Behcet's disease (BD) has been known to be strongly associated with the human leukocyte antigen (HLA) B51. This B51 association has been confirmed in many different ethnic groups between the Middle East and Japan, and it has been proposed that BD is prevalent in those ethnic groups along the old Silk Route. The hypothesis could be made that B51 molecules are primarily involved in BD development through specific antigen presentation. However, polymorphic analyses of the TNFB gene and Tau-a microsatellite between the HLA-B and TNF genes indicate that the pathogenic gene of BD is not the HLA-B51 gene itself but another gene located around the HLA-B gene. HLA-C genotyping by the PCR-SSP method also suggests that the BD pathogenic gene is not the HLA-C gene itself but other gene located near the HLA-B gene. Recently we sequenced a single contig of 236,822 bp from the MICA gene (58.2 kb centromeric of HLA-B) to 90.8 kb telomeric of HLA-C and identified 8 novel genes designated NOB1-8 (NOB: new organization associated with HLA-B). During the course of the genomic sequence analysis we clarified the genetic structure of the MICA (MHC class I chain-related gene A) gene and found a triplet repeat microsatellite polymorphism of (GCT/AGC)n in the transmebrane (TM) region. Furthermore, the microsatellite allele consisting of 6 repetitions of GCT/AGC (MICA A6 allele) was present at a significantly higher frequency in the BD patient group than in the control group and a significant fraction of B51-negative patients were positive for this MICA A6 allele. These results suggest the possibility of a primary association of BD with MICA rather than HLA-B.
Animal ; Behcet's Syndrome/genetics* ; Genotype ; HLA-B Antigens/genetics* ; HLA-C Antigens/genetics* ; Histocompatibility Antigens Class I/genetics* ; Human ; Mice ; Mice, Transgenic ; Microsatellite Repeats ; Polymerase Chain Reaction ; Polymorphism (Genetics)

A 41-year-old man was referred to our hospital suffering from pyrexia. Echocardiogram showed diffuse severe hypokinesis of the left ventricle. The patient was treated medically under a diagnosis of acute myocarditis and anticoagulation therapy had been started. However a large mobile thrombus and multiple small thrombi were detected in the left ventricle 2 days after admission. Because of the deterioration of his left ventricular function (LVEF 14%), he was treated medically with careful monitoring of the thrombi by echocardiogram. His left ventricular function started to improve 3 days after admission (LVEF 27%), and then surgical removal of the thrombi was performed through left ventriculotomy. His postoperative course was uneventful. LVEF was improved to 60% at discharge. He is doing well without any signs of embolic event at 2 years postoperatively. Left ventriculotomy is one of the useful methods for removal of left ventricular thrombus associated with acute myocarditis, if the procedure is performed during the recovery phase.

From May 1975 to August 1991, 90 patients (56 males and 34 females) underwent Bentall's operations or its modified technique. In our modified technique the coronary ostium is cut out like a button and anastomosed to the aortic graft and aortic grafts are not wrapped by the aortic wall. Preoperative diagnoses were AAE (25 patients, 28%), Stanford type A dissection (19, 21%), Marfan's syndrome (16, 18%), aortitis syndrome (12, 13%), AR+ascending aortic aneurysm (6, 7%), syphilitic aortitis (5, 6%), AS+ascending aortic aneurysm (3, 3%), Valsalva's sinus aneurysm (2, 2%) and other diseases (2, 2%). The hospital mortality rate was 17% (15/90) for all cases. The hospital mortality for aortic dissection (37% (7/19)) and reoperation cases (75% (3/4)) were very high. There were 10 cases of late death and the 10 year actuarial survival rate was 66.3%. Among 11 cardiovascular events which occured in the late phase, 5 were dissection at other aortic sites in the type A dissection and Marfan syndrome cases, and 3 were pseudoaneurysm formation at the site of coronary or the aortic anastomosis in the aortitis syndrome cases, and a detachment of the composite graft in the Marfan's syndrome cases. The 10-year event-free rate was 92.0% for non-specific AAE, 68.8% for aortitis syndrome, 61.9% for Marfan's syndrome and 47.3% for Stanford type A dissection. Non-specific AAE had excellent long-term results, but Marfan's syndrome and dissection had poor results. The button technique for coronary reconstruction is effective for all cases and its long term results are good, but, even with this technique, coronary pseudo-aneurysm occured in cases of aortitis syndrome.