Contrasting features of @entity1723 associated with neuroectodermal defects and classical XXXX .

@entity1 @entity1952 @entity66 @entity19278 @entity4106 @entity6 @entity5450 @entity2319 @entity1723 @entity1270 @entity4107 @entity2010
The Wolfram, or @entity19278 , syndrome is a rare @entity66 that is associated with @entity2010 , @entity1723 of an early onset, bilateral @entity1952 and @entity2319 . @entity5450 are usually present as well. We have studied nine @entity1 belonging to five different families. All of the family members were HLA typed (including DR), and islet cell as well as antinuclear antibody determinations were carried out. Although individuals with @entity1723 are very prone to have either HLA- @entity4106 or - @entity4107 antigens, none of our @entity1 had @entity4106 antigens and only one was @entity4107 positive. On the other hand, three of our @entity1 were typed as HLA-DR2 positive. This antigen is uncommon in classical insulin dependent @entity6 . In one of the families, the affected siblings did not share the same HLA haplotype. Islet cell and antinuclear antibodies were not found in any of the cases and six of the @entity1 had a small, but significant, insulin secretory reserve. On the basis of some of the clinical features it was also possible to further distinguish between the @entity19278 and the classical @entity1723 . The differences encountered between classical and @entity1723 --the presence/absence of HLA linkage, HLA-DR2, - @entity4106 and - @entity4107 associations, islet cell or antinuclear antibodies, the tendency to @entity1270 and @entity6 --indicate that their etiopathogenies are triggered by distinct mechanisms.
