Prevalence of rhinosinusitis among atypical cystic fibrosis patients

Marshak, Tal; Rivlin, Y.; Bentur, Lea; Ronen, Ohad; Uri, Nechama

doi:10.1007/s00405-010-1382-0

Cited by 19 publications

(10 citation statements)

References 28 publications

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“…Unlike in other studies, Marshak et al . found that symptoms related to asthma or sinonasal involvement were significantly more common in atypical CF patients than in typical CF patients.…”

Section: Resultsmentioning

confidence: 64%

Sinonasal alterations in computed tomography scans in cystic fibrosis: a literature review of observational studies

Kang

Piltcher

Dalcin

2014

Int Forum Allergy Rhinol

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“…Unlike in other studies, Marshak et al . found that symptoms related to asthma or sinonasal involvement were significantly more common in atypical CF patients than in typical CF patients.…”

Section: Resultsmentioning

confidence: 64%

Sinonasal alterations in computed tomography scans in cystic fibrosis: a literature review of observational studies

Kang

Piltcher

Dalcin

2014

Int Forum Allergy Rhinol

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“…Marshak et al found that chronic rhinosinusitis and nasal polyps were common in patients with CFTR‐related disease. The frequency of severe chronic rhinosinusitis was also somewhat higher in those with CFTR‐related disease than classical CF, but statistical significance was not attained …”

Section: Discussionmentioning

confidence: 92%

Clinical characteristics and genetic analysis of cystic fibrosis transmembrane conductance reseptor‐related disease

Kılınç¹,

Alishbayli²,

Taner³

et al. 2020

Pediatrics International

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Background: Cystic fibrosis (CF) transmembrane conductance receptor (CFTR)-related disease is diagnosed in patients affected by CFTR dysfunction who do not fully meet the CF diagnostic criteria. Only 2% of all CF patients have CFTR-related disease. We define the demographic characteristics of such patients, described the performance of mutational analyses, and describe the clinical findings. Methods: Twenty-four patients were followed-up for CFTR-related disease. Patients with CF symptoms but who did not completely fulfil the CF diagnostic criteria were enrolled. Age, body mass index at the times of diagnosis and admission, symptoms, pulmonary function and fecal elastase test results, gene analyses, and clinical findings during follow-up were evaluated. Results: Ten patients (42%) were female and 14 (58%) male. Their mean age was 15.3 years (minimum-maximum 6-20 years). The mean age at diagnosis was 8.5 years (minimum-maximum 3-14 years) and the most common presenting complaint was a cough (n = 19). During follow up, chronic sinusitis developed in 15 patients, bronchiectasis in 13, nasal polyposis in six, failure to thrive in three, recurrent pancreatitis in two, asthma in one, and congenital bilateral absence of the vas deferens in one. Fecal elastase levels were low in only one of the three patients who failed to thrive. In terms of CFTR gene mutations, two were found in 10 patients, one in eight patients, and none in six. Conclusions: Cystic fibrosis transmembrane conductance receptor-related disease presents with various clinical findings. Serious symptoms may develop later in life. Late diagnosis significantly compromises the quality and duration of life in such patients.

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“…CRS is reported in 30 to 67% of patients with CF (within all age groups) [116][117][118][119]. Some studies have suggested that this may be because of genetic mutation, such as the ∆F508 and 394delTT mutation in Finland [120].…”

Section: Cystic Fibrosis (Cf)mentioning

confidence: 99%

Medical Management of Chronic Rhinosinusitis in Adults

Malaty

2016

Sinusitis

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Chronic rhinosinusitis can be refractory and has detrimental effects not only on symptoms, but also on work absences, work productivity, annual productivity costs, and disease-specific quality of life measures. The pathophysiology of chronic rhinosinusitis continues to evolve. There is evidence that it is driven by various inflammatory pathways and host factors and is not merely an infectious problem, although pathogens, including bacterial biofilms, may certainly contribute to this inflammatory cascade and to treatment resistance. Given this, medical management should be tailored to the specific comorbidities and problems in an individual patient. In addition to treating acute exacerbations of chronic rhinosinusitis with amoxicillin-clavulanate, second or third generation cephalosporins, or fluoroquinolones, one must consider if nasal polyps are present, when symptoms and disease severity correlate to mucosal eosinophilia, and there is the best evidence for intranasal corticosteroids and saline irrigation. Asthma worsens severity of chronic rhinosinusitis and it is felt to be mediated by increased leukotrienes, when leukotriene antagonists may be utilized. Cystic fibrosis has a genetic defect and increased mucin, which are potential treatment targets with dornase alfa showing efficacy. Other comorbidities that may impact treatment include allergies, ciliary dyskinesia, immunodeficiency, and possibly allergic fungal rhinosinusitis.

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Prevalence of rhinosinusitis among atypical cystic fibrosis patients

Cited by 19 publications

References 28 publications

Sinonasal alterations in computed tomography scans in cystic fibrosis: a literature review of observational studies

Sinonasal alterations in computed tomography scans in cystic fibrosis: a literature review of observational studies

Clinical characteristics and genetic analysis of cystic fibrosis transmembrane conductance reseptor‐related disease

Medical Management of Chronic Rhinosinusitis in Adults

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