RESUMO
PURPOSE: Sinunasal symptoms and chronic rhinusinutitis are common in patients with cystic fibrosis. Cystic fibrosis transmembrane regulator (CFTR) modulators have led to dramatic improvements of respiratory symptoms and quality of life in patients with cystic fibrosis. This study aims to evaluate subjective and objective sinunasal symptoms after start of CFTR-modulator triple therapy. METHODS: 43 patients (n = 6 < 18 years), treated with highly effective CFTR-modulator therapy with elexacaftor-tezacaftor-ivacaftor (ELX/TEZ/IVA) were included, as were 20 controls with cystic fibrosis but without CFTR-modulator therapy (n = 6 < 18 years). All assessed their sinunasal symptoms retrospectively and the intervention group at a mean of 9.3 (2-16) months after start of ELX/TEZ/IVA. RESULTS: Improvements in SNOT-22 overall score from m = 32.7 to m = 15.7 points (p < 0.0001) as well in the nasal, emotional, otologic, and sleep subdomains could be demonstrated in the intervention group. No changes were found in the control group. Children showed lower SNOT-22 scores than adults and a reduction of SNOT-22 total score from m = 9.4 to m = 2.2 (p = 0.25) was found. 8 patients were evaluated by an otorhinolaryngologist before and after start of ELX/TEZ/IVA and showed pronounced objective clinical improvement. CONCLUSIONS: Highly effective CFTR-modulator therapy has a significant positive impact on both subjective and objective sinunasal symptoms in patients with CF and some improvement could be demonstrated in children < 18 years as well.
Assuntos
Regulador de Condutância Transmembrana em Fibrose Cística , Fibrose Cística , Humanos , Adulto , Criança , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Fibrose Cística/complicações , Fibrose Cística/tratamento farmacológico , Qualidade de Vida , Estudos Retrospectivos , Emoções , Benzodioxóis/uso terapêutico , MutaçãoRESUMO
Gestational diabetes mellitus (GDM) is a common complication in pregnancy. The effect of GDM on the cardiovascular system after birth is still unclear. Between August 2015 and December 2018, 205 pregnant women were included in the prospective controlled observational study. Patients with GDM were assigned to the study group (n = 99), whereas (n = 107) healthy women served as controls. Postnatal follow up of their offspring was performed at 12 months of age. All included children (n = 125) underwent a specific standardized protocol including anthropometric data, such as weight, height, body mass index (BMI), blood pressure (BP) recordings and ultrasound measurements of the abdominal aortic intima-media-thickness (IMT). Furthermore, at least 10 min 3-channel electrocardiogram recording was done to evaluate the autonomic nervous system (ANS) by phase rectified signal averaging. There were no significant differences in anthropometric data between the groups, neither in the blood pressure nor in the intima-media-thickness of the aorta abdominals. However, in the study group, significantly lower average acceleration capacity (AAC) (study group -20.10 ± 3.04 ms, control group -18.87 ± 4.00 ms, p = 0.02) was found, indicating ANS activation at one year of age. Further studies are required to determine if these results are persistent and if these findings have long-term effects.
RESUMO
Objectives: Gestational diabetes mellitus (GDM) is a common complication in pregnancy, affecting around 14% of all pregnancies each year. It will likely further increase, as obesity becomes more prevalent. The impact of GDM on cardiovascular changes in pregnant women and her child is still unclear. The aim of the study was to measure the effects of physical activity before pregnancy on the cardiovascular system in patients with GDM in pregnancy. Methods: Two hundred and six pregnant women were included in this observational study. All participants were recruited at the tertiary level teaching University Hospital "Klinikum rechts der Isar" between 28 and 32 weeks gestation. Questionnaires dealing with pre-pregnancy daily and physical activity (PA) were evaluated. The cardiovascular status of the mothers included measurements of the intima-media thickness (IMT) of the carotid arteries. PA level was performed with a standardized 6-min-walking-test. Results: Ninety-nine women with GDM with a mean age of 33.84 (± 4.7) years were examined. One hundred seven healthy pregnant women aged 32.6 (± 4.2) years served as controls. The mean weight in the study group was 73.0 (± 20.3) kg and 61.7 (± 9.5) kg in the control group. Based on the higher weight in the study group, the Body Mass Index (BMI) was also significantly higher than in the control group (26.3 ± 7.1 vs. 21.6 ± 3; p < 0.001). The frequency of PA was significantly higher in the control group (p < 0.001). The objective fitness level was worse in pregnant women with GDM compared to healthy controls (472 vs. 523 m, p < 0.001). PA before and during pregnancy was less performed in the study group (86 vs. 64.5%, p = 0.002; 69 vs. 45.7%, p = 0.003). Women who were physically inactive before pregnancy had a 3-times higher risk to develop GDM compared to active women (OR = 2.67). The IMT was significantly thicker in the study group (0.48 ± 0.042 mm vs. 0.45 ± 0.042) mm; p = 0.006). Conclusion: Physical activity before pregnancy and a lower initial weight reduces the risk of developing GDM and cardiovascular risk factors in pregnancy. The development of prevention programs is certainly necessary.