Behçet's disease (BD) is known to be associated with human leukocyte antigen (HLA) B51 in many different ethnic groups. An increased incidence of HLA-B51 in the patient group has also been reported in a Japanese population. Recently, the B51 antigen has been identified to comprise 21 alleles, B*5101-B*5121. Further, not only HLA-B*5101 but also HLA-B*5108 were found to be relatively increased in the patient groups among Italian and Saudi Arabian populations. Therefore, we performed HLA-B*51 allele genotyping by the polymerase chain reaction-sequencing based typing (PCR-SBT) method in order to investigate whether there is any correlation of one particular B51-associated allele with Japanese BD. Ninety-six Japanese patients with BD and 132 healthy Japanese volunteers were enrolled in this study. As a result, the phenotype frequency of the B51 antigen was confirmed to be remarkably increased in the patient group as compared to the ethnically matched control group (59.4% in patients vs. 13.6% in controls; Pc=0.0000000000098, R.R.=9.3). In the B*51 allele genotyping, 56 out of 57 B51-positive patients were defined as B*5101 and the remaining one was B*5102. In contrast, all of 18 B51-positive normal controls were B*5101. None of the Japanese patients and healthy controls carried the HLA-B*5108 allele. This study revealed that B*51 allelic distribution in Japanese was different from those in Italian and Saudi Arabian populations, and that the significantly high incidence of the HLA-B51 antigen in the Japanese BD patient group was mostly caused by the significant increase of the HLA-B*5101 allele.
Object. Cervical spondylotic myelopathy (CSM) or myeloradiculopathy, frequent in adults with athetoid cerebral palsy, is a serious secondary disability in patients with an existing congenital handicap. Although several surgical procedures have been described for CSM in adults with athetoid cerebral palsy, none has had satisfying long-term results. The object of this study was to evaluate the effectiveness and safety of combined anterior—posterior fusion with wave-shaped rods and its influence on the stability of other spinal segments.Methods. Twenty-three patients with CSM and athetoid cerebral palsy underwent posterior fusion with wave-shaped rods combined with anterior interbody fusion with internal fixation; 20 patients, 17 men and three women, were followed for more than 5 years. This procedure yielded good results. The mean follow-up period was 8.7 years (range 5–17 years). At 1-year follow-up examination, ambulation had improved in 12 patients. Upper-extremity pain, deltoid muscle weakness, and ability to self-feed improved in almost all patients. Myelopathy recurred in one patient 8.5 years after surgery. The mean motion angle at the adjacent level to the fixed segment did not change postoperatively, but the mean motion between C-1 and C-2 increased and slight atlantoaxial subluxation occurred postoperatively in five patients.Conclusions. Combined anterior—posterior fusion can effectively improve neurological function in patients with CSM and athetoid cerebral palsy, even in those with severe involuntary movements. Postoperative rigid external fixation is not required.
It is well known that Behçet's disease (BD) is strongly associated with human leukocyte antigen (HLA) B51 in many ethnic groups. However, there has been no published report as yet with respect to this association among the Iranian people. Furthermore, since it is now known that the B51 antigen can be encoded by 21 alleles, B*5101-B*5121, we performed HLA-B*51 allele typing as well as HLA class I genotyping of 48 Iranian patients with this disease. As a result, the frequency of the B*51 allele was significantly higher (62.1%) in the patient group as compared with the ethnically matched control group (31.8%) (Pc=0.067, R.R.=3.51). In the genotyping of B*51 alleles, 33 out of the 36 B*51-positive patients possessed B*5101 and the remaining 3 carried B*5108. This study revealed that Iranian patients with BD also had a strong association with HLA-B51. In addition, this significantly high incidence of HLA-B*51 was found to be caused by an increase in both the HLA-B*5101 and HLA-B*5108 alleles. However, there was no significant difference in the HLA-B*51 allelic distribution between the patient and control groups.
Anterior interbody fusion without decompression is an effective treatment for cervical OPLL myelopathy that resulted in stable long-lasting conditions.
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