CF Fungal Disease in the Age of CFTR Modulators

Bercusson, Amelia; Jarvis, G. A.

doi:10.1007/s11046-021-00541-5

Cited by 21 publications

(6 citation statements)

References 75 publications

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“…Chronic (>4 weeks) pulmonary symptoms (chronic productive cough, tenacious mucus production, dyspnea, and difficult airway clearance) 117…”

Section: Clinical Spectrum Of Pulmonary Aspergillosismentioning

confidence: 99%

Aspergillus and the Lung

Janssens,

Lambrecht,

Van Braeckel

2024

Semin Respir Crit Care Med

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The filamentous fungus Aspergillus causes a wide spectrum of diseases in the human lung, with Aspergillus fumigatus being the most pathogenic and allergenic subspecies. The broad range of clinical syndromes that can develop from the presence of Aspergillus in the respiratory tract is determined by the interaction between host and pathogen. In this review, an oversight of the different clinical entities of pulmonary aspergillosis is given, categorized by their main pathophysiological mechanisms. The underlying immune processes are discussed, and the main clinical, radiological, biochemical, microbiological, and histopathological findings are summarized.

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“…Chronic (>4 weeks) pulmonary symptoms (chronic productive cough, tenacious mucus production, dyspnea, and difficult airway clearance) 117…”

Section: Clinical Spectrum Of Pulmonary Aspergillosismentioning

confidence: 99%

Aspergillus and the Lung

Janssens,

Lambrecht,

Van Braeckel

2024

Semin Respir Crit Care Med

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“…Chest computed tomography (CT) scans often show bronchial wall thickening, segmental atelectasis, and with increasing prevalence with age, bronchiectasis [ 23 , 40 , 41 ]. Bronchiectasis in PCD is most common in the middle and lower lobes, unlike in CF where upper lobe bronchiectasis predominates [ 23 , 42 ]. Bronchiectasis is not specific to PCD, but mucus plugging, tree-in-bud opacities, and atelectasis in conjunction with bronchiectasis are more common in PCD than in other patients with non-CF bronchiectasis due to other etiologies [ 40 , 41 ].…”

Section: Clinical Features Of Pcdmentioning

confidence: 99%

Primary Ciliary Dyskinesia: A Clinical Review

Despotes,

Zariwala,

Davis

et al. 2024

Cells

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Primary ciliary dyskinesia (PCD) is a rare, genetically heterogeneous, motile ciliopathy, characterized by neonatal respiratory distress, recurrent upper and lower respiratory tract infections, subfertility, and laterality defects. Diagnosis relies on a combination of tests for confirmation, including nasal nitric oxide (nNO) measurements, high-speed videomicroscopy analysis (HSVMA), immunofluorescent staining, axonemal ultrastructure analysis via transmission electron microscopy (TEM), and genetic testing. Notably, there is no single gold standard confirmatory or exclusionary test. Currently, 54 causative genes involved in cilia assembly, structure, and function have been linked to PCD; this rare disease has a spectrum of clinical manifestations and emerging genotype–phenotype relationships. In this review, we provide an overview of the structure and function of motile cilia, the emerging genetics and pathophysiology of this rare disease, as well as clinical features associated with motile ciliopathies, novel diagnostic tools, and updates on genotype–phenotype relationships in PCD.

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“…The presence of P. aeruginosa likely contributes to higher host susceptibility to aspergillosis, specifically through a TLR5-mediated shift to a Th2 inflammatory response concordant with ABPA symptoms [ 100 , 116 ]. In addition, a new line of therapy to treat CF was introduced, which corrects effects caused by mutations in CFTR by modulating CFTR [ 37 , 117 , 118 , 119 ]. Considering this, forestalling a TLR5-mediated shift, combined with prophylactic inhibitory drugs that target Aspergilli, could possibly prevent the colonization of A. fumigatus before the development of ABPA.…”

Section: Host Susceptibility—immune Clearance and Evasion Of The Immu...mentioning

confidence: 99%

Novel Treatment Approach for Aspergilloses by Targeting Germination

Verburg

Neer

Duca

et al. 2022

JoF

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Germination of conidia is an essential process within the Aspergillus life cycle and plays a major role during the infection of hosts. Conidia are able to avoid detection by the majority of leukocytes when dormant. Germination can cause severe health problems, specifically in immunocompromised people. Aspergillosis is most often caused by Aspergillus fumigatus (A. fumigatus) and affects neutropenic patients, as well as people with cystic fibrosis (CF). These patients are often unable to effectively detect and clear the conidia or hyphae and can develop chronic non-invasive and/or invasive infections or allergic inflammatory responses. Current treatments with (tri)azoles can be very effective to combat a variety of fungal infections. However, resistance against current azoles has emerged and has been increasing since 1998. As a consequence, patients infected with resistant A. fumigatus have a reported mortality rate of 88% to 100%. Especially with the growing number of patients that harbor azole-resistant Aspergilli, novel antifungals could provide an alternative. Aspergilloses differ in defining characteristics, but germination of conidia is one of the few common denominators. By specifically targeting conidial germination with novel antifungals, early intervention might be possible. In this review, we propose several morphotypes to disrupt conidial germination, as well as potential targets. Hopefully, new antifungals against such targets could contribute to disturbing the ability of Aspergilli to germinate and grow, resulting in a decreased fungal burden on patients.

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CF Fungal Disease in the Age of CFTR Modulators

Cited by 21 publications

References 75 publications

Aspergillus and the Lung

Aspergillus and the Lung

Primary Ciliary Dyskinesia: A Clinical Review

Novel Treatment Approach for Aspergilloses by Targeting Germination

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