Bronchiectasis, a chronic lung disease characterized by irreversible airway destruction, has gained increased attention in recent years. This article highlights the progress made in bronchiectasis ...research, including molecular, translational, and clinical studies, as well as the development of guidelines. World Bronchiectasis Day was established to raise awareness about the disease and address the unmet needs of patients. The article discusses the vicious vortex theory of bronchiectasis pathogenesis, which involves airway dysfunction, chronic infection, inflammation, and structural damage. Targeting all components of the vicious vortex is crucial for treating and preventing disease progression. The article presents several studies that focus on infection, inflammation, and structural damage in bronchiectasis. It also explores the association between bronchiectasis and other respiratory conditions, such as COPD. The authors emphasize the importance of a multidisciplinary approach and global efforts to address the challenges of bronchiectasis.
People with bronchiectasis frequently request specialist advice to prepare for travelling, but there are few publications providing advice on safe travel with bronchiectasis. There is a need for ...recommendations on adapting everyday treatment to the requirements during travelling.
A panel of 13 patient volunteers formulated questions regarding different aspects of travelling, including safety of travel, maintaining regular treatment during travel, and dealing with deterioration while away. Patient input was used to derive a questionnaire and circulated among a panel of bronchiectasis experts. Where 80% or more experts agreed on a response, a recommendation was made.
A total of 26 bronchiectasis experts answered the questionnaire. Recommendations were made on safety of travel, choice of destinations and activities, choice of travel insurance, carrying medications and devices, maintaining regular treatments in transport, documentation to be provided and oxygen requirements. Some statements did not reach an 80% agreement; in many cases these statements may be valid for some, but not all bronchiectasis patients.
The general agreement was that it is considered safe for most people with bronchiectasis to travel. Careful planning and preparation with robust communication between patients and their healthcare provider prior to travel for different scenarios is fundamental to a successful journey.
At interview in the emergency room, the patient reported no periods of lengthy immobilisation—such as a long-haul flight—and said she had not used any hormonal contraceptives. ...upon review of the ...CT scan, we suspected that the filling defects were due to abnormal flow in the pulmonary arteries of the right upper lobe, rather than a pulmonary embolus. 2 days later, a repeat pulmonary CT angiogram showed normal filling of the right pulmonary artery (figure) and coronary CT angiography, with enhanced contrast flow, showed normal coronary arteries. The systemic pressure in the bronchial arteries may cause slowing and even reversal of blood flow in the lower pressure pulmonary arteries leading to affected regions, with uneven contrast filling on imaging.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
36.
Fertility and Pregnancy in Cystic Fibrosis Shteinberg, Michal; Taylor-Cousar, Jennifer L.; Durieu, Isabelle ...
Chest,
December 2021, 2021-12-00, 20211201, Volume:
160, Issue:
6
Journal Article
Peer reviewed
People with cystic fibrosis (pwCF) have experienced increased survival and wellbeing in recent decades, such that more than half of those living with CF are adults. Consequently, sexual and ...reproductive health is increasingly important for pwCF, because many are considering parenthood. Most men and some women with CF (wwCF) will have reduced fertility, which in both sexes is multifactorial. However, unplanned pregnancies in women are not rare, and contraception and its interaction with CF complications need to be addressed by the CF team. Reduced fertility may be overcome in most pwCF through use of assisted reproductive technologies; however, the risk of having offspring with CF must be considered. Most wwCF will have normal pregnancies, but premature birth is common, especially in the setting of reduced lung function and CF-related diabetes (CFRD); optimization of treatment is recommended during pregnancy planning. Parenting imposes an increased burden on pwCF, with the challenges of caring for the newborn, postpartum physiologic changes, and maintaining CF treatments. Most drugs used to treat CF are considered safe in pregnancy and lactation, but exceptions need to be acknowledged, including the limited data regarding safety of CF transmembrane conductance regulator (CFTR) modulators during conception, pregnancy, and lactation. Because most pwCF are eligible for highly effective CFTR modulators, fertility, contraception, and pregnancy in people with CF is changing. Prospective studies regarding these issues in people treated with CFTR modulators are paramount to provide evidence-based guidance for management in the current era of CF care.
Expression of
cystic fibrosis (CF) transmembrane conductance regulator is influenced by a polythymidine (poly-T) tract and a thymidine-guanine (TG) repeat on intron 9, which vary in length and affect ...exon 10 skipping. We compared clinical characteristics and the rate of progression of lung disease of CF patients carrying the
mutation with different intron 9 varying sequences (poly-T) and mutation classes
. Data were collected from patients in Northern Ireland, UK, including diagnostic features, sweat chloride, nutritional status, sputum microbiology, CF-related complications and lung function. Poly-T and TG repeats were determined by PCR. Forced expiratory volume in 1 s (FEV
) decline was determined from linear regression of FEV
measurements of patients over time. We identified 62 patients with
, 55 with a class I/II mutation
and six with
/
. 42 patients had 5T and 13 had 7T. All patients had 12 TG repeats. Patients with
-5T had greater lung function decline, sweat chloride concentrations, pancreatic insufficiency and prevalence of
infection compared with patients with
-7T. Lung function decline and disease severity in
is determined by the poly-T tract length and identity of the mutation
Patients with
-5T and a second class I/II mutation have a severity similar to
homozygous patients, although lung function decline is delayed to an older age. There may be linkage disequilibrium between
and 12 TG repeats.
ABSTRACT
Objectives:
In all patients with cystic fibrosis (CF), gastrointestinal (GI) tract CF transmembrane conductance regulator dysfunction occurs early in life. The identical pathophysiological ...triad of obstruction, infection, and inflammation causes disease of the airways and in the intestinal tract (CF enteropathy). Mucus accumulation within GI tract is a niche for abnormal microbial colonization, leading to dysbiosis. Fecal calprotectin (FC) is a neutrophil cytosolic protein released during apoptosis and necrosis and reflects inflammatory status. Systemic antibiotic treatment for pulmonary exacerbations has been shown to improve systemic inflammatory markers and serum and sputum calprotectin. Antibiotic treatment aimed at pulmonary complaints may improve GI tract inflammatory status. We hypothesized that high levels of FC present during pulmonary exacerbation are due, in part, to multiorgan dysbiosis and thus should diminish with systemic antibiotic treatment.
Methods:
This prospective pilot study enrolled 14 patients with CF, with no current GI symptoms. FC levels and lung function were measured at the beginning and end of systemic antibiotic treatment.
Results:
Compared to preantibiotic treatment baseline values, end of treatment FC levels declined significantly after antibiotic treatment, P = 0.004 and similarly, there was significant improvement in forced expiratory volume in 1 second, P = 0.002.
Conclusions:
High levels of FC during respiratory exacerbation may reflect a systemic exacerbation rather than solely pulmonary. Antibiotic treatment lowered the FC levels possibly by its impact on the intestinal microbiome.
Full text
Available for:
FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
Cystic fibrosis (CF) is a genetic disorder that occurs in people of all genetic ancestries. CF is caused by variants in the CF transmembrane conductance regulator (CFTR) gene that result in ...decreased, absent, or nonfunctional CFTR protein at the cell surface of the mucous membranes. Clinical manifestations include chronic respiratory disease, malabsorption, and decreased fertility. Historically, therapies for CF were focused on the signs and symptoms of the disease. However, more recently, CFTR modulators, therapies directed at the basic defect, are improving the quality and duration of the lives of people with CF. The predicted survival of people with CF in the United States is now 53 years of age. With the hope of longer, healthier lives, female individuals with CF (fwCF) are expressing the desire to discuss fertility, pregnancy, and parenthood. Furthermore, pregnancy rates are increasing substantially. Understanding the impact of use or discontinuation of highly effective modulator therapy on the reproductive health of fwCF is critical. Finally, fwCF and their providers must consider preventative cancer screening.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
PDF
