Since the 1960s, glucocorticoids have been central to induction and maintenance immunosuppression in kidney transplantation, but their long-term use is associated with significant toxicity, including post-transplant diabetes mellitus, hypertension, dyslipidemia, osteoporosis, infection, and cardiovascular disease. Advances in immunosuppressive regimens have enabled many transplant centers to reduce or eliminate steroids in selected recipients. Emerging monitoring tools, such as donor-specific antibody surveillance, donor-derived cell-free DNA, and gene-expression profiling, support individualized approaches, particularly in lower-risk patients. This review examines the rationale, evidence, and implementation strategies for steroid minimization and avoidance in kidney transplantation, highlighting current practice patterns, patient selection considerations, ongoing controversies, and future directions toward appropriate use of steroid-sparing regimens as part of personalized immunosuppression.

COL4A1 variants cause a spectrum of systemic disorders encompassed by Gould syndrome. While kidney involvement is recognized, its long-term progression and detailed kidney pathology remain uncharacterized. We present a 39-year-old man with a heterozygous p.G1239R COL4A1 variant, persistent microscopic hematuria, and a progressive eGFR decline of -1.2 mL/min/1.73 m[2] per year over a 10-year follow-up. Light microscopy showed only focal global glomerulosclerosis and mild tubular dilatation. Immunofluorescence for the α1 chain of type IV collagen (α1[IV]) revealed reduced signal intensity in the tubular basement membrane (TBM). Electron microscopy demonstrated focal thinning of the glomerular basement membrane (GBM), and heterogeneous abnormalities including thinning, focal thickening, reticulation, and lamellation of TBM. Immunoelectron microscopy using FluoroNanogold showed decreased density of gold particles in the TBM, correlating with the fluorescence intensity. This report analyzes COL4A1 expression using correlative light and electron microscopy (CLEM), and describes the long-term kidney function decline. In patients with hematuria and a thin GBM, in addition to Alport syndrome, COL4A1-associated nephropathy should also be considered. Recognizing this entity is essential for accurate systemic risk assessment and genetic counseling. Semi-quantitative assessment of COL4A1 expression in biopsy specimens using CLEM may be a key diagnostic tool for patients with heterozygous COL4A1 variants.

A certain proportion of patients with Mendelian diseases are overlooked, although substantial technical advances in molecular genetics have been achieved. Massively parallel sequencing (MPS) increasingly identifies genetic variants of unknown significance, which may remain clinically unhelpful. Furthermore, difficult niches in the genome exist, which cannot be solved by standard MPS. In the reported family autosomal dominant kidney disease leading to renal failure in middle adulthood runs through the maternal and paternal family. Comprehensive genetic analyses and customized functional evaluation solved the genetic etiologies: autosomal dominant polycystic kidney disease (ADPKD-PKD1, maternal) and autosomal dominant tubulointerstitial kidney disease (ADTKD-UMOD, paternal), with the index patient suffering from both diseases. The pathogenic variant in PKD1 (c.2180T>C, p.(Leu727Pro)) was not identified by exome sequencing (ES) but was unveiled by traditional long-range amplification protocols and whole genome sequencing. The ease of use of MPS increasingly tempts non-specialized genetic laboratories to perform broad analyses of numerous diseases, which in specific cases may worsen the quality of investigations, i.e. for the relatively frequent ADPKD.

Chronic kidney disease (CKD) and kidney failure are associated with disruptions in the hypothalamic-pituitary-gonadal axis leading to alterations in sex-hormone homeostasis, sexual dysfunction, and impaired fertility in women and men. The incidence of CKD among younger adults has increased over the past few decades, with up to 6% of women of childbearing age affected by CKD. Women with CKD have a higher risk of adverse pregnancy outcomes, yet reproductive counseling remains underemphasized in nephrology. In addition, sexual dysfunction and impaired fertility are highly prevalent in men with CKD but often go unaddressed due to underreporting or lack of recognition of their association with CKD. This core curriculum reviews the epidemiology, pathophysiology, and management of reproductive health in both women and men across the spectrum of CKD, dialysis, and kidney transplantation. Embedding nuanced, stage-specific reproductive counseling into routine nephrology care is essential to reduce adverse maternal, fetal, and kidney outcomes and to address persistent knowledge gaps.

The Kidney Disease Outcomes Quality Initiative (KDOQI) convened a work group to review the KDIGO 2024 Clinical Practice Guideline for the Management of Lupus Nephritis (LN). The management of LN has become increasingly individualized, and treatment options are expanding; for example, since the release of the KDIGO guideline, the US Food and Drug Administration has approved obinutuzumab in addition to belimumab and voclosporin for the treatment of LN, and the enrollment of patients in chimeric antigen receptor (CAR) T-cell therapy clinical trials is changing the treatment landscape for autoimmune diseases including systemic lupus erythematosus. The use of combination therapy and the trend toward lower doses of corticosteroids for LN management are also part of this transformation. The KDOQI work group reviewed the KDIGO guideline statements and practice points and provides perspectives for implementation within the context of clinical practice in the United States. This commentary is the product of the KDOQI work group and presents the recommendations and practice points from the KDIGO guideline, followed by commentary and brief notes on clinical utility, implementation, and challenges.

Lower urinary tract symptoms and disorders are commonly encountered by nephrologists, as they often coexist with or may contribute to various kidney diseases. Thus, an understanding of lower urinary tract disorders and when consultation with urology is warranted is essential to nephrology practice. In this review, we examine lower urinary tract physiology and outline a practical, clinically oriented approach to the initial work-up for lower urinary tract symptoms, including history, physical examination and office-based testing. Next, we discuss when referral to urology is indicated in patients with lower urinary tract symptoms, with particular emphasis on the identification of unsafe bladders and the process of urodynamics testing. We also summarize novel methods for evaluating the lower urinary tract which may provide non-invasive alternatives to urodynamics testing in the future. Third, we discuss common lower urinary tract disorders, highlighting key diagnostic considerations and management principles as relevant to nephrologists. Finally, disorders which warrant special attention by the nephrologist in patients with chronic kidney disease, end-stage renal disease and kidney transplantation will be explained.