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1.
Marcus Svedberg Per M. Gustafsson Paul D. Robinson Monica Rosberg Anders Lindblad 《Journal of cystic fibrosis》2018,17(2):236-241
Background
Data on long term variability of Lung Clearance Index (LCI) in Cystic Fibrosis (CF) is urgently needed to guide test result interpretation. Our aim was to evaluate LCI variability in clinically stable CF lung disease in school age children.Methods
Paediatric patients, aged 6 to 17 years, attending the outpatient CF clinic performed Multiple Breath Nitrogen Washout (Exhalyzer® D) and spirometry every third month over a period of one year. Clinical stability was assessed by the Cystic Fibrosis Clinical Score (CFCS) at each visit.Results
Twentyfive children were recruited: baseline median (range) FEV1% pred. 91 (55–122)%, LCI 9.1 (6.4–18.6), CFCS 15 (12 ? 23). A total of 107 visits were included in the study, of which 93% were defined as clinically stable. In clinically stable visits, within-subject variability of LCI and FEV1% pred. were 10% and 16%, respectively. The upper limit of normal (ULN, 95% percentile) of LCI variability during clinical stability was 17%.Conclusions
LCI within-subject variability was low and comparable to FEV1% pred. which strengthen the use of LCI to monitor lung disease progression in CF patients. An increase in LCI > 17% compared to previous LCI-measurement in clinically stable CF patients may therefore indicate early lung disease progression. 相似文献2.
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Kathryn A. Ramsey Caroline McGirr Stephen M. Stick Graham L. Hall Shannon J. Simpson 《Journal of cystic fibrosis》2017,16(6):713-718
Background
We assessed the effect of posture on ventilation distribution and the impact on associations with structural lung disease.Methods
Multiple breath washout (MBW) was performed in seated and supine postures in 25 healthy children and 21 children with CF. Children with CF also underwent a chest CT scan. Functional residual capacity (FRC), lung clearance index (LCI) and moment ratios were calculated from the MBW test. CT scans were evaluated for CF-related structural lung disease.Results
FRC was lower in the supine than in the seated posture, whereas LCI was higher in the supine than in the seated posture. In children with CF, associations between LCI and the extent of structural lung disease were stronger when performed in the supine posture.Conclusions
Body posture influences lung volumes and ventilation distribution in both healthy children and children with CF. MBW testing in the supine posture strengthened associations with structural lung damage. 相似文献5.
Insa Korten Elisabeth Kieninger Sophie Yammine Giulia Cangiano Sylvia Nyilas Pinelopi Anagnostopoulou Florian Singer Claudia E. Kuehni Nicolas Regamey Urs Frey Carmen Casaulta Ben D. Spycher Philipp Latzin 《Journal of cystic fibrosis》2019,18(1):118-126
Background
Lung impairment in cystic fibrosis (CF) starts in infancy. However, tools to monitor early lung disease are limited. Respiratory rate (RR) as a key vital sign is easy to assess during sleep and is elevated during acute respiratory disease. Thus, elevated RR could indicate early lung impairment and potentially serve as a diagnostic tool in disease monitoring.Methods
In a prospective cohort of infants with CF diagnosed by newborn screening and healthy controls RR was measured and respiratory symptoms reported weekly throughout infancy. Infants performed a lung function measurement within the first weeks of life.Results
The analyses included 5656 measurements from 153 infants (43 with CF). RR declined from 43.2 (40.5)/min at 6?weeks of age to 28.3 (24.6)/min at 50?weeks in infants with CF (healthy controls). Infants with CF had consistently higher RR than controls (mean difference: 4.15/min; (95% CI 2.86–5.44); p?<?.001). In both study groups, RR was increased throughout the study period in infants with higher lung clearance indices (LCI) and during episodes of respiratory infections.Conclusions
Infants with CF have a higher RR compared to healthy controls during the first year of life. The association with early LCI measurements, the current gold standard to assess physiology of peripheral airways persisted throughout the study period. This may indicate tracking of lung function by RR. It might thus be an early subtle sign of functional respiratory deficit. Further studies will show if RR can be used as a sensitive and promising marker to monitor early CF lung disease. 相似文献6.
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Elisabeth Kieninger Florian Singer Oliver Fuchs Chiara Abbas Nicolas Regamey Philipp Latzin 《Journal of cystic fibrosis》2011,10(6):487-490
Multiple breath washout (MBW) measurements have recently been shown to be sensitive for detection of early cystic fibrosis (CF) lung disease, with the lung clearance index (LCI) being the most common measure for ventilation inhomogeneity. The aim of this observational study was to describe the longitudinal course of LCI from time of clinical diagnosis during infancy to school-age in eleven children with CF.Elevated LCI during infancy was present in seven subjects, especially in those with later clinical diagnosis. Tracking of LCI at follow-up was evident only in the four most severe cases.We provide the first longitudinal data describing the long-term course of LCI in a small group of infants with CF. Our findings support the clinical usefulness of MBW measurements to detect and monitor early lung disease in children with CF already present shortly after clinical diagnosis. 相似文献
8.
Kent Green Thomas Kongstad Marianne Skov Frederik Buchvald Susanne Rosthøj Jacob Louis Marott Per Gustafsson Tacjana Pressler Kim Gjerum Nielsen 《Journal of cystic fibrosis》2018,17(2):242-248
Background
Knowledge of between-session variability of nitrogen multiple-breath washout (N2MBW) indices is crucial when designing longitudinal interventional studies and in disease monitoring using N2MBW as end-point. Such information is currently sparse.Methods
Monthly triplets of N2MBW were prospectively obtained from 14 children with CF during one year. Linear mixed models were used to analyze variability. Our aim was to assess between-session variability of N2MBW indices from repeated measurements and compare LCI derived from different software packages currently in use (TestPoint® vs. Spiroware®).Results
Baseline LCI (median; range) was 9.37 (6.82; 12.08). Between-session differences in LCI measurements were up to 25%. Intra Class Correlation-Coefficient was 0.82. There was no systematic difference between LCI measurements derived from the two software packages (p = 0.18); however, variability was significantly higher using Spiroware®.Conclusions
We report between-session variability of LCI using N2MBW in school-age children and adolescents with CF. LCI changes exceeding 25% may be considered clinically relevant. TestPoint® and Spiroware® can be used interchangeably in longitudinal studies. 相似文献9.
Vladimir Gatzinsky Göran Wennergren Linus Jönsson Linda Ekerljung Birgitta Houltz Staffan Redfors Ulla Sillén Per Gustafsson 《Journal of pediatric surgery》2014
Background
Esophageal atresia (EA) often leads to persistent symptoms and impaired respiratory function in adulthood. The role of peripheral airways in this impairment has not been previously investigated. Furthermore, asthma-like symptoms are common in these patients.Purpose
The purpose of this study was to investigate pulmonary outcome, including possible peripheral airway dysfunction, perhaps missed by conventional pulmonary function tests and to see if the diagnosis asthma was accurate.Methods
Twenty eight patients operated for EA in Gothenburg 1968–1983 answered a questionnaire regarding respiratory symptoms and underwent pulmonary function tests. Peripheral airway function was measured by multiple breath washout.Results
22/28 (79%) patients had a history of respiratory symptoms. Abnormal peripheral airway function was found in 17 (61%) patients, while only 6 (21%) patients displayed values indicating central obstruction. Nine patients had restrictive disease. Airway hyperresponsiveness was frequent and associated with atopy and airway inflammation. However, respiratory symptoms or doctor-diagnosed asthma did not correlate with any specific lung function test abnormality.Conclusion
Different lung function abnormalities are present in EA survivors, and peripheral airway disease is common. Classical asthma seems to be difficult to diagnose in this patient group. Given the high prevalence of respiratory morbidity, long-term follow-up of pulmonary function, including peripheral airway function, is warranted. 相似文献10.
《Journal of cystic fibrosis》2019,18(3):399-406
BackgroundMultiple-breath washout (MBW) has been shown to detect early impairment of lung function in children with cystic fibrosis (CF). Nitrogen (N2) or sulfur hexafluoride (SF6) can be used as tracer gas for MBW. Recent data indicated higher lung clearance index (LCI) values measured with N2-MBW than concurrent SF6-MBW in older children and adults, however, a comparison in infants and younger children, as well as to other outcome measures of CF lung disease is pending.MethodsN2- and SF6-MBW were performed consecutively in 31 sedated infants and preschool children with CF (mean age, 2.3 ± 0.8 years) and 20 controls (mean age, 2.3 ± 1.1 years) using the Exhalyzer D system. Children with CF also underwent chest magnetic resonance imaging (MRI).ResultsMean difference (95% CI) in LCI between N2- and SF6-MBW was 1.1 ± 0.4 (0.9 to 1.3) in controls and 2.1 ± 1.9 (1.4 to 2.8) in CF. Agreement between N2- and SF6-LCI was poor in children with CF. N2-LCI and SF6-LCI correlated with MRI, however N2-LCI showed a higher concordance with MRI than SF6-LCI. The absolute difference between N2- and SF6-LCI values increased with the severity of CF lung disease as determined by MRI scores.ConclusionN2-LCI values were higher than SF6-LCI values in infants and preschool children with CF and controls. Better concordance of N2-LCI than SF6-LCI with chest MRI scores point towards of a higher sensitivity of N2-LCI to detect early lung disease in children with CF. 相似文献
11.
《Journal of cystic fibrosis》2022,21(5):811-820
BackgroundClub cell secretory protein (CC16) exerts anti-inflammatory functions in lung disease. We sought to determine the relation of serum CC16 deficits and genetic variants that control serum CC16 to lung function among children with cystic fibrosis (CF).MethodsWe used longitudinal data from CF children (EPIC Study) with no positive cultures for Pseudomonas aeruginosa prior to enrollment. Circulating levels of CC16 and an inflammatory score (generated from CRP, SAA, calprotectin, G-CSF) were compared between participants with the lowest and highest FEV1 levels in adolescence (LLF and HLF groups, respectively; N = 130-per-group). Single nucleotide variants (SNVs) in the SCGB1A1, EHF-APIP loci were tested for association with circulating CC16 and with decline of FEV1 and FEV1/FVC% predicted levels between ages 7–16 using mixed models.ResultsCompared with the HLF group, the LLF group had lower levels of CC16 (geometric means: 8.2 vs 6.5 ng/ml, respectively; p = 0.0002) and higher levels of the normalized inflammatory score (-0.21 vs 0.21, p = 0.0007). Participants in the lowest CC16 and highest inflammation tertile had the highest odds for having LLF (p<0.0001 for comparison with participants in the highest CC16 and lowest inflammation tertile). Among seven SNVs associated with circulating CC16, the top SNV rs3741240 was associated with decline of FEV1/FVC and, marginally, FEV1 (p = 0.003 and 0.025, respectively; N = 611 participants, 20,801 lung function observations).ConclusionsSerum CC16 deficits are strongly associated with severity of CF lung disease and their effects are additive with systemic inflammation. The rs3741240 A allele is associated with low circulating CC16 and, possibly, accelerated lung function decline in CF. 相似文献
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Mark O. Wielpütz Oyunbileg von Stackelberg Mirjam Stahl Bertram J. Jobst Monika Eichinger Michael U. Puderbach Lutz Nährlich Sandra Barth Christian Schneider Matthias V. Kopp Isabell Ricklefs Michael Buchholz Burkhard Tümmler Christian Dopfer Jens Vogel-Claussen Hans-Ulrich Kauczor Marcus A. Mall 《Journal of cystic fibrosis》2018,17(4):518-527
Background
A recent single-centre study demonstrated that MRI is sensitive to detect early abnormalities in the lung and response to therapy in infants and preschool children with cystic fibrosis (CF) supporting MRI as an outcome measure of early CF lung disease. However, the feasibility of multicentre standardisation remains unknown.Objective
To determine the feasibility of multicentre standardisation of chest MRI in infants and preschool children with CF.Methods
A standardised chest 1.5 T MRI protocol was implemented across four specialised CF centres. Following training and initiation visits, 42 infants and preschool children (mean age 3.2 ± 1.5 years, range 0–6 years) with clinically stable CF underwent MRI and chest X-ray (CXR). Image quality and lung abnormalities were assessed using a standardised questionnaire and an established CF MRI and CXR score.Results
MRI was successfully performed with diagnostic quality in all patients (100%). Incomplete lung coverage was observed in 6% and artefacts also in 6% of sequence acquisitions, but these were compensated by remaining sequences in all patients. The range of the MRI score in CF patients was similar across centres with a mean global MRI score of 13.3 ± 5.8. Cross-validation of the MRI against the CXR score revealed a moderate correlation (r = 0.43–0.50, p < 0.01).Conclusion
Our results demonstrate that multicentre standardisation of chest MRI is feasible and support its use as radiation-free outcome measure of lung disease in infants and preschool children with CF. 相似文献14.
Daan Caudri Lidija Turkovic Jolyn Ng Nicholas H. de Klerk Tim Rosenow Graham L. Hall Sarath C. Ranganathan Peter D. Sly Stephen M. Stick 《Journal of cystic fibrosis》2018,17(4):462-469
Background
Staphylococcus aureus (S. aureus) may be related to more rapid progression of cystic fibrosis (CF) lung disease.Methods
In the AREST CF cohort study, children diagnosed with CF undergo annual bronchoscopies with bronchoalveolar lavage and ultra-low-dose, chest computed tomography (CT) up to 6-years-old. Spirometry was assessed 3-monthly from the age of 4 years. Associations between de novo S. aureus acquisition before school age and CT and lung function at ages 5–7 years were investigated. Models were adjusted for multiple markers of disease severity at baseline.Results
De novo S. aureus acquisition at 3-years-old (n/N = 12/122) was associated with increased bronchiectasis score at age 5–6 years. This association decreased but remained significant after adjustment for confounders. S. aureus at 3 was associated with significantly reduced FEF25–75 at age 5–7 years, but not with FEV1-%-predicted.Conclusion
De novo S. aureus acquisition at age 3 is associated with later bronchiectasis and FEF25–75 in children with CF. 相似文献15.
Lum S Gustafsson P Ljungberg H Hülskamp G Bush A Carr SB Castle R Hoo AF Price J Ranganathan S Stroobant J Wade A Wallis C Wyatt H Stocks J;London Cystic Fibrosis Collaboration 《Thorax》2007,62(4):341-347
BACKGROUND: Lung clearance index (LCI), a measure of ventilation inhomogeneity derived from the multiple-breath inert gas washout (MBW) technique, has been shown to detect abnormal lung function more readily than spirometry in preschool children with cystic fibrosis, but whether this holds true during infancy is unknown. OBJECTIVES: To compare the extent to which parameters derived from the MBW and the raised lung volume rapid thoraco-abdominal compression (RVRTC) techniques identify diminished airway function in infants with cystic fibrosis when compared with healthy controls. METHODS: Measurements were performed during quiet sleep, with the tidal breathing MBW technique being performed before the forced expiratory manoeuvres. RESULTS: Measurements were obtained in 39 infants with cystic fibrosis (mean (SD) age 41.4 (22.0) weeks) and 21 controls (37.0 (15.1) weeks). Infants with cystic fibrosis had a significantly higher respiratory rate (38 (10) vs 32 (5) bpm) and LCI (8.4 (1.5) vs 7.2 (0.3)), and significantly lower values for all forced expiratory flow-volume parameters compared with controls. Girls with cystic fibrosis had significantly lower forced expiratory volume (FEV(0.5) and FEF(25-75 )) than boys (mean (95% CI girls-boys): -1.2 (-2.1 to -0.3) for FEV(0.5) Z score; FEF(25-75): -1.2 (-2.2 to -0.15)). When using both the MBW and RVRTC techniques, abnormalities were detected in 72% of the infants with cystic fibrosis, with abnormalities detected in 41% using both techniques and a further 15% by each of the two tests performed. CONCLUSIONS: These findings support the view that inflammatory and/or structural changes in the airways of children with cystic fibrosis start early in life, and have important implications regarding early detection and interventions. Monitoring of early lung disease and functional status in infants and young children with cystic fibrosis may be enhanced by using both MBW and the RVRTC. 相似文献
16.
Serial perfusion in native lungs in patients with idiopathic pulmonary fibrosis and other interstitial lung diseases after single lung transplantation
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Akihiko Sokai Tomohiro Handa Fengshi Chen Kiminobu Tanizawa Akihiro Aoyama Takeshi Kubo Kohei Ikezoe Yoshinari Nakatsuka Tsuyoshi Oguma Toyohiro Hirai Sonoko Nagai Kazuo Chin Hiroshi Date Michiaki Mishima 《Clinical transplantation》2016,30(4):407-414
17.
Beres A Aspirot A Paris C Berube D Bouchard S Laberge JM Lands LC Puligandla P 《Journal of pediatric surgery》2011,46(5):829-832
Background/Purpose
The management of asymptomatic congenital lung lesions is controversial. Some centers recommend resection in infancy, and others prefer observation. Our objective was to evaluate the pulmonary function of children who underwent lung resection at 12 months or younger. We hypothesized that these children would not have a significant reduction in pulmonary function when compared with norms for age.Methods
All patients at 2 tertiary-care children's hospitals who underwent lung resection at 12 months or younger and are currently older than 5 years were identified and prospectively recruited. Pulmonary function testing was standardized in all patients.Results
Fourteen children were tested prospectively, whereas results were available for another 5 children. Four children were excluded for inability to perform pulmonary function testing (n = 2) or for preexisting pulmonary hypoplasia/syndrome (n = 2). Pulmonary function testing values were considered normal if they were more than 80% of predicted. Forced vital capacity was normal in 14 (93%) of 15 children, and forced expiratory volume in 1 second was normal in 13 (86%) of 15 children. Diffusion capacity and respiratory muscle strength were normal in all children tested.Conclusions
Most children undergoing lung resection in infancy will have normal pulmonary function tests, supporting our philosophy of early, elective resection of congenital lung lesions. 相似文献18.
Koji Komori Seiichi Hirobe Kenji Okumura Shogo Kasai Mayumi Suenaga 《Journal of pediatric surgery》2009,44(11):2096-2100
Purpose
We evaluated the long-term pulmonary function after lobectomy for congenital cystic lung disease, in both infants and children, using radionuclide imaging (RI).Methods
We performed a retrospective review of 93 patients who underwent resection of cystic lung lesions between 1974 and 2001. The results of postoperative lung volume/perfusion scintigraphy at 1 (n = 64), 5 (n = 32), and 10 years (n = 18) after surgery (V1, 5, 10/Q1, 5, 10) and mean transit time (MTT—a marker for air-trapping) at 1, 5, and 10 years after surgery (MTT1, 5, 10) were compared with respect to age at operation, preoperative infection, underlying disease, and type of surgery.Results
Patients who were younger than 1 year at the time of surgery showed a significantly lower MTT5 (1.09 ± 0.08) and MTT10 (1.15 ± 0.11) than patients who were older than 1 year at the time of surgery (MTT5, 1.49 ± 0.67; MTT10, 1.54 ± 0.33). The noninfected group had significantly higher Q10 and lower MTT10 values (P < .05) compared to the infected group. No significant differences were observed between patients with single lobe vs multiple lobe resection.Conclusions
The optimal age for surgery in patients with congenital cystic lung disease appears to be less than 1 year. 相似文献19.
Jeffrey S. Wagener Michael J. Williams Stefanie J. Millar Wayne J. Morgan David J. Pasta Michael W. Konstan 《Journal of cystic fibrosis》2018,17(4):496-502
Background
Patients with cystic fibrosis (CF) who experience acute declines in percent predicted FEV1 (ppFEV1 decreased ≥10% relative to baseline) are often not treated with antibiotics for pulmonary exacerbations (PEx), whereas other patients are treated even when they have not experienced a decline in lung function.Methods
We analyzed 2 patient cohorts using 3?years of Epidemiologic Study of CF data. Cohort 1 (12,837 patients) experienced a ≥10% acute decline in ppFEV1 (n?=?22,898) and Cohort 2 (10,416 patients) had a clinician-diagnosed PEx (n?=?20,731).Results
70.7% of ≥10% decline events were treated with antibiotics; with intravenous antibiotics used 67.1% of the time. 32.0% of clinician-diagnosed PEx declined <10%; with intravenous antibiotics used 36.9% of the time.Conclusions
A clinician's decision to diagnose a PEx and treat with antibiotics often is not defined by measured lung function: a ≥10% FEV1 decline is not considered an absolute indication of a PEx and the lack of a decline does not contraindicate a PEx. Clinicians appear to use the history of prior PEx plus other variables as factors for diagnosing PEx. 相似文献20.
Bakker EM van der Meijden JC Nieuwhof EM Hop WC Tiddens HA 《Journal of cystic fibrosis》2012,11(3):223-230
BackgroundAccurate assessment of pulmonary status in young children with cystic fibrosis (CF) requires sensitive and objective monitoring techniques.ObjectivesThis study aimed to evaluate the feasibility of lung clearance index (LCI) calculated from multiple breath washout (MBW), home nocturnal pulse oximetry and home nocturnal cough recording in young children with CF, and determine whether these tests can distinguish CF patients from healthy controls.MethodsWe performed a prospective cross-sectional study in 20 CF patients and 30 healthy children aged 0–4 years. MBW was performed in awake and unsedated children at the outpatient clinic using a commercially available device. Measurements of nocturnal oxygen saturation and nocturnal cough were done at home using a pulse oximeter and an audiometer.ResultsThere was a significant difference in mean LCI between healthy children and CF patients (LCI 7.1 vs. 9.3, p < 0.001). Nocturnal oxygen saturation was normal in both groups and did not significantly differ between the groups. Similarly, cough showed no differences between both groups. Cough varied widely between children and between nights. Success rates for saturation and cough measurements were 90% and were similar for CF patients and healthy children. Success rate for LCI was 75% for CF patients and 50% for healthy children.ConclusionsMeasurements of LCI, nocturnal oxygen saturation and cough were feasible in young children; however LCI was the only variable that showed a significant difference between children with CF and healthy children. 相似文献