title: Clinical features of primary ciliary dyskinesia in Cyprus with emphasis on lobectomized patients.
abstract: BACKGROUND
  Despite the manifestations of primary ciliary dyskinesia (PCD) in early life, the diagnosis is often much delayed. Since 1998 in Cyprus, we have established the only national diagnostic and clinical referral center for PCD.
  
  
  OBJECTIVE
  To review the phenotypic features at presentation of PCD patients in Cyprus in relation to age at diagnosis, with emphasis on previously lobectomised patients.
  
  
  METHODS
  The medical records of the diagnosed PCD patients were retrospectively reviewed to obtain clinical data on presentation.
  
  
  RESULTS
  Thirty patients, aged 13.9 years (range 0.1, 58.4 years), were diagnosed with PCD. Twelve of them presented after the age of 18. The most common manifestations were chronic cough (100%), chronic rhinorrhea (96.7%), sputum production (92.9%), laterality defects (63.3%), a history of pneumonia (53.3%) and neonatal respiratory distress (50%). A history of lobectomy in the past was recorded in 16.7% (5 patients). Patients who presented in adulthood had significantly higher frequency of lobectomy (41.7% vs 0%, p-value = 0.006) and had more frequently low FEV1 (58.3% vs 0%, p-value = 0.015) than those who presented before. Serial measurements of FEV1 and FVC indicated significantly lower intercepts in lobectomised compared to the adult non-lobectomised patients both in terms of FEV1 (-4.90 vs -1.80, p-value = 0.022) and FVC (-5.43 vs -1.91, p-value = 0.029) z-score levels. Change in FEV1 and FVC across time was not statistically significant in either group.
  
  
  CONCLUSIONS
  PCD often remains undiagnosed up to adulthood accompanied by appearance of advanced lung disease. Performance of lobectomies seems to be a poor prognostic factor for PCD in adulthood.
authors.affiliation: Cyprus International Institute for Environmental & Public Health in Association with Harvard School of Public Health, Cyprus University of Technology, Limassol, Cyprus; Department of Pediatrics, Hospital "Archbishop Makarios III", Nicosia, Cyprus. Electronic address: p.yiallouros@cut.ac.cy.
authors.forename: Panayiotis K
authors.initials: PK
authors.lastname: Yiallouros
authors.affiliation: Cyprus International Institute for Environmental & Public Health in Association with Harvard School of Public Health, Cyprus University of Technology, Limassol, Cyprus. Electronic address: panagiotis.kouis@cut.ac.cy.
authors.forename: Panayiotis
authors.initials: P
authors.lastname: Kouis
authors.affiliation: Department of Nursing, School of Health Sciences, Cyprus University of Technology, Limassol, Cyprus. Electronic address: nicos.middleton@cut.ac.cy.
authors.forename: Nicos
authors.initials: N
authors.lastname: Middleton
authors.affiliation: Department of Electron Microscopy and Molecular Pathology, Cyprus Institute of Neurology and Genetics, Nicosia, Cyprus. Electronic address: marianan@cing.ac.cy.
authors.forename: Marianna
authors.initials: M
authors.lastname: Nearchou
authors.affiliation: Department of Pulmonology, Nicosia General Hospital, Nicosia, Cyprus. Electronic address: tadamide@mphs.moh.gov.cy.
authors.forename: Tonia
authors.initials: T
authors.lastname: Adamidi
authors.affiliation: Department of Pulmonology, Nicosia General Hospital, Nicosia, Cyprus. Electronic address: angeorgiou@mphs.moh.gov.cy.
authors.forename: Andreas
authors.initials: A
authors.lastname: Georgiou
authors.affiliation: Department of Pulmonology, Pafos General Hospital, Pafos, Cyprus. Electronic address: adonise@cytanet.com.cy.
authors.forename: Adonis
authors.initials: A
authors.lastname: Eleftheriou
authors.affiliation: Department of Pediatrics, Hospital "Archbishop Makarios III", Nicosia, Cyprus. Electronic address: phiv.ioannou@gmail.com.
authors.forename: Phivos
authors.initials: P
authors.lastname: Ioannou
authors.affiliation: Department of Electron Microscopy and Molecular Pathology, Cyprus Institute of Neurology and Genetics, Nicosia, Cyprus; Cyprus School of Molecular Medicine, Nicosia, Cyprus. Electronic address: ahsavvas@cing.ac.cy.
authors.forename: Andreas
authors.initials: A
authors.lastname: Hadjisavvas
authors.affiliation: Department of Electron Microscopy and Molecular Pathology, Cyprus Institute of Neurology and Genetics, Nicosia, Cyprus; Cyprus School of Molecular Medicine, Nicosia, Cyprus. Electronic address: kyriacos@cing.ac.cy.
authors.forename: Kyriacos
authors.initials: K
authors.lastname: Kyriacou
mesh_terms: D000293:Adolescent; D000328:Adult; D001706:Biopsy; D015992:Body Mass Index; D002908:Chronic Disease; D003371:Cough / Q000209:etiology; D003535:Cyprus / Q000453:epidemiology; D057210:Delayed Diagnosis; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007619:Kartagener Syndrome / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D008297:Male; D016347:Medical Records Systems, Computerized; D008875:Middle Aged; D011013:Pneumonectomy / Q000379:methods; D015995:Prevalence; D012189:Retrospective Studies; D012307:Risk Factors; D013147:Spirometry
keywords: Diagnosis; Lobectomy; Lung function; Primary ciliary dyskinesia