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The Experience of adult cystic fibrosis centre in Lithuania in 2009–2013
Date Issued |
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2014-06-11 |
Background: Most cystic fibrosis (CF) patients receive medical care in the specialized multidisciplinary children and adult CF centres worldwide. Objective: To analyze the patients clinical data of the first Adult CF centre in Lithuania. Methods: Data analysis from CF registry database (CFdbase v.2.33, St. Luc University Hospital, Brussels) from January 2009 to December 2013 was done. Results: Twenty-one CF adult patients were at the end of 2013 (two women died in 2009 and 2012, one man in 2013). CF diagnosis was confirmed genetically in 52% of patients (91% of them had DF508). The youngest patient was 18, the oldest one 32 years. The median age of CF patients was 22.4 (2009), 21.7 (2010), 22.7 (2011), 22.6 (2012) and 23.1 years (2013). The mean BMI was 18.2, 18.5, 18.5, 19.4, 19.8 kg/m2, lung function as mean FEV1 was 50.5%, 63.9%, 59.3%, 75.1%, 57.9% of predicted, respectively. The most prevalent respiratory pathogen was methicillin-sensitive Staphylococcus aureus − 50.0, 38.9, 26.1, 33.3 and 21.4%; there were no methicillin-resistant S. aureus. The rate of Pseudomonas spp. was 14.3%, 22.2%, 26.1%, 12.5%, 14.3%, Burkholderia cepacia complex 14.3%, 11.1%, 13.0%, 12.5%, 7.1%, accordingly. The first cases of Stenotrophomonas maltophilia were identified from 2011. The rate of CF exacerbations requiring hospitalization was 0.8, 0.9, 0.9, 1.2, 0.9 per person per year, accordingly. Our Centre has successful experience with pregnant CF woman − she gave birth to a healthy baby without significant complications. Conclusion: Specialized CF Centre is able to provide a comprehensive medical care for CF patients and Centre care model is advisable in Lithuania as well.