[Congenital bronchiectasis (bronchiectasis due to prenatal malformative factors)].
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Abnormalities, Multiple↗
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Genetically predicted associations between blood cell perturbation responses and bronchiectasis through immune mediation: A Mendelian Randomization study.
BACKGROUND: Bronchiectasis is a chronic airway disease characterized by persistent inflammation and structural damage, with substantial clinical and etiologic heterogeneity. Although previous studies have identified associations between blood cells and bronchiectasis, the causal relationships remain unclear. Moreover, the mechanisms underlying blood cell perturbation responses and their potential mediation by immune cells in disease progression are largely unexplored. METHODS: Two-sample Mendelian randomization (MR) analysis was used to explore genetically predicted associations among immune cell traits, blood cell perturbation response phenotypes, and bronchiectasis, based on genome-wide association study summary data. Mediation MR analysis was further applied to assess whether immune cells mediate these associations. Multiple sensitivity analyses, including tests for heterogeneity and horizontal pleiotropy, were performed to evaluate the validity and robustness. RESULTS: Five blood cell perturbation response phenotypes and twenty-nine immune cell traits showed significant genetically predicted associations with bronchiectasis. Mediation analysis showed that natural killer (NK) cell absolute count partially mediated the causal effect between the eosinophil perturbation response and bronchiectasis, with a mediation proportion of 9.626%. CD38 on transitional B cells mediated the causal effect between the monocyte perturbation response and bronchiectasis, with a mediation proportion of 10.580%. Additionally, CD45 on NK cells played a mediating role in the association between the white blood cell perturbation response and bronchiectasis, with a mediation proportion of 10.651%. CONCLUSION: This study systematically explores genetically predicted associations between blood cell perturbation responses and bronchiectasis and highlights potential immune-mediated pathways. These exploratory findings provide novel genetic insights into the pathogenesis of bronchiectasis and identify potential therapeutic targets for future strategies.
Humans↗
Immunological abnormalities in bronchiectasis with chronic bronchial suppuration.
Fifty-three patients with bronchiectasis (chronic bronchial suppuration) of unknown cause, in whom the chronic production of purulent sputum was the prominent clinical feature, were investigated for possible immunological abnormalities. They were compared with two control groups comprising 50 patients with chronic bronchitis and emphysema and 33 patients with bronchial asthma. Forty-two patients with bronchiectasis(79%) had at least one abnormality of immunoglobulin, usually elevation of IgA, IgG or IgM. Eight patients had all three immunoglobulin levels raised and this was related to severity of disease. Similar increases in immunoglobulin levels were observed in the control groups, but the frequency and severity of these changes were significantly greater in the bronchiectasis patients. Two patients had IgA deficiency. There was a very high prevalence of rheumatoid factor (52%) and an increased prevalence of antinuclear factor (10%) in the bronchiectasis patients compared with the control groups. The presence of these autoantibodies did not correlate closely with severity of disease. Ten patients with bronchiectasis (19%) had one or more autoimmune disorders, and the association of severe bronchiectasis, Hashimoto's thyroiditis and pernicious anaemia in one patient is described in detail. The immunoglobulin changes, high incidence of autoantibodies and association with autoimmune disorders raises the possibility that in some patients with bronchiectasis (chronic bronchial suppuration) of apparent unknown cause abnormal immune mechanisms may be important in causing or perpetuating the condition.
Adolescent↗
Bronchography in the recognition of congenital cystic bronchiectasis.
Congenital cystic bronchiectasis is an uncommon disease. Most of the reported cases have been in infants and young children. This paper presents four cases of older children and younger adults who had congenital cystic bronchiectasis. Distribution of the disease is lobar or may even involve an entire lung. Bronchography plays an important role in the diagnosis of congenital cystic bronchiectasis. The three important bronchographic signs of congenital cystic bronchiectasis are: (1) absence of contrast filling of the bronchial glands duct; (2) contrast filling of numerous cysts which communicate directly with the bronchial tree; and (3) nonvisualization of normal lung parenchyma within the area of involvement.
Adolescent↗
[Bilateral pulmonary resections for bilateral bronchiectasis].
The present paper reports on 6 cases of bilateral bronchiectasis in patients aged 11 to 63 years, treated by simultaneous bilateral pulmonary resection and 1 case of bilateral bronchiectasis in which 14 segments were resected successively. Surgery by simultaneous bilateral approach and associated resection of the affected areas is a procedure that should be indicated, in the authors' opinion, only in carefully selected cases in which not more than 13 segments have to be resected. The indications and contraindications of surgery in bilateral bronchiectasis are discussed, preference being given to the simultaneous approach. In the cases reported at most 11 1/2 segments of the four pulmonary lobes affected were resected in a single stage; in 1 case 14 segments were resected successively, the resection exceeding in amplitude all similar one published until now. The immediate and late postoperative evolution (lesional and functional) were favourable, which pleads for a reconsideration of the approach to bilateral bronchiectasis and the application of the surgical treatment recommended, within the limits described.
Adolescent↗
[Bronchiectasis in advanced old-age. Etiopathogenetic and diagnostic considerations and medico-social implications].
A study was carried out on the etiopathogeny and socio-medical implications in 18 cases of bronchiectasis, without a typical clinical symptomatology before admission. All the patients were over the age of 60 years. The bronchographic examination was performed in all the cases. In 8 of the cases bronchiectasis was interpreted as a post-tuberculosis syndrome and in the other 10 cases bronchiectasis was attributed to pulmonary sclerosis of different origin (acute unsuppurated pneumopathy). The gradual changes in pulmonary mechanics, the organic and functional deficiences developing with age lowered the resistance of the body to viral and bacterial infection, contributing to the onset of bronchiectasis in the older group. The suppurative episodes were curred by treatment in all the cases.
Age Factors↗
Experience with bronchiectasis in Nigeria.
Between April 1975 and March 1978, 54 patients were treated for bronchiectasis at the University College Hospital, Ibadan. The sex distribution was equal and ages of the patients ranged from five to 67 years with a peak incidence of the disease in the third and fourth decades of life.One third of the patients had tuberculous bronchiectasis with destroyed left lung syndrome, another third had chronic sinusitis, while nine percent had sickle cell hemoglobinopathy. One case of Kartagener syndrome, pulmonary aspergillosis, poliomyelitis, and aspiration of a foreign body were associated with bronchiectasis.Factors influencing the clinical course and prognosis of this disease in this environment include life threatening hemoptysis, bilateral disease, associated lung abscess or empyema, malnutrition, and chronic anemia.Twenty-six patients were treated conservatively with one death, while 28 patients were treated surgically with three deaths. Twenty percent of the surgical patients developed recurrent bronchiectasis within one to 31/2 years, in lobes which were bronchographically free of disease prior to surgery. These findings suggest that bronchiectasis is a progressive disease and that elective surgery for localized disease probably does not affect its clinical and natural history in this environment.
Adolescent↗
Symptoms and treatment response to florensocatib and inhaled tobramycin in bronchiectasis: Post hoc analysis of two randomized trials.
Inhaled antibiotics and DPP-1 inhibitors improve clinical outcomes in bronchiectasis, but whether baseline symptom burden predicts differential treatment responses remains unclear. In this post hoc analysis of two multicenter randomized trials (SAVE-BE, n = 224; TORNASOL, n = 357), we evaluate the association between baseline Quality of Life-Bronchiectasis Respiratory Symptom Scale (QoL-B-RSS) and treatment effects of florensocatib and inhaled tobramycin. In SAVE-BE, florensocatib reduces exacerbation rates versus placebo (relative risk [RR], 0.47; 95% confidence interval [CI], 0.33-0.67; p < 0.0001), with RRs of 0.53 and 0.40 observed in patients with high and low symptom burdens, respectively, but no significant symptomatic improvement. In TORNASOL, tobramycin produces clinically meaningful QoL-B-RSS improvements (exceeding the 8-point cutoff in high-symptom patients) and ameliorates bronchitic symptoms, with greater benefits in those with higher baseline symptom burden. These hypothesis-generating findings suggest that baseline symptom burden may identify differential responses to anti-inflammatory versus anti-infective therapies in bronchiectasis and support its potential as a simple, practical stratification tool to guide personalized treatment.
Humans↗
Familial middle lobe bronchiectasis.
A rare familial occurrence of middle lobe bronchiectasis is reported in two siblings of a family with seven children. No definite cause for the development of bronchiectasis could be established. Enlarged hilar lymph nodes were found in the resected middle lobe which were compressing the middle lobe bronchus, and this may have contributed to the development of bronchiectasis.
Adolescent↗
Bridging the airway microbiome and targeted therapy in bronchiectasis: multi-omics insights, endotypes and emerging therapies.
Bronchiectasis is a heterogeneous chronic airway disease primarily driven by persistent infection, microbial dysbiosis and dysregulated host immunity. While culture-based microbiology has historically informed clinical management, advances in high-throughput sequencing and multi-omic technologies have transformed our understanding of the airway ecosystem, revealing that disease activity is shaped not only by individual pathogens, but by complex and dynamic host-microbe interactions. Despite the breadth of descriptive microbiome data, translation into clinically actionable diagnostics or therapies has been limited. Importantly, cross-sectional correlations between microbiota and inflammation do not establish cause and effect, underscoring the need to embed host-microbiome profiling within both longitudinal and interventional therapeutic trials. In this review, we critically appraise current microbial and host multi-omics research in bronchiectasis, integrating microbiome studies with host inflammatory, proteomic and immunophenotyping data. We highlight themes emerging across cohorts, including low microbial diversity, pathogen dominance, loss of commensal networks and neutrophil-driven inflammation, and discuss how these features align with biological endotypes associated with exacerbations and treatment response. Drawing on lessons from host-directed therapeutic successes, we examine translational roadblocks limiting microbiome-guided care. We further review emerging microbiome-modulating strategies such as pathogen-specific biologics, bacteriophage therapy, live biotherapeutic products, biofilm-targeting adjuncts and precision antibiotic stewardship. Finally, we propose a roadmap toward microbiome-informed precision medicine through harmonised methodologies, integration of host and microbial biomarkers into clinical trials, and embedding multi-omics pipelines within large international registries. Collectively, these advances have the potential to shift bronchiectasis research and clinical management towards rationally designed, precision medicine-driven therapeutic strategies.
Humans↗
Bronchiectasis and homozygous alpha1-antitrypsin deficiency.
A 34-year-old woman with homozygous a1-antitrypsin deficiency suffered from progressive, generalized cystic bronchiectasis. Although bronchiectasis was reported in the original monograph on the enzyme inhibitor deficiency, it has received minimal attention since then. Alpha1-antitrypsin levels should be measured in patients with severe bronchiectasis.
Adult↗
Bronchiectasis and spontaneous pneumothorax in Marfan's syndrome.
A Chinese girl with Marfan's syndrome had spontaneous pneumothorax and bronchiectasis. Spontaneous pneumothorax is not an infrequent pulmonary manifestation of Marfan's syndrome, but bronchiectasis is rarely reported to occur. So far, it has not been reported that both bronchiectasis and spontaneous pneumothorax can occur in the same patient with Marfan's syndrome.
Adolescent↗
Selective IgA deficiency and Pi ZZ-antitrypsin deficiency. Association with recurrent sinopulmonary infections, emphysema, and bronchiectasis.
We describe a patient in whom selective IgA deficiency and homozygous alpha1-antitrypsin deficiency were discovered. Clinically, the patient suffered from chronic sinopulmonary infections, destructive emphysema, and bronchiectasis. The interrelation of IgA and alpha1-antitrypsin was studied. Twenty-three alpha1-antitrypsin-deficient sera were screened for IgA deficiency. None of these sera were deficient in IgA. Fifteen IgA-deficient sera were screened for alpha1-antitrypsin deficiency. In this group, three patients were found to have variant alpha1-antitrypsin phenotypes. Respiratory infections were a prominent complaint in all three of these patients, with bronchiectasis in two patients. We believe that the combination of IgA and alpha1-antitrypsin deficiencies should be considered in the evaluation of any patient with idiopathic bronchiectasis.
Bronchiectasis↗
[Clinical effects of lividomycin on respiratory tract infections, primarily wet cases of bronchiectasis (author's transl)].
Patients with various respiratory infections, primarily wet cases of bronchiectasis, were treated with lividomycin (LVDM) at a dose of 1 g/day for about a week. The results obtained are as follows: 1. Of the 15 patients with refractory bronchiectasis who had previously been treated with various other antibiotics, 11 patients (73.3%) responded to LVDM. Although there was no patient who responded to LVDM excellently, good cures were achieved in 5 pateints and fair cures in 6 patients. 2. In bronchiectasis patients having infections in association with pulmonary tuberculosis (mixed infections), LVDM did not show any remarkable effects, excepting that fair cures were achieved in 3 patients. 3. In the 7 patients with acute bronchopneumonia, excellent, good and fair cures were achieved in 1, 5 and 1 patient, respectively. Although LVDM was effective in all of the patients with the said infection, the number of the patients responded excellently was meager and the onset of action seemed to be somewhat slow. 4. In one patient with lobar pneumonia, LVDM was not effective. 5. The MIC values of LVDM for clinically isolated various organisms are summarized below: Pseudomonas aeruginosa 50 mcg/ml, Klebsiella pneumoniae 3.13 to 6.25 mcg/ml, Haemophilis influenzae 1.56 to 6.25 mcg/ml, Diplococcus pneumoniae 50 mcg/ml, alpha-Streptococcus 50 to 100 mcg/ml, beta-Streptococcus 100 mcg/ml, and Staphylococcus aureus 1.56 mcg/ml. 6. In spite of the fact that a majority of the patients participated in this study were the aged, LVDM's side effects were limited to an increase of BUN (one patient) and eruption (one patient). Any noticeable changes in hepatic and renal function, hearing acuity, etc. were not observed. As indicated above, LVDM was effective against, respiratory infections as well as infections of the urinary tract on which a considerable number of research has already been reported. Thus, it is considered to be worthwhile to use LVDM for the treatment of respiratory infections.
Adult↗
Unilateral vascular plethora--a sign in advanced unilateral basal bronchiectasis.
Unilateral basal bronchiectasis results in a regional high vascular pressure and the flow of blood is thereby diverted to the uninvolved portion of the lung through bronchopulmonary anastomoses. Unilateral plethora of the upper lobe vessels in significant basal bronchiectasis of the same lung constitutes an important additional sign in the recognition of bronchiectasis on the plain chest radiograph. Attention is drawn to the fact that this will not occur if the adjacent lung is compromised by a related or unrelated disease process.
Adolescent↗
Deficiency of alpha1-antitrypsin and bronchiectasis.
60 consecutive patients with bronchiectasis were screened for alpha1-antitrypsin deficiency. Six cases (10%) with genotype PiMZ (alpha1-antitrypsin less than 66% of normal) were found. This frequency (10%) was statistically significantly (p less than 0.005) higher than the frequency of PiMZ (2.7%) in the control group. In two patients the bronchiectasis was cystic. It is suggested that the deficiency states of alpha1-antitrypsin promoting the proteolysis can participate in the genesis of bronchiectasis.
Adolescent↗
Bronchial vascularization in patients with bronchiectasis.
An analysis of the angiographic patterns of bronchial vascularization in 22 patients with bronchiectasis is presented. The studies disclosed three types of angiographic patterns: (1) bronchial hypervascularization alone; (2) bronchial hypervascularization associated with bronchopulmonary anastomoses, and (3) bronchial hypervascularization with plexiform enlargement of bronchial arteries, vascular collections or angiomatous structures. These types of angiographic pattern show a good correlation with the clinical features and supplment the global assessment of the extent and character of bronchiectasis. Selective bronchial arteriography is useful in patients with bronchiectasis where surgical treatment has been planned, especially if the disease is associated with hemoptysis.
Bronchi↗
Bilateral pulmonary resection for bronchiectasis: a 40-year experience.
Ninety-nine patients underwent bilateral pulmonary resection for severe multisegmental bilateral bronchiectasis at the Overholt Thoracic Clinic during the period 1937 to 1977. A total of 216 operations were performed, and 20 patients underwent three or more procedures. The operative mortality was 1.4% and the incidence of severe complications, 7%. Follow-up ranged from 1 to 30 years (average 10.2 years). Only 1 patient was lost to follow-up. Improvement in pulmonary symptoms was achieved in 83 patients; there was no improvement in 9 patients; and 4 patients were worse following resection. The results suggest that bilateral bronchiectasis need not be a contraindication to operation. In properly selected patients, lasting symptomatic improvement can be provided by resection.
Adolescent↗