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Immunodeficiency with Hyper-lgM (Dysgammaglobulinemia Type I) Accompanied by Aortic Calcification
대동맥 석회화를 동반한 이상 감마글로부린 혈증

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Authors
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
1987-09
Publisher
Seoul National University College of Medicine
Citation
Seoul J Med 1987;28(3):281-286
Keywords
ImmunodeficiencyDysgammaglobulinemiaAortic calcification
Abstract
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.
ISSN
0583-6802
Language
English
URI
http://hdl.handle.net/10371/6448
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Medicine (의학과)The Seoul Journal of MedicineThe Seoul Journal of Medicine Vol. 28 No.3 (1987)
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