Infection, Allergy, and Inflammation: The Role of in Cystic Fibrosis.

(Af) is a mold frequently detected in airway samples from people with cystic fibrosis (pwCF). Abnormal airway mucus may allow Af to germinate, resulting in airway infection or an allergic response. While Af is known to increase morbidity in pwCF, individual responses and the degree of impact on lung disease vary.

Differing rates of fungi in sinonasal cultures from pediatric sinusitis patients.

Objectives: Pediatric chronic rhinosinusitis (PCRS) is a unique clinical entity and the underlying source of inflammation is unknown. Certain subgroups, such as children with nasal polyps and cystic fibrosis (CF) sinusitis are often recalcitrant to standard medical PCRS treatments that target bacterial inflammation. Fungal infection and allergy to fungal proteins drive inflammation in other airway diseases, resulting in chronic inflammation of both the upper and lower airways.

Clinical characteristics of people with cystic fibrosis and frequent fungal infection.

Background: Individuals with cystic fibrosis (CF) and fungal airway infection may present with fungal bronchitis, allergic bronchopulmonary aspergillosis (ABPA) or may appear unaffected despite fungal detection. We sought to characterize people with CF with frequent detection of fungi from airway samples and determine clinical outcomes.

Methods: This retrospective study included individuals with CF with ≥4 lower airway cultures over a 2-year baseline period and ≥2 years of follow-up.

Cardiovascular complications in cystic fibrosis: A review of the literature.

Cystic fibrosis is a genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, leading to dysfunction of the CFTR protein. CFTR dysfunction leads to disease in the respiratory and gastrointestinal systems. Disorders of the cardiovascular system in individuals with CF are usually attributed to secondary effects from progressive lung disease.

Fungal Infection and Inflammation in Cystic Fibrosis.

Fungi are frequently recovered from lower airway samples from people with cystic fibrosis (CF), yet the role of fungi in the progression of lung disease is debated. Recent studies suggest worsening clinical outcomes associated with airway fungal detection, although most studies to date are retrospective or observational. The presence of fungi can elicit a T helper cell type 2 (Th-2) mediated inflammatory reaction known as allergic bronchopulmonary aspergillosis (ABPA), particularly in those with a genetic atopic predisposition.

Potential pathogenicity of Inquilinus limosus in a pediatric patient with cystic fibrosis.

Presentation: Patient is a 6-year-old male with CF, MRSA colonization, and pancreatic insufficiency that presented with worsening ppFEV1 and systemic symptoms despite multiple interventions. BAL grew NTM, Stenotrophomonas maltophilia, and Inquilinus limosus, a rare organism found in patients with CF.

Course: I.

Drug analog inhibition of indoleamine 2,3-dioxygenase (IDO) activity modifies pattern recognition receptor expression and proinflammatory cytokine responses early during influenza virus infection.

Influenza virus is recognized by PRRs, which are critical in the early response to virus infection and induction of proinflammatory cytokines. IDO is increased in the lung of mice immediately following influenza infection, and the presence of IDO has been shown to mediate immune suppression through depletion of trp and reduction in IL-6 production. To determine the role of IDO activity in the early immune response to influenza infection, IDO activity was inhibited using the synthetic analog, 1MT.

Evidence of vascular endothelial dysfunction in young patients with cystic fibrosis.

Background: Cystic fibrosis (CF) is a genetic disorder that affects not only pulmonary function but also multiple organ systems. The fl ow-mediated dilation (FMD) test is a noninvasive assessment of endothelial function and nitric oxide bioavailability. Thus, the purpose of this study was to determine (1) whether endothelial dysfunction is present in young patients with CF and (2) whether endothelial function is associated with pulmonary function and exercise capacity.

Human metapneumovirus establishes persistent infection in the lungs of mice and is reactivated by glucocorticoid treatment.

Human metapneumovirus (HMPV) has been identified as a worldwide agent of serious upper and lower respiratory tract infections in infants and young children. HMPV is second only to respiratory syncytial virus (RSV) as a leading cause of bronchiolitis, and, like RSV, consists of two major genotypes that cocirculate and vary among communities year to year. Children who have experienced acute HMPV infection may develop sequelae of wheezing and asthma; however, the features contributing to this pathology remain unknown.