Evaluation of ventilation maldistribution as an early indicator of lung disease in children with cystic fibrosis

Gustafsson, Per; Aurora, Paul; Lindblad, Anders

doi:10.1183/09031936.03.00049502

Cited by 298 publications

(350 citation statements)

References 30 publications

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“…[1][2][3][4][5][6] Parameters derived from forced expiratory manoeuvres have traditionally been used to monitor lung function in older patients with cystic fibrosis and forced expiratory volume in 1 second (FEV 1 ) is still considered to be a predictor of prognosis in patients with moderate to severe cystic fibrosis lung disease. 7 8 Although infants cannot perform such manoeuvres, forced expiratory flow-volume (FEFV) loops can be obtained by substituting voluntary effort with application of an external pressure to the chest and abdomen to force expiration.…”

mentioning

confidence: 99%

Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests

Lum¹,

Gustafsson

Ljungberg³

et al. 2007

Thorax

200

211

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Background: Lung clearance index (LCI), a measure of ventilation inhomogeneity derived from the multiplebreath 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 ) than boys (mean (95% CI girls-boys): -1.2 (-2.1 to 20.3) for FEV 0.5 Z score; FEF 25-75 : -1.2 (-2.2 to 20.15)). 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.

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mentioning

confidence: 99%

Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests

Lum¹,

Gustafsson

Ljungberg³

et al. 2007

Thorax

200

211

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“…Ventilation distribution can be assessed by analysing the washout curve of an insoluble tracer gas and can be expressed by a variety of indexes, among which lung clearance index and mean dilution number are the most commonly used because of their sensitivity in detecting peripheral airway collapse [9,16]. The regions of the lung that obtain less of the tidal volume than others have a slower clearance compared with those receiving a higher percentage of the tidal volume.…”

Section: Discussionmentioning

confidence: 99%

“…The lung clearance index is calculated as the cumulative expired volume needed to lower the end-tidal tracer gas (SF 6 ) concentration to 1 ⁄ 40 of the starting concentration divided by the FRC, i.e. the number of lung volume turnovers needed to clear the lungs of the marker gas [15,16]. The mean dilution number is the ratio between the first and the zeroth moments of the washout curve.…”

Section: Methodsmentioning

confidence: 99%

The effect of caudal block on functional residual capacity and ventilation homogeneity in healthy children*

Ungern‐Sternberg¹,

Regli²,

Frei³

et al. 2006

Anaesthesia

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SummaryCaudal block results in a motor blockade that can reduce abdominal wall tension. This could interact with the balance between chest wall and lung recoil pressure and tension of the diaphragm, which determines the static resting volume of the lung. On this rationale, we hypothesised that caudal block causes an increase in functional residual capacity and ventilation distribution in anaesthetised children. Fifty-two healthy children (15-30 kg, 3-8 years of age) undergoing elective surgery with general anaesthesia and caudal block were studied and randomly allocated to two groups: caudal block or control. Following induction of anaesthesia, the first measurement was obtained in the supine position (baseline). All children were then turned to the left lateral position and patients in the caudal block group received a caudal block with bupivacaine. No intervention took place in the control group. After 15 min in the supine position, the second assessment was performed. Functional residual capacity and parameters of ventilation distribution were calculated by a blinded reviewer. Functional residual capacity was similar at baseline in both groups. In the caudal block group, the capacity increased significantly (p < 0.0001) following caudal block, while in the control group, it remained unchanged. In both groups, parameters of ventilation distribution were consistent with the changes in functional residual capacity. Caudal block resulted in a significant increase in functional residual capacity and improvement in ventilation homogeneity in comparison with the control group. This indicates that caudal block might have a beneficial effect on gas exchange in anaesthetised, spontaneously breathing preschool-aged children with healthy lungs. Various anaesthetic agents, airway instrumentations and ventilation strategies are used in infants and children undergoing anaesthesia. Each of these factors may affect functional residual capacity (FRC), which is one of the most important respiratory parameters of gas exchange. There is very little information available on the magnitude and the relative contribution of these different factors (e.g. neuroaxial blockade with local anaesthetics) on functional residual capacity in anaesthetised children [1][2][3][4].Muscle relaxation induced by caudal epidural anaesthesia with bupivacaine decreases abdominal muscle tone while sparing the muscles of the respiratory system (diaphragm, intercostal muscles). This could interact with the balance between chest wall and lung recoil pressure and tension of the diaphragm, which determines the static resting volume of the lung. This alteration of the equilibrium between the thoracal and abdominal compartments might affect the functional residual capacity and ventilation homogeneity of the lungs. This study

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“…3 Clinically, CF is characterized by elevated sweat chloride concentration, exocrine pancreatic insufficiency, male infertility and progressive obstructive lung disease, the latter being the primary cause of mortality. 4,5 The respiratory disease progression in CF is expressed by various pulmonary dysfunctions, [6][7][8][9][10] such as ventilation inhomogeneities, 8,11 pulmonary hyperinflation, 9,12 bronchial obstruction, trapped gas and gas exchange disturbances, 10 most of them occurring early in life, 7,12,13 and progressing even in the absence of clinical signs and symptoms. 11,14,15 Owing to the great phenotypic variability in lung disease even observed among patients carrying the same CFTR genotype, several association studies have been conducted to find modifying genetic factors and to study their influence on CF disease outcome.…”

Section: Introductionmentioning

confidence: 99%

Identification of SNPs in the cystic fibrosis interactome influencing pulmonary progression in cystic fibrosis

et al. 2012

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There is growing evidence that the great phenotypic variability in patients with cystic fibrosis (CF) not only depends on the genotype, but apart from a combination of environmental and stochastic factors predominantly also on modifier gene effects. It has been proposed that genes interacting with CF transmembrane conductance regulator (CFTR) and epithelial sodium channel (ENaC) are potential modifiers. Therefore, we assessed the impact of single-nucleotide polymorphisms (SNPs) of several of these interacters on CF disease outcome. SNPs that potentially alter gene function were genotyped in 95 well-characterized p.Phe508del homozygous CF patients. Linear mixed-effect model analysis was used to assess the relationship between sequence variants and the repeated measurements of lung function parameters. In total, we genotyped 72 SNPs in 10 genes. Twenty-five SNPs were used for statistical analysis, where we found strong associations for one SNP in PPP2R4 with the lung clearance index (Pr0.01), the specific effective airway resistance (Pr0.005) and the forced expiratory volume in 1 s (Pr0.005). In addition, we identified one SNP in SNAP23 to be significantly associated with three lung function parameters as well as one SNP in PPP2R1A and three in KRT19 to show a significant influence on one lung function parameter each. Our findings indicate that direct interacters with CFTR, such as SNAP23, PPP2R4 and PPP2R1A, may modify the residual function of p.Phe508del-CFTR while variants in KRT19 may modulate the amount of p.Phe508del-CFTR at the apical membrane and consequently modify CF disease.

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Evaluation of ventilation maldistribution as an early indicator of lung disease in children with cystic fibrosis

Cited by 298 publications

References 30 publications

Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests

Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests

The effect of caudal block on functional residual capacity and ventilation homogeneity in healthy children*

Identification of SNPs in the cystic fibrosis interactome influencing pulmonary progression in cystic fibrosis

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