Ig a nephropathy

Concomitant with nephrotic syndrome and multicentric castleman's disease (MCD) has only been described in a limited number of small studies and case reports. Among those, none confirmed the renal pathology prior to the onset of MCD, and none of the cases had a history of nephrotic syndrome. A 76 year-old Japanese man visited a nephrologist because of incident nephrotic syndrome. He had previously experienced three episodes of nephrotic syndrome, the last one 13 years ago, and had been diagnosed with membranous nephropathy by renal biopsy...

BACKGROUND: Wiskott-Aldrich syndrome (WAS) is an X-linked immunodeficiency characterized by thrombocytopenia and eczema and is caused by a mutation in the WAS gene. WAS has heterogeneous clinical manifestations, and its clinically milder form is called X-linked thrombocytopenia (XLT). Patients with WAS/XLT sometimes have kidney complications, the most common of which is immunoglobulin (Ig)A nephropathy associated with aberrant glycosylation of IgA. CASE DIAGNOSIS/TREATMENT: The patient was a 6-year-old girl who was diagnosed with female XLT at the age of 4 years; she presented with microscopic hematuria and proteinuria at a school urinalysis...

BACKGROUND: Anti-glomerular basement membrane (GBM) disease is a rare rapidly progressive glomerulonephritis, frequently associated with alveolar hemorrhage in the lungs and involving the kidney by crescentic glomerulonephritis. It has been described in association with other glomerulonephritides [such as anti-neutrophilic antibody (ANCA)-glomerulonephritis, membranous nephropathy, and immunoglobulin (Ig)A nephropathy]. CASE SUMMARY: Herein we present an unusual case of concurrent anti-GBM disease, ANCA-associated crescentic glomerulonephritis and diffuse proliferative immune complex mediated glomerulonephritis with predominant staining for IgA and C3 by immunofluorescence...

The monotypic variant of immunotactoid glomerulopathy (ITG), strongly associated with low-grade lymphoproliferative disorders, is characterized histologically by glomerulonephritis and microtubular deposits of monoclonal immunoglobulin G (IgG). We report a patient with high-risk κ light chain multiple myeloma who presented with acute kidney injury, hematuria, proteinuria, and hypocomplementemia. Kidney biopsy revealed immunotactoid glomerulopathy concomitant with κ light chain myeloma cast nephropathy...

BACKGROUND: Glomerular diseases are important causes of morbidity and mortality among children in developing countries. Renal biopsy is the gold standard for determining histological diagnosis, prognosis, and therapy options. This study aimed to investigate the clinical, histopathological, and direct immunofluorescence findings of renal biopsy results in Nepalese children under 18 years old. METHODS: In this retrospective cross-sectional study, the available data from children who had undergone kidney biopsy between 2016 and the end of 2020 were evaluated...

BACKGROUND: This study aimed to investigate the role of neurofascin186 (NF186) in the pathogenesis of the concurrent focal segmental glomerulosclerosis (FSGS) in CIDP-like autoimmune nodopathy patients. METHODS: We presented a case of CIDP-like autoimmune nodopathy complicated with FSGS. We measured NF186 antibodies by cell-binding assay (CBA) method. We performed immunofluorescence analysis in the renal cryosection samples from a patient with minimal nephropathy with rabbit anti-NF186 antibody or NF186 antibody positive human serum...

Fibrosis is the final common finding in patients with advanced diabetic kidney disease. V-set Ig domain containing 4 (VSIG4) is related to fibrosis in several diseases. It also contributes to fibrosis under high-glucose conditions in renal tubule cells. To determine the role of VSIG4 in type 2 diabetes, we examined VSIG4 expression in a type 2 diabetic animal model and podocyte. Urinary excretion of albumin and VSIG4 was significantly higher in db/db mice than in the control group. Urine VSIGs levels for 6 h were about three-fold higher in db/db mice than in db/m mice at 20 weeks of age: 55...

RATIONALE: Amyloidogenic leukocyte chemotactic factor 2 (ALECT2) was recently considered as a new clinicopathologic type of amyloid, which frequently affects kidney in adults and results in different degrees of renal insufficiency and failure with or without proteinuria. Here, we present a case of combining LECT2-associated renal amyloidosis with immunoglobulin (Ig)A nephropathy. PATIENT CONCERNS: A 71-year-old Chinese man presented with edema of both lower extremities...

Background: Immunoglobulin (Ig) A nephropathy (IgAN) with a membranoproliferative pattern of injury that manifests as nephrotic syndrome (NS) is rarely reported in hepatitis C virus (HCV)-induced cirrhosis. It is not known whether eradication of HCV by direct-acting antiviral (DAA) drugs can lead to remission of proteinuria and improve the long-term prognosis. Case Description: We report the case of a 52-year-old woman with HCV cirrhosis for 10 years. She had undergone splenectomy and cholecystectomy due to complications of liver cirrhosis...

Background: Astragaloside IV is the most important bioactive component of Radix Astragali. Previous studies have shown that astragaloside IV plays an important role in the control of early- and mid-stage diabetes and late diabetic nephropathy. However, it is disappointing that the in vivo solubility of astragaloside IV and its bioavailability after oral administration are very low. We recently obtained a new water-soluble derivative of astragaloside IV-astragaloside formic acid (LS-102), which has higher bioavailability than the parent compound...

INTRODUCTION: Immunofluorescence (IF) microscopy is an essential tool for the analysis of glomerular diseases. In this study, we studied the significance of the IF technique together with light microscopy (LM) and clinical details in the diagnosis of different types of diffuse proliferative glomerulonephritis (GN). We intended to evaluate the spectrum of Diffuse Proliferative Glomerulonephritis (DPGN) in our institute. MATERIALS AND METHODS: We evaluated a total of 95 kidney biopsies received in the past 10 years...

The occurrence of kidney diseases associated with a monoclonal gammopathy in the absence of symptomatic multiple myeloma is increasingly recognized. When the kidney is involved, the monoclonal etiology of these diseases results in clinical and laboratory features distinct from those of other disease, necessitating the nomenclature monoclonal gammopathy of renal significance (MGRS). The detection of these monoclonal diseases involving the kidney is important since they are poorly responsive to conventional immunosuppression and instead require clone-directed therapy...

A 60-year-old Polish male with a history of alcoholism, liver cirrhosis, and hepatocellular carcinoma presented via a referral from his primary medical doctor to the emergency room with respiratory distress, acute kidney injury (AKI), and a purpuric rash on both lower extremities. He had received a total of 16 doses of Nivolumab for hepatocellular carcinoma. He had a baseline serum creatinine of 1.5 and Nivolumab was skipped a month prior to presentation because of a rise in creatinine and the onset of the rash...

Panax notoginseng, a traditional Chinese medicine, exerts beneficial effect on diabetic kidney disease (DKD), but its mechanism is not well clarified. In this study we investigated the effects of ginsenoside Rb1 (Rb1), the main active ingredients of Panax notoginseng, in alleviating podocyte injury in diabetic nephropathy and the underlying mechanisms. In cultured mouse podocyte cells, Rb1 (10 μM) significantly inhibited high glucose-induced cell apoptosis and mitochondrial injury. Furthermore, Rb1 treatment reversed high glucose-induced increases in Cyto c, Caspase 9 and mitochondrial regulatory protein NOX4, but did not affect the upregulated expression of aldose reductase (AR)...

Several novel antigens have recently been characterized in membranous nephropathy (MN), but those involved in the rare cases of MN associated with inflammatory neuropathies remain elusive. Although several antibodies have been identified in the serum, there is no evidence so far for their deposition in glomeruli. We report the case of a 73-year-old woman who was referred because of subacute onset of proximal asymmetric lower limb weakness together with ataxic gait. She was diagnosed with inflammatory neuropathy...

BACKGROUND: Polycythemia vera (PV) is a common type of Philadelphia chromosome-negative chronic myeloproliferative disorder. PV-associated kidney disease is rarely reported and remains poorly understood. It has been observed that chronic kidney disease could be a risk factor for poor prognosis in PV. METHODS: We retrospectively analyzed the clinicopathological features of renal presentations in eight patients with confirmed PV-associated kidney disease. RESULTS: The eight patients were 6 males and 2 females, with a mean age of 46...

BACKGROUND: In this study, we investigated the Nrf2/ARE signaling pathway activating capacity of Biphenyl Diester Derivative-39 (BDD-39) in diabetic nephropathy in order to elucidate the mechanism surrounding its antidiabetic potential. OBJECTIVES: Protein expressions of Nrf2, HO-1, NQO-1 and biomarkers of kidney fibrosis were executed after which mRNA levels of Nrf2, HO-1 and NQO-1 were estimated after creating the models following BBD-39 treatment. METHODS: Type 2 diabetes model was established in mice with high-fat diet feeding combined with streptozocin intraperitoneal administration...

Monoclonal gammopathies (MG) encompass a variety of disorders related to clonal expansion and/or malignant transformation of B lymphocytes. Deposition of free immunoglobulin (Ig) components (light or heavy chains, LC/HC) within the kidney during MG may result over time in multiple types and degrees of injury, including acute kidney injury (AKI). AKI is generally a consequence of tubular obstruction by luminal aggregates of LC, a pattern known as "cast nephropathy". Monoclonal Ig LC can also be found as intracellular crystals in glomerular podocytes or proximal tubular cells...

Immunoglobulin (Ig) A nephropathy (IgAN) is the commonest form of primary glomerulonephritis worldwide and is, considered a significant cause of end-stage renal disease in young adults. The precise pathogenesis of IgAN is unclear. The clinical and pathological features vary significantly between individuals and races, which makes treating IgAN difficult. Currently, the therapeutic strategies in IgAN are still optimal blood pressure control and proteinuria remission to improve the renal function in most cases...

Introduction: Immunoglobulin (Ig)A nephropathy (IgAN) is the most frequently diagnosed primary glomerulonephritis worldwide. Despite the common diagnostic feature of mesangial IgA-containing immune complex deposition, the clinical course of the disease is extremely variable, with 30% of patients developing end-stage kidney disease within 20 years of diagnosis. Therefore, identifying which patients are likely to progress is paramount. Results: In this pilot study, we found that urinary exosomal miR-204 expression was significantly reduced in IgAN compared with healthy subjects...