Alport’s Syndrome: Risk of Glomerulonephritis Induced by Anti-Glomerular-Basement-Membrane Antibody after Renal Transplantation

Nephron ◽  
1988 ◽  
Vol 50 (1) ◽  
pp. 34-38 ◽  
Author(s):  
Bharat Shah ◽  
Roy First ◽  
Nina C. Mendoza ◽  
David H. Clyne ◽  
Wesley Alexander ◽  
...  
Keyword(s):  
Renal Transplantation ◽  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Alport's Syndrome ◽  
Alport’S Syndrome

The development of anti-glomerular basement membrane nephritis in two children with Alport's syndrome after renal transplantation: characterization of the antibody target

1989 ◽  
Vol 3 (4) ◽  
pp. 406-413 ◽  
Author(s):  
L. P. W. J. v. d. Heuvel ◽  
C. H. Schröder ◽  
C. O. S. Savage ◽  
D. Menzel ◽  
K. J. M. Assmann ◽  
...  
Keyword(s):  
Renal Transplantation ◽  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Alport's Syndrome ◽  
Alport’S Syndrome

scholarly journals Ultrastructural and immunohistochemical findings in Alport's syndrome: a study of 108 patients from 97 Italian families with particular emphasis on COL4A5 gene mutation correlations.

1998 ◽  
Vol 9 (6) ◽  
pp. 1023-1031
Author(s):  
G Mazzucco ◽  
P Barsotti ◽  
A O Muda ◽  
M Fortunato ◽  
M Mihatsch ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Multidisciplinary Approach ◽  
Genetic Mutation ◽  
Alport's Syndrome ◽  
Col4a5 Gene ◽  
Alport’S Syndrome ◽  
Combined Use ◽  
Definition Of ◽  
Insight Into

A total of 108 patients affected by Alport's syndrome, taken from 97 families, were enrolled in a genetic and ultrastructural study. Sixty-four families (75 patients) were X-linked, seven autosomal recessive, two autosomal dominant, five uninterpretable, and 19 sporadic. The ultrastructural features were consistent with Alport's syndrome in 66, doubtful in 20, and not significant for Alport's syndrome in 22 patients in the X-linked, sporadic, and genetically uninterpretable groups (without significant differences), as well as in the autosomal group. Mutations of the COL4A5 gene were present in 36 patients in the first three groups, without significant differences. More severe mutations were more frequently present in patients with an ultrastructural pattern consistent with Alport's syndrome. Nevertheless, there seems to be no strict correlation between mutation and ultrastructure, because a major rearrangement was found in a patient with no significant lesions, and different morphologic patterns were detected in patients Belonging to the same family. Immunohistochemical investigation into 24 patients for alpha (IV) chains showed that both alpha 3(IV) and alpha 5(IV) were lacking in the glomerular basement membrane of 13 patients (five with mutations) and were expressed in another six (three with mutations and one in the autosomal group). On the contrary, in this study the retained expression of alpha 3(IV) chain was found, despite the lack of alpha 5(IV) in the glomerular basement membrane of five patients (two with mutation). These different patterns could be related to both the type and severity of the COL4A5 mutations. All of the ultrastructural patterns were identified in all three immunohistochemical groups. Ultrastructural features and alpha 5(IV) chain production, even if an expression of a genetic mutation, do not strictly correlate. The combined use of analysis of collagen expression and electron microscopy made it possible to diagnose Alport's syndrome in 92% of the cohort, and therefore this approach is advisable. A multidisciplinary approach is recommended in the study of Alport's syndrome in an attempt to achieve a better diagnostic definition of and insight into the pathogenetic mechanisms.

Immunological Studies on Glomerular Basement Membrane in Alport's Syndrome

1984 ◽  
Vol 67 (s9) ◽  
pp. 38P-38P
Author(s):  
C.O.S. Savage ◽  
C.D. Pusey ◽  
M. Kershaw ◽  
P. Harrison ◽  
J.S. Cameron ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Alport's Syndrome ◽  
Alport’S Syndrome

Allograft Antiglomerular Basement Membrane Glomerulonephritis in a Patient with Alport’s Syndrome

Nephron ◽  
1987 ◽  
Vol 46 (1) ◽  
pp. 43-44 ◽  
Author(s):  
J.L. Teruel ◽  
F. Liaño ◽  
F. Mampaso ◽  
J. Moreno ◽  
A. Serrano ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Alport's Syndrome ◽  
Alport’S Syndrome

scholarly journals Complete atrioventricular block during renal transplantation in a patient with Alport's syndrome: case report

2001 ◽  
Vol 119 (5) ◽  
pp. 184-186 ◽  
Author(s):  
Fábio Ferrari ◽  
Paulo do Nascimento Junior ◽  
Pedro Thadeu Galvão Vianna
Keyword(s):  
Case Report ◽  
Renal Transplantation ◽  
Renal Disease ◽  
Atrioventricular Block ◽  
Conduction System ◽  
Definitive Treatment ◽  
Alport's Syndrome ◽  
Complete Atrioventricular Block ◽  
Alport’S Syndrome ◽  
Complete Atrioventricular

CONTEXT: Patients with Alport's syndrome (causing 5% of end-stage renal disease) have a higher risk of heart conduction abnormalities. OBJECTIVE: To report a case of Alport's syndrome developing complete atrioventricular block during renal transplantation. CASE REPORT: A 21-year-old man with chronic renal failure due to Alport's syndrome was submitted to a renal transplantation under epidural anesthesia and, during the intraoperative period, a complete atrioventricular block was diagnosed and promptly treated with a transcutaneous pacemaker. This extensive sympathetic block can contribute towards disturbances in the heart conduction system, particularly in patients with chronic renal disease in hemodialysis. Even in patients with a normal preoperative electrocardiogram or no conduction system disturbances, some degree of atrioventricular block, including complete atrioventricular block, can occur. In this situation, a transcutaneous pacemaker provides rapid and effective treatment in the operating room, thereby permitting the planning of a definitive treatment.

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