S-Space College of Medicine/School of Medicine (의과대학/대학원) Dept. of Medicine (의학과) The Seoul Journal of Medicine The Seoul Journal of Medicine Vol. 28 No.3 (1987)
Immunodeficiency with Hyper-lgM (Dysgammaglobulinemia Type I) Accompanied by Aortic Calcification
대동맥 석회화를 동반한 이상 감마글로부린 혈증
- Jin, Dong Kyu; Lee, Hoan Jong; Ahn, Hyo Seop; Choi, Yong; Moon, Hyung Ro; Kim, Chul Woo; Chi, Je G.; Park, Myoung Hee; Whang, Eung Sao
- Issue Date
- Seoul J Med, Vol.28 No.3, pp. 281-286
- Immunodeficiency with hyper Ig-M (dysgammaglobulinemia type 1) is characterized
immunologically by decreased or absent serum levels of IgG, IgA, but elevated or normal
IgM. First described by Rosen et al. in 1961, the syndrome is usually seen in young boys with
recurrent bacterial infection, and autoimmune diseases are the frequent manifestation.
We present a 4 9/12 year old boy who showed imnunologic findings compatible with
immunodeficiency with hyper-IgM and associated with huge dilatation and calcification of the
aorta. We studied the Band T cell function in vitro which suggested the excessive suppressor
function of the patient's T lymphocytes, although special staining of the lymph node did not
show predominant suppressor population.