scholarly journals Anti-glomerular basement membrane disease associated with thin basement membrane nephropathy

Medicine ◽  
2021 ◽  
Vol 100 (20) ◽  
pp. e26095
Author(s):  
Chi Young Jung ◽  
Sun-Jae Lee ◽  
Min-Kyung Kim ◽  
Dong Jik Ahn ◽  
In Hee Lee
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Thin Basement Membrane Nephropathy

Pathologic glomerular characteristics and glomerular basement membrane alterations in biopsy-proven thin basement membrane nephropathy

2019 ◽  
Vol 23 (5) ◽  
pp. 638-649 ◽  
Author(s):  
Yusuke Kajimoto ◽  
Yoko Endo ◽  
Mika Terasaki ◽  
Shinobu Kunugi ◽  
Toru Igarashi ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Thin Basement Membrane Nephropathy

scholarly journals Spectrum of collagen type IV nephropathies: From thin basement membrane nephropathy to Alport syndrome

2008 ◽  
Vol 136 (Suppl. 4) ◽  
pp. 323-326 ◽  
Author(s):  
Alenka Vizjak ◽  
Dusan Ferluga
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Alport Syndrome ◽  
Type Iv Collagen ◽  
Mutation Screening ◽  
Glomerular Sclerosis ◽  
Collagen Type Iv ◽  
Dominant Type ◽  
Thin Basement Membrane Nephropathy ◽  
Type Iv

Alport syndrome and thin basement membrane nephropathy are common causes of persistent familial haematuria. They are associated with various mutations in type IV collagen genes. Mutations in genes, coding for ?5 chain of collagen IV, cause X-linked Alport syndrome, whereas mutations in genes for ?3 and ?4 chains can cause the autosomal recessive and autosomal dominant type of Alport syndrome or benign familial haematuria with thin basement membrane nephropathy. In view of the wide spectrum of phenotypes, an exact diagnosis is sometimes difficult to achieve. Few studies of genotype-phenotype correlations in Alport syndrome have shown that various types of mutations may be a significant predictor of the severity of disease. Histopathologic findings in Alport syndrome vary from normal kidney to nonspecific focal segmental and global glomerular sclerosis with characteristic ultrastructural finding of thickening and splitting of the glomerular basement membrane. Thin basement membrane nephropathy is characterized by diffuse thinning of the glomerular basement membrane on an ultrastructural level, while by light microscopy glomeruli are mostly unremarkable. Because of present limitations of mutation screening techniques, kidney biopsy with mandatory ultrastructural analysis and immunohistochemistry examination for type IV collagen ? chains remains a standard approach for establishing diagnosis and determining the mode of transmission of the disease.

Electron microscopic radioautographic studies on the incorporation of 3H proline in the glomerular basement membrane (GBM) in antistreptococcal-cell-membrane (SCM) injected mice

1984 ◽  
Vol 42 ◽  
pp. 188-189
Author(s):  
R.P. Nayyar ◽  
C.F. Lange ◽  
J. L. Borke
Keyword(s):  
Body Weight ◽  
Cell Membrane ◽  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Mesangial Matrix ◽  
Electron Microscopic ◽  
Group A ◽  
Injection Protocol

Streptococcal cell membrane (SCM) antiserum injected mice show a significant thickening of glomerular basement membrane (GBM) and an increase in mesangial matrix within 4 to 24 hours of antiserum administration (1,2,3). This study was undertaken to evaluate the incorporation of 3H proline into glomerular cells and GBM under normal and anti-SCM induced conditions. Mice were administered, intraperitoneally, 0.1 ml of normal or anti-SCM serum followed by a 10 µC/g body weight injection of 3H proline. Details of the preparation of anti-SCM (Group A type 12 streptococcal pyogenes) and other sera and injection protocol have been described elsewhere (2). After 15 minutes of isotope injection a chase of cold proline was given and animal sacrificed at 20 minutes, 1,2,4,8,24 and 48 hours. One of the removed kidneys was processed for immunofluorescence, light and electron microscopic radioautographic studies; second kidney was used for GBM isolation and aminoacid analysis.

Fibronectin binding to glomerular basement membrane is altered in diabetes

Diabetes ◽  
1987 ◽  
Vol 36 (6) ◽  
pp. 758-763 ◽  
Author(s):  
M. P. Cohen ◽  
R. Saini ◽  
H. Klepser ◽  
L. G. Vasanthi
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Fibronectin Binding

Removal of elevated circulating angiopoietin-2 by plasma exchange – A pilot study in critically ill patients with thrombotic microangiopathy and anti-glomerular basement membrane disease

2010 ◽  
Vol 104 (11) ◽  
pp. 1038-1043 ◽  
Author(s):  
Carsten Hafer ◽  
Jan Kielstein ◽  
Marion Haubitz ◽  
Hermann Haller ◽  
Svjetlana Lovric ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Plasma Exchange ◽  
Critically Ill ◽  
Glomerular Basement Membrane ◽  
Critically Ill Patients ◽  
Plasma Levels ◽  
Vascular Inflammation ◽  
Endothelial Damage ◽  
Healthy Controls ◽  
Angiopoietin 2

SummaryIn critically ill patients, the massive release of angiopoietin-2 (Ang-2) from Weibel-Palade bodies interferes with protective angiopoietin-1 (Ang-1)/Tie2 signalling in endothelial cells, thus leading to vascular inflammation and subsequent organ-dysfunction. We hypothesised that plasma exchange (PE) is efficient for lowering excess Ang-2 levels in critically ill patients with thrombocytic microangiopathy (TMA) or anti-glomerular basement membrane (anti-GBM) disease. Plasma Ang-1 and Ang-2 were measured by immuno-luminometric assays in patients with TMA (n=9) or anti-GBM disease (n=4) before and after up to four PE sessions. Twenty apparently healthy volunteers served as controls. Median (IQR) plasma levels of Ang-2 were markedly increased in patients with TMA (7.3 (2.4–21.1) ng/ml) and anti-GBM disease (5.8 (3.4–7.0) ng/ml) compared to healthy controls (1.0 (0.9–1.4) ng/ml, p <0.001). Moreover, Ang-1 plasma levels were decreased in both, TMA (1.02 (0.62–1.62) ng/ml) and anti-GBM disease patients (0.74 (0.59–3.62) ng/ml) compared to healthy controls (2.5 (1.93–3.47) ng/ ml, p <0.005). During a total of 32 treatments, PE effectively lowered elevated mean (SD) Ang-2 plasma levels by 36.7 ± 19.6 % per treatment (p <0.0001), whereas low Ang-1 plasma levels remained unchanged (0.3 ± 58.5 %; p =0.147). Ang-2 levels declined to almost normal values during ≤4 PE treatments (Friedman´s test p <0.0001). PE is an effective method to remove excess circulating Ang-2. It remains to be elucidated if the removal of Ang-2 is crucial to ameliorate endothelial damage in critically ill patients with severely altered endothelial integrity.

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