Effects of sirolimus on Lung function in patients with Lymphangioleiomyomatosis

Background: Lymphangioleiomyomatosis (LAM) is a progressive lungs disease that affects women at reproductive years. Sirolimus inhibits mammalian target of rapamycin (mTOR) and its administration in past studies was hopeful in treatment of patients with LAM. The aim of this study was to evaluate sirolimus therapy on lung function in LAM patients. Methods: We conducted a trial to evaluate the effect of sirolimus on six patients with LAM who had severe or very severe obstructive lung disease, and one-year follow up. Maintenance level of Sirolimus was 10-15 ng/ml. Serial visits (including physical examination, evaluation of signs and symptoms of disease and adverse events due to treatment), spirometry, 6MWT done at baseline 3, 6, 9 and 12 months after. Spirometric parameters walk distance and O2 saturationbefore and after exercise at first and the end of treatment were measured. Results: Four patients had TSC-LAM while the other 2 patients had S-LAM. The mean level of sirolimus was 13 ng/ml after one-year treatment. Mean FEV1 at enrollment and end of study was 1000cc (33 predict) and 1228cc (42 predict) respectively (P=0.674). The mean FVC at baseline and end of study was 1648cc (49 predict) and 1866cc (55 predict) (P=0.996). The mean FEV1/FVC at enrollment and the end of treatment was 58 and 62 respectively (P=0.753). The mean FEF25-75 at first and at the end of treatment was 16 and 26, respectively (P=0.028). The mean walk distance in 6MWT at first and at the end of study was 315 meters (P=0.9). The mean percentage of O2 saturation at rest was 84 and 92 at first and at the end of study (P=0.104). Conclusion: In LAM patients, sirolimus has been shown stabilizeto or improve lung function, rest and exertional O2 saturation. Sirolimus was effective in LAM patients who had severe or very severe physiological disorders

Diagnostic values of bronchodilator response versus 9-question questionnaire for asthma

Introduction: Several studies have investigated different tools for asthma diagnosis in order to reduce the cost and improve its early recognition. The goal of this study is to establish a short questionnaire to be used in practice for asthma screening and compare diagnostic values between this method and spirometric response to bronchodilators.Material and method: 208 patients presenting with chronic stable dyspnea (> 6 months) and definite clinical diagnosis of chronic obstructive pulmonary disease, bronchiectasis, pulmonary fibrosis or asthma, were enrolled. 9 questions out of 43 based on the literature search were selected by regression analysis. Patients were asked to complete the questionnaire and then their spirometric responses to bronchodilators were evaluated. Results: Of all, 53.8% of cases were diagnosed clinically to have asthma. For establishing diagnosis of asthma, the bronchodilator test had 48.2% sensitivity, 78.1% specificity, 72% positive, 56.4% negative predictive values, 2.2 positive, 0.66 negative likeli-hood ratios, and false positive, false negative and accuracy of 21.9%, 51.8% and 62.01%, respectively. The revised 9 questions from the questionnaire had 97.3% sensitivity, 77.1% specificity, 83.2% positive, 96.1% negative predictive values, 4.24 positive,  0.03 negative likelihood ratios, 22.9% false positive, 2.7% false negative and 87.98% accuracy.Conclusions: The 9-question questionnaire had better diagnostic values in defining asthma in patients with chronic dyspnea than reversibility of airway obstruction to salbutamol and can be used as a useful screening test for diagnosis of asthma in clinical practice and for investigational purposes

Holy month of Ramadan and increase in organ donation willingness

Organ shortage is the most significant factor in restricting the activities of trans-plantation systems. We herein report the positive impact of Muslims′ holy month of Ramadan on willingness to donate organs in Iran. Data were derived from the database of Donation Willing-ness Registries, affiliated to the organ procurement unit of Masih Daneshvari Hospital during March 2007 till March 2008. The number of applications for organ donation was compared bet-ween Ramadan and its previous month, and the socio-economic characteristics of the applicants were compared between those who applied in Ramadan and those who did so in the previous month. In addition, the mean number of daily applications was compared between Ramadan and the other months of the same year. A total of 11528 applications for organ donation cards were registered for the Ramadan of 2007 as opposed to 4538 applications in the previous month, sho-wing an increasing rate of 154%. The mean number of daily applications was significantly higher in Ramadan than that of the other months of the same year (P< 0.001). There was also a significant difference in terms of the socio-economic characteristics between the applicants in Ramadan and those in the previous month. The increase in organ donation willingness in Ramadan may be the result of the propagation of altruism by the mass media and religious organizations. Ramadan seems to provide a great opportunity to promote organ donation across the Muslim world

Diagnostic Values of Bronchodilator Response versus 9-Question Questionnaire for Asthma

Introduction: Several studies have investigated different tools for asthma diagnosis in order to reduce the cost and improve its early recognition. The goal of this study is to establish ashort questionnaire to be used in practice for asthma screening and compare diagnostic values between this method and spirometric response to bronchodilators. Material and method: 208 patients presenting with chronic stable dyspnea (> 6 months) and definite clinical diagnosis of chronic obstructive pulmonary disease, bronchiectasis, pulmonary fibrosis or asthma, were enrolled. 9 questions out of 43 based on the literature search were selected by regression analysis. Patients were asked to complete the questionnaire and then their spirometric responses to bronchodilators were evaluated. Results: Of all, 53.8% of cases were diagnosed clinically to have asthma. For establishing diagnosis of asthma, the bronchodilator test had 48.2% sensitivity, 78.1% specificity, 72% positive, 56.4% negative predictive values, 2.2 positive, 0.66 negative likeli-hood ratios, and false positive, false negative and accuracy of 21.9%, 51.8% and 62.01%, respectively. The revised 9 questions from the questionnaire had 97.3% sensitivity, 77.1% specificity, 83.2% positive, 96.1% negative predictive values, 4.24 positive, 0.03 negative likelihood ratios, 22.9% false positive, 2.7% false negative and 87.98% accuracy. Conclusions: The 9-question questionnaire had better diagnostic values in defining asthma in patients with chronic dyspnea than reversibility of airway obstruction to salbutamol and can be used as auseful screening test for diagnosis of asthma in clinical practice and for investigational purposes

The Reality of Inadequate Patient Care and the Need for a Global Action Framework in Organ Donation and Transplantation

Background. Transplant therapy is considered the best and often the only available treatment for thousands of patients with organ failure that results from communicable and noncommunicable diseases. The number of annual organ transplants is insufficient for the worldwide need. Methods. We elaborate the proceedings of the workshop entitled "The Role of Science in the Development of International Standards of Organ Donation and Transplantation,"organized by the Pontifical Academy of Sciences and cosponsored by the World Health Organization in June 2021. Results. We detail the urgency and importance of achieving national self-sufficiency in organ transplantation as a public health priority and an important contributor to reaching relevant targets of the United Nations Agenda for Sustainable Development. It details the elements of a global action framework intended for countries at every level of economic development to facilitate either the establishment or enhancement of transplant activity. It sets forth a proposed plan, by addressing the technical considerations for developing and optimizing organ transplantation from both deceased and living organ donors and the regulatory oversight of practices. Conclusions. This document can be used in governmental and policy circles as a call to action and as a checklist for actions needed to enable organ transplantation as treatment for organ failure.Fil: Domínguez Gil, Beatriz. Ministerio de Sanidad; EspañaFil: Ascher, Nancy L.. University of California; Estados UnidosFil: Fadhil, Riadh A.S.. Weill Cornell Medicine-qatar; QatarFil: Muller, Elmi. University of Cape Town; SudáfricaFil: Cantarovich, Marcelo. Centre Universitaire de Santé Mcgill; CanadáFil: Ahn, Curie. Asian Society of Transplantation; Corea del SurFil: Berenguer, Marina. La Fe University Hospital; España. Universidad de Valencia; EspañaFil: Bušić, Mirela. Ministry of Health of the Republic of Croatia; CroaciaFil: Egawa, Hiroto. Tokyo Women's Medical University; JapónFil: Gondolesi, Gabriel Eduardo. Fundación Favaloro; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Medicina Traslacional, Trasplante y Bioingeniería. Fundación Favaloro. Instituto de Medicina Traslacional, Trasplante y Bioingeniería; ArgentinaFil: Haberal, Mehmet. Başkent University; TurquíaFil: Harris, David. The University Of Sydney; AustraliaFil: Hirose, Ryutaro. Scientific Registry of Transplant Recipients; ArgentinaFil: Ilbawi, André. Organizacion Mundial de la Salud; ArgentinaFil: Jha, Vivekanand. Imperial College London; Reino UnidoFil: López Fraga, Marta. Council Of Europe; FranciaFil: Andrés Madera, Sergio. Secretaria de Gobierno de Salud. Instituto Nacional Central Único Coordinador de Ablación e Implante; ArgentinaFil: Najafizadeh, Katayoun. Shahid Beheshti University Of Medical Sciences; IránFil: O'Connell, Philip J.. The University of Sydney; AustraliaFil: Rahmel, Axel. German Organ Transplantation Foundation; AlemaniaFil: Shaheen, Faissal A.M.. King Fahad Hospital; Arabia SauditaFil: Twahir, Ahmed. Aga Khan University Hospital; KeniaFil: Van Assche, Kristof. Universiteit Antwerp; BélgicaFil: Wang, Haibo. China Organ Transplant Response System; ChinaFil: Haraldsson, Boerje. University Goteborg; SueciaFil: Chatzixiros, Efstratios. Organizacion Mundial de la Salud; ArgentinaFil: Delmonico, Francis L.. Harvard Medical School; Estados Unido