Primary ciliary dyskinesia is an inherited disorder in which respiratory cilia are stationary, or beat in a slow or dyskinetic manner, leading to impaired mucociliary clearance and significant sinopulmonary disease. One diagnostic test is ciliary functional analysis using digital high‐speed video microscopy (DHSV), which allows real‐time analysis of complete ciliary function, comprising ciliary beat frequency (CBF) and ciliary beat pattern (CBP). However, DHSV lacks standardization. In this paper, the current knowledge of DHSV ciliary functional analysis is presented, and recommendations given for a standardized protocol for ciliary sample collection and processing. A proposal is presented for a quantitative and qualitative CBP evaluation system, to be used to develop international consensus agreement, and future DHSV research areas are identified.
Background: Prior to introducing social needs screening into our subspecialty clinics, we first wanted to understand the health effects of the major social challenges facing children with chronic diseases in British Columbia. Methods: Using a strict prospective methodology, avoiding use of health databases and proxy end points, we studied the effects of five social health determinants (distance from care, family income, gender, ethnicity, caregiver education), on health outcomes in three groups of children with chronic diseases: cystic fibrosis (CF), type 1 diabetes (T1D), chronic kidney disease (CKD). Social determinant data were collected at a face-to-face interview during a clinic visit. These were correlated with diagnosis-specific health outcomes, measured at the same visit. Main outcomes were: forced expired volume in 1 second (FEV 1 ) (CF group), HbA1c (T1D group), estimated glomerular filtration rate (CKD group). Results: We studied 270 children: 85 CF, 89 T1D and 96 CKD. In all three groups, children from families with annual income less than $45,000 had significantly worse health than those from families above this cut-off. Lower caregiver education was related to worse health in the CKD and T1D groups. We found no adverse health effects associated with distance from subspecialty care, patient ethnicity or gender. Conclusion: Even in a prosperous province, family poverty and lack of caregiver education still impose measurable adverse effects on the health of children with chronic diseases. We hope these results help support the integration of social needs screening into routine multidisciplinary outpatient clinics. Early detection of social problems and targeted interventions will hopefully help to equalize health outcomes between children from different social groups.
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