XXXX --an autoaggressive immunopathy?

@entity1 @entity8 @entity3686 @entity177 @entity1200 @entity631 @entity6103
@entity3686 , or @entity3686 , is a pathologically and clinically very inhomogeneous disease, the main features of which are @entity8 combined with deafness of varying degrees. Our experience with 18 @entity1 from 6 families demonstrates the variability in the manifestation of the clinical defect syndromes with @entity8 . In the majority of our cases, @entity177 was diagnosed. 7 @entity1 are undergoing chronic intermittent hemodialysis, and 4 @entity1 survive with a kidney transplant. Following kidney transplantation, histological changes in the sense of a recurrent @entity631 were demonstrated in some cases. The existence of prevailing histocompatibility antigen @entity6103 serves as a clue to the possibility of an underlying @entity1200 . We calculate a relative risk of 6.0. The HLA-D region controls the expression of surface antigens. They are responsible for the communication with lymphoid cells. Defect immune response genes in combination with missing reaction to autoantibodies against @entity631 may lead to an increase of susceptibility for the disease.
