XXXX : report of four new cases and a review of literature.

@entity1 @entity1952 @entity2011 @entity6 @entity19278 @entity4683 @entity2319 @entity1723 @entity393 @entity2010
The Wolfram, or @entity19278 , syndrome consists of @entity2010 , @entity6 , @entity1952 , and @entity2319 . @entity6 usually occurs as the first manifestation of this syndrome, followed by the development of @entity1952 , @entity393 , and finally @entity2010 . We report on four cases with a review of the literature. The @entity6 occurring in these @entity1 is clinically indistinguishable from classic @entity6 . Two of three @entity1 continue to have measurable C-peptide secretion 8 yr after onset of @entity6 . Two of three @entity1 with @entity19278 had the HLA-DR2 antigen. Combining our cases with those described in the literature, 7 of 11 @entity1 have the HLA-DR2 antigen. The preponderance of the HLA-DR2 antigen in the @entity19278 is different from classic @entity1723 . This is further evidence of the genetic heterogeneity of @entity6 . Although the @entity19278 is rare, it should be considered in @entity6 @entity1 with unexplained @entity1952 and @entity393 or with @entity2011 and @entity4683 in the presence of adequate blood sugar control.
