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Pediatr Transplant 2022 May 19:e14307. Epub 2022 May 19.

University of Wisconsin Madison, Madison, Wisconsin, USA.

Background: FSGS is a common indication for kidney transplant with a high-risk of posttransplant recurrence.

Methods: In this review, we summarize current knowledge about FSGS recurrence after kidney transplantation, including epidemiology, pretransplant planning, posttransplant management, and investigational treatments.

Results: FSGS recurs in 14%-60% of first transplants, likely associated with a circulating permeability factor. Read More

J Am Soc Nephrol 2022 May 17. Epub 2022 May 17.

Department of Pathology, Division of AI and Computational Pathology, and Department of Medicine, Division of Nephrology, Duke University, Durham, North Carolina

Background: Heterogeneity in disease course and treatment response among patients with MCD/FSGS necessitates a granular evaluation of kidney tissue features. This study aimed to identify histologic and ultrastructural descriptors of structural changes most predictive of clinical outcomes in the Nephrotic Syndrome Study Network (NEPTUNE).

Methods: Forty-eight histologic (37 glomerular, 9 tubulointerstitial, 2 vascular) and 20 ultrastructural descriptors were quantified by applying the NEPTUNE Digital Pathology Scoring System to NEPTUNE kidney biopsies. Read More

J Bras Nefrol 2022 May 13. Epub 2022 May 13.

Centro Hospitalar de Lisboa Central, Hospital Curry Cabral, Departamento de Nefrologia, Lisboa, Portugal.

Background: Kidney disease is a rare manifestation of ankylosing spondylitis (AS) and its pathological alterations remain poorly described. The aim of this study was to investigate the clinical presentation and pathological alterations on kidney biopsy of AS patients and review and discuss the current literature on the issue.

Methods: We retrospectively studied the clinical presentation and kidney pathological alterations of 15 Caucasian AS patients submitted to kidney biopsy between October 1985 and March 2021. Read More

Clin Nephrol Case Stud 2022 10;10:37-41. Epub 2022 May 10.

Department of Pediatrics West Virginia University School of Medicine, Children's Hospital Morgantown, WV, and.

Introduction: The medical literature is scant with reports of twins diagnosed with nephrotic syndrome associated with genetic mutations. Mutations in the protein coding paired box gene 2 () and in the non-muscle class I myosin, myosin 1E, () have been implicated in the development of steroid-resistant nephrotic syndrome. We describe the first case, to our knowledge, of the concordant presentation of nephrotic syndrome in twins with simultaneous mutations in and . Read More

Exp Clin Transplant 2022 May;20(Suppl 3):129-136

From the Department of Pediatrics, Gazi University, Ankara, Turkey.

Objectives: Neutrophil-to-lymphocyte ratio and platelet (thrombocyte)-to-lymphocyte ratio have become accepted markers of inflammation in recent years and are used to assess disease activity in some diseases. In this study, we investigated the relationship between these values and acute rejection attacks, as well as their role in determining chronic allograft nephropathy, in follow-up of pediatric kidney transplant recipients.

Materials And Methods: Our study included 58 kidney transplant recipients (age 5-18 years) with at least 5-year follow-up at our center. Read More

Intern Med 2022 May 14. Epub 2022 May 14.

Nephrology Center, Toranomon Hospital Kajigaya, Japan.

We experienced a 77-year-old Japanese man who presented with nephrotic-range proteinuria 20 days after receiving ramucirumab treatment for metastatic sigmoid colon cancer. A kidney biopsy showed two characteristic histological findings. The first finding was podocyte injury with cellular crescent-like formation, although focal segmental glomerulosclerosis (FSGS) (collapsing variant) according to the Colombia classification may have been a more appropriate name for this injury, as hypertrophy and hyperplasia of epithelial cells, presumably resulting from podocyte injury, were seen between Bowman's capsule and the glomerular basement membrane (GBM); these changes appeared to be due to the collapse of the GBM rather than to GBM destruction with fibrinoid necrosis. Read More

J Investig Med High Impact Case Rep 2022 Jan-Dec;10:23247096221097522

Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY, USA.

Sarcoidosis is well known for heterogeneity of its presentation and multisystem organ involvement. It commonly involves respiratory tract, skin, eyes, and lymph nodes, as well as hematologic and renal systems. While anemia and lymphopenia are the most common hematologic abnormalities seen in sarcoidosis, immune thrombocytopenic purpura (ITP) is considered rare. Read More

Pediatr Nephrol 2022 May 13. Epub 2022 May 13.

Department of Pediatrics, Division of Pediatric Nephrology, University of California at San Diego and Rady Children's Hospital, San Diego, CA, USA.

BMC Nephrol 2022 May 12;23(1):182. Epub 2022 May 12.

Nephrology Department, Heraklion University Hospital, Voutes, 71500, Heraklion, Crete, Greece.

Background: Dent disease is an X-linked disorder characterized by low molecular weight proteinuria (LMWP), hypercalciuria, nephrolithiasis and chronic kidney disease (CKD). It is caused by mutations in the chloride voltage-gated channel 5 (CLCN5) gene (Dent disease-1), or in the OCRL gene (Dent disease-2). It is associated with chronic metabolic acidosis; however metabolic alkalosis has rarely been reported. Read More

Ren Fail 2022 Dec;44(1):826-830

Department of Dermato-Venereology, The Second Hospital of Shandong University, Jinan, China.

J Clin Apher 2022 May 9. Epub 2022 May 9.

Division of Nephrology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Background: Primary focal segmental glomerulosclerosis (FSGS) treatment is based on immunosuppressive therapies. Since refractory disease is common, alternative methods are emerging. One of these methods is plasmapheresis with intravenous cyclosporine and corticosteroids, and it could be an option in post-transplant recurrent FSGS. Read More

Kidney Dis (Basel) 2022 Mar 24;8(2):148-159. Epub 2022 Jan 24.

Department of Paediatrics, Region Västra Götaland, Sahlgrenska University Hospital, Gothenburg, Sweden.

Aims: This study aimed to investigate associations between renal and extrarenal manifestations of mitochondrial diseases and their natural history as well as predictors of renal disease severity and overall disease outcome. The secondary aim was to generate a protocol of presymptomatic assessment and monitoring of renal function in patients with a defined mitochondrial disease.

Methods: A multicenter, retrospective cohort study was performed by the Mitochondrial Clinical and Research Network (MCRN). Read More

Kidney Med 2022 May 24;4(5):100457. Epub 2022 Mar 24.

NYU Grossman School of Medicine, NYU Langone Health, New York, NY.

Rationale And Objective: Angiotensin-converting enzyme inhibitor or angiotensin receptor blocker therapy (renin-angiotensin-aldosterone system [RAAS] inhibitor) to control proteinuria in primary and genetic focal segmental glomerulosclerosis (FSGS) follows guidelines based on other proteinuria-related kidney diseases. There is no consensus on the efficacy and safety of RAAS inhibitor therapies in primary and genetic FSGS. This systematic review assessed the effects of RAAS inhibitor therapy on kidney outcomes in these patients. Read More

Mol Ther 2022 May 5. Epub 2022 May 5.

The Key Laboratory of Infection and Immunity of Shandong Province, Department of Pharmacology, School of Basic Medical Sciences, Shandong University, Jinan, Shandong 250012, China. Electronic address:

Although tissue-resident-memory T (T) cells, a recently identified non-circulating memory T cell population, play a crucial role in mediating local immune responses and protect against pathogens upon local reinfection, the composition, effector function, and specificity of T cells in the kidney and their relevance for chronic kidney disease remain unknown. In this study, we found that renal tissue displayed high abundance of tissue-resident lymphocytes, and the proportion of CD8 T cells was significantly increased in the kidney from patients and mice with focal segmental glomerulosclerosis (FSGS), diabetic kidney disease (DKD), and lupus nephritis (LN). Mechanistically, IL-15 significantly promoted CD8 T cell formation and activation, thereby promoting podocyte injury and glomerulosclerosis. Read More

Kidney Int 2022 May 2. Epub 2022 May 2.

Institute of Translational Medicine, Semmelweis University, Budapest, Hungary; International Nephrology Research and Training Center, Semmelweis University, Budapest.

Patients with chronic kidney disease and experimental animal models of kidney fibrosis manifest diverse progression rates. Genetic susceptibility may contribute to this diversity, but the causes remain largely unknown. We have previously described kidney fibrosis with a mild or severe phenotype in mice expressing transforming growth factor-beta1 (TGF-β1) under the control of a mouse albumin promoter (Alb/TGF-β1), on a mixed genetic background with CBAxC57Bl6 mice. Read More

Pediatr Nephrol 2022 May 4. Epub 2022 May 4.

Division of Nephrology, Department of Pediatrics, University of Florida School of Medicine, Gainesville, FL, 32610, USA.

Background: Primary FSGS manifests with nephrotic syndrome and may recur following KT. Failure to respond to conventional therapy after recurrence results in poor outcomes. Evaluation of podocyte B7-1 expression and treatment with abatacept (a B7-1 antagonist) has shown promise but remains controversial. Read More

Clin Sci (Lond) 2022 May;136(10):715-731

Department of Medicine, Université de Montréal, Centre de recherche du Centre hospitalier de l'Université de Montréal (CRCHUM), 900 Saint Denis Street, Montréal, QC, Canada H2X 0A9.

Podocyte damage and loss are the early event in the development of focal segmental glomerulosclerosis (FSGS). Podocytes express angiotensin II type-2-receptor (AT2R), which may play a key role in maintaining kidney integrity and function. Here, we examined the effects of AT2R deletion and AT2R agonist compound 21 (C21) on the evolution of FSGS. Read More

J Clin Invest 2022 May;132(9)

III. Department of Medicine and.

The glomerular filtration barrier (GFB) produces primary urine and is composed of a fenestrated endothelium, a glomerular basement membrane (GBM), podocytes, and a slit diaphragm. Impairment of the GFB leads to albuminuria and microhematuria. The GBM is generated via secreted proteins from both endothelial cells and podocytes and is supposed to majorly contribute to filtration selectivity. Read More

J Cell Mol Med 2022 Apr 29. Epub 2022 Apr 29.

Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.

Transcriptome profiling of tubulointerstitial tissue in glomerulonephritis may reveal a potential tubulointerstitial injury-related biomarker. We profiled manually microdissected tubulointerstitial tissue from biopsy cores of 65 glomerulonephritis cases, including 43 patients with IgA nephropathy, 3 with diabetes mellitus nephropathy, 3 with focal segmental glomerulosclerosis, 3 with lupus nephritis, 4 with membranous nephropathy and 9 with minimal change disease, and additional 22 nephrectomy controls by RNA sequencing. A potential biomarker was selected based on the false discovery rate, and experiments were performed in TNF-α-stimulated primary cultured human tubular epithelial cells (hTECs). Read More

Pediatr Nephrol 2022 Apr 29. Epub 2022 Apr 29.

Division of Nephrology, The Children's Hospital of Philadelphia, Philadelphia, USA.

Kidney Int 2022 Apr 25. Epub 2022 Apr 25.

Department of Pathology, School of Basic Medical Sciences, Fudan University, Shanghai, China. Electronic address:

Ribosomal protein S6 (rpS6) phosphorylation mediates the hypertrophic growth of kidney proximal tubule cells. However, the role of rpS6 phosphorylation in podocyte hypertrophy and podocyte loss during the pathogenesis of focal segmental glomerulosclerosis (FSGS) remains undefined. Here, we examined rpS6 phosphorylation levels in kidney biopsy specimens from patients with FSGS and in podocytes from mouse kidneys with Adriamycin-induced FSGS. Read More

Am J Transplant 2022 Apr 26. Epub 2022 Apr 26.

Department of Pediatric Nephrology, Tokyo Women's Medical University, Tokyo, Japan.

Pediatr Rep 2022 Apr 3;14(2):166-169. Epub 2022 Apr 3.

Department of Pediatrics and Adolescent Medicine, Tokyo Medical University, Tokyo 160-0023, Japan.

In Japan, the prevalence of low birth weight (LBW) has been estimated to be approximately 10%, which is the highest among developed countries. This high prevalence might affect the occurrence of LBW-associated diseases in the adult population of Japan. LBW has been recognized as a contributing factor to post-adaptive focal segmental glomerulosclerosis (FSGS) in adulthood; however, few reports to date have evaluated the clinical and pathological characteristics of post-adaptive FSGS. Read More

Can J Kidney Health Dis 2022 19;9:20543581221090010. Epub 2022 Apr 19.

BC Provincial Renal Agency, Vancouver, Canada.

Background: Adults with primary focal segmental glomerulosclerosis (FSGS) are frequently resistant to multiple immunosuppressive agents, which is associated with ongoing complications of nephrotic syndrome and a high risk of progression to end-stage renal disease (ESRD). Rituximab, a monoclonal antibody against the B cell CD20 antigen, has shown some preliminary evidence in treating nephrotic syndrome.

Objectives: Describe the usage and the efficacy of rituximab for adults with FSGS in British Columbia (BC) (Canada) and perform a literature review of multi-immunosuppressive drug resistant FSGS in adult patients treated with rituximab to estimate the overall response rate. Read More

Kidney Int 2022 05;101(5):880-882

Department of Pathology and Immunology, Washington University School of Medicine in St. Louis, St. Louis, Missouri, USA; Department of Internal Medicine, Washington University School of Medicine in St. Louis, St. Louis, Missouri, USA. Electronic address:

Collapsing glomerulopathy frequently shows focal lesions on biopsy, creating challenges with transcriptomic investigations because of inadequate tissue sample. This challenge is overcome with spatial transcriptomics, technology linking transcriptomic data to histology. Applying this technology to investigate patients with collapsing glomerulopathy related to HIV infection or severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Smith et al. Read More

Can J Kidney Health Dis 2022 8;9:20543581221089094. Epub 2022 Apr 8.

Division of Nephrology, Department of Medicine, University Health Network, University of Toronto, ON, Canada.

Background: Glomerulonephritis (GN) is a leading cause of kidney failure and accounts for 20% of incident cases of end-stage kidney disease (ESKD) in Canada annually. Reversal of kidney injury and prevention of progression to kidney failure is possible; however, limited knowledge of underlying disease mechanisms and lack of noninvasive biomarkers and therapeutic targets are major barriers to successful therapeutic intervention. Multicenter approaches that link longitudinal clinical and outcomes data with serial biologic specimen collection would help bridge this gap. Read More

Front Mol Biosci 2022 4;9:871615. Epub 2022 Apr 4.

Department of Nephrology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.

Aberrant O-glycosylation of IgA1 plays an important role in IgA nephropathy pathogenesis. Previous proteomic studies analyzed O-glycans of the circulating IgA1 hinge region and found that the N-acetylgalactosamine (GalNAc) and galactose numbers in the hinge region of IgA1 of patients with IgA nephropathy were lower than those in healthy participants. However, the diagnostic performance of the O-glycosylation traits in the hinge region of plasma IgA1 for IgA nephropathy remains unelucidated. Read More

Front Med (Lausanne) 2022 4;9:705908. Epub 2022 Apr 4.

Division of Pulmonary and Critical Care, College of Medicine-Jacksonville, University of Florida, Jacksonville, FL, United States.

Introduction: Acute kidney injury (AKI) has been associated with an increased mortality rate among hospitalized patients with Coronavirus disease 2019 (COVID-19). The current review aimed to evaluate the symptoms, complications, and treatments performed to manage AKI in patients with COVID-19.

Methods: We searched PubMed/Medline, Web of Science, and Embase for the relevant scientific literature published up to February 1, 2022. Read More

Cureus 2022 Mar 15;14(3):e23175. Epub 2022 Mar 15.

Gastroenterology and Hepatology, Ascension Health, Grand Blanc, USA.

Collapsing glomerulopathy (CG) is a rare variant of focal segmental glomerulosclerosis (FSGS) that commonly presents as nephrotic syndrome in patients. CG is almost always associated with human immunodeficiency virus (HIV) infection but is rarely from other infectious sources such as parvovirus, Epstein-Barr virus, cytomegalovirus, and SARS-CoV-2. CG has also been reported to be related to other etiologies such as genetic disorders, lupus, malignancy, and post-renal transplant but is exceedingly rare when related to hepatitis C virus (HCV). Read More

J Assoc Physicians India 2022 Apr;70(4):11-12

Sms Medical College and Hospital, Jaipur.

Diabetes mellitus (DM) is the leading cause of chronic kidney disease worldwide chiefly attributable to diabetic nephropathy (DN). In these patients, non diabetic kidney disease (NDKD) can also occur either alone or superimposed on diabetic nephropathy. This study aimed to identify the prevalence and the etiology of NDKD in our center and also the clinical and laboratory parameters to help distinguish these two entities. Read More