Nasal polyposis in cystic fibrosis: follow-up of children and adolescents for a 3-year period

Weber, Silke Anna Theresa; Iyomasa, Renata Mizusaki; Corrêa, Camila de Castro; Florentino, Wellington Novais Mafra; Ferrari, Giesela Fleischer

doi:10.1016/j.bjorl.2016.09.005

Cited by 14 publications

(7 citation statements)

References 27 publications

(40 reference statements)

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“…Nasal polyps in PCD have already been reported in the literature as occurring in 25% to 52% of adults [11,19] which is similar to our findings. The prevalence of nasal polyps in PCD is similar to cystic fibrosis (prevalence of 39–48%) [20,21]. Nasal polyps in PCD differ from the classic eosinophilic sinonasal polyposis, since they are associated with oedema, congestive mucosa, and sticky or purulent secretions.…”

Section: Discussionmentioning

confidence: 99%

Critical Evaluation of Sinonasal Disease in 64 Adults with Primary Ciliary Dyskinesia

Béquignon

Dupuy

Zerah-Lancner

et al. 2019

JCM

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To date, no study precisely described ear, nose and throat (ENT) disease in adults with primary ciliary dyskinesia (PCD) and its relationship with ciliary function/ultrastructure. A retrospective study of standardized ENT data (exam, audiogram, sinus Computed tomography (CT), and bacteriology) was conducted in 64 adults with confirmed PCD who were followed in two ENT reference centers. Rhinorrhoea and hearing loss were the main symptoms. Symptom scores were higher in older patients. Nasal endoscopy was abnormal in all patients except one, showing nasal polyps in one-third of the patients and stagnant nasal mucus secretions in 87.5% of the patients. Sinus CT opacities were mainly incomplete and showed one-third of the patients with sinus hypoplasia and/or agenesis. Middle meatus mainly grew Haemophilus influenzae, Streptoccocus pneumoniae and Pseudomonas aeruginosa. Otitis media with effusion (OME), which is constant in childhood, was diagnosed in less than one-quarter of the patients. In two-thirds of the patients, audiogram showed hearing loss that was sensorineural in half of the patients. ENT disease severity was not correlated with ciliary function and ultrastructure, but the presence of OME was significantly associated with a forced expiratory volume (FEV1) < 70%. Rhinosinusitis is the most common clinical feature of PCD in adults, while OME is less frequent. The presence of active OME in adults with PCD could be a severity marker of lung function and lead to closer monitoring.

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Section: Discussionmentioning

confidence: 99%

Critical Evaluation of Sinonasal Disease in 64 Adults with Primary Ciliary Dyskinesia

Béquignon

Dupuy

Zerah-Lancner

et al. 2019

JCM

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“…We also found some degrees of hypoplasia in at least one of the sinuses (51.5%) and the frontal sinus accounted for the highest percentage (48.9%). Frontal sinus agenesis is one of the notable features among patients with cystic fibrosis and chronic rhino sinusitis (9,22,24) . In this study, there was no statistical relationship between the presence of polyps with the examination, age, and symptoms, as well as examination findings, such as rhinorrhea, and sinonasal CT findings.…”

Section: Discussionmentioning

confidence: 99%

Sinonasal Manifestations in Children with Cystic Fibrosis

2019

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Background: Involvement of the upper respiratory tract and pathological changes in the nose and paranasal sinuses are common in patients with cystic fibrosis. Objectives: We aimed to identify sinonasal manifestations in Iranian children with cystic fibrosis. Methods: Forthy seven children with cystic fibrosis were enrolled and symptoms related to the nose and paranasal sinuses were recorded using a questionnaire. The patients' nasal and sinus CT scan were investigated in terms of findings related to cystic fibrosis. Results: The most common complaint was nasal congestion (61.7%). Ground glass opacity in the nasal cavity was found in 63.8% of the patients. Postnasal drip was observed in 36.2% of the patients. In nasal endoscopic examination, 15 (31.9%) patients had clear polyps in the nasal cavity. Conclusions: History, CT scans, and initial clinical examination (rhinorrhea in anterior rhinoscopy) does not allow accurate diagnosis of chronic rhino sinusitis or nasal polyps in children with cystic fibrosis.

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“…In patients with CF, quality of life especially in children and adolescents is seriously affected by severe damage to the lungs, digestive system and other organs in the body [2]. Pancreatitis [3], gastrointestinal complications [4], chronic endobronchial airway infection [5], pulmonary complications [6], sinusitis [7], diabetes mellitus [8], nasal polyps [9], antibiotic resistance [10], depression and anxiety [11], liver disease [12], osteoporosis [13], bronchiectasis [14] and arthritis [15], remain the most common complications for patients with CF. CF is characterized by pancreatic insufficiency and chronic endobronchial airway infection [16].…”

Section: Introductionmentioning

confidence: 99%

Identification of Key Genes and Underlying Mechanisms in Cystic Fibrosis and its Associated Complications Based on Bioinformatics Analysis of Next Generation Sequencing Data Analysis

Vastrad¹,

Vastrad²

2022

Preprint

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Cystic fibrosis (CF) is one of the inherited autosomal recessive disorders with very complicated pathogenesis. Identifying the molecular signatures and specific biomarkers of CF might provide novel clues for CF and and CF associated complications prognosis and targeted therapy. Based on the nexgeneration sequencing (NGS) dataset GSE136371 downloaded from the Gene Expression Omnibus (GEO) database, the differentially expressed genes (DEGs) between CF samples and normal controls were identified by using DESeq2 bioconductor package of R software. Gene ontology (GO) and REACTOME pathway enrichment analyses were applied for the DEGs. Then protein-protein interaction (PPI) network of these DEGs was visualized by Cytoscape with IMEx interactome database. The most significant module from the PPI network was selected for GO and pathway enrichment analysis. Subsequently, a miRNA-hub gene regulatory network and TF-hub gene regulatory network were constructed to identify hub genes, miRNAs and TFs. Finally, receiver operating characteristic curve (ROC) analysis was established to validate these hub genes. Total of 917 DEGs were identified between CF and normal control samples in GSE136371 dataset, including 479 up regulated and 438 down regulated genes. The most enriched DEGs in GO and pathway enrichment analysis were mainly associated with response to stimulus, regulation of cellular process, Neutrophil degranulation and rRNA processing. PPI network, module analysis, miRNA-hub gene regulatory network and TF-hub gene regulatory network predicted ten hub genes (FN1, UBE2D1, SRPK1, MAPK14, CEBPB, HSP90AB1, HSPA8, XRCC6, NCL and PARP1). In conclusion, the DEGs, relative pathways and hub genes identified in the present study might aid in understanding of the molecular mechanisms underlying CF and CF associated complications progression and provide potential molecular targets and biomarkers for CF and CF associated complications.

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Nasal polyposis in cystic fibrosis: follow-up of children and adolescents for a 3-year period

Cited by 14 publications

References 27 publications

Critical Evaluation of Sinonasal Disease in 64 Adults with Primary Ciliary Dyskinesia

Critical Evaluation of Sinonasal Disease in 64 Adults with Primary Ciliary Dyskinesia

Sinonasal Manifestations in Children with Cystic Fibrosis

Identification of Key Genes and Underlying Mechanisms in Cystic Fibrosis and its Associated Complications Based on Bioinformatics Analysis of Next Generation Sequencing Data Analysis

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