The DRB1*04:02 and DQB1*05:03 alleles are associated with pemphigus vulgaris in our study as well as in various populations. The association with HLA-DRB1*08:04 in our study was confirmed to be specific to this allele and not to linkage disequilibrium to any adjacent gene. The association between HLA-B*57 and pemphigus vulgaris is reported for the first time in the present study.

BackgroundBullous pemphigoid (BP) is an autoimmune disease with bullous vesicles and an incidence of 0.2 to 1.4 per 100,000 inhabitants. Many studies have been published demonstrating the association of pemphigoid with HLA class II system alleles in different populations, however there are no data on the BP, one of the most heterogeneous in the world.ObjectiveTo typify HLA alleles in Brazilians with Bullous pemphigoid.MethodsThe study group included 17 Brazilian patients with a confirmed diagnosis of BP from a hospital in Sao Paulo city, southeast Brazil. DNA was extracted from peripheral blood using Qiagen kits and HLA A, B, C, DR and DQ typing was performed using polymerase chain reaction. The control group was composed of a database of 297 deceased donors from the city of Sao Paulo. The statistical significance level was adjusted using the Bonferroni correction depending on the phenotypic frequencies evaluated for HLA class I (A, B and C) and class II (DRB1, DQB1 and DQA1).ResultsOur findings show that alleles HLA C*17, DQB1*03:01, DQA1*01:03 and DQA1*05:05 are associated with the onset of the disease in the Brazilian population, with relative risks of 8.31 (2.46 to 28.16), 3.76 (1.81 to 7.79), 3.57 (1.53 to 8.33), and 4.02 (1.87 to 8.64), respectively (p<0.005).ConclusionOur data indicate that Brazilian patients with BP present the same genetic predisposition linked to HLA-DQB1*03:01 previously reported in Caucasian and Iranian individuals and our study introduces three new alleles (C*17, DQA1*01:03 and DQA1*05:05) involved in the pathophysiology of BP.

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ORAL PRESENTATIONSpatients were stage Ib, 4 patients were stage IIa, and 3 patients were stage IIb. The tumor stages, feeding vessels, operating time, complications, and recurrence were observed and recorded. Eight patients received preoperative angiographic embolization, and 3 patients received intraoperative external carotid artery clamping. Transnasal or transpterygoid and posterolateral wall of maxillary sinus approaches are used for tumor resection. Results:The mean duration of the surgery was 2 hours. The mean intraoperative blood loss of patients who received preoperative hyperselective embolization was 470 mL, patients who received intraoperative external carotid artery clamping was 510 mL, and patients who did not receive arterial supply blocking was 930 mL. After surgery, CT scan or MR image showed total removal of the tumor was achieved in all patients. No postoperative complications were observed. All patients were followed-up for 9 months to 3 years (mean 1.5 years), and no recurrence was founded. Conclusion:Endoscopic resection of JNA is a difficult but effective operation. The key techniques to remove tumor are bleeding control, drilling-out the bone that the tumor invaded. Endonasal surgery combined with a preoperative embolization of the arterial supply can control blood loss. For small and intermediatesized JNA (Radkowski Ia-IIb), endoscopic surgery is an appropriate choice. If the tumor extends into lateral fossa infratemporalis or deep into the skull base,we do not recommend it. General OtolaryngologyEvidence-Based long-term Outcomes in ESS Martin L. Hopp, MD, PhD (presenter); Narine Vardanyan, MHA; Shawn Nasseri, MD Objective: 1) Analyze appropriateness of endoscopic sinus surgery. 2) Understand the long-term results of endoscopic sinus surgery at an academic institution with an academic and private practice blend of patients using the Sino Nasal Outcome Test ("SNOT 22") statistically validated tool.Method: Included consecutive patients undergoing sinus surgery at CSMC since January 2009 who were able to complete SNOT-22 prior to surgery. Three hundred fifty patients were included from 17 physicians. Patients mailed the postop form 3, 6, and 12 months following surgery. Patients ranged from 22 years to 67 years split between 220 females and 330 males. Results:The results were quite impressive for patients from immediate to long-term (12 month) postoperative results. The results were statistically significant in demonstrating an improvement of symptoms overall and in all 4 subcategories with a univariate analysis demonstrating P values of <.001. These results were very exciting due to the multi-cultural and varied nature of the patients ranging from tertiary care academic referrals to community otolaryngology patients. Conclusion:It was concluded that endoscopic sinus surgery was effective with results reaching statistical significance in demonstrating an improvement of symptoms overall and in all 4 sub-categories with a univariate analysis demonstrating P values of <.001. Scientific findings...

por todos os estimados ensinamentos em otorrinolaringologia, estomatologia e na área de pesquisa. Obrigado pela confiança e paciência em todo o percurso da pós-graduação. Ao Prof. Dr. Luiz Ubirajara Sennes, pelos conselhos e sugestões na etapa final da tese. Por me aceitar no Programa de Pós-Graduação em Otorrinolaringologia da FMUSP e pelo modo como conduz o Programa de Pós-Graduação. Ao Prof. Dr. Ricardo Ferreira Bento, professor titular de Otorrinolaringologia da FMUSP, por ter me aberto todas as portas que precisei, desde a residência médica até os dias de hoje. Ao Dr. Helcio Rodrigues e ao Prof. Dr. Jorge Kalil, sem os quais o trabalho não seria possível. Disponibilizaram o Laboratório de Imunologia do INCOR e auxiliaram em todos os meios possíveis. À Claudia Borba Rosales, do Laboratório de Imunologia do InCor, por auxiliar na extração e tipificação de DNA, além de ter ensinado todo o processo detalhadamente inúmeras vezes. Ao Dr. Raimar Weber, por me incentivar ao estudo de HLA em pacientes com Pênfigo Vulgar e me permitir realizar a continuidade do seu trabalho. Pela sua prontidão em me ajudar em todos os passos da tese. Ao Dr. Azis Arruda Chagury, pelos conselhos, sugestões e parceria na tipificação e extração do DNA, assim como na descrição do processo. Ao Dr. Ali Mahmoud, por ser um grande amigo, pelas experiências compartilhadas na área de Estomatologia, ajuda no desenvolvimento da pesquisa e por disponibilizar imagens que foram utilizadas na tese. Às secretarias Márcia, Luci e Marileide pelo auxílio em todas os momentos que foi preciso. Sempre com muita generosidade e simpatia. Aos funcionários do Laboratório de Imunologia do InCor, do departamento de Patologia e de Dermatologia do HC-FMUSP, pela parceria durante o período de realização desta pesquisa. À Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES) pela concessão de bolsa de estudo de doutorado direto. À Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) pelo financiamento na compra dos kits que viabilizaram o projeto. Aos pacientes dos nossos ambulatórios que se prontificaram a participar do estudo e aceitaram a coleta de sangue.