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Ther Apher Dial 2022 May 18. Epub 2022 May 18.

Nephrology, hemodialysis, apheresis and kidney transplantation department, Grenoble University Hospital, Grenoble, France.

Background: Cryoglobulinemia is defined as the presence of an abnormal immunoglobulin that may be responsible for vasculitis of small-caliber vessels. Apheresis can be used in order to temporarily eliminate circulating cryoglobulins. The aim of this study was to assess the effectiveness of apheresis (double-filtration plasmapheresis-DFPP-) in symptomatic and/or severe cryoglobulinemias. 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

Kidney Int Rep 2022 May 13;7(5):1123-1127. Epub 2022 Jan 13.

Division of Nephrology, Department of Internal Medicine, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA.

Exp Clin Transplant 2022 May;20(Suppl 3):45-48

From the Department of Pediatric Nephrology, Dr. Sami Ulus Maternity and Child Health and Diseases Training and Research Hospital, Ankara, Turkey.

Membranoproliferative glomerulonephritis and renal microangiopathies may manifest similar clinical presentations and histology. Many genetic mutations that cause these diseases have been reported. Studies on mutations in the gene encoding diacylglycerol kinase epsilon identified a novel pathophysiologic mechanism leading to atypical hemolytic uremic syndrome and/or membranoproliferative glomerulonephritis. Read More

BMC Nephrol 2022 May 12;23(1):181. Epub 2022 May 12.

Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA.

Background: Bartonella endocarditis is often a diagnostic challenge due to its variable clinical manifestations, especially when it is first presented with involvement of organs other than skin and lymph nodes, such as the kidney.

Case Presentation: This was a 13-year-old girl presenting with fever, chest and abdominal pain, acute kidney injury, nephrotic-range proteinuria and low complement levels. Her kidney biopsy showed diffuse crescentic proliferative glomerulonephritis with a full-house pattern of immune complex deposition shown by immunofluorescence, which was initially considered consistent with systemic lupus erythematous-associated glomerulonephritis (lupus nephritis). Read More

Saudi J Kidney Dis Transpl 2021 Sep-Oct;32(5):1479-1481

Department of Medicine, NKP Salve Institute of Medical Sciences, Nagpur, Maharashtra, India.

C3 glomerulopathy is usually seen with the presence of C3 nephritic factor, homozygous or heterozygous mutations in the regulatory complement proteins factor H, factor I, or C3. We describe the presence of heterozygous laminin β2 mutation in a patient of C3 glomerulonephritis with ocular and central nervous system involvement, the significance of which is unknown. Read More

Saudi J Kidney Dis Transpl 2021 Sep-Oct;32(5):1388-1396

Department of Nephrology, Dialysis and Transplantation, Charles Nicolle Hospital; Faculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.

The elderly population has significantly increased in the world. Nephrotic syndrome (NS) is one manifestation of glomerular nephropathy in the elderly. The objective of our study is to determine NS particularities in the elderly and to identify the factors predicting progression to chronic end-stage renal disease (ESRD). Read More

J Nephrol 2022 May 6;35(4):1289-1293. Epub 2022 May 6.

Department of Organ Transplant Medicine, Tokyo Women's Medical University, Tokyo, Japan.

Proliferative glomerulonephritis with monoclonal immunoglobulin IgG deposits (PGNMID) is an already described form of renal involvement by monoclonal gammopathy. PGNMID is known to recur in kidney allografts. Bortezomib has shown clinical success in the treatment of multiple myeloma. 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

Clin Nephrol 2022 Apr 21. Epub 2022 Apr 21.

Aims: Donor-derived cell-free DNA (dd-cfDNA) surveillance testing has never been studied in comparison with other surveillance tests. In this study we aim to describe our center's clinical experience with routine dd-cfDNA monitoring and to assess whether monitoring dd-cfDNA by protocol provides additional information that aids in detection of acute rejection.

Materials And Methods: We implemented the dd-cfDNA (Allosure) surveillance protocol in addition to measurements of serum creatinine, proteinuria, and donor-Specific antibody. Read More

BMJ Case Rep 2022 Apr 20;15(4). Epub 2022 Apr 20.

Complement Therapeutics Research Group, Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK.

A male child presented initially well with a mixed nephrotic-nephritic syndrome and was commenced on standard high-dose oral corticosteroids. Clinical deterioration occurred 3 weeks later with rapidly progressing renal dysfunction, seizures and diminished urinary output, requiring renal replacement therapy. Once stabilised, renal biopsy demonstrated mesangial and capillary C3, minimal IgG deposition, with mesangial electron dense deposits felt consistent with postinfectious glomerulonephritis or C3 glomerulopathy. Read More

Mol Genet Genomic Med 2022 Apr 18:e1945. Epub 2022 Apr 18.

Department of Nephrology, Children's Hospital of Chongqing Medical University, Chongqing, China.

Background: Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a monogenic immunodeficiency disease caused by forkhead box protein3 (FOXP3) mutation. The kidney is commonly involved in IPEX syndrome, but there were few studies focusing on renal involvement.

Methods: Whole-exome sequencing was used to identify the novel FOXP3 mutation. Read More

Case Rep Nephrol Dial 2022 Jan-Apr;12(1):22-30. Epub 2022 Mar 14.

Department of Nephrology, General University Hospital, Prague, Czechia.

Patients on long-term home parenteral nutrition (HPN) occasionally develop glomerulonephritis due to chronic central venous catheter (CVC)-related infection. Most previously reported cases were membranoproliferative glomerulonephritis (MPGN). This is a case report of a 16-year-old girl receiving HPN for short bowel syndrome. Read More

Cureus 2022 Mar 10;14(3):e23041. Epub 2022 Mar 10.

Hematology and Oncology, St. Joseph's Regional Medical Center, Paterson, USA.

Herein we present the case of a patient who was diagnosed with membranoproliferative glomerulonephritis and underwent renal transplant 27 years prior to presentation with new kidney failure. Although our patient did not undergo renal biopsy, it is our thought that she developed recurrent membranoproliferative disease, as she was well maintained on immunosuppressants and steroids for many years. This case is unique, because she was outside of the typical window for both chronic rejection and recurrent disease. Read More

Front Med (Lausanne) 2022 28;9:819320. Epub 2022 Mar 28.

Nephrology and Dialysis Unit (The European Rare Kidney Disease Reference Network, The European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases, and the European Reference Network That Aims at Improving the Care of Patients With Rare Immunological Disorders), Center of Research of Immunopathology and Rare Diseases- Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, Department of Clinical and Biological Sciences, University of Turin and S. Giovanni Bosco Hub Hospital, Turin, Italy.

In the countries where HCV infection is still endemic, about 90% of subjects with mixed cryoglobulinemia had previously been infected with HCV and about 80% are RNA positive. Remarkable results in severe HCV-related cryoglobulinemic vasculitis have been obtained with Rituximab. Details of the clinical characteristics and effective treatment of non HCV-related cryogloulinemic syndromes are presently lacking. Read More

Nephrol Dial Transplant 2022 04 11. Epub 2022 Apr 11.

Instituto de Investigación Hospital 12 de octubre (imas12), Madrid, Spain.

Introduction: Membranoproliferative glomerulonephritis (MPGN) represents a histologic pattern of glomerular injury which may be due to several etiologies. Few studies have comprehensively analyzed the recurrence of MPGN according to the current classification system.

Methods: We collected a multicenter, retrospective cohort of 220 kidney graft recipients with biopsy-proven native kidney disease due to MPGN between 1981-2021 in 11 hospitals. Read More

Stem Cell Res 2022 05 14;61:102751. Epub 2022 Mar 14.

Department of Urology, The Second Hospital, Cheeloo College of Medicine, Shandong University, Jinan 250033, China; Engineering Laboratory of Urinary Organ and Functional Reconstruction of Shandong Province, Jinan, China; Binzhou Medical University, Yantai, China. Electronic address:

Glomerulopathy with fibronectin deposits (GFND) is an autosomal dominant kidney disease exhibiting microscopic hematuria, proteinuria, and hypertension that may lead to end-stage renal failure. In this study, using non-integrative episomal vectors an induced pluripotent stem cell (iPSC) line, FHUSTCi001-A, was derived from peripheral blood mononuclear cells of an 11-year-old boy with GFND carrying a heterozygous c.5602G > A (p. Read More

Medicine (Baltimore) 2022 Feb;101(7):e28754

Department of Internal Medicine, Hamad Medical Corporation, Doha, Qatar.

Rationale: Kidney involvement with COVID-19 infection is a well-known complication, and the majority of kidney involvement is related to ischemic injury/acute tubular injury. However, there are some cases of glomerulonephritis, the etiology of which is not yet known, but an immune process is likely to be the trigger.

Patient Concerns: A 27-year-old man presented to our hospital with facial puffiness and lower-limb swelling. Read More

Am J Nephrol 2022 30;53(4):325-330. Epub 2022 Mar 30.

University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-net, ERN-Reconnect and RITA-ERN Member) including the Nephrology and Dialysis Unit, Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley (North-West Italy), Department of Clinical and Biological Sciences, San Giovanni Bosco Hub Hospital, University of Turin, Turin, Italy.

Introduction: To date, almost 7 billion doses of the different types of vaccine against SARS-CoV-2 have been administered worldwide. Although the severity of new cases of SARS-CoV-2 has progressively decreased, and the pressure on national health systems has declined, the development of de novo glomerular injuries has been suggested.

Methods: This study aimed to examine the patients who were hospitalized in our Unit between April and November 2021 and underwent renal biopsy for new-onset urinary abnormalities (UA) and/or renal impairment within 3 months of SARS-CoV-2 vaccination. Read More

Int Urol Nephrol 2022 Mar 30. Epub 2022 Mar 30.

Department of Renal Medicine, Singapore General Hospital, Academia Level 3, 20 College Road, Singapore, 169856, Singapore.

Front Pediatr 2022 4;10:827466. Epub 2022 Mar 4.

Department of Pathology, Carver College of Medicine, University of Iowa, Iowa City, IA, United States.

Coronavirus disease 2019 (COVID-19) may cause a wide spectrum of kidney pathologies. The impact of COVID-19 is unclear in the context of the complement system abnormalities, including C3 glomerulopathy (C3G). In this report, we describe a young adult receiving a kidney transplant for C3 glomerulopathy (C3G), a disorder of the alternative complement pathway. Read More

Turk J Pediatr 2022 ;64(1):59-68

Divisions of Pediatric Pathology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Background: Crescentic glomerulonephritis (CGN) is a rapidly progressive and rare cause of glomerulonephritis in childhood. The aim of this study is to evaluate demographic data of children with crescentic glomerulonephritis, to classify the etiologies and to investigate the correlation between the severity of kidney disease and the expression of CD163+ macrophages.

Methods: Between the years 2000 and 2016 in a single center, patients under 18 years of age with kidney biopsies containing crescents were included in the study. Read More

J Immunol 2022 04 11;208(7):1772-1781. Epub 2022 Mar 11.

Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Centro Anna Maria Astori, Bergamo, Italy; and

Alternative pathway complement dysregulation with abnormal glomerular C3 deposits and glomerular damage is a key mechanism of pathology in C3 glomerulopathy (C3G). No disease-specific treatments are currently available for C3G. Therapeutics inhibiting complement are emerging as a potential strategy for the treatment of C3G. Read More

Minerva Med 2022 Mar 10. Epub 2022 Mar 10.

Department of Nephrology, Jinhua People's Hospital, Jinhua, China.

Saudi J Kidney Dis Transpl 2021 Jul-Aug;32(4):1166-1170

Department of Nephrology, Liaquat National Hospital, Karachi, Pakistan.

About 3% of the population aged more than 50 years, is affected by monoclonal gammopathy of undetermined significance (MGUS), a premalignant condition that may progress to lymphoproliferative disorders. Since MGUS does not represent the diseases associated with end organ damage, a new term, monoclonal gammopathy of renal significance (MGRS) is coined for the monoclonal gammopathies that are associated with renal disorders. MGRS is classified into various types, including monoclonal immunoglobulin deposition disease (MIDD) and proliferative glomerulonephritis with monoclonal immunoglobulin deposition (PGNMID). Read More

Saudi J Kidney Dis Transpl 2021 Jul-Aug;32(4):1128-1133

Department of Pathology, University College of Medical Sciences and Guru Teg Bahadur Hospital, New Delhi, India.

C3 glomerulopathy (C3G) is a rare entity that is defined by glomerular pathology characterized by predominant deposition of C3 in the glomeruli, with absent or scant immunoglobulin deposition. The aim of this study was to diagnose this rare entity, using clinical features, light microscopy (LM) and direct immunofluorescence (DIF) findings. A retrospective study was done from January 2016 to December 2018. Read More

Pediatr Nephrol 2022 Feb 24. Epub 2022 Feb 24.

Queensland Fertility Group, Virtus Genetics, Brisbane, Australia.

Background: Intronic WT1 mutations are usually causative of Frasier syndrome with focal segmental glomerulosclerosis as the characteristic nephropathy. Membranoproliferative glomerulonephritis is not commonly associated with disorders of sex development but has been recently identified as a WT1-associated nephropathy, but usually in cases of exonic mutations in either isolated Wilms tumor or Denys-Drash syndrome.

Methods: The clinical and genetic data from 3 individuals are reported. Read More

G Ital Nefrol 2022 Feb 16;39(1). Epub 2022 Feb 16.

Nephrology and Dialysis Unit, ASL TO4, Turin, Italy.

Monoclonal gammopathy of renal significance (MGRS) designates disorders induced by a monoclonal protein secreted by plasma cells or B-cell clones in patients who do not meet the diagnostic criteria for multiple myeloma or other B-cell malignancies. Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is a form MGRS. Until now, no guidelines to decide the best therapeutic approach to manage PGNMID exist, and most patients progress to End Stage Renal Disease (ESRD) without therapy. Read More

BMC Nephrol 2022 02 16;23(1):67. Epub 2022 Feb 16.

Department of Nephrology, Aix-Marseille Univ, Hôpital de la Conception, AP-HM, 147 Bd Baille, 13005, Marseille, France.

Background: Hypocomplementemic urticarial vasculitis (HUV) is a rare systemic vasculitis. We aimed to describe the kidney involvement of HUV in a multicenter national cohort with an extended follow-up.

Methods: All patients with HUV (international Schwartz criteria) with a biopsy-proven kidney involvement, identified through a survey of the French Vasculitis Study Group (FVSG), were included. Read More

Intern Med 2022 Feb 1. Epub 2022 Feb 1.

Department of Nephrology, Gifu Prefectural Tajimi Hospital, Japan.

A 49-year-old woman presented with nephrotic-range proteinuria, microhematuria, and moderate renal dysfunction. Diuretic-resistant refractory ascites associated with nephrotic syndrome were observed. Based on the histopathological findings, the patient was diagnosed with proliferative glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID). Read More