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BMJ Case Rep 2021 Apr 23;14(4). Epub 2021 Apr 23.

Division of Nephrology, Department of Medicine, University of the Philippines Manila, Manila, Philippines.

Crescentic glomerulonephritis is usually associated with an acute nephritic syndrome with rapidly declining renal function. Postinfectious cases usually have a higher possibility of recovery. Juvenile nasopharyngeal angiofibroma (JNA) is a rare, locally aggressive tumour affecting mostly young men. Read More

BMC Nephrol 2021 Apr 21;22(1):143. Epub 2021 Apr 21.

Renal Division, Department of Medicine, West China Hospital of Sichuan University, 610041, Chengdu, China.

Background: C3 glomerulopathy (C3G) is a recent disease classification that is characterized by the presence of glomerular deposits (composed of C3) in the absence of significant amounts of immunoglobulin and comprises dense deposit disease and C3 glomerulonephritis (C3GN). Most C3GN manifests as membranoproliferative, mesangial proliferative glomerulonephritis patterns via light microscopy. Pure membranous nephropathy (MN)-like glomerular lesions are rare manifestations of C3GN. Read More

J Med Case Rep 2021 Apr 3;15(1):186. Epub 2021 Apr 3.

Division of Nephrology, University of Massachusetts Medical School -Baystate, Springfield, MA, USA.

Background: Immune checkpoint inhibitors and mitogen-activated protein kinase inhibitors have become the standard of care in patients with advanced melanoma bearing V600 mutations. However, little is known about their nephrotoxicity. To date, only two cases of anti-glomerular basement membrane glomerulonephritis after exposure to checkpoint inhibitors have been documented. Read More

Front Med (Lausanne) 2020 2;7:582272. Epub 2021 Mar 2.

Nephrology, Dialysis and Transplantation Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy.

Acute kidney injury (AKI) is increasingly emerging as a global emergency. Sepsis, major surgery, and nephrotoxic drugs are the main causes of AKI in hospitalized patients. However, glomerulonephritis accounts for about 10% of AKI episodes in adults, mainly related to rapidly progressive glomerulonephritis resulting from granulomatous polyangiitis (GPA, Wegener granulomatosis), microscopic polyangiitis (MPA), and anti-glomerular basement membrane (GBM) disease. Read More

Am J Clin Pathol 2021 Feb 24. Epub 2021 Feb 24.

Department of Pathology, Microbiology and Immunology, Vanderbilt University School of Medicine, Nashville, TN, USA.

Objectives: To study the pathologic spectrum of kidney diseases in patients with hepatitis C virus infection (HCV+).

Methods: Native kidney biopsy specimens in HCV+ patients were reviewed.

Results: A total of 9,836 native kidney biopsy specimens were evaluated from January 2007 to December 2016, of which 273 (2. Read More

BMJ Case Rep 2021 Feb 5;14(2). Epub 2021 Feb 5.

Research Laboratory of Renal Pathology LR00SP01, Charles Nicolle Hospital, Bab Saadoun, Tunis, Tunisia.

Renal involvement in mantle cell lymphoma (MCL) is rare. We present the case of a man followed for MCL presented with acute kidney injury and positive antineutrophil cytoplasmic antibody (ANCA) type anti proteinase 3 (PR3). He was treated as for a rapidly progressing glomerulonephritis with cyclophosphamide and methylprednisolone followed by oral prednisone. Read More

Nephrol Dial Transplant 2021 01;36(2):208-215

Division of Nephrology and Hypertension and Division of Hematology, Mayo Clinic, Rochester, MN, USA.

Proliferative glomerulonephritis (GN) with monoclonal immunoglobulin deposits (PGNMIDs) is a recently described entity among the spectrum of monoclonal gammopathy of renal significance (MGRS). The disease is renal limited and manifests with chronic glomerular disease, altered renal function and albuminuria, sometimes in the nephrotic range. Acute nephritic syndrome is rare. Read More

Front Pediatr 2020 27;8:580178. Epub 2020 Nov 27.

Department of Pediatrics, Al Jalila Children's Specialty Hospital, Dubai, United Arab Emirates.

Congenital nephrotic syndrome, historically defined by the onset of large proteinuria during the first 3 months of life, is a rare clinical disorder, generally with poor outcome. It is caused by pathogenic variants in genes associated with this syndrome or by fetal infections disrupting podocyte and/or glomerular basement membrane integrity. Here we describe an infant with congenital CMV infection and nephrotic syndrome that failed to respond to targeted antiviral therapy. Read More

Indian J Nephrol 2020 Jul-Aug;30(4):264-269. Epub 2020 Feb 26.

Department of Nephrology, SMS Medical College, Jaipur, Rajasthan, India.

Introduction: The spectrum of renal disorder in the elderly differs from the younger population. There is a paucity of literature regarding kidney biopsy in elderly. This study aims to highlight the clinical profile and histopathological spectrum of the elderly patient undergoing a renal biopsy. Read More

J Rheumatol 2021 02 1;48(2):228-231. Epub 2020 Sep 1.

K. Tselios, MD, PhD, D.D. Gladman, MD, FRCPC, C. Taheri, BHSc, J. Su, MB, MSc, M.B. Urowitz, MD, FRCPC, University of Toronto Lupus Clinic, Centre of Prognosis Studies in the Rheumatic Diseases, University Health Network, Toronto, Ontario, Canada.

Objective: Lupus nephritis (LN) may lead to endstage kidney disease (ESKD) in 22% of patients over a period of 15 years, with the risk being particularly higher in diffuse proliferative forms. The rate of kidney function decline varies. However, a catastrophic course leading to ESKD within a few years from onset is uncommon. Read More

BMC Nephrol 2020 11 7;21(1):463. Epub 2020 Nov 7.

Department of Nephrology, University of Hong Kong - Shenzhen Hospital, Shenzhen, China.

Background: Angioimmunoblastic T cell lymphoma (AITL) is an infrequent hematological malignancy with variable and often atypical presentations. The presence of dysproteinemia, autoantibodies and systemic involvement in AITL has often led to a delay in diagnosis or even misdiagnosis in practice. We herewith present a case of AITL that primarily presented with acute kidney injury associated with type II Cryoglobulinemia, the underlying cause was only identified 8 months after the emergence of initial symptoms. Read More

BMC Nephrol 2020 08 24;21(1):362. Epub 2020 Aug 24.

Second Department of Internal Medicine, Division of Nephrology, Kansai Medical University, 2-5-1 Shinmachi Hirakata, Osaka, 573-1191, Japan.

Background: Mutations in the Wilms tumor 1 gene cause a spectrum of podocytopathy ranging from diffuse mesangial sclerosis to focal segmental glomerulosclerosis. In a considerable fraction of patients with Wilms tumor 1 mutations, the distinctive histology of immune-complex-type glomerulonephritis has been reported. However, the clinical relevance and etiologic mechanisms remain unknown. Read More

Clin Nephrol 2020 Nov;94(5):260-265

Mastocytosis is a rare myeloproliferative disease in which mast cells abnormally accumulate in the skin, bone marrow, intestine, liver, spleen, and lymph nodes. Characterized by uncontrolled proliferation of aberrant mast cells, the disease can present either cutaneously or systemically. Mast cells facilitate the immune response and inflammation, and mastocytosis with renal involvement has been rarely reported in adults. Read More

Medicine (Baltimore) 2020 Jun;99(25):e20572

Department of Nephrology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Bunkyo.

Rationale: Infection is a major trigger or pathogenic origin in a substantial proportion of glomerulonephritis (GN) patients, typically manifesting infection-related GN (IRGN). Various microorganisms, infection sites, and clinical and histopathological features are involved in IRGN. Once an infectious origin is identified and successfully eradicated, nephrotic syndrome or kidney dysfunction is spontaneously resolved. Read More

Diagn Pathol 2020 May 27;15(1):62. Epub 2020 May 27.

Université de Tours, PRES Centre-Val de Loire, Tours, France.

Background: Infection-related glomerulonephritis with IgA deposits (IRGN-IgA) is a rare disease but it is increasingly reported in the literature. Data regarding epidemiology and outcome are lacking, especially in Europe. We aimed to assess the clinical, pathologic and outcome data of IRGN-IgA. Read More

CEN Case Rep 2020 11 30;9(4):326-332. Epub 2020 Apr 30.

Division of Nephrology, Department of Internal Medicine, Toyonaka Municipal Hospital, 4-14-1, Shibahara-cho, Toyonaka, Osaka, 560-8565, Japan.

A 74-year-old man presented with nephrotic syndrome and kidney insufficiency. Laboratory tests revealed monoclonal gammopathy of immunoglobulin A-lambda. Renal biopsy revealed diffuse mesangial proliferation and double-contoured basement membranes. Read More

Nephron 2020 10;144(5):228-235. Epub 2020 Mar 10.

Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.

Background: Complement component 3 glomerulopathy (C3G) is a disease diagnosed based on the predominance of C3 immunostaining in glomeruli. The popular electron microscopic subtyping of C3G into dense deposit disease and C3 glomerulonephritis (GN) is not without limitations. We aimed to study the light microscopic (LM) patterns of C3G along with their clinicopathological correlation and treatment outcome. Read More

Case Rep Rheumatol 2019 14;2019:8750306. Epub 2019 Dec 14.

Oakland University William Beaumont School of Medicine, 586 Pioneer Dr, Rochester, MI 48309, USA.

Thrombotic thrombocytopenic purpura (TTP) is a potentially fatal disorder that requires urgent identification and treatment. The association of TTP with systemic lupus erythematosus (SLE) and vasculitis has been reported, however, never simultaneously. A 33-year-old woman with a history of SLE presented with acute abdominal pain, fever, arthralgias, and skin rash. Read More

Pan Afr Med J 2019 23;34:107. Epub 2019 Oct 23.

Service de Médecine Interne, CHU Hédi Chaker, Sfax, Tunisie.

Henoch-Schonlein purpura (HSP) is a small-vessel vasculitis secondary to IgA deposits. We conducted a retrospective study of 14 cases of HSP in adults based on EULAR/PRINTO/PRES classification criteria for HSP over a period of 18 years (1996 to 2014) in Department of Internal Medicine at the Hedi Chaker University Hospital, Sfax, South Tunisia. The purpose of our study was to highlight the epidemiological, clinical, therapeutic and evolutionary features of HSP in our adult patients. Read More

J Int Med Res 2020 Apr 27;48(4):300060519894456. Epub 2019 Dec 27.

Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Graduate School of Peking Union Medical College, Beijing, China.

Objective: We investigated renal injury characteristics in Chinese patients with systemic sclerosis (SSc) who had undergone renal biopsy.

Methods: We searched the medical records of patients with SSc who were hospitalized at Peking Union Medical College Hospital between January 1990 and August 2019. We analyzed the clinical characteristics and pathological results of these patients. Read More

Adv Chronic Kidney Dis 2019 09;26(5):369-375

Division of Nephrology, Department of Medicine, Columbia University Irving Medical Center, New York, NY. Electronic address:

In systemic lupus erythematosus, nephrotic-range proteinuria typically signals the presence of a proliferative lupus nephritis (class III/IV) and/or membranous lupus nephritis (class V, with or without concomitant class III or IV lesions). However, in rare instances, systemic lupus erythematosus patients with nephrotic syndrome have kidney biopsy findings of normal glomeruli or focal segmental glomerulosclerosis lesions, with or without mesangial proliferation, on light microscopy; the absence of subepithelial or subendothelial deposits on immunofluorescence and electron microscopy; and diffuse foot process effacement on electron microscopy. This pattern, termed lupus podocytopathy, is a unique form of lupus nephritis that mimics minimal change disease or primary focal segmental glomerulosclerosis and represents approximately 1% of lupus nephritis biopsies. Read More

Intern Med 2019 1;58(21):3173-3178. Epub 2019 Nov 1.

The First Department of Internal Medicine, University of Occupational & Environmental Health, School of Medicine, Japan.

Diffuse proliferative lupus nephritis (DPLN) is a serious organ complication. Drug resistance correlates with P-glycoprotein (P-gp) expression on activated lymphocytes. We encountered a refractory DPLN patient with expansion of peripheral CD69/CXCR3-co-expressing P-gpCD4 cells producing IL-2 and IL-6. Read More

Clin Rheumatol 2020 Feb 26;39(2):509-514. Epub 2019 Oct 26.

Laboratory of Medical Investigation (LIM-36), Instituto da Criança, Faculdade de Medicina, Universidade de São Paulo, Av. Dr. Enéas de Carvalho Aguiar, 647, 5° andar, Cerqueira Cesar, São Paulo, São Paulo, CEP:05403-900, Brazil.

Introduction/objectives: Tyro3, Axl, and Mer (TAM) receptors and ligands mediate apoptotic bodies engulfment which alteration has been related with juvenile systemic lupus erythematosus (JSLE) pathogenesis. Thus, the aim was to determine their soluble levels.

Methods: Serum sTyro3, sAxl, sMer, and Gas6 levels were measured using ELISA in 67 JSLE patients, 12 juvenile idiopathic arthritis (JIA) inflammatory and 20 healthy controls and related with SLEDAI-2K score, anti-dsDNA antibody, ESR, CRP, C3, C4 levels, and nephritis. Read More

BMC Nephrol 2019 10 17;20(1):375. Epub 2019 Oct 17.

Department of Internal Medicine IV, Teikyo University School of medicine, University Hospital, Mizonokuchi, Kawasaki, Japan.

Background: TAFRO syndrome is a systemic inflammatory disorder that manifests as thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Renal dysfunction is frequently complicated with TAFRO syndrome, however, it is challenging to perform kidney biopsy in patients with TAFRO syndrome in the presence of thrombocytopenia. Renal histology in TAFRO syndrome mainly shows membranoproliferative glomerulonephritis (MPGN)-like lesions or thrombotic microangiopathy (TMA)-like glomerulopathy. Read More

BMC Nephrol 2019 10 17;20(1):377. Epub 2019 Oct 17.

Department of Pathology, 900 Hospital of the jiont logistic, Fuzhou, China.

Background: To investigate the clinicopathological characteristics of renal damage caused by long-term exposure to carbon disulfide (CS2) in nine patients.

Methods: All the patients underwent ultrasound-guided renal biopsy. All specimens were examined by light microscopy and immunohistochemistry (IHC). Read More

Front Immunol 2019 14;10:1951. Epub 2019 Aug 14.

Graduate Institute of Aerospace and Undersea Medicine, Department of Medicine, National Defense Medical Center, Taipei, Taiwan.

Chinese herbal medicines used in combination have long-term been shown to be mild remedies with "integrated effects." However, our study provides the first demonstration that M1, an active metabolite of ginsenoside, exerted its dramatic therapeutic effects on accelerated and severe lupus nephritis (ASLN) mice, featuring acute renal function impairment, heavy proteinuria, high serum levels of anti-dsDNA, and high-grade, diffuse proliferative renal lesions. In the present study, NZB/WF1 mice were given injections of lipopolysaccharide to induce the ASLN model. Read More

Transplant Proc 2019 Jul - Aug;51(6):1845-1847

Clínica de Nefrologia e Transplantes, Hospital BP-Beneficência Portuguesa, São Paulo, Brazil.

Background: There are numerous reports of successful pregnancies following kidney transplantation. However, little information is available regarding the management and evolution of multiple pregnancies in a kidney-transplanted woman.

Case Report: We report the case of successful consecutive spontaneous triplet and twin pregnancies in a woman who had undergone kidney transplantation at 30 years of age, 12 years before the first pregnancy, as a result of end-stage renal disease secondary to chronic glomerulonephritis due to diffuse proliferative lupus nephritis. Read More

Rheumatology (Oxford) 2019 11;58(11):2039-2050

Department of Nephrology, Xuzhou, Jiangsu, China.

Objectives: We previously reported that ex vivo TGF-β and IL-2-induced CD8+CD103+ regulatory T cells (CD8+CD103+ iTregs) displayed similar immunosuppressive effect and therapeutic function on lupus mice nephritis to that of CD4+Foxp3+ Tregs. In view of the important role of glomerular endothelial cell (GEC) injury in inflammatory processes in SLE, this study aimed to investigate the nature and mechanism of CD8+CD103+ iTregs-mediated amelioration of LN by attenuating GEC injury.

Methods: Treg cells from patients with SLE and from healthy controls were characterized by flow cytometry analysis. Read More

Lupus 2019 Aug 11;28(9):1082-1090. Epub 2019 Jul 11.

1 Department of Medicine, Nephrology Section, King Abdulaziz Medical City, Ministry of National Guard, Jeddah, Saudi Arabia.

Background: Few data are available about the rate of short-term remission and its impact on the long-term outcomes of proliferative lupus nephritis in the Middle East.

Methods: An observational study was carried out involving 96 adult patients with biopsy-proven focal or diffuse proliferative lupus nephritis (PLN) from four different hospitals. Data on induction, remission and long-term outcomes were collected and analyzed. Read More