scholarly journals What Is Most Suitable for Children With Cystic Fibrosis—The Relationship Between Spirometry, Oscillometry, and Multiple Breath Nitrogen Washout

2022 ◽  
Vol 9 ◽  
Author(s):  
Magdalena Postek ◽  
Katarzyna Walicka-Serzysko ◽  
Justyna Milczewska ◽  
Dorota Sands

IntroductionIn cystic fibrosis (CF), pathological lung changes begin early in life. The technological progress currently gives many diagnostic possibilities. However, pulmonary function testing in children remains problematic.ObjectivesOur study aimed to correlate the results of impulse oscillometry (IOS) with those of multiple breath nitrogen washout (MBNW) in our pediatric CF population. We also compared those parameters between the groups with and without spirometric features of obturation.MethodsWe collected 150 pulmonary function test sets, including spirometry, IOS, and MBNW in patients with CF aged 12.08 ± 3.85 years [6–18]. The study group was divided into two subgroups: IA (without obturation) and IB (with obturation). We also compared Sacin, Scond, and oscillometry parameters of 20 patients aged 14–18 years who reached the appropriate tidal volume (VT) during MBNW.ResultsStatistical analysis showed a negative correlation between lung clearance index (LCI) and spimoetric parameters. Comparison of subgroups IA (n = 102) and IB (n = 48) indicated a statistically significant difference in LCI (p < 0.001) and FEV1z-score (p < 0.001), FEV1% pred (p < 0.001), MEF25z-score (p < 0.001), MEF50 z-score (p < 0.001), MEF75 z-score (p < 0.001), R5% pred (p < 0.05), and R20% pred (p < 0.01). LCI higher than 7.91 was found in 75.33% of the study group, in subgroup IB—91.67%, and IA−67.6%.ConclusionsLCI derived from MBNW may be a better tool than IOS for assessing pulmonary function in patients with CF, particularly those who cannot perform spirometry.

1992 ◽  
Vol 39 (5) ◽  
pp. 392-399
Author(s):  
Se Kyu Kim ◽  
Seon Hee Cheon ◽  
Joon Ha Chang ◽  
Won Hong Jong ◽  
Soo Chein ◽  
...  

2017 ◽  
Author(s):  
Maryl Kreider

Intelligent management of pulmonary disease requires a fundamental understanding of the complex interrelationships between multiple elements that interact to maintain homeostasis in the respiratory system. This chapter discusses the physiologic basis for pulmonary function testing and the use of these tests to diagnose disease, quantitate functional impairments, and follow the effects of treatment. Figures illustrate the relationships between lung volume, airway conductance, and airway resistance; the relationship between forced expiratory volume and time (spirogram); the relationship between spirometry and maximum expiratory flow volume; dynamic airway compression; patterns of abnormalities seen on flow-volume curves; lung volumes and capacities; pressure-volume relationships in health and disease; and the clinical assessment of lung function. Tables list the capacities and volumes of gas contained in the lungs during various breathing maneuvers, conditions associated with alterations in diffusing capacity, and recommendations for tests for various clinical scenarios. This review contains 7 highly rendered figures, 3 tables, and 86 references.


2018 ◽  
Vol 28 (2) ◽  
pp. 29566
Author(s):  
Magali Santos Lumertz ◽  
Ângela De Moura ◽  
Leonardo Araújo Pinto ◽  
Paulo Augusto Moreira Camargos ◽  
Paulo José Cauduro Marostica

AIMS: To compare clinical, laboratory and spirometric parameters of preschoolers (ages from three to six years old) with cystic fibrosis identified by abnormal newborn screening test (NS group), with data of patients whose diagnostic suspicion arose from characteristic clinical signs and symptoms (CS group).METHODS:  Retrospective cohort study, with a sample obtained from the medical charts of patients who received specialized and multidisciplinary outpatient follow-up. All the included children had a confirmed diagnosis of cystic fibrosis by sweat electrolyte testing and/or genetic sequencing. Variables included sex, weight, height, body mass index, number of hospitalizations per year, Pseudomonas aeruginosa colonization, presence of pancreatic insufficiency and Shwachman score, which covers four domains: general activity, nutrition, radiological examination and physical evaluation, each one scored between 5 and 25 points. A total score ≤40 points indicates severe state, and ≥86 points indicates excellent state. For comparison of pulmonary function, data from the last spirometry performed before the age of six years were used. Outcomes were compared by chi-square or Student's t test, with a significance limit of 0.05.RESULTS: Twenty-four patients were included in the study, seven children in the NS group and 17 children in the CS group. The children of the NS group started follow-up with a mean age of 1.51±2.04 months, and those of the CS group started with 3.77±1.80 months (p=0.014). The mean Shwachman score was 94.40±2.19 in the NS vs. 87.67±8.00 in the CS group (p=0.018). The mean Z score for body mass index was 0.95±1.02 in the NS group vs. 0.51±1.05 in the CS group (p=0.051). Spirometric variables had slightly higher values in the NS group, with no statistically significant difference between groups.CONCLUSIONS: The results suggest that those patients diagnosed with cystic fibrosis from the neonatal screening test suspicion benefited from an early intervention, being able to initiate pulmonary function tests and receive treatment and counseling earlier. Evaluation of clinical parameters with the Shwachman score showed that benefits could be already observed at the preschool age.


2013 ◽  
Vol 103 (2) ◽  
pp. 162-167 ◽  
Author(s):  
Janna W Woestenenk ◽  
Rebecca K Stellato ◽  
Suzanne W Terheggen−Lagro ◽  
Cornelis K van der Ent ◽  
Roderick HJ Houwen

2017 ◽  
Vol 50 (5) ◽  
pp. 1700326 ◽  
Author(s):  
Gwyneth Davies ◽  
Janet Stocks ◽  
Lena P. Thia ◽  
Ah-Fong Hoo ◽  
Andrew Bush ◽  
...  

With the advent of novel designer molecules for cystic fibrosis (CF) treatment, there is huge need for early-life clinical trial outcomes, such as infant lung function (ILF). We investigated the degree and tracking of ILF abnormality during the first 2 years of life in CF newborn screened infants.Forced expiratory volume in 0.5 s (FEV0.5), lung clearance index (LCI) and plethysmographic functional residual capacity were measured at ∼3 months, 1 year and 2 years in 62 infants with CF and 34 controls.By 2 years there was no significant difference in FEV0.5 z-score between CF and controls, whereas mean LCI z-score was 0.81 (95% CI 0.45–1.17) higher in CF. However, there was no significant association between LCI z-score at 2 years with either 3-month or 1-year results. Despite minimal average group changes in any ILF outcome during the second year of life, marked within-subject changes occurred. No child had abnormal LCI or FEV0.5 on all test occasions, precluding the ability to identify “high-risk” infants in early life.In conclusion, changes in lung function are mild and transient during the first 2 years of life in newborn screened infants with CF when managed according to a standardised UK treatment protocol. Their potential role in tracking disease to later childhood will be ascertained by ongoing follow-up.


2002 ◽  
Vol 21 (9) ◽  
pp. 1271-1287 ◽  
Author(s):  
Mark D. Schluchter ◽  
Michael W. Konstan ◽  
Pamela B. Davis

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