Rhinosinusitis in Pediatric Primary Ciliary Dyskinesia: Impact of Disease
| Autor: | Jay M. Bhatt, Ethan G Muhonen, Scott D. Sagel, Maxene Meier, Kenny H. Chan |
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| Rok vydání: | 2019 |
| Předmět: |
Male
Pathology medicine.medical_specialty Adolescent Mucociliary clearance Ciliary dyskinesia Disease Severity of Illness Index 03 medical and health sciences 0302 clinical medicine Risk Factors Humans Medicine Sinusitis Respiratory system Child 030223 otorhinolaryngology Retrospective Studies Rhinitis Primary ciliary dyskinesia business.industry Cilium Age Factors Infant Newborn Genetic disorder Infant Sinus surgery medicine.disease 030228 respiratory system Otorhinolaryngology Child Preschool Female Surgery business Ciliary Motility Disorders |
| Zdroj: | Otolaryngology–Head and Neck Surgery. 161:877-880 |
| ISSN: | 1097-6817 0194-5998 |
| Popis: | Primary ciliary dyskinesia (PCD) is a genetic disorder characterized by abnormal respiratory cilia ultrastructure and/or function causing defective mucociliary clearance. We investigated the extent and severity of rhinosinusitis in a large cohort of children with PCD and explored associations among risk factors, including genotype and sinus disease.Retrospective chart review.Tertiary academic children's hospital.A review was conducted with a patient registry at the PCD Foundation Center at our institution. Demographic, imaging, clinical, and operative data were reviewed through the institutional electronic health record system.Fifty-four subjects were identified with mean and median age at diagnosis of 5.2 and 4.0 years. The male:female ratio was 35%:65%. Sinus symptoms were present in 46 (85%) subjects, 22 of whom had chronic rhinosinusitis. Nineteen (35%) subjects underwent operative intervention, consisting of endoscopic sinus surgery (ESS; 16 patients) and maxillary lavage (3 patients). Nineteen subjects underwent adenoidectomy for PCD-related indications. Five sinus-related admissions in 3 subjects were noted during the study period, and no complication of rhinosinusitis occurred in the cohort. Genetic test results were available in 27 subjects, in whom 23 (85%) had biallelic mutations in a PCD gene. Demographic factors, Lund-Mackay score, and PCD genotype were not found to be predictors for ESS or hospitalization in our cohort.While rhinosinusitis was common in our PCD cohort, most patients did not require ESS. Since complications of rhinosinusitis were uncommon, we recommend judicious surgical management tailored to the patient's symptoms. |
| Databáze: | OpenAIRE |
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