- Health Perception and Behaviors in Adults With Bronchiectasis. [Journal Article]
- RCRespir Care 2018 Dec 11
- CONCLUSIONS: The diverse symptom perception and health-related behaviors highlighted the need for evaluation and intervention in bronchiectasis. These findings will provide rationales for refining future health care through comprehensive (particularly psychological) interventions worldwide.
- Coronary-to-bronchial artery fistula in a patient with angina. [Journal Article]
- JCJ Cardiol Cases 2013; 7(2):e45-e47
- Coronary-to-bronchial artery fistulae are the most common found in adulthood and have often been associated with bronchiectasis and other abnormalities of pulmonary parenchyma. In this study we descr...
Coronary-to-bronchial artery fistulae are the most common found in adulthood and have often been associated with bronchiectasis and other abnormalities of pulmonary parenchyma. In this study we describe an unusual case in which circumflex coronary artery-to-left bronchial artery fistula was associated with the presence of extensive aneurysmatic dilatation of thoracic arteries. <Learning objective: This report deals with the case of a coronary-to-bronchial artery fistula (CAF) as the cause of atypical angina in a patient with normal coronary arteries. The concomitant finding of aneurysmatic dilatation of thoracic arteries controindicated an invasive approach. Medical therapy with vasodilators and anti-anginal drugs was soon started proving to be efficacious in symptoms relief.>.
- Allergic bronchopulmonary aspergillosis successfully treated with mepolizumab: Case report and review of the literature. [Journal Article]
- RMRespir Med Case Rep 2019; 26:59-62
- A 56-year-old woman was referred to our hospital for recurrent asthma of 20 years duration. She was diagnosed as having allergic bronchopulmonary aspergillosis on the basis of clinical symptoms, peri...
A 56-year-old woman was referred to our hospital for recurrent asthma of 20 years duration. She was diagnosed as having allergic bronchopulmonary aspergillosis on the basis of clinical symptoms, peripheral blood eosinophilia, elevated total serum immunoglobulin E value, positive results of specific IgE and precipitating antibodies against Aspergillus sp., central bronchiectasis, and mucoid impaction. Systemic corticosteroids and anti-fungal therapy improved her symptoms, but the cessation of these treatments led to frequent exacerbations. Omalizumab improved her asthmatic symptoms to the point that corticosteroids could be stopped; however, radiological findings were not improved, and coexisting eosinophilic sinusitis and otitis media worsened. After her treatment was changed from omalizumab to mepolizumab, not only her asthmatic symptoms but also her sinusitis and otitis media became well controlled, and chest radiological findings improved.
- The association between computed tomography scan findings of pulmonary infection caused by atypical mycobacteria and bacillus count in sputum samples. [Journal Article]
- IJInt J Mycobacteriol 2018 Oct-Dec; 7(4):355-357
- CONCLUSIONS: Some CT scan findings, such as nodule diameter smaller than 5 mm, consolidation, bronchiectasis, and pleural thickening, may be indicators of atypical mycobacterial infection. Increased number of involved lobes with bronchiectasis can promote early diagnosis in patients with higher smear and culture grading.
- Traction bronchiectasis on high-resolution computed tomography may predict fatal acute eosinophilic pneumonia. [Journal Article]
- RIRespir Investig 2018 Oct 24
- CONCLUSIONS: We observed the characteristics of patients with fatal AEP who did not respond to treatment. TBE was observed in all fatal cases and may be associated with a poor prognosis.
- New Insights Into the Epidemiology of Bronchiectasis. [Editorial]
- ChestChest 2018; 154(6):1272-1273
- [Respiratory manifestations in primary immunodeficiencies]. [Journal Article]
- OHOrv Hetil 2018; 159(49):2043-2049
- The respiratory infections are the most common presentations and leading cause of morbidity and mortality in primary immunodeficiencies. The pathogen or spectrum of pathogens may be characteristic fo...
The respiratory infections are the most common presentations and leading cause of morbidity and mortality in primary immunodeficiencies. The pathogen or spectrum of pathogens may be characteristic for the underlying primary immunodeficiency, and that knowledge plays an important role in the empirical treatment or prevention of the infections, or, conversely, the identification of an unusual pathogen may raise the suspicion on an immunodeficiency. Apart from the acute respiratory infections, chronic lung diseases are also common complications. The recurrent infections result in bronchiectasis, an irreversible structural damage of the respiratory tract, that frequently serve as locus for subsequent infections. The other group of chronic pulmonary complications are the interstitial lung diseases, which seem to be independent from infections and can be regarded as the pulmonary manifestation of the immune dysregulation. It is important to identify those patients with immunodeficiency who are at increased risk of secondary lung complications and to regularly screen this group for the early detection of the pulmonary complications. Orv Hetil. 2018; 159(49): 2043-2049.
- Loss of ARHGEF1 causes a human primary antibody deficiency. [Journal Article]
- JCIJ Clin Invest 2018 Dec 06
- ARHGEF1 is a RhoA-specific guanine nucleotide exchange factor expressed in hematopoietic cells. We used whole-exome sequencing to identify compound heterozygous mutations in ARHGEF1, resulting in the...
ARHGEF1 is a RhoA-specific guanine nucleotide exchange factor expressed in hematopoietic cells. We used whole-exome sequencing to identify compound heterozygous mutations in ARHGEF1, resulting in the loss of ARHGEF1 protein expression in two primary-antibody-deficient siblings presenting with recurrent severe respiratory tract infections and bronchiectasis. Both ARHGEF1-deficient patients showed an abnormal B cell immunophenotype, with a deficiency in marginal-zone and memory B cells and an increased frequency of transitional B cells. Furthermore, the patients' blood contained immature myeloid cells. Analysis of a mediastinal lymph node from one patient highlighted the small size of the germinal centres and an abnormally high plasma cell content. On the molecular level, T and B lymphocytes from both patients displayed low RhoA activity and low steady-state actin polymerization (even after stimulation of lysophospholipid receptors). As a consequence of disturbed regulation of the RhoA downstream target ROCK, the patients' lymphocytes failed to efficiently restrain AKT phosphorylation. Enforced ARHGEF1 expression or drug-induced activation of RhoA in patients' cells corrected the impaired actin polymerization and AKT regulation. Our results indicate that ARHGEF1 activity in human lymphocytes is involved in controlling actin cytoskeleton dynamics, restraining PI3K/AKT signalling, and confining B lymphocytes and myelocytes within their dedicated functional environment.
- [Individualised treatment of patients with bronchoectasis]. [Journal Article]
- ULUgeskr Laeger 2018 Dec 03; 180(49)
- Bronchiectasis is characterised by chronic cough, airway inflammation, infection and bronchial dilatation. The disease may lead to impaired lung function and premature death. An underlying inflammato...
Bronchiectasis is characterised by chronic cough, airway inflammation, infection and bronchial dilatation. The disease may lead to impaired lung function and premature death. An underlying inflammatory disease or immunodeficiency may be present, but 40% of the cases are idiopathic. The diagnosis is based on high-resolution CT scan. Treatment is aimed at reducing symptoms and risk of exacerbations, and includes antibiotics, bronchodilators and pulmonary rehabilitation. Assessment of disease severity and individual phenotypes may help to modify treatment strategies in the future.
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- Mucolytic Agents and Statins Use is Associated with a Lower Risk of Acute Exacerbations in Patients with Bronchiectasis-Chronic Obstructive Pulmonary Disease Overlap. [Journal Article]
- JCJ Clin Med 2018 Dec 04; 7(12)
- CONCLUSIONS: Statins and mucolytic agents use may lower risk of acute exacerbation in patients with BCO.