results expand the mutation spectrum of PKHD1 that leads to ARPKD, which may improve genetic counseling and prenatal diagnosis for families with ARPKD.