scholarly journals Comparison of alpha5(IV) collagen chain expression in skin with disease severity in women with X-linked Alport syndrome.

1998 ◽  
Vol 9 (8) ◽  
pp. 1433-1440
Author(s):  
K Nakanishi ◽  
K Iijima ◽  
N Kuroda ◽  
Y Inoue ◽  
Y Sado ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Renal Disease ◽  
Disease Severity ◽  
End Stage Renal Disease ◽  
Alport Syndrome ◽  
Expression Ratio ◽  
Female Patients ◽  
Stage Renal Disease ◽  
End Stage ◽  
Epidermal Basement Membrane

X-Linked Alport syndrome is caused by mutations in the type IV collagen alpha5 chain gene. Male patients usually develop end-stage renal disease, whereas female patients have more variable phenotypes from asymptomatic hematuria to end-stage renal disease. The variable phenotypes in female patients may be attributable to different X-chromosome inactivation patterns. Therefore, the correlation between disease severity and the degree of alpha5 chain expression in the epidermal basement membrane of female patients with X-linked Alport syndrome was examined. To estimate the disease severity in X-linked Alport syndrome, the ratios of protein to creatinine in single voided urine samples were used. Expression of the alpha5 chain in the epidermal basement membrane was examined by an indirect immunofluorescence method using an anti-alpha5 chain monoclonal antibody. A total of 25 female patients with X-linked Alport syndrome from 17 families was examined. Multiple regression analysis using disease severity as the response variable, and age, family history of nephritis, female and male family history of end-stage renal disease, serum creatinine concentration, and alpha5(IV) expression ratio in the epidermal basement membrane as explanatory variables showed that only alpha5(IV) expression ratio was a significant factor, and that it showed a highly significant negative association with disease severity (adjusted r2=0.71, P=0.0001). These findings suggest that variable alpha5 chain expression, possibly caused by different X-inactivation patterns, is responsible for the variable disease severity in female patients with X-linked Alport syndrome, and that immunohistochemical examination of alpha5 chain expression in the epidermal basement membrane may be a simple and useful method for predicting patient outcome.

scholarly journals Anti-glomerular basement membrane antibody disease is an uncommon cause of end-stage renal disease

2013 ◽  
Vol 83 (3) ◽  
pp. 503-510 ◽  
Author(s):  
Wen Tang ◽  
Stephen P. McDonald ◽  
Carmel M. Hawley ◽  
Sunil V. Badve ◽  
Neil C. Boudville ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Renal Disease ◽  
Glomerular Basement Membrane ◽  
End Stage Renal Disease ◽  
Stage Renal Disease ◽  
End Stage

Measuring disease severity in patients with end-stage renal disease: validity of the Craven et al. ESRD Severity Index

1995 ◽  
Vol 25 (1) ◽  
pp. 189-193 ◽  
Author(s):  
K. W. Griffin ◽  
R. Friend ◽  
N. K. Wadhwa
Keyword(s):  
Renal Disease ◽  
Disease Severity ◽  
End Stage Renal Disease ◽  
Burden Of Illness ◽  
Severity Index ◽  
Entire Group ◽  
Psychological Burden ◽  
Severity Scores ◽  
Stage Renal Disease ◽  
End Stage

SynopsisThe validity of a recently developed measure of disease severity, the End-stage Renal Disease Severity Index (Craven et al. 1991) was examined in haemodialysis (HD) and continuous ambulatory peritoneal dialysis (CAPD) patients (total N = 82). Scores on the ESRD Severity Index were compared with three commonly identified components of disease severity: physiological indices of severity, functional status, and psychological burden of illness. For the entire group of subjects, scores on the ESRD Severity Index were negatively associated with functional ability and positively related to physiological severity. ESRD Severity Index scores showed a weaker relationship with psychological burden of illness which depended in part on treatment mode. Disease severity scores were positively related to depression in CAPD patients but not in HD patients. These findings suggest that the ESRD Severity Index is a valuable research tool with construct validity.

scholarly journals The Genetics, Current Research, and Future Treatment of Alport Syndrome

2017 ◽  
Vol 6 (1) ◽  
pp. 1-7
Author(s):  
Elise Alexandra Kikis ◽  
Emily Holland Williams
Keyword(s):  
Basement Membrane ◽  
End Stage Renal Disease ◽  
Alport Syndrome ◽  
Autosomal Recessive ◽  
Type Iv Collagen ◽  
Autosomal Dominant ◽  
Basement Membranes ◽  
Type Iv ◽  
Stage Renal Disease ◽  
End Stage

Alport syndrome is a type IV collagen disease that affects the glomerular basement membrane of approximately one in every 5000 people. The disease was first described by A. Cecil Alport in 1927 as “a dominantly inherited hereditary nephritis.” The three genotypes of the disease are X-linked dominant, autosomal recessive, and autosomal dominant. The X-linked dominant genotype is the most common, accounting for 80% of all cases of Alport syndrome, affecting mainly men. The autosomal recessive and autosomal dominant types affect men and women equally. Alport syndrome is caused by mutations on the COL4A3, COL4A4, and COL4A5 genes, which code the ?3, ?4, and ?5 (IV) chains that make up type IV collagen molecules, an important component of basement membranes. Thus, Alport syndrome results in malformed basement membranes, with symptoms including renal impairment, hematuria, bilateral sensorineural hearing loss, and an abnormal structure of the glomerular basement membrane. Alport syndrome also often progresses to end-stage renal disease, especially in men with X-linked Alport syndrome. At this point, there is no cure for Alport syndrome. However, there are many successful treatments for its symptoms. Angiotensin-converting enzyme (ACE) inhibitors are often given to patients in the early stages of Alport syndrome. For patients with end-stage renal disease, dialysis or kidney transplants are considered the best course of action.

scholarly journals Potential Hepatoprotective Role of Galectin-3 during HCV Infection in End-Stage Renal Disease Patients

2017 ◽  
Vol 2017 ◽  
pp. 1-8 ◽  
Author(s):  
Ruzica Lukic ◽  
Nevena Gajovic ◽  
Ivan Jovanovic ◽  
Milena Jurisevic ◽  
Zeljko Mijailovic ◽  
...  
Keyword(s):  
Renal Disease ◽  
Disease Severity ◽  
End Stage Renal Disease ◽  
Chronic Renal Disease ◽  
Hcv Infection ◽  
Galectin 3 ◽  
Stage Renal Disease ◽  
End Stage ◽  
Esrd Patients

Hepatitis C virus infection (HCV), one of the greatest causes of liver disease, is a frequent complication in patients with end-stage renal disease (ESRD) on dialysis. ESRD is defined as decreased glomerular filtration and also accompanied by impaired function of the immune system. Galectin-3 is aβ-galactoside-binding lectin, involved in various biological processes including pathogenesis of chronic renal disease. The aim of our study was to estimate disease severity in ESRD HCV+patients and analyze the serum concentrations of IL-1β, IL-4, IL-23, and IL-6; anti-HCV antibodies; and galectin-3. Also, we attempted to determine potential correlation between galectin-3 level and parameters of disease severity ALT and AST. Our results showed decreased levels of ALT and AST (p=0.00), demonstrating less liver destruction in ESRD HCV+patients in comparison to HCV+patients. Increased levels of IL-6 (p=0.03) implicate a hepatoprotective role of IL-6 in these patients. Also, level of galectin-3 (p=0.00) in the serum of ESRD HCV+patients was higher than that of HCV+patients. This alteration was accompanied with negative correlation between galectin-3 and AST and ALT, respectively (p=0.029;p=0.033). The presence of increased systemic levels of IL-6 and Gal-3 in ESRD HCV+patients may be an attempt to counteract or limit ongoing proinflammatory processes and to downregulate chronic inflammation, suggesting the new aspects of HCV infection in ESRD patients.

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