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KMID : 0882419950480060828
Korean Journal of Medicine
1995 Volume.48 No. 6 p.828 ~ p.835
A Case of Type2 Autoimmune Polyglandular Syndrome
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Abstract
Autoimmune polyglandular syndrome encompasses a number of diseases, ranging from unusual endocrine and nonendocrine disorders to the co-occurrence of common organ specific autoimmune disease. Knowledge of these syndrome is important for several
respects: given the presence of one disease, certain others become more likely(in relatives as well as in the primary patient); there are similarities in the etiology and genetics of the different diseases; and immunologic treatment of one
disease
may
be applicable to others.
We recently experienced type II autoimmune polyglandular syndrome in a twenty-two-year old female patient who had insulin dependent diabetes mellitus associated with diabetic ketoacidosis, Hashimoto's thyroiditis, autoimmune Addison's disease,
primary
gonadal failure and alopecia totalis.
Among the family member, twenty-year-old sister showed primary gonadal afilure.
Evaluation of the histocompatibility leukocyte antigene(HLA) was done in the patient and 4 relatives by two-step polymerase chain reaction(PCR). In HLA typing, the patient showed HLA-A1, B8, DR3, DR4 haplotypes, which had been known to be related
with
the autoimmune polyglancular syndrome.
KEYWORD
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