scholarly journals P0415MONOCLONAL GAMMOPATHY-RELATED RENAL DISEASES: NEW CLINICAL AND HISTOLOGICAL PATTERNS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Giulia Schiavone ◽  
Marisa Santostefano ◽  
Benedetta Fabbrizio ◽  
Elena Mancini
Keyword(s):  
Renal Biopsy ◽  
Light Chain ◽  
Monoclonal Gammopathy ◽  
Kidney Tissue ◽  
Renal Diseases ◽  
Type I ◽  
Diagnostic Challenge ◽  
Cytoplasmic Inclusions ◽  
Ckd Stage

Abstract Background and Aims In the last few years new clinical-histopathological forms of paraproteinemia associated kidney disease have emerged: light chain proximal tubulopathies, proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) and C3-glomerulopathy. It is now widely recognized that the pathogenic role of the monoclonal protein depends on the free light chain chemical-physical features, even in the case of “dangerous small B cell clones”. Thus MGRS (monoclonal gammopathy of renal significance) associated disease is now considered a different entity from monoclonal gammopathy of undetermined significance (MGUS). Method We retrospectively evaluated the clinical-histopathological features of the biopsies performed in our Nephrological Unit on patients with MGUS in 2008-2018, in order to re-classify them in accordance with the recent scientific literature. Results Nine patients (7 M/2 F; age 46-77 y) were analyzed: five of them suffering from CKD stage 2, four of them with AKI or rapidly progressive CKD, 7 with proteinuria > 1 g/day, 2 with physiological proteinuria. Bone marrow biopsy: 7 patients with MGUS, 2 with smoldering multiple myeloma. Renal biopsy: 4 glomerulopathies (monoclonal fibrillary glomerulonephritis, cryoglobulinaemic (type I) glomerulonephritis, C3 glomerulonephritis, PGNMID), 5 proximal tubulopathies (2 LCPT with Fanconi syndrome, 1 LCPT without cytoplasmic inclusions and with interstitial inflammatory reaction, 2 LCPT with acute tubular necrosis) Table 1, Table 2, Light microscopy and IF of PGNMID. Conclusion MGRS is responsible for the pathogenesis of new histopathological renal lesions, based on new pathogenetic pathways. The clinical and histological features of the different disease states are dependent on the structure of FLCs. Our retrospective analysis of MGRS biopsies confirms how difficult and complex the diagnostic challenge of monoclonal renal injury really is. The differentiation between MGUS and MGRS is based on renal biopsy and demonstration of monoclonality on kidney tissue even if serum/urine immunofixation is negative. Early biomarkers of the pathogenetic role of monoclonal FLC should be identified both for diagnosis and therapeutical monitoring.

scholarly journals Epidemiological and Clinical Characteristics of Monoclonal Gammopathy of Renal Significance (MGRS) in South America

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5856-5856
Author(s):  
Camila Peña ◽  
Gonzalo P Mendez ◽  
Natalia Paola Schutz ◽  
Eloisa Riva ◽  
Ricardo Valjalo ◽  
...  
Keyword(s):  
Renal Failure ◽  
Renal Biopsy ◽  
Plasma Cell ◽  
Light Chain ◽  
Monoclonal Gammopathy ◽  
Clinical Characteristics ◽  
Protein Electrophoresis ◽  
Renal Response ◽  
Series Study

Abstract Background Monoclonal gammopathy of renal significance (MGRS) is a recently defined entity. It is a group of renal diseases due to paraprotein deposition from a small B lymphocyte or plasma cell clon, not meeting the criteria for an overt gammopathy-associated neoplasm. Despite this feature, the secondary kidney damage may be severe and irreversible; therefore, its early recognition and treatment are crucial. There are few studies on MGRS in the international literature, and no reported data from Latin America (LA). Aims To describe epidemiological and clinical characteristics of patients diagnosed with MGRS in LA. To evaluate patients outcomes. Material and methods This is an international multicentric retrospective case series study. All members of GELAMM (Grupo de estudio latinoamericano de Mieloma Múltiple) were invited to participate. Patients with diagnosis of MGRS according to the IMWG definition were included. All cases had pathological diagnosis provided by a renal biopsy. Epidemiological and clinical data were collected from clinical records in a standardized report form. Renal response was arbitrarily defined as the partial or total recovery of renal failure or renal symtoms at the end of treatment. Statistical analysis was performed by descriptive statistics using STATA 12. Results We received data from 18 patients, from centers in Chile, Argentina and Uruguay. Median follow up was 22,5 months. The patients characteristics are shown in table 1. The median age was 58 years (36 to 78 years). Male to female ratio was 1:1,25. Twelve had history of hypertension and one patient of renal transplantation. Anemia was present in 78% of cases (mean 10,7g/dL +/-2,3), hypoalbuminemia in 72% (mean 2,8g/dL +/-0,7), renal failure in 83% (mean creatinine of 4,6mg/dL +/- 4,8) with 47% of these (7 patients) requiring renal replacement therapy (RRT). Proteinuria was measured in 16 patients. Its average was 4,4gr (range 0,12 - 11,5gr/24hrs). LDH and calcemia were normal in all cases. Half of the patients presented as a nephrotic syndrome. Regarding histological subtypes, the most frequently diagnosed was the proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID). The paraprotein most frequently found was Kappa, and in the renal biopsy was IgG Kappa deposition (table 1). Serum protein electrophoresis (sPEP) was performed in all cases. Only 8 out 18 patients underwent urine protein electrophoresis (uPEP) and 14 had urine and serum immunofixation (IFX) done. Serum free light chain (sFLC) were performed in 94% of the patients. The paraprotein identification according to each of this exams is shown in figure 1. Seventeen patients received treatment; 13 received an anti-plasma cell drug, 7 a thalidomide based regimen and 6 a bortezomib based regimen. The patient with IgM MGRS was treated with a rituximab based regimen. Regarding renal responses, there were no data in 5 patients. Nine out 13 of the patients achieved renal response: 3 achieved partial recovery and 9 complete recovery. Three patients become RRT independent. There was no mortality in our cohort. No patient relapsed, but 3 progressed: 1 to multiple myeloma (MM), 1 to systemic amyloidosis and another to systemic light chain deposition disease (LCDD). Discussion Only 18 cases from 3 South American countries were collected. The lack of hematologists in some countries, difficulties in achieving a renal biopsy, non-availability of immunofluorescence in the histological studies and few experienced pathologists could be some of the problems in our region. Our cohort is of rather young patients, which is probably related to the fact that these patients are mostly undergoing renal biopsy. We believe, however, that the incidence of MGRS (similar to what happens with MGUS), increases with age, which could mean a problem of underdiagnoses. As expected, nephropathies frequently associated with MM were found: AL amyloidosis and LCDD. However, the most common renal pathology was PGNMID, a rare entity. This data must be corroborated with a larger study. The high sensitivity of sFLC to identify the paraprotein was corroborated and highlighted the importance of this test in the follow up. Half of patients achieved a renal response, which reinforces the fact that they must be promptly treated. Conclusion According to our knowledge, this is the larger cases series study in LA, and we hope it will be a contribution to the knowledge of this pathology. Disclosures No relevant conflicts of interest to declare.

scholarly journals Human Glucose Transporters in Renal Glucose Homeostasis

2021 ◽  
Vol 22 (24) ◽  
pp. 13522
Author(s):  
Aleksandra Sędzikowska ◽  
Leszek Szablewski
Keyword(s):  
Glucose Homeostasis ◽  
Kidney Tissue ◽  
Glucose Transporters ◽  
Blood Stream ◽  
Renal Physiology ◽  
Renal Diseases ◽  
Carrier Proteins ◽  
Sodium Dependent ◽  
Pass Through

The kidney plays an important role in glucose homeostasis by releasing glucose into the blood stream to prevent hypoglycemia. It is also responsible for the filtration and subsequent reabsorption or excretion of glucose. As glucose is hydrophilic and soluble in water, it is unable to pass through the lipid bilayer on its own; therefore, transport takes place using carrier proteins localized to the plasma membrane. Both sodium-independent glucose transporters (GLUT proteins) and sodium-dependent glucose transporters (SGLT proteins) are expressed in kidney tissue, and mutations of the genes coding for these glucose transporters lead to renal disorders and diseases, including renal cancers. In addition, several diseases may disturb the expression and/or function of renal glucose transporters. The aim of this review is to describe the role of the kidney in glucose homeostasis and the contribution of glucose transporters in renal physiology and renal diseases.

scholarly journals The role of adiponectin and its receptor in patients with idiopathic membranous nephropathy complicated with hyperuricemia

2021 ◽  
pp. 31-31
Author(s):  
Tong Liu ◽  
Mengdi Xia ◽  
Yongji Zhang ◽  
Yibin Wang ◽  
Yun Zhou
Keyword(s):  
Membranous Nephropathy ◽  
Kidney Tissue ◽  
Renal Tissue ◽  
Idiopathic Membranous Nephropathy ◽  
Elisa Method ◽  
Adiponectin Receptor 1 ◽  
Ckd Stage ◽  
The Relationship ◽  
Nlrp3 Expression

Introduction/Objective. This study aimed to assess the changes of adiponectin (APN), IL-1?, adiponectin receptor 1 (Adipo R1), and NLRP3 expression of patients with idiopathic membranous nephropathy (IMN) complicated with hyperuricemia (HUA) and analyze the relationship between the APN pathway and the NLRP3 pathway. Methods. Forty-eight patients with IMN+HUA group, 49 patients with IMN group, 30 healthy controls, and 24 samples of healthy renal tissue were evaluated. APN and IL-1? of each group were detected by the ELISA method. AdipoR1 and NLRP3 in kidney tissue were detected by immunohistochemistry. The clinical data of each group were collected, and the relationship between APN, IL-1?, AdipoR1, NLRP3, and other indexes was analyzed. Results. (1) The concentration of UA, APN, IL-1?, and NLRP3 in the IMN+HUA group are significantly higher than those in the IMN group, but the AdipoR1 was lower. (2) With the severity of CKD stage, APN, IL-1?, and NLRP3 gradually increased in IMN+HUA group, but AdipoR1 gradually decreased. However, the above indicators did not change significantly in the IMN stages. Conclusion. The AdipoR1-AMPK and NLRP3-caspase-1-IL-1? signaling pathway may play an essential role in IMN+HUA patients. The intervention of these two pathways may make a great significance to the occurrence and progression on IMN+HUA patients.

scholarly journals Role of renal biopsy in managing pediatric renal diseases: A midterm analysis of a series at bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh

2017 ◽  
Vol 28 (1) ◽  
pp. 125 ◽  
Author(s):  
RanjitRanjan Roy ◽  
Abdullah Al Mamun ◽  
SM Shamsul Haque ◽  
Golam Muinuddin ◽  
Md. Habibur Rahman
Keyword(s):  
Renal Biopsy ◽  
Renal Diseases ◽  
Medical University

Renal involvement in plasma cell dyscrasias, immunoglobulin-based amyloidoses, and fibrillary glomerulopathies, lymphomas, and leukaemias

2010 ◽  
pp. 4055-4065
Author(s):  
P Ronco ◽  
F Bridoux ◽  
G Touchard
Keyword(s):  
Plasma Cell ◽  
Light Chain ◽  
Monoclonal Gammopathy ◽  
Renal Involvement ◽  
Renal Diseases ◽  
Progressive Decline ◽  
Wide Range ◽  
Single Clone ◽  
Plasma Cell Dyscrasias ◽  
First Line Treatment

Plasma cell dyscrasias are characterized by uncontrolled proliferation of a single clone of B cells which is responsible for the secretion of a monoclonal immunoglobulin (Ig) or Ig subunit that can become deposited in tissues. They can cause a wide range of renal diseases. Light-chain amyloidosis—renal presentation is usually with proteinuria, often progressing to nephrotic syndrome. Progressive decline in renal function usually occurs, leading finally to endstage renal failure. Diagnosis is made by the detection of monoclonal gammopathy in serum and/or urine (90% of cases) in combination with biopsy evidence of amyloid- forming light chain deposits. Chemotherapy with oral mephalan plus dexamethasone should be considered as first line treatment....

Emerging Role of Circular RNAs in Kidney Diseases in Nephrology

2021 ◽  
Vol 22 ◽  
Author(s):  
Cong Ma ◽  
Junjun Luan ◽  
Jeffrey B. Kopp ◽  
Hua Zhou
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Kidney Diseases ◽  
Kidney Tissue ◽  
Current Status ◽  
Renal Diseases ◽  
Circular Rnas ◽  
Systemic Lupus

Background: Circular RNAs (circRNAs) have been identified to be involved in a variety of human diseases such as cancers, cardiovascular diseases, and autoimmune diseases. In recent years, the role of circRNAs in the development of kidney diseases in nephrology has been gradually recognized. Objective: We updated and described the current status of circRNAs in kidney diseases in nephrology. We particularly focused on the roles and mechanisms of circRNAs in systemic lupus erythematosus and lupus nephritis. Methods: We summarized recent reports published on PubMed, Web of Science, Scopus, Scielo databases using key words circRNAs, kidney diseases or renal diseases, or systemic lupus erythematosus. Results: Studies of circRNAs in certain kidney diseases such as acute kidney injury, focal segmental glomerulosclerosis, idiopathic membranous nephropathy, IgA nephropathy, diabetic nephropathy, hypertensive renal damage and particular lupus nephritis address the function and pathogenesis of circRNAs in these diseases. Mechanisms of circRNAs in the above human kidney diseases so far have focused on the role of sponging microRNAs and regulating the expression of target genes. Moreover, circRNAs have been detected in blood, urine, and kidney tissue samples. These results suggest that circRNAs can serve as biomarkers for the diagnosis and monitoring the progression of kidney diseases. Conclusion: CircRNAs play important roles in the pathogenesis, diagnosis, and treatment of kidney diseases emphasizing lupus nephritis in nephrology.

scholarly journals Approach to native medical renal biopsy interpretation of glomerular disease

2019 ◽  
Vol 9 (2) ◽  
pp. 1571-1579
Author(s):  
Gopi Aryal ◽  
Sameer Chhetri Aryal
Keyword(s):  
Renal Biopsy ◽  
Renal Tissue ◽  
Clinical Syndrome ◽  
Renal Diseases ◽  
Primary Role ◽  
Pathology Laboratory ◽  
Glomerular Diseases ◽  
Clinical Presentations ◽  
Biopsy Interpretation

Diverse pathogenetic mechanisms and clinical manifestation of renal diseases may produce the same renal morphologic pattern or variety of renal morphologic pattern can lead to the same clinical syndrome. The primary role of the renal biopsy is to provide a diagnosis and information about disease activity and chronicity. The systematic approach to native medical renal biopsy includes evaluation of the four compartments of the kidney sequentially (glomeruli, tubules, interstitium, and blood vessels). The diagnosis in renal pathology is an integrated process in which we must analyze all clinical data, light microscopy, immunohistology, and electron microscopy studies for diagnosis. The aim of the article is to describe the handling of the renal tissue in the anatomical pathology laboratory. It also provides the guideline to renal biopsy evaluation and approach to arrive at the diagnosis of glomerular diseases with similar clinical presentations.

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