Browsing by Author "Raposo, M"
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- Noninvasive Ventilation: Education and Training. A Narrative Analysis and an International Consensus DocumentPublication . Karim, H; Burns, K; Ciobanu, L; El-Khatib, M; Nicolini, A; Vargas, N; Hernández-Gilsoul, T; Skoczyński, S; Falcone, V; Arnal, JM; Bach, J; De Santo, L; Lucchini, A; Steier, J; Purro, A; Petroianni, A; Sassoon, C; Bambi, S; Aguiar, M; Soubani, A; Taniguchi, C; Mollica, C; Berlin, D; Piervincenzi, E; Rao, F; Luigi, F; Ferrari, R; Garuti, G; Laier-Groeneveld, G; Fiorentino, G; Ho, K; Alqahtani, J; Luján, M; Moerer, O; Resta, O; Pierucci, P; Papadakos, P; Steiner, S; Stieglitz, S; Dikmen, Y; Duan, J; Bhakta, P; Iglesias, A; Corcione, N; Caldeira, V; Karakurt, Z; Valli, G; Kondili, E; Ruggieri, M; Raposo, M; Bottino, F; Soler-González, R; Gurjar, M; Sandoval-Gutierrez, JL; Jafari, B; Arroyo-Cozar, M; Noval, A; Corcione, N; Barjaktarevic, I; Sarc, I; Mina, B; Szkulmowski, Z; Taniguchi, C; Esquinas, ANoninvasive ventilation (NIV) is an increasingly used method of respiratory support. The use of NIV is expanding over the time and if properly applied, it can save patients' lives and improve long-term prognosis. However, both knowledge and skills of its proper use as life support are paramount. This systematic review aimed to assess the importance of NIV education and training. Literature search was conducted (MEDLINE: 1990 to June, 2018) to identify randomized controlled studies and systematic reviews with the results analyzed by a team of experts across the world through e-mail based communications. Clinical trials examining the impact of education and training in NIV as the primary objective was not found. A few studies with indirect evidence, a simulation-based training study, and narrative reviews were identified. Currently organized training in NIV is implemented only in a few developed countries. Due to a lack of high-grade experimental evidence, an international consensus on NIV education and training based on opinions from 64 experts across the twenty-one different countries of the world was formulated. Education and training have the potential to increase knowledge and skills of the clinical staff who deliver medical care using NIV. There is a genuine need to develop structured, organized NIV education and training programs, especially for the developing countries.
- Prevalence of Late-Onset Pompe Disease in Portuguese Patients with Diaphragmatic Paralysis - DIPPER StudyPublication . Guimarães, MJ; Winck, JC; Conde, B; Mineiro, A; Raposo, M; Moita, J; Marinho, A; Silva, JM; Pires, N; André, S; Loureiro, CPompe disease is a rare autosomal recessive neuromuscular disorder caused by acid α-glucosidase enzyme (GAA) deficiency and divided into two distinct variants, infantile- and late-onset. The late-onset variant is characterized by a spectrum of phenotypic variation that may range from asymptomatic, to reduced muscle strength and/or diaphragmatic paralysis. Since muscle strength loss is characteristic of several different conditions, which may also cause diaphragmatic paralysis, a protocol was created to search for the diagnosis of Pompe disease and exclude other possible causes. METHODS: We collected a sample size of 18 patients (10 females, 8 males) with a median age of 60 years and diagnosis of diaphragmatic paralysis of unknown etiology, followed in the Pulmonology outpatient consultation of 9 centers in Portugal, over a 24-month study period. We evaluated data from patient's clinical and demographic characteristics as well as complementary diagnostic tests including blood tests, imaging, neurophysiologic and respiratory function evaluation. All patients were evaluated for GAA activity with DBS (dried blood test) or serum quantification and positive results confirmed by serum quantification and sequencing. RESULTS: Three patients were diagnosed with Pompe's disease and recommended for enzyme replacement therapy. The prevalence of Pompe, a rare disease, in our diaphragmatic paralysis patient sample was 16.8%. CONCLUSION: We conclude that DBS test for GAA activity should be recommended for all patients with diaphragmatic paralysis which, despite looking at all the most common causes, remains of unknown etiology; this would improve both the timing and accuracy of diagnosis for Pompe disease in this patient population. Accurate diagnosis will lead to improved care for this rare, progressively debilitating but treatable neuromuscular disease.