Factors associated with cystic fibrosis mortality before the age of 30: retrospective analysis of a cohort in southern Brazil

The aim of this study was to retrospectively evaluate the factors associated with mortality before the age of 30 in adults with cystic fibrosis (CF) followed up at a referral center in southern Brazil. This study included individuals over 18 years of age. Clinical data related to childhood and the period of transition to an adult healthcare of individuals with CF were recorded, as well as spirometric and mortality data of individuals between 18 and 30 years of age. A total of 48 patients were included in this study, of which 28 (58.3%) were male. Comparing groups, we observed a higher prevalence of homozygosis for the F508del mutation (P=0.028), massive hemoptysis before the age of 18 (P=0.027), and lower values of pulmonary function, forced expiratory volume in the first second (FEV1) (%) (P=0.002), forced vital capacity (FVC) (%) (P=0.01), and FEV1/FVC (%) (P=0.001) in the group that died before age 30. F508del homozygosis, episodes of massive hemoptysis in childhood, and lower FEV1 values at age 18 were related to mortality before age 30 in a cohort of individuals with CF in southern Brazil.

Effects of an early rehabilitation program for adult cystic fibrosis patients during hospitalization: a randomized clinical trial

There is little information on pulmonary rehabilitation in patients with cystic fibrosis (CF) with pulmonary exacerbation. This study aimed to evaluate the effects of an early rehabilitation program on lung function, muscle strength, inflammatory markers, and quality of life in adults with CF hospitalized for pulmonary exacerbation. In this randomized controlled trial, 19 patients were included in the intervention group and 15 in the control group. The intervention group underwent an early rehabilitation program for 14 days after admission. All patients underwent spirometry, one-repetition maximum tests (1RM), and the 6-min walk test, and answered the Revised Cystic Fibrosis Questionnaire (CFQ-R) for quality of life and the International Physical Activity Questionnaire. Serum levels of interleukin and tumor necrosis factor alpha (TNF-α) were measured. In the intervention group, there were increases in 1RM biceps (P=0.009), triceps (P=0.005), shoulder abductors (P=0.002), shoulder flexors (P=0.004), hamstrings (P<0.001), and quadriceps values (P<0.001). In addition, there were improvements in CFQ-R-emotion (P=0.002), treatment burden (P=0.002), vitality (P=0.011), and physical scores (P=0.026), and a reduction in the Borg resting fatigue score (P=0.037). The interleukins levels did not change after the intervention. In adult CF patients with pulmonary exacerbation, early hospital rehabilitation had a significant impact on improving resting fatigue, muscle strength, and quality of life.

Dyspnea perception in cystic fibrosis patients

We evaluated dyspnea perception in cystic fibrosis patients compared with normal subjects, during an inspiratory resistive loading test and 6-min walk test. We also evaluated the correlation between dyspnea scores induced by resistive loads and by the 6-min walk test. In this prospective, cross-sectional study, 31 patients with cystic fibrosis (≥15 years of age) and 31 age-, gender-, and ethnicity-matched healthy volunteers (20 females and 11 males per group) underwent inspiratory resistive loading, spirometry, and the 6-min walk test. As the magnitude of the inspiratory loads increased, dyspnea scores increased (P<0.001), but there was no difference between groups in dyspnea score (P=0.654). Twenty-six (84%) normal subjects completed all the resistive loads, compared with only 12 (39%) cystic fibrosis patients (P<0.001). Dyspnea scores were higher after the 6-min walk test than at rest (P<0.001), but did not differ between groups (P=0.080). Post-6-min walk test dyspnea scores correlated significantly with dyspnea scores induced by resistive loads. We conclude that dyspnea perception induced in cystic fibrosis patients by inspiratory resistive loading and by 6-min walk test did not differ from that induced in normal subjects. However, cystic fibrosis patients discontinued inspiratory resistive loading more frequently. There were significant correlations between dyspnea perception scores induced by inspiratory resistance loading and by the 6-min walk test. This study should alert clinicians to the fact that some cystic fibrosis patients fail to discriminate dyspnea perception and could be at risk for delay in seeking medical care.

Duración del tratamiento adyuvante con tamoxifeno en el cáncer de mama operable / Duration of adjuvant treatment with tamoxifen in patients with resectable breast cancer

Tamoxifen administered inpatients after breast cancer surgert is one of the most effective adjuvant treatments in this pathology. A large number of randomized studies including thousands of patients allowed to quantify the treatment benefits in terms of recurrence, contralateral breast cancer and overall survival rates. The treatment is generally given for more than 2 years. However, the optimal treatment duration is unknown. Uncertainties appeared when adverse effects were described after some years of treatment (eg a higher incidence of endometrial adenocarcinoma). New ongoing multicentric trials will clearly define in the next years the optimal duration of this adjuvant treatment