We recently identified a trinucleotide repeat polymorphism, (GCT)n, within the transmembrane (TM) segment of the human MHC class I MICA gene (MHC class I chain-related gene A). Five distinct alleles (A4, A5, A5.1, A6, A9) corresponding to 4, 5, 5 with one nucleotide insertion, 6 and 9 repetitions, respectively, have been detected in various HLA-homozygous B cell lines. Here we present allele frequencies for this trimeric short tandem repeat (STR) in 604 unrelated individuals collected from nine human populations (Japanese, Northern Han, Hui, Uygur, Kazakhstan, Iranian, Saudi Arabian, Greek and Italian) determined using the polymerase chain reaction (PCR) combined with fluorescent-based automated fragment detection technology. All alleles were present in each population, but allelic distributions varied from one population to another. No new alleles (such as A7 or A8) were identified. The evolutionary and structural significance of these data as well as the potential application to forensic medicine is discussed.
Objective: To investigate the clinical features of ocular lesions in Behçet's disease in different countries. Methods: A descriptive questionnaire survey was performed. Results: 25 eye centres in 14 countries returned questionnaires on prevalent cases in 2006. Clinical data were analysed on 1,465 patients with ocular lesions. Recurrent oral aphthous ulcers were reported in 94.5%, skin lesions in 69.5% and genital ulcers in 61.4%. Most of the patients had bilateral and recurrent intraocular inflammation. Poor visual acuity was seen in 18.9% in women, but 24.8% in men (p,0.01). Panuveitis was seen more in men than in women (p,0.01). 23% of the patients had visual acuity equal to or worse than 20/ 200 at the final visit. The patients with poor vision were more frequently in India, Iran and Japan than in other countries (p,0.01). Conclusions: We report the largest contemporary international case series of patients with ocular involvement in Behçet's disease. Panuveitis was significantly more frequent in men than women, and men tended to have a worse visual prognosis. There were some differences in the clinical pattern of Behçet's disease in different countries. Despite modern treatment, the disease still carries a poor visual prognosis with one-quarter of the patients blind. B ehçet's disease is a multisystem disorder characterised by recurrent oral aphthous ulcers, skin lesions, genital ulcers and ocular lesions. The disease often leads to blindness in severely affected individuals. It is most prevalent between the second and fourth decades of life. Behçet's disease is found predominantly between East Asia and the Mediterranean basin, and is uncommon in the American continents, Oceania and sub-Saharan Africa.1 The distribution of uveitis and intraocular inflammation may differ in different regions of the world. In Japan, Behçet's disease is one of the three most frequent diagnoses in patients with uveitis.2 The highest prevalence rate of the disease has been reported from Turkey.3 This disease is strongly associated with the major histocompatibility complex antigen HLA-B51, first reported in 1973. 4 Populations with a high prevalence of HLA-B51 lie predominantly north of the equator, spanning Japan and Western Europe between 30˚and 45˚N. 5 The frequency of ocular involvement in patients is thought to be between 50 and 70%.6-10 The characterisitic ocular feature is a relapsing uveitis, which may involve the anterior segment, posterior segment or both. The classification of the patient's uveitis is important both therapeutically and prognostically, because those lesions affecting the posterior part of the eye tend to be persistent and blinding.11 The disease may be more severe in men than in women. 12In the present study, we examined the differences in ocular features in Behçet's disease between regions and ethnic groups on a worldwide scale and retrospectively analysed the clinical features, ocular manifestations, visual outcomes and complications in more than 1400 Behçet's patients with ocular involvement. METHODSD...
These data suggest that transvitreal endoresection of posterior uveal melanoma is an acceptable management option to conserve the globe and functional vision in selected patients. Distant metastasis is an infrequent event in this modality of treatment.
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.
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