A 5-year-old Korean boy with diagnosis of cutis marmorata telangiectatica congenita had a bluish-red, widespread livedo network over the entire body surface. There was skin atrophy as well as ulceration and crust over some of the lesions. These abnormalities have been associated with mental retardation with abnormal EEG findings, speech disability, defective growth, cleft palate, presence of simian lines and an increase in the atd angles of both palms, diffuse demineralization of bony structure and weakness of the long extensor muscles of both thumbs. Based on our clinical datas and review of world literature, it would seem to be necessary to change the terminology.
To determine laboratory evidence suggesting immunological abnormalities in persons with hemophilia, we evaluated the immunological status of 75 Korean hemophiliacs, seronegative for human immunodeficiency virus (HIV) antibodies, who have been treated only with Korean factor VIII concentrates. From this study, it was shown that Korean hemophiliacs had decreased CD4 levels, increased CD8 levels, and decreased CD4:CD8 ratios. Diminished lymphocyte response to the mitogens, phytohemagglutinin and concanavalin A, and decreased natural killer cell activity were observed in the hemophiliacs. In addition, production of interleukin-II in the hemophiliacs was lower than in the healthy controls. The percentage of B lymphocytes was significantly reduced but the serum levels of immunoglobulin (Ig) G were elevated. However, the serum Ig A and Ig M levels were normal. This study demonstrated a high frequency of immunological abnormalities in HIV antibody negative Korean hemophiliacs treated only with domestic factor VIII concentrates.
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