Sinonasal quality of life in primary ciliary dyskinesia

Abstract

Key Points Our findings suggest that primary ciliary dyskinesia (PCD)‐related chronic rhinosinusitis (CRS) has a more significant impact on quality of life than CRS without nasal polyps and cystic fibrosis (CF). PCD and CF have similar mucociliary clearance defects, yet sinonasal symptom severity varies between the two.

Letter to the editor regarding “Sinonasal quality of life in primary ciliary dyskinesia”

“…However, as illustrated by Stack et al's second supplementary figure, these two conditions can present very differently in pediatric patients, with young CF patients complaining of minimal sinonasal QoL impact. 1 Our cohort, which adds further data regarding pediatric sinonasal QoL in PCD, demonstrates that the same cannot be said for young PCD patients, even for common sinonasal and respiratory tract symptoms. We found that PCD particularly impacts sleep symptoms in teenage patients, consistent with other work which has revealed obstructive sleep apnea to be a common finding in the pediatric PCD population.…”

“…In this work, the authors compare sinonasal QoL in patients with PCD to those with cystic fibrosis (CF). 1 The authors demonstrate that patients with PCD have more severe sinusitis symptomatology despite a common foundation in defective mucociliary clearance between the two conditions. Patients with PCD had a wide variety of rhinosinusitis disability index scores (30.4 ± 23.4), particularly when compared to CF (14.2 ± 15.8).…”

“…In writing this reply, we seek to add our experience in exploring the impact of PCD on pediatric sinonasal QoL to the information provided in the aforementioned study. 1 Pediatric patients with PCD represent a unique patient population; chronic sinus disease presents at an early age when aggressive medical or surgical interventions may be less desirable. At our center, we surveyed 17 patients under age 21 years (11.9 ± 5.7), of whom 10 were male (58.8%) and seven (41.2%) were female.…”

Histologic characterization of primary ciliary dyskinesia chronic rhinosinusitis

“…3 Despite a similar mechanism, the disease presents distinct endoscopic features (Figure 1), and PCDrelated CRS presents with worse QOL than CF-related CRS. 4 Therefore, this study aimed to compare SHRs outcomes of CF and PCD patients. Secondarily, we compared F I G U R E 1 Cystic fibrosis-related and primary ciliary dyskinesia-related chronic rhinosinusitis (CRS) appear distinct on nasal endoscopy.…”