ABSTRACT. Background. Ciliary ultrastructural defects with total lack of dynein arms (DA) cause abnormal mucociliary function leading to the chronic infections observed in primary ciliary dyskinesia. The role of partial ciliary ultrastructural defects, especially those involving the central complex, and their relationship with respiratory symptoms have been less thoroughly investigated.Objective. In a pediatric population with partial ciliary defects, we determined the relationship(s) between ultrastructural findings, ciliary motility, and clinical and functional features, and evaluated the outcome of this population.Design. We analyzed the clinical presentation and pulmonary function of 43 children with chronic bronchitis and partial ultrastructural defects (from 15% to 90%) of their respiratory cilia demonstrated on bronchial biopsies. The study population was divided into 3 groups according to ciliary ultrastructure: the main ultrastructural defect concerned the central complex in 23 patients (CC group), peripheral microtubules in 8 patients (PMT group), and DA in 12 patients (DA group).Results. The percentage of ciliary defects was lower in the PMT group than in the CC and DA groups. Patients in the PMT group had less severe disease with frequent normal ciliary motility. Patients in the CC group had initially a higher incidence of respiratory tract infections, extensive bronchiectasis frequently requiring surgery, and arguments in favor of a congenital origin (high proportion of sibling form). Partial absence of DA, although of congenital origin, was associated with a good prognosis. In all groups, follow-up showed that the functional prognosis remained good with appropriate treatment.Conclusions. In children with chronic respiratory infections, presence of situs inversus, sibling form, obstructive pulmonary syndrome, or bronchiectasis required ultrastructural analysis, regardless of ciliary motility. Detection of CC abnormalities is a marker of severity and required intensive therapy and close follow-up. A irways are lined by respiratory epithelium composed of 2 main cell types, ciliated and goblet cells, which together ensure efficient mucociliary transport. 1,2 Mucociliary transport is an important defense mechanism, and ciliary beat frequency (CBF) is a major parameter in airway clearance. Each cilium beats in a coordinated fashion with its neighbors, producing unidirectional mucus flow. The structural components of the core of the cilium, known as the axoneme, is highly conserved and include 9 peripheral doublet microtubules with attached dyneis and radial spokes, and 2 central single microtubules (Fig 1A). Inner and outer dynein arms (DA) are the transducers of mechanical force necessary for ciliary motion. Failure of ciln armiary structure and function impairs airway clearance and can be responsible for respiratory tract infections. [3][4][5] Ciliary insufficiency can be caused by either an inborn error or damage inflicted on the cilia by a noxious agent. 6 -10 Congenital defects of ciliary ultrastruc...