Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction (HR) mediated by antigens to Aspergillus fumigatus. It is estimated that 2–15% of patients with cystic fibrosis (CF) and between 1% and 5% of asthmatics develop ABPA, affecting approximately 4.8 million people worldwide. The goals of treatment are controlling inflammation, reducing the number of exacerbations and limiting the progression of lung damage. Systemic steroids are therefore used as the mainstay therapy, along with antifungal medications. However, many patients do not respond or develop side effects to treatment. In this scenario, biological drugs such as Omalizumab, Mepolizumab, Benralizumab and Dupilumab have been implemented in clinical practice, even though there is a lack of scientific evidence to support their use. We performed a literature review of the studies carried out which analyzed biologics for the management of ABPA in adult populations with asthma and CF. To our knowledge this is the first literature review that included all biologics. We included a total of 32 studies, all but one were descriptive studies, and the vast majority evaluated the use of Omalizumab. Biologics appeared to have more benefit for patients with ABPA and asthma than CF, specifically at decreasing the frequency of acute exacerbations and by having a steroid-sparing effect. Although a decrease in serum IgE level is considered a measure of therapy success, values may not decline as expected in the context of a significant clinical improvement, highlighting the importance of measuring patient-oriented outcomes. As evidence comes mainly from case series and case reports, randomized controlled trials are needed to evaluate further the safety and efficacy of biologics in ABPA. The reviews of this paper are available via the supplemental material section.
Plastic bronchitis is a rare and underdiagnosed disease characterized by the formation and expectoration of bronchial casts of amorphous material, which can be potentially fatal. It is more frequent in pediatric population. Symptoms can range from chronic cough and dyspnea to respiratory failure depending on the area of the compromised airway. Casts are classified as type I when constituted by inflammatory cells and accompany diseases such as asthma and pneumonias; and type II when acellular and are associated with congenital heart diseases following procedures such as Fontan. We report the case of a male schoolchild with a history of complex congenital heart disease, treated with palliative surgery, evaluated in the emergency department for cough and respiratory distress. The mother referred expulsion of gelatinous material after coughing. During clinical evaluation, expulsion of bronchial casts was evidenced, suggesting a plastic bronchitis. He underwent a diagnostic and therapeutic bronchoscopy and received initial treatment with respiratory therapy, nebulized hypertonic saline solution, mucolytics, dornase alpha and nebulized heparin. The hospitalization revealed a stenosis of the right pulmonary artery, which was corrected with stent. The patient progressed satisfactorily with improvement of cough and expectoration. He was discharged with combined treatment, nebulized medications and those concerning his underlying disease.
Solitary fibrous tumors of the pleura (SFTP) are rare mesenchymal neoplasms that originate from mesenchymal growth in the pleura, tend to be single tumors, usually have an indolent course and show nonspecific symptoms. SFTP can be often diagnosed from an incidental finding of a single mass in the thorax and should be confirmed by biopsy and immunohistochemistry. A minority of cases may present Doege–Potter syndrome (DPS, episodes of refractory hypoglycemia) associated with production of insulin-like growth factor 2 (IGF-2). Both SFTP and DPS are rare occurrences with less than 2000 cases reported worldwide. The curative treatment is tumor resection. Two cases of patients with DPS caused by SFTP are presented below.
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