Cystic fibrosis – Comparison between patients in paediatric and adult age

AbstractCystic fibrosis (CF) is the most common autosomal recessive disease in Caucasians. Although most cases are diagnosed in childhood, diagnosis in adults is apparently increasing.ObjectiveEvaluate the adult population with CF, comparing patients who were diagnosed before and after 18 years of age.MethodsRetrospective analysis of patients followed in three main medical centres in Portugal in 2012. Comparison of two groups: G1 – patients diagnosed at <18 years and G2 – patients diagnosed at ≥18 years.Results89 adults were identified: 61.8% in G1, 38.2% in G2. Gender distribution was similar in both groups. Average age in G2 was higher (38.3±8.4 vs. 26.8±6.1 years, p<0.001). Respiratory symptoms most frequently led to CF diagnosis in all patients, mainly in adulthood. There was a greater percentage of patients homozygous for the mutation delF508 in G1 (43.6 vs. 8.8%, p=0.02). Respiratory and pancreatic function, and body mass index (BMI) showed a higher severity in G1 (G1 vs. G2: FEV1: 54.6±27.3 vs. 29.9±64.6%, p=0.177; pancreatic insufficiency 72.7 vs. 26.5%, p<0.001; BMI 20.2±3.4 vs. 22.2±4.8, p=0.018). Pseudomonas aeruginosa and methicillin-sensitive Staphylococcus aureus were the most frequently isolated microorganisms. Lung transplantation rate was higher in G2 (20.6 vs. 10.9%, p=0.231) while mortality rate was higher in G1 (0 vs. 3.6%, p=0.261). Hospital admission rate was higher in G1 as well as mortality rate.ConclusionThe results suggest that patients with CF diagnosed in childhood have characteristics that distinguish them from those diagnosed in adulthood, and these differences may have implications for diagnosis, prognosis and life expectancy

Coexistence or Separation of the Superconducting, Antiferromagnetic, and Paramagnetic Phases in Quasi One-Dimensional (TMTSF)2PF6 ?

We report on experimental studies of the character of phase transitions in the quasi-1D organic compound (TMTSF)2PF6 in the close vicinity of the borders between the paramagnetic metal PM, antiferromagnetic insulator AF, and superconducting SC states. In order to drive the system through the phase border P_0(T_0), the sample was maintained at fixed temperature T and pressure P, whereas the critical pressure P_0 was tuned by applying the magnetic field B. In this approach, the magnetic field was used (i) for tuning (P-P_0), and (ii) for identifying the phase composition (due to qualitatively different magnetoresistance behavior in different phases). Experimentally, we measured R(B) and its temperature dependence R(B,T) in the pressure range (0 - 1)GPa. Our studies focus on the features of the magnetoresistance at the phase transition between the PM and AF phases, in the close vicinity to the superconducting transition at T~1K. We found pronounced history effects arising when the AF/PM phase border is crossed by sweeping the magnetic field: the resistance depends on a trajectory which the system arrives at a given point of the P-B-T phase space. In the transition from the PM to AF phase, the features of the PM phase extends well into the AF phase. At the opposite transition from the AF to PM phase, the features of the AF phase are observed in the PM phase. These results evidence for a macroscopically inhomogeneous state, which contains macroscopic inclusions of the minority phase. When the system is driven away from the transition, the homogeneous state is restored; upon a return motion to the phase boundary, no signatures of the minority phase are observed up to the very phase boundary.Comment: 10 figures, 23 page

Occupational Exposure And Occurrence Of Pneumoconioses In Campinas, Brazil, 1978-2003 [exposição Ocupacional E Ocorrência De Pneumoconioses Na Região De Campinas (sp) Brasil, 1978-2003]

Objective: To develop and consolidate a comprehensive database on the occurrence of pneumoconioses in an industrialized region of Brazil, with a special focus on the activities most frequently related to these diseases. Methods: A retrospective, observational study was conducted in order to gather data on cases of pneumoconioses treated at the outpatient clinic of the State University at Campinas Hospital das Clínicas between 1978 and 2003. Individuals diagnosed with pneumoconiosis, based on their occupational history and on chest X-ray findings of abnormalities consistent with interstitial lung disease involving the parenchyma, in accordance with the 1980 and 2000 recommendations of the International Labour Organization, were included in the study. Results: A total of 1147 cases of pneumoconiosis were identified (1075 in males and 72 in females): 1061 cases of silicosis (92.5%); 51 cases of mixed-dust pneumoconiosis (4.45%); 15 cases of asbestosis (1.31%); 13 cases of phosphate rock-related pneumoconiosis (1.13%); and 7 cases of other types of pneumoconiosis (0.6%), including those related to exposure to coal, graphite and hard metals. The most common chest X-ray findings were 1/0, 1/1 or 1/2 profusion and small regular opacities (p, q or r), although 192 patients (16.74%) presented large opacities. There has been a substantial decline in the occurrence of the disease since the 1990s, and the duration of exposure was typically shorter than that observed in a study conducted in the United States. Conclusions: Our findings have been compiled into a comprehensive database for the investigation of pneumoconiosis in an industrialized area of Brazil. These data make it possible to conduct follow-up studies and develop health policies related to occupational respiratory disorders.346367372Corn JK. Historical aspects of industrial hygiene - II. Silicosis. Am Ind Hyg Assoc J. 1980;41(2):125-33(2002) Health Effects of Occupational Exposure to Respirable Crystalline Silica. NIOSH hazard review, , National Institute for Occupational Safety and Health, Cincinnati: Dept. of Health and Human Services, Centers for Disease Control and Prevention, National Institute for Occupational Safety and HealthRosenman, K.D., Reilly, M.J., Henneberger, P.K., Estimating the total number of newly-recognized silicosis cases in the United States (2003) Am J Ind Med, 44 (2), pp. 141-147(2003) Work-Related Lung Disease Surveillance Report, 2002, , National Institute for Occupational Safety and Health, Cincinnati: U.S. Dept. of Health and Human Services, Public Health Service, Centers for Disease Control and PreventionFinkelstein, M.M., Silicosis surveillance in Ontario from 1979 to 1992 (1995) Scand J Work Environ Health, 21 (SUPPL. 2), pp. 55-57Algranti E. Epidemiologia das doenças ocupacionais respiratórias no Brasil. In: Menezes AM. Epidemiologia das doenças respiratórias. Rio de Janeiro: Revier2001. p.119-43International Labour Office. Guidelines for the Use of ILO International Classification of Radiographs of Pneumoconiosis. Occupational Safety and Health Series n. 22 (rev). Genebra: ILO2000Valiante, D.J., Rosenman, K.D., Does silicosis still occur? (1989) JAMA, 262 (21), pp. 3003-3007Mulloy, K.B., Coultas, D.B., Samet, J.M., Use of chest radiographs in epidemiological investigations of pneumoconioses (1993) Br J Ind Med, 50 (3), pp. 273-275Morrone, L.C., Epidemiologia da silicose no estado de São Paulo. Rev Bras (1980) Saúde Ocup, 8 (1), pp. 6-30Nogueira, D.P., Certain, D., Brolio, R., Garrafa, N.M., Shibata, H., Ocorrência de silicose entre trabalhadores da indústria cerâmica da cidade de Jundiaí, SP (Brasil). (1981) Rev Saúde Pública, 15 (3), pp. 263-271Bagatin, E., Jardim, J.R., Nery, L.E., Capitani, E.M., Marchi, E., Sabino, M.O., Ocorrencia de silicose pulmonar na regiao de Campinas - SP (1995) Jornal De Pneumologia, 21 (1), pp. 17-26Oliveira, J.I., (1998) Prevalência da silicose entre os trabalhadores das indústrias cerâmicas do município de Pedreira, , SP, thesis, Campinas: Universidade Estadual de Campinas;Algranti, E., Mendonça, E.M., DeCapitani, E.M., Freitas, J.B., Silva, H.C., Bussacos, M.A., Non-malignant asbestos-related diseases in Brazilian asbestos-cement workers (2001) Am J Ind Med, 40 (3), pp. 240-254Bagatin, E., Neder, J.A., Nery, L.E., Terra-Filho, M., Kavakama, J., Castelo, A., Non-malignant consequences of decreasing asbestos exposure in the Brazil chrysotile mines and mills (2005) Occup Environ Med, 62 (6), pp. 381-389Prowse, K., Allen, M.B., Bradbury, S.P., Respiratory symptoms and pulmonary impairment in male and female subjects with pottery workers' silicosis (1989) Ann Occup Hyg, 33 (3), pp. 375-38