{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T07:35:47Z","timestamp":1791272147051,"version":"4.1.0"},"reference-count":30,"publisher":"American Academy of Pediatrics (AAP)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2001,11,1]]},"abstract":"<jats:sec><jats:title>Background.<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective.<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Design.<\/jats:title><jats:p>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).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results.<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions.<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec>","DOI":"10.1542\/peds.108.5.e86","type":"journal-article","created":{"date-parts":[[2004,8,13]],"date-time":"2004-08-13T23:55:51Z","timestamp":1092441351000},"page":"e86-e86","source":"Crossref","is-referenced-by-count":41,"title":["Abnormal Central Complex Is a Marker of Severity in the Presence of Partial Ciliary Defect"],"prefix":"10.1542","volume":"108","author":[{"given":"Aline","family":"Tamalet","sequence":"first","affiliation":[{"name":"From the Pediatric Pulmonology and ENT Departments, Armand Trousseau Hospital (AP-HP), Paris, France; the"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annick","family":"Clement","sequence":"additional","affiliation":[{"name":"From the Pediatric Pulmonology and ENT Departments, Armand Trousseau Hospital (AP-HP), Paris, France; the"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francoise","family":"Roudot-Thoraval","sequence":"additional","affiliation":[{"name":"Evaluation and Biostatistics Department, H. Mondor Hospital (AP-HP), Cre\u0301teil, France; the"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascale","family":"Desmarquest","sequence":"additional","affiliation":[{"name":"From the Pediatric Pulmonology and ENT Departments, Armand Trousseau Hospital (AP-HP), Paris, France; the"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gilles","family":"Roger","sequence":"additional","affiliation":[{"name":"From the Pediatric Pulmonology and ENT Departments, Armand Trousseau Hospital (AP-HP), Paris, France; the"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miche\u0300le","family":"Boule\u0301","sequence":"additional","affiliation":[{"name":"From the Pediatric Pulmonology and ENT Departments, Armand Trousseau Hospital (AP-HP), Paris, France; the"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie Claude","family":"Millepied","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"TA\u00a7; Armelle","family":"Baculard","sequence":"additional","affiliation":[{"name":"From the Pediatric Pulmonology and ENT Departments, Armand Trousseau Hospital (AP-HP), Paris, France; the"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estelle","family":"Escudier","sequence":"additional","affiliation":[{"name":"Departments of Genetics, Cytogenetics and Embryology (Biology of Reproduction Unit), Pitie\u0301 Salpetrie\u0300re Hospital (AP-HP), Paris, France; and INSERM U492, Cre\u0301teil, France."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2001,11,1]]},"reference":[{"key":"2021121003544953000_B1","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1164\/arrd.1977.116.4.705","article-title":"The cells of the pulmonary airways.","volume":"116","author":"Breeze","year":"1977","journal-title":"Am Rev Respir Dis"},{"key":"2021121003544953000_B2","first-page":"287","article-title":"Ciliary function in transport of mucus.","volume":"64","author":"Sleigh","year":"1983","journal-title":"Eur J Respir Dis"},{"key":"2021121003544953000_B3","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1126\/science.1084576","article-title":"A human syndrome caused by immobile cilia.","volume":"193","author":"Afzelius","year":"1976","journal-title":"Science"},{"key":"2021121003544953000_B4","first-page":"71","article-title":"Screening for ciliary dyskinesia. A spectrum of defects of motility and structure.","volume":"64","author":"Rutland","year":"1983","journal-title":"Eur J Respir Dis"},{"key":"2021121003544953000_B5","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1002\/ppul.1950050109","article-title":"Primary ciliary dyskinesia: evaluation and Management.","volume":"5","author":"Rossman","year":"1988","journal-title":"Pediatr Pulmonol"},{"key":"2021121003544953000_B6","doi-asserted-by":"crossref","first-page":"688","DOI":"10.3109\/00016488309139463","article-title":"Primary and secondary ciliary dyskinesia.","volume":"95","author":"Mygind","year":"1983","journal-title":"Acta Otolaryngol"},{"key":"2021121003544953000_B7","first-page":"5","article-title":"Acquired ciliary defects compared to those seen in the immobile-cilia syndrome.","volume":"64","author":"Afzelius","year":"1983","journal-title":"Eur J Respir Dis"},{"key":"2021121003544953000_B8","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1007\/BF02713856","article-title":"Abnormalities of bronchial cilia in patients with chronic bronchitis. An ultrastructural and quantitative analysis.","volume":"161","author":"Lungarella","year":"1983","journal-title":"Lung"},{"key":"2021121003544953000_B9","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1056\/NEJM198502213120802","article-title":"Acquired ciliary defects in nasal epithelium of children with acute viral upper respiratory infections.","volume":"312","author":"Carson","year":"1985","journal-title":"N Engl J Med"},{"key":"2021121003544953000_B10","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1177\/000348948809700308","article-title":"Acquired ultrastructural alterations of respiratory cilia and clinical disease. A review.","volume":"97","author":"Ballenger","year":"1988","journal-title":"Ann Otol Rhinol Laryngol"},{"key":"2021121003544953000_B11","first-page":"271","article-title":"Abnormalities of the ciliary axonemal complex in children: an ultrastructural and kinetic study in a series of 34 cases.","volume":"44","author":"Escalier","year":"1982","journal-title":"Biol Cell"},{"key":"2021121003544953000_B12","first-page":"161","article-title":"Nasal ciliary ultrastructure and function in patients with primary ciliary dyskinesia compared with that in normal subjects and in subjects with various respiratory diseases.","volume":"129","author":"Rossman","year":"1984","journal-title":"Am Rev Respir Dis"},{"key":"2021121003544953000_B13","first-page":"287","article-title":"Determination of capillary oxygen tension in infants and children.","volume":"14","author":"Gaultier","year":"1979","journal-title":"Bull Eur Physiopath Resp"},{"key":"2021121003544953000_B14","first-page":"1045","article-title":"Re\u0301sistance pulmonaire totale, compliance pulmonaire dynamique chez l'enfant de 3a\u0300 15 ans.","volume":"50","author":"Gaultier","year":"1974","journal-title":"Sem Hop Paris"},{"key":"2021121003544953000_B15","doi-asserted-by":"crossref","first-page":"908","DOI":"10.1164\/ajrccm\/147.4.908","article-title":"Do nasal ciliary changes reflect bronchial changes? An ultrastructural study.","volume":"147","author":"Verra","year":"1993","journal-title":"Am Rev Respir Dis"},{"key":"2021121003544953000_B16","doi-asserted-by":"crossref","first-page":"1559","DOI":"10.1164\/ajrccm.151.5.7735615","article-title":"Ciliary defects in healthy subjects, bronchiectasis, and primary ciliary dyskinesia.","volume":"151","author":"de Iongh","year":"1995","journal-title":"Am J Respir Crit Care Med"},{"key":"2021121003544953000_B17","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1007\/BF00464443","article-title":"A method for measuring the orientation (beat direction) of respiratory cilia.","volume":"243","author":"Rautiainen","year":"1986","journal-title":"Arch Otorhinolaryngol"},{"key":"2021121003544953000_B18","doi-asserted-by":"crossref","first-page":"140","DOI":"10.3109\/00016489709124106","article-title":"Clinical relevance of tubulus anomalies and compound cilia.","volume":"529","author":"Torkkeli","year":"1997","journal-title":"Acta Otolaryngol"},{"key":"2021121003544953000_B19","first-page":"189","article-title":"Primary ciliary dyskinesia: ultrastructural defects and clinical features.","volume":"33","author":"Min","year":"1995","journal-title":"Rhinology"},{"key":"2021121003544953000_B20","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1016\/S0344-0338(84)80093-X","article-title":"Atypical bronchial cilia in children with recurrent respiratory tract infections: a comparative ultrastructural study.","volume":"178","author":"Cornillie","year":"1984","journal-title":"Path Res Pract"},{"key":"2021121003544953000_B21","doi-asserted-by":"crossref","first-page":"3091","DOI":"10.1016\/S0021-9258(17)30186-2","article-title":"Paralysed flagella mutants ofChlamydomonas reinhardtii defective for axonemal doublet microtubule arms.","volume":"254","author":"Huang","year":"1979","journal-title":"J Biol Chem"},{"key":"2021121003544953000_B22","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1056\/NEJM197707072970101","article-title":"The immotile-cilia sndrome. A congenital ciliary abnormality as an etiologic factor in chronic airway infections and male sterility","volume":"297","author":"Eliasson","year":"1977","journal-title":". N Engl J Med"},{"key":"2021121003544953000_B23","first-page":"1","article-title":"The immobile-cilia syndrome and other ciliary disease.","volume":"19","author":"Afzelius","year":"1979","journal-title":"Int Rev Exp Path"},{"key":"2021121003544953000_B24","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1016\/S0022-5320(79)80041-6","article-title":"Flagellar mutants in man: on heterogeneity of the immobile-cilia syndrome.","volume":"69","author":"Afzelius","year":"1979","journal-title":"J Ultrastruct Res"},{"key":"2021121003544953000_B25","doi-asserted-by":"crossref","first-page":"63","DOI":"10.3109\/10409238509086788","article-title":"The immobile-cilia syndrome: a microtubule-associated defect.","volume":"19","author":"Afzelius","year":"1985","journal-title":"Rev Biochem"},{"key":"2021121003544953000_B26","doi-asserted-by":"crossref","first-page":"364","DOI":"10.1083\/jcb.84.2.364","article-title":"Microtubule-membrane interactions in cilia. Isolation and characterization of ciliary membranes from Tetrahymena pyriforis.","volume":"84","author":"Dentler","year":"1980","journal-title":"J Cell Biol"},{"key":"2021121003544953000_B27","doi-asserted-by":"crossref","first-page":"2376","DOI":"10.1183\/09031936.97.10102376","article-title":"Longitudinal study of lung function in a cohort of primary ciliary dyskinesia.","volume":"10","author":"Ellerman","year":"1997","journal-title":"Eur Respir J"},{"key":"2021121003544953000_B28","first-page":"544","article-title":"The immobile cilia syndrome, a longitudinal survey.","volume":"124","author":"Corkey","year":"1981","journal-title":"Am Rev Respir Dis"},{"key":"2021121003544953000_B29","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1148\/radiology.154.3.3969467","article-title":"The immobile cilia syndrome: radiological manifestations.","volume":"154","author":"Nadel","year":"1985","journal-title":"Radiology"},{"key":"2021121003544953000_B30","first-page":"301","article-title":"The chest film in immobile cilia syndrome in children.","volume":"29","author":"Faure\u0301","year":"1986","journal-title":"Ann Radiol"}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/108\/5\/e86\/892819\/pe11010000o1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/108\/5\/e86\/892819\/pe11010000o1.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,14]],"date-time":"2024-01-14T05:06:14Z","timestamp":1705208774000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/108\/5\/e86\/63739\/Abnormal-Central-Complex-Is-a-Marker-of-Severity"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2001,11,1]]},"references-count":30,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2001,11,1]]},"published-print":{"date-parts":[[2001,11,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.108.5.e86","relation":{},"ISSN":["1098-4275","0031-4005"],"issn-type":[{"value":"1098-4275","type":"electronic"},{"value":"0031-4005","type":"print"}],"subject":[],"published":{"date-parts":[[2001,11,1]]}}}