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PEDIATRICS Vol. 108 No. 5 November 2001, p. e86

ELECTRONIC ARTICLE:
Abnormal Central Complex Is a Marker of Severity in the Presence of Partial Ciliary Defect

Received Jan 16, 2001; accepted Jun 27, 2001.

Aline Tamalet*, Annick Clement*, Francoise Roudot-ThoravalDagger , Pascale Desmarquest*, Gilles Roger*, Michèle Boulé*, Marie Claude Millepied, TA§; Armelle Baculard*, and Estelle Escudierparallel

From the * Pediatric Pulmonology and ENT Departments, Armand Trousseau Hospital (AP-HP), Paris, France; the Dagger  Evaluation and Biostatistics Department, H. Mondor Hospital (AP-HP), Créteil, France; the § Department of Pathology (Electron Microscopy Laboratory), Intercommunal Hospital, Créteil, France; the parallel  Departments of Genetics, Cytogenetics and Embryology (Biology of Reproduction Unit), Pitié Salpetrière Hospital (AP-HP), Paris, France; and INSERM U492, Créteil, France.

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.  Key words:  respiratory tract infection, pediatric population, ciliary beat frequency, ciliary ultrastructure, bronchiectasis, pulmonary function.




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