Exacerbations aiguës au cours de la fibrose pulmonaire idiopathique [Acute exacerbations of idiopathic pulmonary fibrosis].

Although generally considered as a slowly evolving disease, idiopathic pulmonary fibrosis (IPF) is also characterized by episods of rapid deterioration with worsening of dyspnea and hypoxemia, and new ground glass opacities at imaging. These events called "acute exacerbations" (AE) are responsible for half of all deaths in IPF. Pathophysiologic mechanisms of AE are poorly understood. The effectiveness of corticosteroids and immunosuppressive agents appears limited. The mortality of AE is 60-70%. Preventing or controlling AE could improve the overall prognosis of IPF. AE also exist in other interstitial lung diseases

Bosentan and/or Sildenafil for non-operable chronic thromboembolic pulmonary hypertension

Objectives: To evaluate outcome of patients treated "off-label" by bosentan and/or sildenafil for chronic thromboembolic pulmonary hypertension (CTEPH). Patients and methods: Since 2003, 18 patients (mean age 69 ± 11 years) have been treated with bosentan and/or sildenafil for CTEPH (mean pulmonary arterial resistance 8.1 ± 3.7 U Wood) in Lausanne University Hospital, with a follow-up of at least 12 months. Sixteen of them were inoperable because of distal disease and/or age or significant co-morbidities and 2 had persistent or recurrent pulmonary hypertension despite surgery. Efficacy of treatment was evaluated by comparison of New York Heart Association functional class (NYHA), six-minute walk test (6-MWT) and serum levels of N-terminal-pro brain natriuretic peptide (NT pro-BNP) at baseline (T0) and at 12 months (T12). Wilcoxon rank test was used for statistics. Results: At T0, median NYHA class was III (range II-IV), 6-MWT was 348 meters (5 and 95 centiles:0, 539) and NT pro-BNP was 387 mmol/l (58, 3508). At T12, 11 patients were treated with bosentan, 5 with sildenafil, 1 with inhaled Iloprost (because of failure of the two other treatments) and 1 with a combination of sildenafil and Iloprost. NYHA had improved in 10 patients, remained stable in 7 and worsened in 1 (median decrease 0.5 (-2; 0.2) p = 0.013). Six-MWT improved by a median of 15 meters (-142, +270) (p = 0.047) and NT pro-BNP decreased by a median of 65 mmol/l (-2988, +187) (p = n.s.). Among the 10 patients with a follow-up of 2 years or longer, two thirds remained stable and one third had worsened at 24 month. Treatments were well tolerated and only one patient had significant side effects (cutaneous reaction to bosentan) necessitating a switch to another treatment. Conclusion: In agreement with published data, bosentan and sildenafil improved functional status (NYHA, 6-MWT) and haemodynamics (NT pro BNP) in our patients with inoperable CTEPH. However these medications should not be used as substitute for surgery when the latter is applicable

Genetic diversity among Brazilian soybean cultivars based on SSR loci and pedigree data

In this study, simple sequence repeats (SSR) loci and pedigree data were used to investigate the genetic relationship in a group of 168 Brazilian soybean cultivars. Eighteen SSR loci produced an average of 5.06 alleles and a mean gene diversity of 0.58 for the cultivars studied. Genetic distance (GD) was determined using the modified Roger's Wright distance, and a final dendrogram was in agreement with the cultivar pedigree. A distance matrix based on the coefficient of parentage scores was also generated for the cultivars, which ranged from 0 to 1, with a mean of 0.18, whereas SSR-based genetic similarity (1- GD) ranged from 0.01 to 0.90, with a mean of 0.25. Mantel's Z test showed that the similarity matrices generated from both the data sets were low, but significantly correlated (r = 0.31, p<0.001). The results showed that SSR data and pedigree analyses could help to quantify more accurately the degree of relationship among the soybean cultivars.<br>Locos microssatélites e dados de genealogia foram utilizados para avaliar a diversidade genética de um grupo de 168 cultivares brasileiras de soja. Os dezoito locos utilizados apresentaram em média 5,06 alelos por loco e coeficiente de diversidade genética médio de 0,58. O dendrograma final resultante da matriz de distância genética de Roger modificado por Wright, apresentou boa concordância com a ancestralidade dos grupos formados. Também foi estimado os coeficientes de parentesco entre as cultivares, sendo observada variação de 0 a 1 com média de 0,18, enquanto que as similaridades para os locos microssatélites (1- GD) variou de 0,01 a 0,90 com média de 0,25. A correlação entre as duas matrizes obtidas determinada pelo teste Z de Mantel apresentou valor baixo, 0,31, mas significativo (p<0,001). Os resultados obtidos sugerem que os locos microssatélites aliados às informações de genealogia proporcionam melhor análise da diversidade genética de cultivares de soja