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Rosai‐Dorfman disease (RDD) is a rare, idiopathic histiocytic proliferative disorder. We report two cases of RDD related to the breast which showed common distinctive imaging characteristics which can help facilitate accurate diagnosis and appropriate management. [ABSTRACT FROM AUTHOR]

Of note, HNF1A and HNF1B are associated with Fanconi syndrome and kidney cystic diseases in humans.4,5 Gene set enrichment analysis (GSEA) of Hnf4 target genes (Table S3) corroborated the motif enrichment analysis, as it suggested that Hnf4 target genes were upregulated in PG rats (Figure 3E). The PG rat tubules were dilated compared to WT, thus accounting for the discrepant diameters of the tubules between the WT and PG rats. No histological kidney abnormalities were noted in WT and PS rats, while the PG rats had minimal to moderate histological changes (n = 3 for WT and PS rats, n = 6 for PG rats). Dear editor, We identified angiomotin ( I AMOT i ) as a novel candidate gene for X-linked recessive nephropathy associated with glomerular disease, tubulopathy and progressive kidney cystic disease. [Extracted from the article]

Aim. To characterise infiltrating T cells in kidneys and circulating lymphocyte subsets of adult patients with primary/idiopathic minimal change disease. Methods. In a cohort of 9 adult patients with primary/idiopathic minimal change recruited consecutively at disease onset, we characterized (1) infiltrating immune cells in the kidneys using immunohistochemistry and (2) circulating lymphocyte subsets using flow cytometry. As an exploratory analysis, association of the numbers and percentages of both kidney-infiltrating immune cells and the circulating lymphocyte subsets with kidney outcomes including deterioration of kidney function and proteinuria, as well as time to complete clinical remission up to 48 months of follow-up, was investigated. Results. In the recruited patients with primary/idiopathic minimal change disease, we observed (a) a dominance of infiltrating T helper 17 cells and cytotoxic cells, comprising cytotoxic T cells and natural killer cells, over Foxp3+ Treg cells in the renal interstitium; (b) an increase in the circulating total CD8+ T cells in peripheral blood; and (c) an association of some of these parameters with kidney function and proteinuria. Conclusions. In primary/idiopathic minimal change disease, a relative numerical dominance of effector over regulatory T cells can be observed in kidney tissue and peripheral blood. However, larger confirmatory studies are necessary. [ABSTRACT FROM AUTHOR]

Minimal change disease constitutes a major cause of nephrotic syndrome. It is regarded as a non-immune-complex mediated primary glomerulopathy and pathogenetically is characterised by podocyte injury and effacement of foot processes; therefore, it is also classified as a type of podocytopathy. T cell dysfunction with increased levels of a soluble glomerular permeability factor has been proposed to play a major role in the pathogenesis of minimal change disease. It has been therefore suggested that a dysfunction of regulatory T cells, the orchestrators of immune homeostasis, could be implicated in perpetuating T cell activation in this condition. However, the actual contribution of regulatory T cell dysfunction in the immunopathogenesis of primary minimal change disease is still largely unclear. We here propose a theoretical model based on the available evidence. [ABSTRACT FROM AUTHOR]

Minimal change disease constitutes a major cause of nephrotic syndrome. It is regarded as a non-immune-complex mediated primary glomerulopathy and pathogenetically is characterised by podocyte injury and effacement of foot processes; therefore, it is also classified as a type of podocytopathy. T cell dysfunction with increased levels of a soluble glomerular permeability factor has been proposed to play a major role in the pathogenesis of minimal change disease. It has been therefore suggested that a dysfunction of regulatory T cells, the orchestrators of immune homeostasis, could be implicated in perpetuating T cell activation in this condition. However, the actual contribution of regulatory T cell dysfunction in the immunopathogenesis of primary minimal change disease is still largely unclear. We here propose a theoretical model based on the available evidence. [ABSTRACT FROM AUTHOR]

Minimal change disease constitutes a major cause of nephrotic syndrome. It is regarded as a non-immune-complex mediated primary glomerulopathy and pathogenetically is characterised by podocyte injury and effacement of foot processes; therefore, it is also classified as a type of podocytopathy. T cell dysfunction with increased levels of a soluble glomerular permeability factor has been proposed to play a major role in the pathogenesis of minimal change disease. It has been therefore suggested that a dysfunction of regulatory T cells, the orchestrators of immune homeostasis, could be implicated in perpetuating T cell activation in this condition. However, the actual contribution of regulatory T cell dysfunction in the immunopathogenesis of primary minimal change disease is still largely unclear. We here propose a theoretical model based on the available evidence. [ABSTRACT FROM AUTHOR]

Background BK virus-induced nephropathy in renal transplantation is well recognized but its oncogenic potential is less appreciated. Methods We report a case of high-grade urothelial tumor in a renal transplant recipient with chronic BK viremia in the absence of BK nephropathy successfully treated with intravesical Bacillus Calmette-Guerin (BCG) instillation. Following BCG therapy, there was also spontaneous clearance of BK viremia. Results In July 2011, Mr. LYY, a 57-year-old Chinese man with end-stage renal failure secondary to chronic glomerulonephritis underwent uncomplicated deceased donor renal transplantation. One year later, he developed persistent high-level BK viremia despite reduction in immunosuppression. He was also treated with a course of ciprofloxacin from January to October 2013, and intravenous immunoglobulin (IVIG) in February 2013, but high-level BK viremia persisted. In spite of this, his graft function was preserved. He was subsequently placed on BK surveillance alone. Chronic BK viremia persisted for the next 5 years with stable graft function. See Figure 1. In February 2018, an incidental urinary bladder nodule was picked up on computed tomography (CT) scan performed for evaluation of urosepsis. Follow-up flexible cystoscopy in May 2018 showed a 1.5 cm papillary tumor over the left superior bladder wall. Transurethral resection of bladder tumor (TURBT) was performed and histopathological examination revealed high-grade papillary urothelial carcinoma. In addition, the biopsy specimen was stained positive for SV40, suggesting the possible association between chronic BK virus replication in the bladder and oncogenesis. Relook TURBT in July 2018 revealed a persistent erythematous patch over the posterior bladder wall. Given the cystoscopic findings, Mr. LYY received a weekly instillation of intravesical BCG for 6 weeks. He tolerated the intravesical BCG treatment with no local or systemic complications. Interestingly, his serum BK viral loads dropped below lower limits of detection thereafter and continued to remain so for the next 4 months. Conclusion We postulate that the intravesical BCG therapy triggered an immune response that targeted not only the tumor but also the BK virus infection, resulting in successful clearance of chronic BK viremia. Disclosures All authors: No reported disclosures. [ABSTRACT FROM AUTHOR]