PubMed · 15658439
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Yutaka Yatomi. 2004. [Thrombomodulin].. https://pubmed.ncbi.nlm.nih.gov/15658439/
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PURPOSE OF REVIEW: In this review, we aimed to highlight recent findings from "-omics" studies in Behçet's disease. RECENT FINDINGS: Recent genomic studies in Behçet's disease identified possible risk loci associated with Behçet's disease related uveitis, neurologic involvement and gastrointestinal involvement. Additionally, sex-specific genetic effects were determined in Behçet's disease. Transcriptomic analyses of immune cells in Behçet's disease revealed that key inflammatory pathways such as NF-κB and MAPK have roles in Behçet's disease pathogenesis. Proteomic studies have highlighted the role of immune cell derived extracellular vesicles and identified potential biomarkers for vascular involvement and examined HLA I-bound immunopeptidomes. Metabolomics studies are still limited, but recent research has pointed to alterations in fatty acid metabolism and lipid profiles in Behçet's disease patient. SUMMARY: Omics studies have gained importance in the field of Behçet's disease through the generation of large data sets and efforts to extend their application are intensifying. These studies can provide opportunities for understanding Behçet's disease pathogenesis when they lead to testable hypotheses. Current challenges include the choice of appropriately homogeneous patient and control groups, effective data management and sharing, high cost and a rapidly increasing gap between the wealth of observational data generated and the relative paucity of controlled experimental efforts that could lead to mechanistic understanding.
Behçet's disease is a multisystemic, recurrent, inflammatory disorder, which has a three-symptom complex comprising uveitis, oral aphtae and genital ulcerations. It is rare in childhood. The prevalence of neurologic involvement in BD is range of 10-49%, and shows a wide spectrum from isolated headache to subacute encephalopathy and severe psychosis. We report a 12-year-old Behçet's disease patient with secondary pseudotumor syndrome due to cerebral vein thrombosis and aim to review the literature.
Adamantiades-Behçet disease (ABD) is a chronic multisystemic vasculitis that is able to affect any human organ or system. Recurrent oral ulcers are a very important clinical sign. ABD is a worldwide pathology, which prevalence varies according to the population and geographic location. Although ABD has been known for ages, its aetiology remains an enigma. Genetic, immunological and microbiological factors have been associated. A wide spectrum of clinical manifestations (oral, genital, cutaneous, ocular, neurological, vascular and gastrointestinal) and an unpredictable evolution with repeated periods of exacerbation and remission are the most representative aspects of this pathology. The complex treatment of ABD requires a deep multidisciplinary cooperation; therefore, there is an extensive development of new therapeutic agents that have improved the prognosis of ABD. In this review were analysed the main etiological, clinical and therapeutic aspects of the disease.