Twenty-nine unrelated Japanese patients with insulin dependent diabetes mellitus with juvenileonset (JOD) were HLA typed with special reference to family history.
The following results were obtained.
1) The frequency of BJW 22-2, a Japanese specific subclass of BW 22, was significantly (x2y= 20.7) increased in JOD, as a whole, compared with the control group.
2) The frequency of B 5 was significantly (x2=16.4) decreased in JOD as a whole.
3) Of twenty-nine JOD patients, twelve had a family history of maturity-onset diabetes (MOD), and seventeen had no apparent family history of diabetes mellitus. Among JOD patients with a positive family history of MOD, two with an insidious onset and one with a history of obesity before the onset were found. The incidence of ketonuria and emaciation was less frequent in this group than in the group without a positive family history of MOD.
4) In the group with a positive family history no difference in phenotype frequencies of HLA was found when compared with a control.
5) In the group without a family history, the frequency of BJW 22-2 was significantly (x2=19.7) increased and no patient had B 5. The frequency of a positive response in the leucocyte migration inhibition test, using mitochondrial fraction of islet cell as an antigen, was significantly higher in this group than in the group with a family history.
These results suggest a genetical as well as clinical heterogeneity of so-called JOD.
