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Arthritis Research & Therapy May 2024Epidemiological observational studies have elucidated a correlation between rheumatoid arthritis (RA) and bronchiectasis. However, the causal nature of this association...
BACKGROUND
Epidemiological observational studies have elucidated a correlation between rheumatoid arthritis (RA) and bronchiectasis. However, the causal nature of this association remains ambiguous. To clarify this potential causal linkage, we conducted a two-sample Mendelian randomization (MR) analysis to explore the bidirectional causality between RA and bronchiectasis.
METHODS
Summary statistics for RA and bronchiectasis were obtained from the IEU OpenGWAS database We employed various methods, including inverse variance weighting (IVW), MR-Egger, weighted median, weighted mode, and simple mode, to explore potential causal links between RA and bronchiectasis. Additionally, a series of sensitivity studies, such as Cochran's Q test, MR Egger intercept test, and leave-one-out analysis, were conducted to assess the MR analysis's accuracy further.
RESULTS
In the forward MR analysis, the primary analysis indicated that a genetic predisposition to RA correlated with an increased risk of bronchiectasis in European populations (IVW odds ratio (OR): 1.28, 95% confidence interval (CI): 1.20-1.37, p = 1.18E-13). Comparable results were noted in the East Asian subjects (IVW OR: 1.55, 95% CI: 1.30-1.34, p = 8.33E-07). The OR estimates from the other four methods were consistent with those obtained from the IVW method. Sensitivity analysis detected no evidence of horizontal pleiotropy or heterogeneity. Conversely, in the reverse MR analysis, we found no evidence to support a genetic causality between bronchiectasis and RA in either European or East Asian populations.
CONCLUSION
This study indicates that genetic predisposition to RA correlates with a heightened risk of bronchiectasis in both European and East Asian populations. These results imply that routine screening for bronchiectasis in RA patients could be beneficial, and effective management of RA may contribute to a reduced risk of bronchiectasis. Future research should aim to clarify the underlying mechanisms linking these two conditions.
Topics: Humans; Arthritis, Rheumatoid; Bronchiectasis; Causality; Genetic Predisposition to Disease; Mendelian Randomization Analysis; Polymorphism, Single Nucleotide; Risk Factors; White People; East Asian People
PubMed: 38783321
DOI: 10.1186/s13075-024-03336-3 -
Cureus Apr 2024Kartagener syndrome (KS), also known as primary ciliary dyskinesia, is a rare genetic disorder commonly diagnosed early in childhood. It is characterized by a triad of...
Kartagener syndrome (KS), also known as primary ciliary dyskinesia, is a rare genetic disorder commonly diagnosed early in childhood. It is characterized by a triad of findings, namely, situs inversus, chronic sinusitis, and bronchiectasis. Here, we present the case of a 73-year-old female who incidentally presented the KS triad during her imaging tests in the emergency department of our institution for COVID-19 symptoms.
PubMed: 38779262
DOI: 10.7759/cureus.58747 -
Transplantation Proceedings May 2024The shortage of donors for lung transplants is the main limitation of the preceding. Lobar transplantation is an alternative especially useful in patients with short...
BACKGROUND
The shortage of donors for lung transplants is the main limitation of the preceding. Lobar transplantation is an alternative especially useful in patients with short stature and small thoracic cavities. The aim of this study was to perform a descriptive analysis of Portuguese patients who underwent lobar lung transplantation.
METHODS
A retrospective study was conducted, and patients submitted to lobar lung transplantation from January 2012 to December 2023 were evaluated. A descriptive analysis was made, including demographic data, lung diseases, waiting list dynamics, pre-transplant evaluations, and post-transplant outcomes.
RESULTS
Sixteen lobar transplants were performed with a predominance of female patients and a median age of 47 years. Most patients had interstitial lung disease or bronchiectasis either due to cystic fibrosis or non-cystic fibrosis. The median predicted total lung capacity (pTLC) ratio was 0.73. The median waiting list time was 6 months with 9 urgent transplants and 1 emergent lobar retransplant. Extracorporeal membrane oxygenation (ECMO) was used in pre-, intra-, and postoperative periods. Most transplanted lobes were the median lobe (ML) + right upper lobe (RUL) and left upper lobe (LUL). The median length of stay was 58 days, with complications such as PDG grade 3, bronchial tree ischemia, and concentrical stenosis of bronchial anastomosis. Six patients died in this period, 1 in the immediate postoperative period and 5 during the post-transplant hospitalization, with a median survival of 20.7 months and a 1-year and 5-year survival rate of 60%.
CONCLUSION
Our results show a population with an increased waiting list converging in many urgent cases, with an early mortality and high primary graft dysfunction rate. Nevertheless, mid- and long-term survival are promising.
PubMed: 38777711
DOI: 10.1016/j.transproceed.2024.04.017 -
Frontiers in Medicine 2024The prevalence of bronchiectasis among adult Aboriginal Australians is higher than that of non-Aboriginal Australians. However, despite evidence to suggest higher...
BACKGROUND
The prevalence of bronchiectasis among adult Aboriginal Australians is higher than that of non-Aboriginal Australians. However, despite evidence to suggest higher prevalence of bronchiectasis among Aboriginal people in Australia, there is sparce evidence in the literature assessing clinical parameters that may predict survival or mortality in this population.
METHODS
Aboriginal Australians residing in the Top End Health Service region of the Northern Territory of Australia aged >18 years with chest computed tomography (CT) confirmed bronchiectasis between 2011 and 2020 were included. Demographics, body mass index (BMI), medical co-morbidities, lung function data, sputum microbiology, chest CT scan results, hospital admissions restricted to respiratory conditions and all-cause mortality were assessed.
RESULTS
A total of 459 patients were included, of whom 146 were recorded deceased (median age at death 59 years). Among the deceased cohort, patients were older (median age 52 vs. 45 years, = 0.023), had a higher prevalence of chronic obstructive pulmonary disease (91 vs. 79%, = 0.126), lower lung function parameters (median percentage predicted forced expiratory volume in 1 s 29 vs. 40%, = 0.149), a significantly greater proportion cultured non- fungi (65 vs. 46%, = 0.007) and (46 vs. 28%, = 0.007) on sputum microbiology and demonstrated bilateral involvement on radiology. In multivariate models advancing age, prior culture and Intensive care unit (ICU) visits were associated with increased odds of mortality. Higher BMI, better lung function on spirometry, prior positive sputum microbiology for and use of inhaled long-acting beta antagonist/muscarinic agents may have a favourable effect.
CONCLUSION
The results of this study may be of use to stratify high risk adult Aboriginal patients with bronchiectasis and to develop strategies to prevent future mortality.
PubMed: 38774399
DOI: 10.3389/fmed.2024.1366037 -
Cureus Apr 2024Kartagener's syndrome is a genetic condition that is caused by a triad of symptoms, which includes bronchiectasis, chronic sinusitis, and situs inversus, and is...
Kartagener's syndrome is a genetic condition that is caused by a triad of symptoms, which includes bronchiectasis, chronic sinusitis, and situs inversus, and is considered rare. It is caused by defective ciliary motility, leading to impaired mucociliary clearance. We report a case of a 24-year-old male who presented with primary infertility and a long-standing history of recurrent respiratory infections. Physical examination revealed dextrocardia and digital clubbing. Radiological investigations confirmed situs inversus totalis and bronchiectasis. Semen analysis revealed azoospermia. Genetic analysis was not done due to financial constraints. However, the constellation of clinical features was diagnostic of Kartagener's syndrome. The patient was managed with antibiotics and chest physiotherapy. In vitro fertilization (IVF) was advised for infertility. A successful pregnancy was achieved through IVF, indicating viable sperm despite immotility. The aforementioned case underscores the significance of maintaining a heightened sense of suspicion for Kartagener's syndrome in individuals exhibiting unexplained bronchiectasis and infertility. Early diagnosis can prevent chronic respiratory morbidity and enable parenthood through assisted reproduction.
PubMed: 38770502
DOI: 10.7759/cureus.58635 -
Case Reports in Medicine 2024Common variable immunodeficiency (CVID) is a rare immunodeficiency syndrome which presents with wide manifestations leading to delayed diagnosis. A 34-year-old woman...
Common variable immunodeficiency (CVID) is a rare immunodeficiency syndrome which presents with wide manifestations leading to delayed diagnosis. A 34-year-old woman presented to our hospital complaining of dyspnea and productive cough. Lung CT scan revealed loculated right-sided pleural effusion with bronchiectasis and consolidation in right lower lobes. After taking medical history and physical examination, we suspected CVID and ordered serum immunoglobulin levels. The laboratory results were in line with CVID diagnosis and showed decreased levels of IgG, IgM, and IgA. The patient was started on intravenous immune globulin (IVIG) therapy every month. After 3-month follow-up, the patient reported no problem and felt better.
PubMed: 38765317
DOI: 10.1155/2024/4028888 -
Respiratory Research May 2024Bronchiectasis is a condition characterized by abnormal and irreversible bronchial dilation resulting from lung tissue damage and can be categorized into two main... (Comparative Study)
Comparative Study
BACKGROUND
Bronchiectasis is a condition characterized by abnormal and irreversible bronchial dilation resulting from lung tissue damage and can be categorized into two main groups: cystic fibrosis (CF) and non-CF bronchiectasis (NCFB). Both diseases are marked by recurrent infections, inflammatory exacerbations, and lung damage. Given that infections are the primary drivers of disease progression, characterization of the respiratory microbiome can shed light on compositional alterations and susceptibility to antimicrobial drugs in these cases compared to healthy individuals.
METHODS
To assess the microbiota in the two studied diseases, 35 subjects were recruited, comprising 10 NCFB and 13 CF patients and 12 healthy individuals. Nasopharyngeal swabs and induced sputum were collected, and total DNA was extracted. The DNA was then sequenced by the shotgun method and evaluated using the SqueezeMeta pipeline and R.
RESULTS
We observed reduced species diversity in both disease cohorts, along with distinct microbial compositions and profiles of antimicrobial resistance genes, compared to healthy individuals. The nasopharynx exhibited a consistent microbiota composition across all cohorts. Enrichment of members of the Burkholderiaceae family and an increased Firmicutes/Bacteroidetes ratio in the CF cohort emerged as key distinguishing factors compared to NCFB group. Staphylococcus aureus and Prevotella shahii also presented differential abundance in the CF and NCFB cohorts, respectively, in the lower respiratory tract. Considering antimicrobial resistance, a high number of genes related to antibiotic efflux were detected in both disease groups, which correlated with the patient's clinical data.
CONCLUSIONS
Bronchiectasis is associated with reduced microbial diversity and a shift in microbial and resistome composition compared to healthy subjects. Despite some similarities, CF and NCFB present significant differences in microbiome composition and antimicrobial resistance profiles, suggesting the need for customized management strategies for each disease.
Topics: Humans; Bronchiectasis; Cystic Fibrosis; Male; Female; Microbiota; Adult; Middle Aged; Sputum; Young Adult; Cohort Studies; Aged
PubMed: 38762736
DOI: 10.1186/s12931-024-02835-w -
Allergologie Select 2024None.
None.
PubMed: 38756207
DOI: 10.5414/ALX02444E -
Transplantation Proceedings May 2024A 21-year-old woman diagnosed with cystic fibrosis developed cirrhosis, exocrine pancreatic insufficiency, and insulin-dependent diabetes mellitus. The patient qualified...
BACKGROUND
A 21-year-old woman diagnosed with cystic fibrosis developed cirrhosis, exocrine pancreatic insufficiency, and insulin-dependent diabetes mellitus. The patient qualified for double organ liver-pancreas transplantation beyond typical indications. The respiratory symptoms of cystic fibrosis were moderate and well-treated. The patient was endangered mainly by liver insufficiency and recurrent hypoglycemia, which was due to the treatment of diabetes with high doses of insulin. Computed tomography showed mild bronchiectasis, cirrhotic liver, splenomegaly, and atrophy of the pancreas. Pseudomonas aeruginosa colonized the upper respiratory tract. Gastrointestinal complications were sufficient for the patient to be qualified for combined liver-pancreas transplantation.
METHODS
First, a standard hepatectomy was performed. The liver was transplanted orthotopically. Subsequently, the team performed pancreas transplantation through a separate incision. The donor's duodenum was anastomosed to the recipient's jejunum, close to the ligament of Treitz.
RESULTS
No serious complications were noted during the postoperative period. Transplanted organs started functioning without delay. The patient was discharged after 6 weeks in general good condition. Twenty months later, the patient felt well, and the grafts kept functioning properly.
CONCLUSION
Combined liver-pancreas transplantation in patients with CF restores exocrine and endocrine pancreatic function and minimizes the risk of life-threatening complications associated with liver insufficiency. Improvement of life quality coincides with the possibility of discontinuing insulin and pancreatic enzyme supplementation. The combination of liver and pancreas transplantation may prevent advanced pulmonary complications, extend the prognosis of survival, and improve the long-term life quality.
PubMed: 38749862
DOI: 10.1016/j.transproceed.2024.03.034