Figures
- FIGURE 1
Flowchart describing the selection procedure.
- FIGURE 2
Forced expiratory volume in 1 s (FEV1) in primary ciliary dyskinesia patients: forest plot showing the heterogeneity and weighted mean value of FEV1 % pred in the included studies.
- FIGURE 3
Forced expiratory volume in 1 s (FEV1) in primary ciliary dyskinesia patients by age group: forest plot showing the heterogeneity and weighted mean value of FEV1 % pred in the included studies.
- FIGURE 4
Forced vital capacity (FVC) in primary ciliary dyskinesia patients: forest plot showing the heterogeneity and weighted mean value of FVC % pred in the included studies.
Tables
- TABLE 1
Detailed characteristics of included studies describing spirometric indices of primary ciliary dyskinesia patients, stratified by age group of patients
First author [ref.] Country Year of publication Patients n Age years Type of study Study design Diagnostics Symptoms stratified by age Longitudinal data FEV1 FVC Children Al Saadi [51] Saudi Arabia 2013 22 10.86±3.09# Prospective Case–control EM+HFVM NA − + − Davis [55] USA/Canada 2015 118 8 (5–11)¶ Prospective Case series EM or genetic analysis NA − + − Gokdemir [58] Turkey 2014 24 12.9±2.7# Prospective Clinical trial EM or Kartagener NA − + + Karadag [61] UK 1999 21 10.8±3.2# Retrospective Case–control EM, HFVM NA − + + Koh [62] Korea 2000 19 12 (7–16)+ Prospective Clinical trial EM NA − + − Maglione [63] Denmark, Italy and UK 2014 158 8.7 (4.2–17.4)§ Retrospective Cohort According to consensus NA + + + Paff [68] The Netherlands 2013 25 10.7 (7.1–14.5)¶ Prospective Case–control According to consensus NA − + − Adults Frija-Masson [57] France 2017 78 34.8 (28.6–47.1)¶ Retrospective Case series EM, genetic analysis or Kartagener NA + + + Horvath [60] UK 2003 14 35±4.6# Prospective Case–control EM, HFVM NA − + + Mossberg [65] Sweden 1983 24 30 (19–47)§ Prospective Case series Clinical suspicion NA − + − Both children and adults Amirav [52] Israel 2016 13 10 (5–43)¶ Retrospective Case series All tests, including genetic analysis − − + − Boon [53] Belgium 2014 122 17.7 (9.5–28.1)¶ Retrospective Case series EM, HFVM, genetic analysis − − + + Cohen-Cymberknoh [54] Israel 2014 14 15.9±8.6# Retrospective Case series EM, HFVM, nNO, genetic analysis − − + − Ellerman [56] Denmark 1997 24 21±13.6# Prospective Case series HFVM + + + + Hellinckx [59] Belgium 1998 11 15.2±7# Prospective Case series EM, HFVM + + + − Mahut [64] France 2006 16 13.5 (12–17)¶ Retrospective Case series Clinical, EM + − + − Noone [4] USA and Canada 2004 78 Adults: 36 (19–73)+; children: 8 (1–17)+ Prospective Case series EM, HFVM, genetic analysis + − + − Nyilas [66] Germany and Switzerland 2017 49 14.7±6.6# Prospective Case–control All tests − − + + Olm [67] Brazil 2011 10 13.4±4.7# Prospective Case–control EM+HFVM + − + + Pifferi [69] Italy 2015 45 14 (22.25)¶ Prospective Case–control EM, HFVM, nNO − − + + Shah [70] UK 2016 118 23.5 (10–36)¶ Retrospective Case series EM, HFVM, nNO, 2% clinical − + + − Tolusakow [71] Germany 1981 21 3–43ƒ Retrospective Case series Clinical suspicion − − + + Vallet [72] France 2013 41 6 (3–11)¶ Retrospective Case series EM, HFVM − + + + Yiallouros [73] Cyprus 2015 20 0.1–58.4ƒ Retrospective Case series EM or HFVM+nNO + + + + FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; EM: electron microscopy; HFVM: high-frequency video-microscopy; NA: not applicable; nNO: nasal nitric oxide. Age is reported in different formats depending on the available information: #: mean±sd; ¶: median (interquartile range); +: median (range); §: mean (range); ƒ: range.
- TABLE 2
Characteristics of included studies describing spirometry indices of primary ciliary dyskinesia patients
Total studies 24 (100) Study size ≤20 patients 8 (33) 21–50 patients 10 (42) 51–100 patients 2 (8) >100 patients 4 (17) Number of centres Single centre 17 (71) Multicentre 7 (29) Publication period ≤1996 2 (8) 1997–2007 7 (29) ≥2008 15 (63) Study region Europe 15 (62.5) Asia 6 (25) North and South America 3 (12.5) Age of participants Children <18 years 7 (29) Adults ≥18 years 3 (13) Children and adults 14 (58) Study design Retrospective 11 (46) Prospective 13 (54) Diagnostics performed Diagnosis based only on strong clinical suspicion 2 (8) Diagnosis based on EM or a combination of tests in most patients; few only on strong clinical suspicion# 3 (13) Diagnosis based on EM or a combination of tests in all patients# 19 (79) Spirometry indices reported FEV1 24 (100) FVC 12 (50) Information for different age groups 6 (43)¶ Longitudinal information 8 (33) Data are presented as n (%). EM: electron microscopy; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity. #: combination of the following: EM, nasal nitric oxide, high-frequency video-microscopy or light microscopy, or genetic analysis. ¶: of 14 studies including children and adults.
Supplementary Material
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TABLE S1 Characteristics of included studies describing spirometric indices of PCD patients by country, including studies with overlapping population 00231-2018.tableS1
TABLE S2 Metaregression results on spirometric indices (FEV1 and FVC) of PCD patients 00231-2018.tableS2
FIGURE S1 Forest plot showing the heterogeneity and weighted mean value of FVC percent predicted in the included publications 00231-2018.figureS1
