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Post-infectious glomerulonephritis (PIGN) is an immune complex mediated glomerular injury occurring because of an infection, most commonly with group A beta-hemolytic streptococcus in children. C3 glomerulopathy (C3G) is a distinct clinicopathological entity occurring secondary to dysregulation of alternate complement pathway encompassing both C3 glomerulonephritis (C3GN) and dense deposit disease (DDD). While most patients with PIGN attain complete remission with normalized complement levels by 6-8 weeks after presentation, patients with C3G continue to have hypocomplementemia with high rates of progressive kidney disease. Here, we report a patient diagnosed with dense deposit disease after his initial presentation with PIGN three years prior. While current literature continues to explore the overlapping and distinguishing features of PIGN and C3G, including how underlying defects in the alternate complement pathway may commonly contribute to both diseases, this case further exemplifies the importance of recognizing the clinico-pathogenic features of PIGN and C3G in pediatric patients with glomerulonephritis., (© 2023. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Objective: The objective of this study was to evaluate renal function and renal parenchymal length changes secondary to the coverage or preservation of accessory renal arteries (ARAs) in complex aortic repair., Methods: This was a single-center retrospective study identifying all patients undergoing fenestrated or branched endovascular aortic repair (f-b EVAR) who presented with ARAs. Two groups were created, a preserved ARA group, with incorporation of the vessel as a dedicated fenestration or branch in the endograft plan, and a non-preserved ARA group, without incorporation of them. Early >30% decline of glomerular filtration rate (GFR), kidney infarcts, and endoleaks were evaluated. Mid-term results with freedom from kidney shrinkage (defined as length decrease >10%) at follow-up, freedom from GFR decrease >30%, or need for postoperative dialysis at follow-up were also analyzed. Primary assisted patency of incorporated ARAs was calculated., Results: From 2011 through 2020, 145 patients undergoing complex aortic repair presented with an ARA. After excluding ruptured aneurysms, 33 patients had the ARA preserved with their incorporation into the stent graft (preserved ARA group), and 99 did not have preservation of them (not-preserved ARA group). There were no statistical differences in demographics or type of aneurysm. Patients in the ARA-preserved group had more ARAs (median of two per patient vs one in the non-preserved ARA group; P = .01) and bigger ARAs (median 4 vs 3 mm in the non-preserved ARA group; P = .001). Early postoperative worsening >30% of GFR (23% vs 6%; P = .03) as well as postoperative renal infarction (57% vs 6%; P = .001) and ARA-related endoleaks (20% vs 0%; P = .01) were statistically higher for the not-preserved ARA group. Mid-term kidney length showed significant shrinkage in the not-preserved ARA group compared with the ARA preserved group (9.7% vs 0%; P = .001). Freedom from >30% GFR decline at 2 years was significantly higher for the preserved ARA group (83% vs 47%; P = .01).Two-year primary assisted patency of incorporated ARA was 94%., Conclusions: Complex aortic repair incorporation of ARA is feasible, with low complications and good primary assisted patency at 2 years. It leads to less postoperative early renal dysfunction as well as higher freedom for mid-term renal disfunction and kidney shrinkage., (Copyright © 2022 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Morphological changes that occur during kidney injury involve actin skeleton remodeling. Here we tested whether heat-shock protein 27 (HSP27), a small stress response protein involved in cytoskeletal remodeling, protects the kidney from tubulointerstitial fibrosis in obstructive nephropathy. Tubular cell HSP27 immunostaining was significantly increased in human kidneys with ureteropelvic junction obstruction, supporting the clinical relevance of our studies. To develop an animal model for mechanistic studies, we generated transgenic mice that specifically overexpress human HSP27 in renal tubules, under the kidney androgen-regulated protein promoter, and determined the effects of HSP27 overexpression on epithelial-to-mesenchymal transition and tubulointerstitial fibrosis following unilateral ureteral obstruction. This was associated with decreased fibrogenesis as evidenced by significant declines in phosphorylated p38MAPK, collagen III, α-smooth muscle actin, 4-hydroxynonenal, and reduced trichrome staining following obstruction. Notably, E-cadherin and β-catenin remained at the cell membrane of tubular cells in transgenic mice with an obstructed ureter. Monocyte/macrophage infiltration, however, was not significantly affected in these transgenic mice. Thus, tubular HSP27 inhibits fibrogenesis in obstructive nephropathy. Further studies are needed to determine pathways regulating the interactions between HSP27 and the E-cadherin-β-catenin complex.

Bile cast nephropathy (BCN) is seen in patients who have acute kidney injury and severe hyperbilirubinemia due to a wide range of hepatobiliary system diseases. Findings seen by renal biopsy include acute tubular injury with necrotic and sloughed epithelial cells, yellow-green pigment within tubular epithelial cells, and pigmented granular casts. Hall's special stain for bile turns these casts green. In recent years, BCN has been described in a small number of case reports and clinical studies primarily in the setting of severe liver dysfunction. We present 2 diverse cases of BCN. The first involves an adult with hepatorenal syndrome secondary to alcoholic steatohepatitis and early cirrhosis. Second, we describe the first reported case of BCN in a child with fulminant hepatic failure due to Wilson's disease. Our cases expand the spectrum of causative diseases, and they provide further evidence that BCN is a distinct pathologic entity which may be found in both adult and pediatric patients with a variety of severe liver diseases. [ABSTRACT FROM AUTHOR]

Tubulointerstitial nephritis in patients with inflammatory bowel disease has been linked to the use of 5-ASA derivatives. Various aspects of this theory have been challenged with a potential role for the underlying autoimmune disorder. Steroids are the mainstay of treatment and mycophenolate mofetil might be an effective alternative. We report a patient who responded well to mycophenolate despite continuing mesalamine, the suspected offending agent. [ABSTRACT FROM AUTHOR]