Characterising the allergic profile of children with cystic fibrosis.
Ontology highlight
ABSTRACT: Background
Cystic fibrosis (CF) is a genetic condition that affects multiple organ systems. Allergic bronchopulmonary aspergillosis (ABPA) is a well-recognised problem but other allergic conditions are less well documented in CF.Objective
To characterise the allergic profile of a cohort of children with CF, with a focus on those with ABPA.Methods
A cohort of children with CF were interviewed and retrospective data were collected regarding their allergic histories and other relevant clinical features.Results
The cohort included 37 children with median age of 9 years (interquartile range: 6-12). There was a history of ≥1 allergic condition(s) in 28/37 children (76%). The most common allergic condition was allergic rhinitis (AR) in 21/37 (57%) and 16 of these 21 children (76%) had another allergic condition. All children with ABPA (8) had another allergic condition. In some children ABPA exacerbations appeared to be seasonal, suggesting possible cross-sensitisation between Aspergillus fumigatus and aeroallergens associated with seasonal AR. Allergic conditions were also common in children with Pseudomonas aeruginosa infection.
SUBMITTER: Faulkner AL
PROVIDER: S-EPMC8669696 | biostudies-literature |
REPOSITORIES: biostudies-literature
Similar Datasets
Project description:Allergic bronchopulmonary aspergillosis (ABPA) is a pulmonary disorder that often occurs in patients with asthma or cystic fibrosis (CF) and is characterized by a hypersensitivity response to the allergens of the fungus Aspergillus fumigatus. In patients with CF, growth of A. fumigatus hyphae within the bronchial lumen triggers an immunoglobulin E (IgE)-mediated hypersensitivity response that results in airway inflammation, bronchospasm, and bronchiectasis. In most published studies, the prevalence of ABPA is about 8.9% in patients with CF. Since the clinical features of this condition overlap significantly with that of CF, ABPA is challenging to diagnose and remains underdiagnosed in many patients. Diagnosis of ABPA in CF patients should be sought in those with evidence of clinical and radiologic deterioration that is not attributable to another etiology, a markedly elevated total serum IgE level (while off steroid therapy) and evidence of A. fumigatus sensitization. Management of ABPA involves the use of systemic steroids to reduce inflammation and modulate the immune response. In patients who do not respond to steroids or cannot tolerate them, antifungal agents should be used to reduce the burden of A. fumigatus allergens. Recent studies suggest that omalizumab may be an effective option to reduce the frequency of ABPA exacerbations in patients with CF. Further randomized controlled trials are needed to better establish the efficacy of omalizumab in managing patients with CF and ABPA.
| S-EPMC5399694 | biostudies-literature
Project description:Allergic bronchopulmonary aspergillosis (ABPA) is a complex pulmonary disorder characterized by a hypersensitivity reaction to Aspergillus fumigatus, and almost always seen in patients with cystic fibrosis (CF) and asthma. Fungal hyphae leads to an ongoing inflammation in the airways that may result in bronchiectasis, fibrosis, and eventually loss of lung function. Despite the fact that ABPA is thought to be more prevalent in CF than in asthma, the literature on ABPA in CF is more limited. The diagnosis is challenging and may be delayed because it is made based on a combination of clinical features, and radiologic and immunologic findings. With clinical deterioration of a patient with CF, ABPA is important to be kept in mind because clinical manifestations mimic pulmonary exacerbations of CF. Early diagnosis and appropriate treatment are important in preventing complications related to ABPA. Treatment modalities involve the use of anti-inflammatory agents to suppress the immune hyperreactivity and the use of antifungal agents to reduce fungal burden. Recently, in an effort to treat refractory patients or to reduce adverse effects of steroids, other treatment options such as monoclonal antibodies have started to be used. Intensive research of these new agents in the treatment of children is being conducted to address insufficient data.
| S-EPMC7606581 | biostudies-literature
Project description:This multi-center study will compare multi-target DNA and quantitative FIT stool-based testing to colonoscopy in individuals with Cystic Fibrosis (CF) undergoing colon cancer screening with colonoscopy. The primary endpoint is detection of any adenomas, including advanced adenomas and colorectal cancer (CRC).
| 2726863 | ecrin-mdr-crc
Project description:BackgroundAllergic bronchopulmonary aspergillosis (ABPA) is an allergic reaction to colonisation of the lungs with the fungus Aspergillus fumigatus, and affects around 10% of people with cystic fibrosis. ABPA is associated with an accelerated decline in lung function. High doses of corticosteroids are the main treatment for ABPA; although the long-term benefits are not clear, and their many side effects are well-documented. A group of compounds, the azoles, have activity against A fumigatus, and have been proposed as an alternative treatment for ABPA. Of this group, itraconazole is the most active. A separate antifungal compound, amphotericin B, has been used in aerosolised form to treat invasive infection with A fumigatus, and may have potential for the treatment of ABPA. Antifungal therapy for ABPA in cystic fibrosis needs to be evaluated. This is an update of a previously published review.ObjectivesThe review aimed to test the hypotheses that antifungal interventions for the treatment of ABPA in cystic fibrosis: 1. improve clinical status compared to placebo or standard therapy (no placebo); and 2. do not have unacceptable adverse effects. If benefit was demonstrated, we planned to assess the optimal type, duration, and dose of antifungal therapy.Search methodsWe searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register, which comprises references identified from comprehensive electronic database searches, handsearches of relevant journals, and abstract books of conference proceedings. Date of the most recent search of the Group's Trials Register was 28 September 2021. We searched ongoing trials registries, most recently on 11 March 2022. Earlier, we also approached pharmaceutical companies regarding possible unpublished trials.Selection criteriaPublished or unpublished randomised controlled trials, in which antifungal treatments were compared to either placebo or no treatment, or where different doses of the same treatment were used in the treatment of ABPA in people with cystic fibrosis.Data collection and analysisThe searches identified six trials; none of which met the inclusion criteria for the review.Main resultsWe included no completed randomised controlled trials. There is currently one ongoing trial, which we may find eligible for a future update.Authors' conclusionsAt present, there are no randomised controlled trials that evaluate the use of antifungal therapies for the treatment of ABPA in people with cystic fibrosis, although one trial is currently ongoing. Trials with clear outcome measures are needed to properly evaluate the use of corticosteroids in people with ABPA and cystic fibrosis.
| S-EPMC9438469 | biostudies-literature
Project description:Aspergillus spp. are spore forming molds; a subset of which are clinically relevant to humans and can cause significant morbidity and mortality. A. fumigatus causes chronic infection in patients with chronic lung disease such as asthma, chronic obstructive pulmonary disease (COPD) and cystic fibrosis (CF). In patients with CF, A. fumigatus infection can lead to allergic disease, such as allergic bronchopulmonary aspergillosis (ABPA) which is associated with high rates of hospitalizations for acute exacerbations and lower lung function. ABPA results from TH2 immune response to Aspergillus antigens produced during hyphal growth, marked by high levels of IgE and eosinophil activation. Clinically, patients with ABPA experience difficulty breathing; exacerbations of disease and are at high risk for bronchiectasis and lung fibrosis. Oral corticosteroids are used to manage aspects of the inflammatory response and antifungal agents are used to reduce fungal burden and lower the exposure to fungal antigens. As the appreciation for the severity of fungal infections has grown, new therapies have emerged that aim to improve treatment and outcomes for patients with CF.
| S-EPMC8067098 | biostudies-literature
Project description:To determine the clinical presentation, diagnostic investigations and laboratory workup done in admitted children with cystic fibrosis at Aga Khan University Hospital Karachi, Pakistan.This is athree years retrospective study from January 2013 to December 2015 conducted at The Aga Khan University Hospital Karachi Pakistan, enrolling admitted patient from birth to 15 years of either gender, diagnosed with CF on the basis of clinical features and positive sweat chloride test. Different clinical presentations were noted including initial presentations. Sweat chloride values more than 60mmol/L were labeled as positive and consistent with diagnosis of CF. Available Delta F-508 mutation analyses were noted. Relevant laboratory and radiological investigations including sputum culture and HR-CT chest findings were documented. Results were analyzed using SPSS version 20.Total 43 children were selected according to the inclusion criteria. Chronic cough (69.76%) was the most common initial clinical presentation. Mean age at onset of symptoms was 14.41± 26.18 months and mean age at diagnosis was 47.20 ± 45.80 months Respiratory features were most common in our cohort including chronic productive cough (90.71%), recurrent bronchopneumonia (72.09%) and asthma like presentation (44.19%) with wheezing and cough. 86% patients presented with failure to thrive. Gastroenterological features including steatorrhea were seen in 55.81% patients and 44.19% patients had abdominal distension. Mean sweat chloride value in our population was 82.70± 22.74. Gene analysis for Delta F-508 was identified in 12 (27.90%) patients. Bronchiectatic pulmonary changes on HRCT were seen in 18 patients (41.86%). Pseudomonas grew in 12 patients (27.90%) in sputum cultures at the time of diagnosis.Respiratory presentations predominate in CF children followed by gastrointestinal features. Nearly half of our patient had bronchiectatic changes on CT scan chest and more than quarter had pseudomonas colonization in the airways at the time of diagnosis. Delta F-508 mutation was found to be uncommon in our study population. There is significant delay in diagnosing patients with CF.
| S-EPMC5510102 | biostudies-other
Project description:We report the implementation of a pediatric home spirometry program at our institution. A respiratory therapist provided either a virtual or an in-person initiation visit that included a coached spirometry session. Families were instructed to perform daily uncoached spirometry sessions for 5 days. The program's quality assurance component was deemed not to be human research by the local IRB. In total, 52 subjects completed an initiation visit (34 with at least 3 additional uncoached spirometry sessions). The clinic spirometry and coached (same-day) sessions and uncoached (same-week) sessions were completed by 12 and 17 subjects, respectively. The median (99% CI) coefficients of variation for FEV1% of the uncoached maneuvers were 3.5% (2.9-5.9%). The median (IQR) FEV1% and FEV1 (mL) absolute differences between coached and uncoached home spirometry were -2% (-4 and +3%) and -25 mL (-93 and +93 mL), respectively. The median (IQR) absolute differences in FEV1% and FEV1 (mL) between coached or uncoached home spirometry and clinic spirometry were -6% (-10 and -2%) and -155 mL (-275 and -88 mL), and -4% (-10 and +5%), and -110 mL (-280 and +9 mL), respectively. Differences in absolute FEV1 (L) and FEV1% were found among different modalities of spirometry performed by people with cystic fibrosis. Understanding the variability of uncoached home spirometry and the differences among coached and uncoached home spirometry, hospital and coached home spirometry, and hospital and uncoached home spirometry for any given individual is crucial to effectively utilize this tool in clinical care.
| S-EPMC9952128 | biostudies-literature
Project description:This SuperSeries is composed of the SubSeries listed below.
2020-06-15 | GSE141537 | GEO
Project description:BackgroundCystic fibrosis is an autosomal recessive multisystem disorder with an approximate prevalence of 1 in 3500 live births. Allergic bronchopulmonary aspergillosis is a lung disease caused by aspergillus-induced hypersensitivity with a prevalence of 2% to 15% in people with cystic fibrosis. The mainstay of treatment includes corticosteroids and itraconazole. The treatment with corticosteroids for prolonged periods of time, or repeatedly for exacerbations of allergic bronchopulmonary aspergillosis, may lead to many adverse effects. The monoclonal anti-IgE antibody, omalizumab, has improved asthma control in severely allergic asthmatics. The drug is given as a subcutaneous injection every two to four weeks. Since allergic bronchopulmonary aspergillosis is also a condition resulting from hypersensitivity to specific allergens, as in asthma, it may be a candidate for therapy using anti-IgE antibodies. Therefore, anti-IgE therapy, using agents like omalizumab, may be a potential therapy for allergic bronchopulmonary aspergillosis in people with cystic fibrosis. This is an updated version of the review.ObjectivesTo evaluate the efficacy and adverse effects of anti-IgE therapy for allergic bronchopulmonary aspergillosis in people with cystic fibrosis.Search methodsWe searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched the reference lists of relevant articles and reviews. Last search: 09 September 2021. We searched two ongoing trial registries (Clinicaltrials.gov and the WHO trials platform). Date of latest search: 16 August 2021.Selection criteriaRandomized and quasi-randomized controlled trials comparing anti-IgE therapy to placebo or other therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis.Data collection and analysisTwo review authors independently extracted data and assessed the risk of bias in the included study. They planned to perform data analysis using Review Manager.Main resultsOnly one study enrolling 14 participants was eligible for inclusion in the review. The double-blind study compared a daily dose of 600 mg omalizumab or placebo along with twice daily itraconazole and oral corticosteroids, with a maximum daily dose of 400 mg. Treatment lasted six months but the study was terminated prematurely and complete data were not available. We contacted the study investigator and were told that the study was terminated due to the inability to recruit participants into the study despite all reasonable attempts. One or more serious side effects were encountered in six out of nine (66.67%) and one out of five (20%) participants in omalizumab group and placebo group respectively.Authors' conclusionsThere is lack of evidence for the efficacy and safety of anti-IgE (omalizumab) therapy in people with cystic fibrosis and allergic bronchopulmonary aspergillosis. There is a need for large prospective randomized controlled studies of anti-IgE therapy in people with cystic fibrosis and allergic bronchopulmonary aspergillosis with both clinical and laboratory outcome measures such as steroid requirement, allergic bronchopulmonary aspergillosis exacerbations and lung function.
| S-EPMC8457344 | biostudies-literature
Project description:The transcriptomic profile of cystic fibrosis human lung fibroblasts was compared to normal human lung fibroblasts.
2020-06-15 | GSE141535 | GEO