Longitudinal lung function and structural changes in children with primary ciliary dyskinesia

Pediatric Pulmonology

Volumn 47, Issue 8, 2012, Pages 816-825

  Magnin, Marie Lémery ^a   Cros, Pierrick ^a   Beydon, Nicole ^a   Mahloul, Malika ^b   Tamalet, Aline ^a   Escudier, Estelle ^a,c   Clément, Annick ^a,c   Le Pointe, Hubert Ducou ^a,c   Blanchon, Sylvain ^a  

^a ARMAND TROUSSEAU HOSPITAL   (France)

^b HÔPITAL SAINT ANTOINE   (France)

^c SORBONNE UNIVERSITÉ   (France)

Author keywords

blood gas; computed tomography; Kartagener syndrome; lung function test

Indexed keywords

ADOLESCENT; ARTICLE; BLOOD GAS ANALYSIS; BRONCHIECTASIS; CHILD; CILIARY DYSKINESIA; CLINICAL ARTICLE; CLINICAL EVALUATION; COMPUTER ASSISTED TOMOGRAPHY; CONTROLLED STUDY; DATA ANALYSIS; FEMALE; FOLLOW UP; FORCED EXPIRATORY FLOW; FORCED VITAL CAPACITY; HUMAN; HYPERINFLATION; LONGITUDINAL STUDY; LUNG FUNCTION; LUNG FUNCTION TEST; LUNG PARENCHYMA; LUNG STRUCTURE; MALE; MEDICAL RECORD REVIEW; PRESCHOOL CHILD; PRIORITY JOURNAL; SCHOOL CHILD; SPIROMETRY;

ADOLESCENT; ADULT; BLOOD GAS ANALYSIS; CHILD; CHILD, PRESCHOOL; DISEASE PROGRESSION; FEMALE; HUMANS; INFANT; KARTAGENER SYNDROME; LONGITUDINAL STUDIES; LUNG; MALE; RESPIRATORY FUNCTION TESTS; RETROSPECTIVE STUDIES; TOMOGRAPHY, X-RAY COMPUTED;

EID: 84863728715     PISSN: 87556863     EISSN: 10990496     Source Type: Journal    
DOI: 10.1002/ppul.22577     Document Type: Article

References (49)

1. Afzelius BA,. A human syndrome caused by immotile cilia. Science 1976; 193: 317-319.
2. Escudier E, Duquesnoy P, Papon JF, Amselem S,. Ciliary defects and genetics of primary ciliary dyskinesia. Paediatr Respir Rev 2009; 10: 51-54.
3. Afzelius BA,. The immotile-cilia syndrome: a microtubule-associated defect. CRC Crit Rev Biochem 1985; 19: 63-87.
4. Papon JF, Coste A, Roudot-Thoraval F, Boucherat M, Roger G, Tamalet A, Vojtek AM, Amselem S, Escudier E,. A 20-year experience of electron microscopy in the diagnosis of primary ciliary dyskinesia. Eur Respir J 2009; 35: 1057-1063.
5. Barbato A, Frischer T, Kuehni CE, Snijders D, Azevedo I, Baktai G, Bartoloni L, Eber E, Escribano A, Haarman E, Hesselmar B, Hogg C, Jorissen M, Lucas J, Nielsen KG, O'Callaghan C, Omran H, Pohunek P, Strippoli MP, Bush A,. Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children. Eur Respir J 2009; 34: 1264-1276.
6. Jain K, Padley SP, Goldstraw EJ, Kidd SJ, Hogg C, Biggart E, Bush A,. Primary ciliary dyskinesia in the paediatric population: range and severity of radiological findings in a cohort of patients receiving tertiary care. Clin Radiol 2007; 62: 986-993. (Pubitemid 47317140)
7. Kennedy MP, Noone PG, Leigh MW, Zariwala MA, Minnix SL, Knowles MR, Molina PL,. High-resolution CT of patients with primary ciliary dyskinesia. AJR Am J Roentgenol 2007; 188: 1232-1238. (Pubitemid 46668503)
8. Noone PG, Leigh MW, Sannuti A, Minnix SL, Carson JL, Hazucha M, Zariwala MA, Knowles MR,. Primary ciliary dyskinesia: diagnostic and phenotypic features. Am J Respir Crit Care Med 2004; 169: 459-467. (Pubitemid 38187117)
9. Santamaria F, Montella S, Tiddens HA, Guidi G, Casotti V, Maglione M, de Jong PA,. Structural and functional lung disease in primary ciliary dyskinesia. Chest 2008; 134: 351-357.
10. Corkey CW, Levison H, Turner JA,. The immotile cilia syndrome. A longitudinal survey. Am Rev Respir Dis 1981; 124: 544-548. (Pubitemid 12236675)
11. Ellerman A, Bisgaard H,. Longitudinal study of lung function in a cohort of primary ciliary dyskinesia. Eur Respir J 1997; 10: 2376-2379. (Pubitemid 27479564)
12. Hellinckx J, Demedts M, De Boeck K,. Primary ciliary dyskinesia: evolution of pulmonary function. Eur J Pediatr 1998; 157: 422-426. (Pubitemid 28225448)
13. Marthin JK, Petersen N, Skovgaard LT, Nielsen KG,. Lung function in patients with primary ciliary dyskinesia: a cross-sectional and 3-decade longitudinal study. Am J Respir Crit Care Med 2010; 181: 1262-1268.
14. Sarria EE, Mattiello R, Rao L, Wanner MR, Raske ME, Tiller C, Kimmel R, Tepper RS,. Computed tomography score and pulmonary function in infants with chronic lung disease of infancy. Eur Respir J 2011; 38: 918-923.
15. Mastellari P, Biggi S, Lombardi A, Zompatori M, Grzincich G, Pisi G, Spaggiari C,. Correlation between HRCT and pulmonary functional tests in cystic fibrosis. Radiol Med 2005; 110: 325-333. (Pubitemid 43028135)
16. Santamaria F, Montella S, Camera L, Palumbo C, Greco L, Boner AL,. Lung structure abnormalities, but normal lung function in pediatric bronchiectasis. Chest 2006; 130: 480-486. (Pubitemid 44267016)
17. Maglione M, Bush A, Montella S, Mollica C, Manna A, Esposito A, Santamaria F,. Progression of lung disease in primary ciliary dyskinesia: is spirometry less accurate than CT? Pediatr Pulmonol 2012; 47: 498-504.
18. de Jong PA, Lindblad A, Rubin L, Hop WC, de Jongste JC, Brink M, Tiddens HA,. Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax 2006; 61: 80-85. (Pubitemid 43106983)
19. Judge EP, Dodd JD, Masterson JB, Gallagher CG,. Pulmonary abnormalities on high-resolution CT demonstrate more rapid decline than FEV1 in adults with cystic fibrosis. Chest 2006; 130: 1424-1432. (Pubitemid 44786736)
20. Tamalet A, Clement A, Roudot-Thoraval F, Desmarquest P, Roger G, Boule M, Millepied MC, Baculard TA, Escudier E,. Abnormal central complex is a marker of severity in the presence of partial ciliary defect. Pediatrics 2001; 108: E86.
21. Escudier E, Couprie M, Duriez B, Roudot-Thoraval F, Millepied MC, Pruliere-Escabasse V, Labatte L, Coste A,. Computer-assisted analysis helps detect inner dynein arm abnormalities. Am J Respir Crit Care Med 2002; 166: 1257-1262. (Pubitemid 35222604)
22. Soni R, Dobbin CJ, Milross MA, Young IH, Bye PP,. Gas exchange in stable patients with moderate-to-severe lung disease from cystic fibrosis. J Cyst Fibros 2008; 7: 285-291.
23. Gaultier C, Boule M, Allaire Y, Clement A, Buvry A, Girard F,. Determination of capillary oxygen tension in infants and children: assessment of methodology and normal values during growth. Bull Eur Physiopathol Respir 1979; 14: 287-297.
24. Miller MR, Hankinson J, Brusasco V, Burgos F, Casaburi R, Coates A, Crapo R, Enright P, van der Grinten CP, Gustafsson P, Jensen R, Johnson DC, MacIntyre N, McKay R, Navajas D, Pedersen OF, Pellegrino R, Viegi G, Wanger J,. Standardisation of spirometry. Eur Respir J 2005; 26: 319-338. (Pubitemid 41079078)
25. Stanojevic S, Wade A, Cole TJ, Lum S, Custovic A, Silverman M, Hall GL, Welsh L, Kirkby J, Nystad W, Badier M, Davis S, Turner S, Piccioni P, Vilozni D, Eigen H, Vlachos-Mayer H, Zheng J, Tomalak W, Jones M, Hankinson JL, Stocks J,. Spirometry centile charts for young Caucasian children: the Asthma UK Collaborative Initiative. Am J Respir Crit Care Med 2009; 180: 547-552.
26. Brisse HJ, Aubert B,. CT exposure from pediatric MDCT: results from the 2007-2008 SFIPP/ISRN survey. J Radiol 2009; 90: 207-215.
27. Bhalla M, Turcios N, Aponte V, Jenkins M, Leitman BS, McCauley DI, Naidich DP,. Cystic fibrosis: scoring system with thin-section CT. Radiology 1991; 179: 783-788.
28. Brody AS, Klein JS, Molina PL, Quan J, Bean JA, Wilmott RW,. High-resolution computed tomography in young patients with cystic fibrosis: distribution of abnormalities and correlation with pulmonary function tests. J Pediatr 2004; 145: 32-38. (Pubitemid 38962449)
29. Fauroux B, Nicot F, Essouri S, Hart N, Clement A, Polkey MI, Lofaso F,. Setting of noninvasive pressure support in young patients with cystic fibrosis. Eur Respir J 2004; 24: 624-630. (Pubitemid 39406136)
30. Hart N, Polkey MI, Clement A, Boule M, Moxham J, Lofaso F, Fauroux B,. Changes in pulmonary mechanics with increasing disease severity in children and young adults with cystic fibrosis. Am J Respir Crit Care Med 2002; 166: 61-66. (Pubitemid 34693964)
31. Hart N, Tounian P, Clement A, Boule M, Polkey MI, Lofaso F, Fauroux B,. Nutritional status is an important predictor of diaphragm strength in young patients with cystic fibrosis. Am J Clin Nutr 2004; 80: 1201-1206. (Pubitemid 41350679)
32. Zavorsky GS, Cao J, Mayo NE, Gabbay R, Murias JM,. Arterial versus capillary blood gases: a meta-analysis. Respir Physiol Neurobiol 2007; 155: 268-279. (Pubitemid 46177810)
33. Kraemer R, Latzin P, Pramana I, Ballinari P, Gallati S, Frey U,. Long-term gas exchange characteristics as markers of deterioration in patients with cystic fibrosis. Respir Res 2009; 10: 106.
34. Narang I, Ersu R, Wilson NM, Bush A,. Nitric oxide in chronic airway inflammation in children: diagnostic use and pathophysiological significance. Thorax 2002; 57: 586-589. (Pubitemid 34761759)
35. Lundberg JO,. Airborne nitric oxide: inflammatory marker and aerocrine messenger in man. Acta Physiol Scand Suppl 1996; 633: 1-27.
36. Beydon N, Davis SD, Lombardi E, Allen JL, Arets HG, Aurora P, Bisgaard H, Davis GM, Ducharme FM, Eigen H, Gappa M, Gaultier C, Gustafsson PM, Hall GL, Hantos Z, Healy MJ, Jones MH, Klug B, Lodrup Carlsen KC, McKenzie SA, Marchal F, Mayer OH, Merkus PJ, Morris MG, Oostveen E, Pillow JJ, Seddon PC, Silverman M, Sly PD, Stocks J, Tepper RS, Vilozni D, Wilson NM,. An official American Thoracic Society/European Respiratory Society statement: pulmonary function testing in preschool children. Am J Respir Crit Care Med 2007; 175: 1304-1345. (Pubitemid 46905757)
37. Eastham KM, Fall AJ, Mitchell L, Spencer DA,. The need to redefine non-cystic fibrosis bronchiectasis in childhood. Thorax 2004; 59: 324-327. (Pubitemid 38496751)
38. Ron E,. Cancer risks from medical radiation. Health Phys 2003; 85: 47-59. (Pubitemid 36706040)
39. Donnelly LF, Emery KH, Brody AS, Laor T, Gylys-Morin VM, Anton CG, Thomas SR, Frush DP,. Minimizing radiation dose for pediatric body applications of single-detector helical CT: strategies at a large Children's Hospital. AJR Am J Roentgenol 2001; 176: 303-306. (Pubitemid 32105630)
40. Montella S, Santamaria F, Salvatore M, Pignata C, Maglione M, Iacotucci P, Mollica C,. Assessment of chest high-field magnetic resonance imaging in children and young adults with noncystic fibrosis chronic lung disease: comparison to high-resolution computed tomography and correlation with pulmonary function. Invest Radiol 2009; 44: 532-538.
41. Eichinger M, Optazaite DE, Kopp-Schneider A, Hintze C, Biederer J, Niemann A, Mall MA, Wielputz MO, Kauczor HU, Puderbach M,. Morphologic and functional scoring of cystic fibrosis lung disease using MRI. Eur J Radiol 2011; doi: 10.1016/j.ejrad.2011.02.045.
42. Brillet PY, Cottin V, Letoumelin P, Landino F, Brauner MW, Valeyre D, Cordier JF, Nunes H,. Combined apical emphysema and basal fibrosis syndrome (emphysema/fibrosis syndrome): CT imaging features and pulmonary function tests. J Radiol 2009; 90: 43-51.
43. Ichikado K, Suga M, Muranaka H, Gushima Y, Miyakawa H, Tsubamoto M, Johkoh T, Hirata N, Yoshinaga T, Kinoshita Y, Yamashita Y, Sasaki Y,. Prediction of prognosis for acute respiratory distress syndrome with thin-section CT: validation in 44 cases. Radiology 2006; 238: 321-329. (Pubitemid 43010610)
44. Sumikawa H, Johkoh T, Colby TV, Ichikado K, Suga M, Taniguchi H, Kondoh Y, Ogura T, Arakawa H, Fujimoto K, Inoue A, Mihara N, Honda O, Tomiyama N, Nakamura H, Muller NL,. Computed tomography findings in pathological usual interstitial pneumonia: relationship to survival. Am J Respir Crit Care Med 2008; 177: 433-439. (Pubitemid 351317587)
45. Brunelli A, Xiume F, Refai M, Salati M, Marasco R, Sciarra V, Sabbatini A,. Evaluation of expiratory volume, diffusion capacity, and exercise tolerance following major lung resection: a prospective follow-up analysis. Chest 2007; 131: 141-147. (Pubitemid 46122986)
46. Werner HA, Pirie GE, Nadel HR, Fleisher AG, LeBlanc JG,. Lung volumes, mechanics, and perfusion after pulmonary resection in infancy. J Thorac Cardiovasc Surg 1993; 105: 737-742. (Pubitemid 23127734)
47. Wisser W, Senbaklavaci O, Ozpeker C, Ploner M, Wanke T, Tschernko E, Wolner E, Klepetko W,. Is long-term functional outcome after lung volume reduction surgery predictable? Eur J Cardiothorac Surg 2000; 17: 666-672. (Pubitemid 30364943)
48. Nadel HR, Stringer DA, Levison H, Turner JA, Sturgess JM,. The immotile cilia syndrome: radiological manifestations. Radiology 1985; 154: 651-655. (Pubitemid 15162356)
49. de Jong PA, Tiddens HA,. Cystic fibrosis specific computed tomography scoring. Proc Am Thorac Soc 2007; 4: 338-342. (Pubitemid 47330867)