{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T05:44:31Z","timestamp":1770788671519,"version":"3.50.0"},"reference-count":64,"publisher":"Archives of Pathology and Laboratory Medicine","issue":"11","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2008,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    <jats:italic>Context.<\/jats:italic>\n                    \u2014Ciliary dyskinesia is a rare, but significant, cause of chronic respiratory infections, and transmission electron microscopy is a critical adjunct to making the diagnosis.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:italic>Objective.<\/jats:italic>\n                    \u2014To investigate a single institution's experience with patients demonstrating abnormal ciliary ultrastructure.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:italic>Design.<\/jats:italic>\n                    \u2014Retrospective clinicopathologic review of 278 bronchial or nasal turbinate brushings or biopsies from 1983 through 2007.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:italic>Results.<\/jats:italic>\n                    \u2014There were 12 women and 9 men (mean age, 19.6 years; range, 1\u201354 years) with abnormal ciliary ultrastructure. Clinical history was unavailable in 3 patients, 15 (83%) of 18 patients presented with chronic or recurrent upper respiratory infections, and 3 (17%) presented with infertility. Seven (39%) of 18 patients had findings of Kartagener syndrome with situs inversus, dextrocardia, and bronchiectasis. Truncation or absence of inner or outer dynein arms occurred in 15 (71%) of 21 cases, and 5 (24%) revealed transposition defects with displacement of the central microtubules and peripheral doublets in 9 + 0 and 8 + 1 patterns. Radial spoke defects with microtubular disarray occurred in 4 (19%) of 21 cases. Compound cilia with multiple axonemes within a single outer sheath and supernumerary microtubules each occurred in 2 (10%) of the cases. Random ciliary orientation was also found in 2 (10%) of the cases, and dense granular basal body inclusions occurred in 1 case (5%). Multiple abnormalities occurred in 6 (29%) of the 21 cases.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:italic>Conclusions.<\/jats:italic>\n                    \u2014Most patients presented with chronic respiratory tract infections or infertility. Dynein arm defects, transposition defects, and radial spoke defects were the most commonly encountered abnormal findings. Less-frequent abnormal findings included compound cilia, supernumerary microtubules, and dense granular basal body inclusions.\n                  <\/jats:p>","DOI":"10.5858\/132.11.1786","type":"journal-article","created":{"date-parts":[[2021,2,12]],"date-time":"2021-02-12T00:03:47Z","timestamp":1613088227000},"page":"1786-1791","source":"Crossref","is-referenced-by-count":27,"title":["Ultrastructural Abnormalities of Respiratory Cilia: A 25-Year Experience"],"prefix":"10.5858","volume":"132","author":[{"given":"Thomas P.","family":"Plesec","sequence":"first","affiliation":[{"name":"From the Department of Anatomic Pathology, Cleveland Clinic Foundation, Cleveland, Ohio"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angela","family":"Ruiz","sequence":"additional","affiliation":[{"name":"From the Department of Anatomic Pathology, Cleveland Clinic Foundation, Cleveland, Ohio"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James T.","family":"McMahon","sequence":"additional","affiliation":[{"name":"From the Department of Anatomic Pathology, Cleveland Clinic Foundation, Cleveland, Ohio"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard A.","family":"Prayson","sequence":"additional","affiliation":[{"name":"From the Department of Anatomic Pathology, Cleveland Clinic Foundation, Cleveland, Ohio"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"3906","reference":[{"key":"i1543-2165-132-11-1786-b1","series-title":"Am J Med Sci","first-page":"3","article-title":"Disorders of ciliary motility.","volume":"321","author":"Cowan, M. 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Diagnostic approach to primary ciliary dyskinesia: a review.Eur J Pediatr2000. 159:95\u201398."},{"key":"i1543-2165-132-11-1786-b4","series-title":"Acta Paediatr","first-page":"667","article-title":"Primary ciliary dyskinesia: age at diagnosis and symptom history.","volume":"91","author":"Coren, M. E.","year":"2002","unstructured":"Coren, M. E., M.Meeks, I.Morrison, R. M.Buchdahl, and A.Bush. Primary ciliary dyskinesia: age at diagnosis and symptom history.Acta Paediatr2002. 91:667\u2013669."},{"key":"i1543-2165-132-11-1786-b5","series-title":"Ultrastruct Pathol","first-page":"243","article-title":"Clinico-pathological evaluation of ciliary dyskinesia: diagnostic role of electron microscopy.","volume":"27","author":"Pizzi, S.","year":"2003","unstructured":"Pizzi, S., S.Cazzato, F.Bernardi, W.Mantovani, and G.Cenacchi. Clinico-pathological evaluation of ciliary dyskinesia: diagnostic role of electron microscopy.Ultrastruct Pathol2003. 27:243\u2013252."},{"key":"i1543-2165-132-11-1786-b6","series-title":"Am J Respir Crit Care Med","first-page":"459","article-title":"Primary ciliary dyskinesia: diagnostic and phenotypic features.","volume":"169","author":"Noone, P. G.","year":"2004","unstructured":"Noone, P. G., M. W.Leigh, and A.Sannuti. et al. Primary ciliary dyskinesia: diagnostic and phenotypic features.Am J Respir Crit Care Med2004. 169:459\u2013467."},{"key":"i1543-2165-132-11-1786-b7","series-title":"Otolaryngol Head Neck Surg","first-page":"68","article-title":"New developments in the diagnosis of Kartagener's syndrome.","volume":"116","author":"Teknos, T. N.","year":"1997","unstructured":"Teknos, T. N., R.Metson, T.Chasse, G.Balercia, and G. R.Dickersin. 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Primary ciliary dyskinesia: clinical presentation, diagnosis and genetics.Ann Med2005. 37:439\u2013449."},{"key":"i1543-2165-132-11-1786-b24","series-title":"Acta Otolaryngol","first-page":"527","article-title":"The secondary nature of ciliary (dis)orientation in secondary and primary ciliary dyskinesia.","volume":"124","author":"Jorissen, M.","year":"2004","unstructured":"Jorissen, M. and T.Willems. The secondary nature of ciliary (dis)orientation in secondary and primary ciliary dyskinesia.Acta Otolaryngol2004. 124:527\u2013531."},{"key":"i1543-2165-132-11-1786-b25","series-title":"Annu Rev Physiol","first-page":"423","article-title":"Genetic defects in ciliary structure and function.","volume":"69","author":"Zariwala, M. A.","year":"2007","unstructured":"Zariwala, M. A., M. R.Knowles, and H.Omran. Genetic defects in ciliary structure and function.Annu Rev Physiol2007. 69:423\u2013450."},{"key":"i1543-2165-132-11-1786-b26","series-title":"Pediatr Pulmonol","first-page":"307","article-title":"Primary ciliary dyskinesia (PCD).","volume":"29","author":"Meeks, M.","year":"2000","unstructured":"Meeks, M. and A.Bush. Primary ciliary dyskinesia (PCD).Pediatr Pulmonol2000. 29:307\u2013316."},{"key":"i1543-2165-132-11-1786-b27","series-title":"Nat Genet","first-page":"143","article-title":"Mutations in DNAH5 cause primary ciliary dyskinesia and randomization of left-right asymmetry.","volume":"30","author":"Olbrich, H.","year":"2002","unstructured":"Olbrich, H., K.H\u00e4ffner, and A.Kispert. et al. 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Ciliary function analysis for the diagnosis of primary ciliary dyskinesia: advantages of ciliogenesis in culture.Acta Otolaryngol2000. 120:291\u2013295."},{"key":"i1543-2165-132-11-1786-b21","series-title":"Hum Mutat","first-page":"289","article-title":"Primary ciliary dyskinesia associated with normal axoneme ultrastructure is caused by DNAH11 mutations.","volume":"29","author":"Schwabe, G. C.","year":"2008","unstructured":"Schwabe, G. C., K.Hoffmann, and N. T.Loges. et al. Primary ciliary dyskinesia associated with normal axoneme ultrastructure is caused by DNAH11 mutations.Hum Mutat2008. 29:289\u2013298."},{"key":"i1543-2165-132-11-1786-b22","series-title":"Ann Otol Rhinol Laryngol","first-page":"264","article-title":"Primary ciliary dyskinesia: quantitative investigation of the ciliary ultrastructure with statistical analysis.","volume":"96","author":"Van der Baan, S.","year":"1987","unstructured":"Van der Baan, S., A. J. P.Veerman, P. D.Bezemer, and L.Feenstra. Primary ciliary dyskinesia: quantitative investigation of the ciliary ultrastructure with statistical analysis.Ann Otol Rhinol Laryngol1987. 96:264\u2013272."},{"key":"i1543-2165-132-11-1786-b23","series-title":"Ann Med","first-page":"439","article-title":"Primary ciliary dyskinesia: clinical presentation, diagnosis and genetics.","volume":"37","author":"Storm van's Gravesande, K.","year":"2005","unstructured":"Storm van's Gravesande, K. and H.Omran. Primary ciliary dyskinesia: clinical presentation, diagnosis and genetics.Ann Med2005. 37:439\u2013449."},{"key":"i1543-2165-132-11-1786-b24","series-title":"Acta Otolaryngol","first-page":"527","article-title":"The secondary nature of ciliary (dis)orientation in secondary and primary ciliary dyskinesia.","volume":"124","author":"Jorissen, M.","year":"2004","unstructured":"Jorissen, M. and T.Willems. The secondary nature of ciliary (dis)orientation in secondary and primary ciliary dyskinesia.Acta Otolaryngol2004. 124:527\u2013531."},{"key":"i1543-2165-132-11-1786-b25","series-title":"Annu Rev Physiol","first-page":"423","article-title":"Genetic defects in ciliary structure and function.","volume":"69","author":"Zariwala, M. A.","year":"2007","unstructured":"Zariwala, M. A., M. R.Knowles, and H.Omran. Genetic defects in ciliary structure and function.Annu Rev Physiol2007. 69:423\u2013450."},{"key":"i1543-2165-132-11-1786-b26","series-title":"Pediatr Pulmonol","first-page":"307","article-title":"Primary ciliary dyskinesia (PCD).","volume":"29","author":"Meeks, M.","year":"2000","unstructured":"Meeks, M. and A.Bush. Primary ciliary dyskinesia (PCD).Pediatr Pulmonol2000. 29:307\u2013316."},{"key":"i1543-2165-132-11-1786-b27","series-title":"Nat Genet","first-page":"143","article-title":"Mutations in DNAH5 cause primary ciliary dyskinesia and randomization of left-right asymmetry.","volume":"30","author":"Olbrich, H.","year":"2002","unstructured":"Olbrich, H., K.H\u00e4ffner, and A.Kispert. et al. Mutations in DNAH5 cause primary ciliary dyskinesia and randomization of left-right asymmetry.Nat Genet2002. 30:143\u2013144."},{"key":"i1543-2165-132-11-1786-b28","series-title":"Proc Natl Acad Sci U S A","first-page":"10282","article-title":"Mutations in the DNAH11 (axonemal heavy chain dynein type 11) gene cause one form of situs inversus totalis and most likely primary ciliary dyskinesia.","volume":"99","author":"Bartoloni, L.","year":"2002","unstructured":"Bartoloni, L., J. L.Blouin, and Y.Pan. et al. Mutations in the DNAH11 (axonemal heavy chain dynein type 11) gene cause one form of situs inversus totalis and most likely primary ciliary dyskinesia.Proc Natl Acad Sci U S A2002. 99:10282\u201310286."},{"key":"i1543-2165-132-11-1786-b29","series-title":"N Engl J Med","first-page":"318","article-title":"Transposition of ciliary microtubules: another cause of impaired ciliary motility.","volume":"303","author":"Sturgess, J. M.","year":"1980","unstructured":"Sturgess, J. M., J.Chao, and J. A. P.Turner. Transposition of ciliary microtubules: another cause of impaired ciliary motility.N Engl J Med1980. 303:318\u2013322."},{"key":"i1543-2165-132-11-1786-b30","series-title":"N Engl J Med","first-page":"53","article-title":"Cilia with defective radial spokes: a cause of human respiratory disease.","volume":"300","author":"Sturgess, J. M.","year":"1979","unstructured":"Sturgess, J. M., J.Chao, J.Wong, N.Aspin, and J. A.Turner. Cilia with defective radial spokes: a cause of human respiratory disease.N Engl J Med1979. 300:53\u201356."},{"key":"i1543-2165-132-11-1786-b31","series-title":"Eur J Respir Dis","first-page":"607","article-title":"Supernumerary microtubules in the cilia of two siblings causing \u201cimmotile cilia syndrome.\u201d.","volume":"64","author":"Antonelli, M.","year":"1983","unstructured":"Antonelli, M., A.Modesti, S.Quattrucci, and M.de Angelis. Supernumerary microtubules in the cilia of two siblings causing \u201cimmotile cilia syndrome.\u201d.Eur J Respir Dis1983. 64:607\u2013612."},{"key":"i1543-2165-132-11-1786-b32","series-title":"New Engl J Med","first-page":"1681","article-title":"Random ciliary orientation: a cause of respiratory tract disease.","volume":"323","author":"Rutland, J.","year":"1990","unstructured":"Rutland, J. and R. U.de Iongh. 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