Lung transplantation for pulmonary fibrosis in dyskeratosis congenita: Case Report and systematic literature review

<p>Abstract</p> <p>Background</p> <p>Dyskeratosis congenita (DC) is a progressive, multi-system, inherited disorder of telomere biology with high risks of morbidity and mortality from bone marrow failure, hematologic malignancy, solid tumors and pulmonary fibrosis. Hematopoietic stem cell transplantation (HSCT) can cure the bone marrow failure, but it does not eliminate the risks of other complications, for which life-long surveillance is required. Pulmonary fibrosis is a progressive and lethal complication of DC.</p> <p>Case presentation</p> <p>In this report, we describe a patient with DC who developed pulmonary fibrosis seven years after HSCT for severe aplastic anemia, and was successfully treated with bilateral lung transplantation. We also performed a systematic literature review to understand the burden of pulmonary disease in patients with DC who did or did not receive an HSCT. Including our patient, we identified 49 DC patients with pulmonary disease (12 after HSCT and 37 without HSCT), and 509 with no reported pulmonary complications.</p> <p>Conclusion</p> <p>Our current case and literature review indicate that pulmonary morbidity is one of the major contributors to poor quality of life and reduced long-term survival in DC. We suggest that lung transplantation be considered for patients with DC who develop pulmonary fibrosis with no concurrent evidence of multi-organ failure.</p

Cost effectiveness of tobacco control policies in Vietnam: The case of population-level interventions

Background: Tobacco smoking is one of the leading public health problems in the world. It is also possible to prevent and/or reduce the harm from tobacco use through the use of cost-effective tobacco control measures. However, most of this evidence comes from developed countries and little research has been conducted on this issue in developing countries. Objective: The objective of this study was to analyse the cost effectiveness of four population-level tobacco control interventions in Vietnam. Methods: Four tobacco control interventions were evaluated: excise tax increase; graphic warning labels on cigarette packs; mass media campaigns; and smoking bans (in public or in work places). A multi-state life table model was constructed in Microsoft Excel to examine the cost effectiveness of the tobacco control intervention options. A government perspective was adopted, with costing conducted using a bottom-up approach. Health improvement was considered in terms of disability-adjusted life-years (DALYs) averted. All assumptions were subject to sensitivity and uncertainty analysis. Results: All the interventions fell within the definition of being very cost effective according to the threshold level suggested by the WHO (i.e. < GDP per capita). Graphic warning labels on cigarette packs was the most costeffective option, followed by excise tax increases, mass media campaigns, public smoking bans and work place smoking bans. If the cost offset was included in the analysis, all interventions would provide cost savings to the government health sector. Conclusions: All four interventions to reduce the harm from tobacco use appear to be highly cost effective and should be considered as priorities in the context of Vietnam. The government may initially consider graphic warning labels and tax increase, followed by other interventions. © 2011 Adis Data Information BV. All rights reserved

Portland Daily Press: July 31,1888

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