Σκοπός: 1) Η μελέτη της επίπτωσης της στυτικής δυσλειτουργίας σε ασθενείς με σύνδρομο επικάλυψης ( ΣΑΑΥ και ΧΑΠ ) και η συγκρισή της με την επίπτωση της στυτικής δυσλειτουργίας σε ασθενείς που παρουσίαζαν μόνο ΣΑΑΥ, και 2) η εκτίμηση της βελτίωσης της στυτικής δυσλειτουργίας (ED) σε άνδρες με σύνδρομο επικάλυψης (αποφρακτική άπνοια του ύπνου και χρόνια αποφρακτική πνευμονοπάθεια) υπό θεραπεία με cPAP (συνεχής θετική πίεση αεραγωγών) και βρογχοδιασταλτικά. Υλικό και μέθοδος : Μελετήθηκαν 48 άνδρες με μέσο όρο ηλικίας τα 52.8 + 10 χρόνια με αποφρακτική άπνοια του ύπνου (ΣΑΑΥ) και χρόνια αποφρακτική πνευμονοπάθεια (ΧΑΠ) και παράλληλα στυτική δυσλειτουργία ED. Επίσης 30 άνδρες με στυτική δυσλειτουργία και ΣΑΑΥ χωρίς αποφρακτική νόσο. Έλαβαν την κατάλληλη θεραπεία για αποφρακτική πνευμονοπάθεια (cPAP και βρογχοδιασταλτικά) για 6 μήνες και έπειτα το επίπεδο της στυτικής λειτουργίας (EF) επανεκτιμήθηκε. Η ED θεωρήθηκε ότι βελτιώθηκε εάν ο βαθμός βελτίωσης (score) αυξήθηκε για τουλάχιστον 5 μονάδες σε σύγκριση με αυτό της βασικής τιμής. Οι καθοριστικοί παράγοντες για βελτίωση της ED επίσης εκτιμήθηκαν, καθώς επίσης και ο βαθμός ικανοποίησης του κάθε ασθενή κατά τη διάρκεια της θεραπείας Αποτελέσματα : Η επίπτωση της ED σε ασθενείς με σύνδρομο επικάλυψης ήταν 81,08%. Η επίπτωση της ED σε ασθενείς πάσχοντες από ΣΑΑΥ και ED ήταν 29,17%. Η ED βελτιώθηκε σε 12 ασθενείς (25%), αλλά μόνο τα 2 /3 αυτών ήταν ικανοποιημένοι με τον βαθμό βελτίωσης μετά από θεραπεία. Η βελτίωση της ED συσχετίστηκε θετικά με την ηλικία, δείκτη άπνοιας / υπόπνοιας και αρνητικά με την διάρκεια της ED. Το score της ED, ο κορεσμός της αιμοσφαιρίνης σε Ο2 κατά τη διάρκεια της νύχτας και το BMI δεν συσχετίστηκαν σημαντικά με το βαθμό βελτίωσης της EF. Συμπεράσματα : 1) Η επίπτωση της ED σε ασθενείς με σύνδρομο επικάλυψης είναι μεγαλύτερη συγκριτικά με αυτή των ασθενών που έπασχαν μόνο από ΣΑΑΥ και συγκριτικά με το γενικό πληθυσμό. 2) Η συνήθης θεραπεία σε ασθενείς με ΣΑΑΥ και ΧΑΠ με βρογχοδιασταλτικά και cPAP έχει θετικά αποτελέσματα στην παράλληλη ED στην μειονότητα των ασθενών αυτών. Από τους άνδρες που βελτιώθηκαν, 1/3 από αυτούς δεν ήταν ικανοποιημένοι με το αποτέλεσμα αυτής της θεραπείας. Διαφορετική θεραπευτική αντιμετώπιση ή συνδυασμός αγωγής θα πρέπει πιθανά να χορηγηθεί στους ασθενείς αυτούς. / Aim: 1) To determine the prevalence of erectile dysfunction in patients with overlap syndrome (obstructive sleep apnea and chronic obstructive pulmonary disease) compared with patients suffered from sleep apnea syndrome and erectile dysfunction and 2)To assess the improvement of concurrent erectile dysfunction (ED) in these patients treated with continuous positive airway pressure (cPAP) and bronchodilators Material and Methods: We evaluated 48 men suffering from OSA, COPD and concurrent ED, and 30 men suffered from OSA and ED. They were treated with conventional therapy for pulmonary obstructive disease (cPAP and bronchodilators) for 6 months and then their erectile function status was reassessed. ED was considered as improved, if ED intensity score increased for at least five points compared to that of baseline. Results: The prevalence of ED in patients with overlap syndrome was 81,08%. The prevalence of ED in men with OSA was 29,17%. ED was improved in 12 patients (25%), but only two thirds of them were satisfied with the grade of improvement after treatment. ED improvement was related positively with age and apnea/hypopnea index and negatively with ED duration. ED intensity score, O2 saturation at night and BMI were not significantly related to the outcome of EF improvement. Conclusions: 1) The prevalence of ED in patients with overlap syndrome was higher compared with the prevalence of ED in men with OSA. 2) Conventional treatment for OSA and COPD, has a positive effect on concurrent ED on the minority of patients. The effect is possibly due to the improvement of respiration during sleep with cPAP and of oxygenation with bronchodilators continuously

JGH Open: An open access journal of gastroenterology and hepatology published by Journal of Gastroenterology and Hepatology Foundation and John Wiley and Sons Australia, Ltd. Background and Aim: The United Kingdom-primary biliary cholangitis (UK-PBC) and global primary biliary cholangitis group (GLOBE) prognostic models have been recently developed to predict long-term outcomes in primary biliary cholangitis (PBC). However, these predictive scores have not yet been well evaluated in the U.S. population. Methods: We retrospectively reviewed newly diagnosed PBC patients at the Cleveland Clinic between November 1998 and February 2017. Adverse events were defined as liver transplantation, liver-related mortality, and all-cause mortality. Transplant-free survival (TFS) was estimated using the Kaplan–Meier method. Predictive performances of all prognostic models were evaluated using the C-statistic. Results: We identified 352 patients who used ursodeoxycholic acid therapy. Of them, 311 (88.4%) only had PBC, while 41 (11.6%) were diagnosed with PBC-autoimmune hepatitis overlap. A total of 22 (6%), 47 (13%), and 55 (16%) patients had adverse events within 5, 10, and 15 years after diagnosis, respectively. In patients with PBC only, the C-statistic in predicting 15-year adverse events was 0.75 per GLOBE compared to 0.74 per UK-PBC (P = 0.94), 0.73 per Rotterdam (P = 0.44), 0.66 per Barcelona (P = 0.004), 0.65 per Paris 1 (P = 0.005), 0.62 per Paris 2 (P < 0.0001), 0.60 per Toronto (P < 0.0001), and 0.60 per Mayo (P < 0.0001) scores. Median follow-up was 9.2 years. Ten-year TFS for patients who had optimal versus suboptimal treatment response was 92 versus 74% per Paris 1 (P < 0.0001), 95 versus 79% per Paris 2 (P = 0.0002), 93 versus 65% per Barcelona (P < 0.0001), and 96 versus 68% per Rotterdam (P < 0.0001) risk scores, respectively. Conclusion: In our cohort of PBC patients, the UK-PBC and GLOBE scores were both accurate and reasonably valid prognostic models in the U.S. population.

京都大学 / 0048 / 新制・課程博士 / 博士(医学) / 甲第20987号 / 医博第4333号 / 新制||医||1027(附属図書館) / 京都大学大学院医学研究科医学専攻 / (主査)教授 伊達 洋至, 教授 長船 健二, 教授 一山 智 / 学位規則第4条第1項該当 / Doctor of Medical Science / Kyoto University / DFAM

Submitted by Nadir Basilio (nadirsb@uninove.br) on 2018-07-23T18:30:00Z No. of bitstreams: 1 Julio Cesar Mendes de Oliveira.pdf: 1398345 bytes, checksum: ec1db982cfbc37dacc81fc94295a19b1 (MD5) / Made available in DSpace on 2018-07-23T18:30:00Z (GMT). No. of bitstreams: 1 Julio Cesar Mendes de Oliveira.pdf: 1398345 bytes, checksum: ec1db982cfbc37dacc81fc94295a19b1 (MD5) Previous issue date: 2016-12-15 / Introduction: Currently, chronic obstructive pulmonary disease (COPD) is considered an important cause of morbidity and mortality, and the twelfth most prevalent disease in the world, thus representing a serious public health problem. It is common for patients with COPD to have poor sleep quality. As causes of this poor quality of sleep we highlight nocturnal cough, dyspnea, use of medications such as theophylline and recurrent hypoxemia. The term overlap syndrome is used when we have COPD associated with obstructive sleep apnea (OSA). Overlap syndrome, with a prevalence of 9.5-28%, results in marked hypoxemia during sleep with a greater tendency to hypercapnia, pulmonary hypertension and cor pulmonale, giving these patients a worse prognosis. Objectives: To verify the prevalence of overlap syndrome and to analyze the physiological sleep variables and the quality of life of patients with COPD undergoing a home pulmonary rehabilitation program (PRPD). The study will be conducted in a private clinic, located in the city of Cascavel in the interior of the state of Paraná (PR). Patients will be submitted to clinical evaluation, pulmonary function tests (plethysmography), polysomnography and will respond to quality of life questionnaires, excessive daytime sleepiness and risk for OSA before and after participation in PRPD. Results: The main cause related to the development of COPD was current or previous smoking (83.3% of cases). The most frequent symptom reported was dyspnea (88.8%), followed by productive cough (66.6%) and bronchial secretion (40.0%). By evaluating the clinical history of the cases, a significant number of comorbidities were identified, in addition to COPD, and cardiovascular and neurological diseases were more prevalent. The prevalence of OSA with AHI greater than 5 events was 59.3% (70 patients) and AHI> 15 was 26.2% (31 patients). Conclusion: According to the preliminary data, it was possible to delineate the profile of COPD patients associated with a high prevalence of OSA, with characteristics of an elderly population with multiple comorbidities, suggesting a sleep quality lower than desired. / Introdução: Atualmente, a doença pulmonar obstrutiva crônica (DPOC) é considerada uma importante causa de morbidade e mortalidade, sendo a décima segunda enfermidade mais prevalente no mundo, representando assim um sério problema de saúde pública. É comum pacientes com DPOC apresentarem uma má qualidade do sono. Como causas desta má qualidade do sono destacamos a tosse noturna, dispneia, uso de medicações, como a teofilina e hipoxemia recorrente. O termo overlap syndrome é empregado quando temos a DPOC associada a apneia obstrutiva do sono (AOS). A síndrome da overlap, com uma prevalência entre 9,5-28%, resulta em acentuada hipoxemia durante o sono com maior tendência a hipercapnia, hipertensão pulmonar e cor pulmonale, conferindo a esses pacientes um pior prognóstico. Objetivos: Verificar a prevalência da síndrome de overlap e analisar as variáveis fisiológicas do sono e a qualidade de vida de pacientes com DPOC submetidos a programa de reabilitação pulmonar domiciliar (PRPD). O estudo será realizado em uma clínica privada, localizada na Cidade de Cascavel no interior do estado do Paraná (PR). Os pacientes serão submetidos a avaliação clínica, provas de função pulmonar (pletismografia), polissonografia e responderão a questionários de qualidade de vida, sonolência excessiva diurna e risco para AOS antes e após a participação no PRPD. Resultados: A principal causa relacionada ao desenvolvimento da DPOC foi o tabagismo atual ou prévio (83,3% dos casos). O sintoma mais frequente relatado foi a dispneia (88,8%), seguida de tosse produtiva (66,6%) e secreção brônquica (40,0%). Avaliando-se o histórico clínico dos casos identificou-se, além da DPOC, um número significativo de comorbidades, sendo as cardiovasculares e neurológicas mais prevalentes. A prevalência de AOS com um IAH maior de cinco eventos foi 59,3% (70 pacientes) e IAH > 15 foi de 26,2% (31 pacientes). Conclusão: De acordo com os dados preliminares, pôde-se delinear o perfil de pacientes com DPOC associado a uma alta prevalência de AOS, com características de uma população idosa, com múltiplas comorbidades, sugerindo uma qualidade de sono inferior à desejada.

Background: Anti-NMDA receptor encephalitis (NMDAR-E) is an autoimmune encephalitis (AE) mainly affecting young females. It typically presents with isolated psychiatric symptoms (e.g. depressed mood) at first and neurological abnormalities (e.g. seizures, movement disorders) only develop later. Thus, there is a high risk of overlooking NMDAR-E in patients with preexisting psychiatric illness due to symptom overlap in the prodromal period of the disease when treatment is most effective. Although rare, concomitant or sequential development of a demyelinating disorder is increasingly recognized as an associated disease entity (overlap syndrome), with immediate diagnostic and therapeutic implications. Case presentation: We report a patient with a borderline personality disorder (BPD), which developed NMDAR-E and an overlapping demyelinating disorder with anti-Myelin oligodendrocyte glycoprotein (MOG) -IgG positivity. The initial clinical presentation with predominantly affective symptoms (e.g. mood lability, anxiety, depressed mood) lead us to suspect an exacerbation of the BPD at first. However, acute changes in premorbid behavior, newly developed psychotic symptoms and memory deficits lead us to the correct diagnosis of an AE, which was further complicated by the development of a demyelinating disorder. As a result of impaired illness awareness and psychosis, diagnostic and treatment was difficult to carry out. The symptoms completely remitted after treatment with methylprednisolone 1 g daily for 5 days and 5 cycles of plasma exchange. Conclusions: Continuous awareness for neuropsychiatric clinical warning signs in patients with a pre-diagnosed psychiatric disorder is important for a timely diagnosis. Therefore, we believe that the diagnostic and therapeutic algorithm provided here, for the first time specifically addressing patients with preexisting psychiatric illness and integrating overlap syndromes, can be a useful tool. Moreover, in order to timely perform diagnostics and treatment, judicial approval should be obtained rapidly.

Maladies fréquentes, l’asthme touche 8,4% de la population canadienne âgée de 12 ans et plus et la maladie pulmonaire obstructive chronique (MPOC) touche de 5 à 15% de la population âgée entre 35 et 79 ans. L’asthme et la MPOC peuvent coexister chez un patient. Ce phénomène appelé syndrome de chevauchement de l’asthme et de la MPOC (ACOS) toucherait environ 10% à 55% des patients MPOC. Afin de mieux caractériser l’ACOS et les effets indésirables des médicaments utilisés pour traiter la MPOC, deux études ont été mises en place. Une première étude réalisée auprès de pneumologues a permis de décrire les méthodes de diagnostic, de traitement et d’évaluation de la maitrise de l’ACOS dans la pratique clinique. Les pneumologues rapportent que le diagnostic devrait être basé sur les caractéristiques cliniques, les tests de fonction pulmonaire et l'intuition clinique du médecin. De plus, un corticostéroïde inhalé en combinaison et un bronchodilatateur inhalé à longue durée d’action devraient être introduits rapidement dans le plan de traitement. La deuxième étude a permis d’évaluer la fréquence des effets indésirables chez les patients MPOC/ACOS traités avec un bronchodilatateur inhalé à longue durée d’action. Cette étude démontre que les effets indésirables sont fréquents chez les patients MPOC/ACOS et que la sécheresse buccale et la gorge sèche sont les plus rapportés. Ces résultats démontrent que la mise en place de lignes directrices pour l’ACOS ainsi qu’une meilleure connaissance du profil de tolérance des bronchodilatateurs inhalés à longue durée d’action seraient bénéfiques pour les patients / Asthma and chronic obstructive pulmonary disease (COPD) are frequent in Canada with a prevalence of 8.4% among Canadians aged 12 and over for asthma and a prevalence of 5 to 15% among Canadians aged 35 to 79 years. Asthma and COPD can coexist in a patient and this Asthma-COPD overlap syndrome (ACOS) affects about 10% to 55% of COPD patients. To better understand this syndrome, two studies were designed and conducted. First, focus groups evaluated how pulmonologists diagnose and treat ACOS, and how they assess its control in routine clinical practice. The pulmonologists reported that the diagnosis must be based on clinical characteristics, pulmonary function tests, and clinical intuition. They also agreed that the treatment should target the features of both asthma and COPD. The second study assessed the prevalence of adverse events in COPD/ACOS patients on long-acting inhaled anticholinergics (LAAC) and β2-agonists (LABA) in a real-world setting. This study demonstrates that side effects are common among COPD/ACOS patients. Dry mouth and dry throat were the most reported side effects. These results demonstrate that more explicit guidelines for ACOS and a better understanding of the safety profile of long-acting bronchodilators would be beneficial for patients.