scholarly journals Prevalence, Characteristics, and Outcomes of Incidental IgA Glomerular Deposits in Donor Kidneys

2020 ◽  
Vol 5 (11) ◽  
pp. 1914-1924 ◽  
Author(s):  
Lillian W. Gaber ◽  
Faiza N. Khan ◽  
Edward A. Graviss ◽  
Duc T. Nguyen ◽  
Linda W. Moore ◽  
...  
Keyword(s):  
Glomerular Deposits

Immunolabelling of glomerular deposits in kidney biopsies routinely fixed in glutaraldehyde

1989 ◽  
Vol 47 ◽  
pp. 910-911
Author(s):  
Ś Lhoták ◽  
I. Alexopoulou ◽  
G. T. Simon
Keyword(s):  
Uv Light ◽  
Kidney Diseases ◽  
Light Chains ◽  
Microscopical Level ◽  
Accurate Identification ◽  
Light Microscopical Level ◽  
Dense Deposits ◽  
Cacodylate Buffer ◽  
Glomerular Deposits ◽  
Prognostic Importance

Various kidney diseases are characterized by the presence of dense deposits in the glomeruli. The type(s) of immunoglobulins (Igs) present in the dense deposits are characteristic of the disease. The accurate Identification of the deposits is therefore of utmost diagnostic and prognostic importance. Immunofluorescence (IF) used routinely at the light microscopical level is unable to detect and characterize small deposits found in early stages of glomerulonephritis. Although conventional TEM is able to localize such deposits, it is not capable of determining their nature. It was therefore attempted to immunolabel at EM level IgG, IgA IgM, C3, fibrinogen and kappa and lambda Ig light chains commonly found in glomerular deposits on routinely fixed ( 2% glutaraldehyde (GA) in 0.1M cacodylate buffer) kidney biopsies.The unosmicated tissue was embedded in LR White resin polymerized by UV light at -10°C. A postembedding immunogold technique was employed

Fibrillary glomerulonephritis or complement 3 glomerulopathy: a rare case of diffuse necrotising crescentic glomerulonephritis with C3-dominant glomerular deposition and positive DNAJB9

2021 ◽  
Vol 14 (2) ◽  
pp. e239868
Author(s):  
Lyle Wesley Baker ◽  
Mahnoor Khan ◽  
Cherise Cortese ◽  
Nabeel Aslam
Keyword(s):  
Rare Case ◽  
Crescentic Glomerulonephritis ◽  
Complement Pathway ◽  
Hepatitis C Infection ◽  
Fibrillary Glomerulonephritis ◽  
Complement 3 ◽  
Nephritic Syndrome ◽  
Mean Diameter ◽  
Alternative Complement ◽  
Glomerular Deposits

Fibrillary glomerulonephritis (FGN) and complement 3 glomerulopathy (C3G) are rare forms of glomerulonephritis with distinct aetiologies. Both FGN and C3G can present with nephritic syndrome. FGN is associated with autoimmune disease, dysproteinaemia, malignancy and hepatitis C infection. C3G is caused by the unregulated activation of the alternative complement pathway. We present a rare case of diffuse necrotising crescentic glomerulonephritis with dominant C3 glomerular staining on immunofluorescence—consistent with C3G—but electron microscopy (EM) findings of randomly oriented fibrils with a mean diameter of 14 nm and positive immunohistochemistry for DNAJB9—suggestive of FGN. To the best of our knowledge, this is the first reported case of FGN to show dominant C3 glomerular deposits. This case report reaffirms the utility of EM in the evaluation of nephritic syndrome and highlights the value of DNAJB9—a novel biomarker with a sensitivity and specificity near 100% for FGN.

Ultrastructural ‘Fingerprint’ in Cryoprecipitates and Glomerular Deposits: A Clinicopathologic Analysis of Fingerprint Deposits

Nephron ◽  
2001 ◽  
Vol 90 (1) ◽  
pp. 37-42 ◽  
Author(s):  
Cheng-Fang Su ◽  
Han-Hsiang Chen ◽  
Jui-Chi Yeh ◽  
Shiu-Ching Chen ◽  
Chuan-Chuan Liu ◽  
...  
Keyword(s):  
Glomerular Deposits

Membranoproliferative Glomerulonephritis and α1-Antitrypsin Deficiency in Children

PEDIATRICS ◽  
1983 ◽  
Vol 71 (1) ◽  
pp. 88-92
Author(s):  
C. Frederic Strife ◽  
George Hug ◽  
Gail Chuck ◽  
A. James McAdams ◽  
Charles A. Davis ◽  
...  
Keyword(s):  
Liver Disease ◽  
Membranoproliferative Glomerulonephritis ◽  
Immunoglobulin A ◽  
Clinical Signs ◽  
Type I ◽  
Complement Protein ◽  
Protein Levels ◽  
Antitrypsin Deficiency ◽  
Glomerular Deposits ◽  
At Deficiency

Two white girls had reduced serum concentration of α1-antitrypsin (α-AT), phenotype ZZ, and liver disease. Hepatocytes exhibited the microscopic criteria of α-AT deficiency. Hypocomplementemia, elevated circulating immune complexes (patient 1), clinical signs of renal disease, and the histologic findings of membranoproliferative glomerulonephritis (MPGN) type I developed. Immunoglobulin A (but not α-AT) was demonstrable immunologically as a component of glomerular deposits in patient 1. Among 53 patients with MPGN but without clinical signs of liver disease, none had Pi type Z. Among 23 patients with phenotype ZZ but without clinical signs of kidney disease, six had abnormal complement protein levels, but the pattern did not resemble that of idiopathic MPGN type I. These results are consistent with the conclusion that MPGN in the two patients reported here is a consequence of their chronic liver disease and is not directly related to the presence of the allelic α-AT variant PiZ.

Neural Epidermal Growth Factor–Like 1 Protein–Positive Membranous Nephropathy in Chinese Patients

2021 ◽  
pp. CJN.11860720
Author(s):  
Guoqin Wang ◽  
Lijun Sun ◽  
Hongrui Dong ◽  
Yanyan Wang ◽  
Xiaoyi Xu ◽  
...  
Keyword(s):  
Membranous Nephropathy ◽  
Clinical Characteristics ◽  
Chinese Patients ◽  
Double Positive ◽  
Immunohistochemistry Staining ◽  
Phospholipase A2 Receptor ◽  
Pathologic Features ◽  
Glomerular Deposits ◽  
Epidermal Growth

Background and objectivesThe neural EGF-like 1 (NELL-1) protein is a novel antigen in primary membranous nephropathy. The prevalence and clinical characteristics of NELL-1–positive membranous nephropathy in Chinese individuals with primary membranous nephropathy are unclear.Design, setting, participants, & measurementsA total of 832 consecutive patients with biopsy-proven primary membranous nephropathy were enrolled. The glomerular expression of phospholipase A2 receptor (PLA2R) and thrombospondin type 1 domain-containing 7A (THSD7A) was screened. Glomerular immunohistochemistry staining for NELL-1 was performed in 43 patients with PLA2R- and THSD7A-negative membranous nephropathy, 31 patients with PLA2R-positive membranous nephropathy, and two patients with PLA2R and THSD7A double positivity. The NELL-1 antibody was also detected in the sera of patients with NELL-1–positive membranous nephropathy by western blot. Clinical and pathologic features were comparable between patients with isolated NELL-1–positive, isolated PLA2R/THSD7A-positive, and triple antigen–negative membranous nephropathy.ResultsAmong the 832 patients with primary membranous nephropathy, 11 of 54 (20%) patients with PLA2R-negative membranous nephropathy had THSD7A-positive membranous nephropathy. NELL-1–positive membranous nephropathy accounted for 35% (15 of 43) of all patients with PLA2R- and THSD7A-negative membranous nephropathy. One patient was double positive for NELL-1 and PLA2R in glomerular deposits and positive for only the PLA2R antibody in the serum. Most patients with NELL-1–positive membranous nephropathy were women. No tumors were found. There were significant differences in the prevalence of IgG subtypes between patients with different antigen positivity. Among patients with isolated NELL-1–positive membranous nephropathy, although 80% (12 of 15) were IgG4 staining positive, the proportion of IgG4 dominance was only 67% (ten of 15).ConclusionsAbout one third of patients who were PLA2R and THSD7A negative were NELL-1 positive in Chinese patients with primary membranous nephropathy. NELL-1–positive membranous nephropathy was more common than THSD7A-positive membranous nephropathy in PLA2R-negative membranous nephropathy.

Hereditary and acquired complement dysregulation in membranoproliferative glomerulonephritis

2009 ◽  
Vol 101 (02) ◽  
pp. 271-278 ◽  
Author(s):  
Veronique Fremeaux-Bacchi ◽  
Christoph Licht
Keyword(s):  
Membranoproliferative Glomerulonephritis ◽  
Age Of Onset ◽  
Current Knowledge ◽  
Treatment Strategies ◽  
Specific Treatment ◽  
Disease Recurrence ◽  
C3 Nephritic Factor ◽  
Dense Deposit ◽  
Glomerular Deposits ◽  
End Stage

SummaryMembranoproliferative glomerulonephritis (MPGN) is a chronic progressive renal disease that is diagnosed on the basis of renal histological features. Several MPGN subtypes have been defined by the localization and composition of glomerular deposits (electron dense, Ig and C3). MPGN II or dense deposit disease (DDD) which is defined by the occurrence of electron dense deposits within the lamina densa of the glomerular basement membrane (GBM) is strongly associated with dysregulation of the alternative complement pathway (AP). However, C3 Nephritic Factor (C3NeF), an autoantibody against the alternative C3 convertase C3bBb, and mutations in regulatory proteins of the AP have also been identified in other subtypes of MPGN and even in glomerulonephritis with mesangial C3 deposits. Clinically, MPGN is characterized by proteinuria (up to nephrotic range) and hypertension, frequent progression to end-stage kidney disease and disease recurrence after renal transplantation. The age of onset varies from childhood to adulthood. In the following we will review our current knowledge of pathogenesis of MPGN and will present a novel classification system of the disease based on pathogenesis rather than on morphology. A better understanding of the pathogenesis of MPGN is crucial for the development of novel, specific treatment strategies.

The Importance of Glomerular Deposits of Von Willebrand Factor in Human Renal Allografts

Renal Failure ◽  
2006 ◽  
Vol 28 (4) ◽  
pp. 315-321 ◽  
Author(s):  
B. Handan Özdemir ◽  
Aylin Sar ◽  
Mehmet Haberal
Keyword(s):  
Von Willebrand Factor ◽  
Renal Allografts ◽  
Glomerular Deposits ◽  
Von Willebrand ◽  
Willebrand Factor

Seropositive PLA2R-associated membranous nephropathy but biopsy-negative PLA2R staining

2020 ◽  
Author(s):  
Jiao Luo ◽  
Wang Zhang ◽  
Cailing Su ◽  
Zhanmei Zhou ◽  
Guobao Wang
Keyword(s):  
Membranous Nephropathy ◽  
Interstitial Fibrosis ◽  
Clinicopathological Characteristics ◽  
Enzyme Linked Immunosorbent Assay ◽  
Double Positive ◽  
Lower Serum ◽  
Tubular Atrophy ◽  
Phospholipase A2 Receptor ◽  
Glomerular Deposits ◽  
Paraffin Tissue

Abstract Background Serum phospholipase A2 receptor (PLA2R) antibody (SAb) and glomerular deposits of PLA2R antigen (GAg) have been tested widely in idiopathic membranous nephropathy (MN). Recently, we noticed a special form of PLA2R-associated MN with positive circulating PLA2R antibody but negative PLA2R deposits in the glomeruli by immunofluorescence on frozen tissue (IF-F). The significance of this form of PLA2R-associated MN is yet to be elucidated. This study aimed to explore the clinicopathological features of these PLA2R-associated MN patients. Methods This study enrolled 229 biopsy-proven PLA2R-associated MN patients with SAb+. SAb was measured by enzyme-linked immunosorbent assay, and GAg was detected by IF-F. These patients were divided into SAb+/GAg+ and SAb+/GAg− groups. Clinicopathological characteristics of SAb+/GAg+ and SAb+/GAg− PLA2R-associated MN patients were compared. PLA2R antigens of 19 SAb+/GAg− PLA2R-associated MN patients were verified by immunohistochemistry on paraffin tissue (IHC-P). Results Among 229 SAb+ PLA2R-associated MN patients, 210 (91.70%) were GAg+ and 19 (8.3%) were GAg−. These 19 SAb+/GAg− PLA2R-associated MN patients presented positive PLA2R deposits by IHC-P. Compared with SAb+/GAg+ PLA2R-associated MN patients, SAb+/GAg- PLA2R-associated MN patients had higher levels of serum PLA2R antibody (P = 0.004), increased proteinuria (P = 0.008), lower serum albumin (P = 0.019), more prominent chronic pathological lesions in terms of glomerulosclerosis score (P = 0.025), interstitial fibrosis score (P = 0.016), tubular atrophy score (P = 0.010) and total renal chronicity score (P = 0.010), and were more likely to be accompanied by focal segmental glomerulosclerosis (P = 0.014). Higher SAb level was associated with the total renal chronicity score (odds ratio per 100 RU/mL, 1.16; 95% confidence interval 1.01–1.33; P = 0.033). Conclusions PLA2R-associated MN patients with seropositive PLA2R antibody but negative PLA2R deposits in the glomeruli by IF-F have higher levels of SAb and worse clinicopathological manifestations compared with their double-positive counterparts. IHC-P can be an alternative technique to reveal PLA2R glomerular deposits.

Atypical Structured Glomerular Deposits: An Immunohistochemical Study

1991 ◽  
Vol 11 (2) ◽  
pp. 151-156 ◽  
Author(s):  
Mitsuru Nakajima ◽  
Tim D. Hewitson ◽  
Douglas C. Mathews ◽  
Ian Forbes ◽  
Priscilla Kincaid-Smith
Keyword(s):  
Immunohistochemical Study ◽  
Glomerular Deposits
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