Respiratory system parameters and clinical markers in children with cystic fibrosis

Castilho, Tayná; Wamosy, Renata Maba Gonçalves; Keil, Patricia Morgana Rentz; Santos, Bruna Weber; Bobbio, Tatiana Godoy; Schivinski, Camila Isabel Santos

doi:10.1590/1809-2950/e24008324en

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ORIGINAL RESEARCH • Fisioter. Pesqui. 32 • 2025 • https://doi.org/10.1590/1809-2950/e24008324en linkcopy

Respiratory system parameters and clinical markers in children with cystic fibrosis

Parámetros del sistema respiratorio y marcadores clínicos para niños con fibrosis quística

Authorship SCIMAGO INSTITUTIONS RANKINGS

ABSTRACT

This study aimed to investigate the relationship between respiratory system parameters and clinical markers in individuals with cystic fibrosis (CF), as well as to compare individuals with altered oscillometric parameters to those with values within normal range. This cross-sectional analytical study involved children and adolescents with CF. Data collected included anthropometric measurements, disease severity, bacterial colonization, genetic mutations, and parameters from impulse oscillometry (IOS) and spirometry. A total of 115 evaluations were conducted. Correlation was found between respiratory system parameters and the association of total airway resistance (R5) and 5 Hz reactance (X5) with genetic mutations, BMI percentiles, and disease severity. Significant differences were observed between the TypicalX5 and AlteredX5 groups (FEV_1% and FEF_25-75%, p<0.001) and the TypicalR5 and AlteredR5 groups (FEV_1% and FEF_25-75%, p<0.001). Height, FEV₁ (L), and FEF_25-75% (L) explained 69.2% of variations in X5 (kPa), while height and FEV1 (L) together explained 68.2% of variations in R5. Conclusion: This study verified the relationship between spirometric and oscillometric parameters, and found that R5 and X5 are linked to genetic mutation, BMI percentile, and disease severity. Routine spirometry, as well as weight and height assessment, are essential in the management of lung disease.

Keywords
Adolescent; Cystic Fibrosis; Clinical Markers; Child; Pulmonary Function; Respiratory Mechanics

	Total Sample (n=115)	AlteredX5 (n=72)	TypicalX5 (n=43)	AlteredR5 (n=19)	TypicalR5 (n=96)
Parameter	Mean±SD
FEV₁ (L)	1.44±0.67	1.23±0.56	1.78±0.70	0.90±0.57	1.55±0.63
FEV₁%	70.90±26.85	58.80±23.66	91.16±18.40	46.06±27.81	75.81±23.88
FEF_25-75% (L/s)	1.34±1.09	0.97±0.69	1.96±1.34	0.60±0.50	1.49±1.12
FEF_25-75% %	52.45±35.14	37.77±26.60	77.02±34.18	26.21±21.63	57.64±35.05
Z5 (kPa/L/s)	0.79±0.32	0.87±0.33	0.64±0.22	1.23±0.32	0.69±0.23
Z5%	166.84±65.35	195.90±65.33	118.18±22.50	270.67±58.14	146.29±43.61
R5 (kPa/L/s)	0.72±0.29	0.78±0.30	0.61±0.22	1.10±0.31	0.64±0.21
R5%	112.23±36.30	125.89±37.26	89.34±19.32	174.21±30.76	99.95±21.94
R20 (kPa/L/s)	0.50±0.13	0.50±0.14	0.48±0.10	0.60±0.16	0.47±0.11
R20%	96.13±17.16	98.81±19.19	91.62±11.98	115.69±17.11	92.25±14.36
X5 (kPa/L/s)	-0.31±0.17	-0.37±0.17	-0.18±0.06	-0.52±0.21	-0.26±0.12
X5%	207.00±123.29	265.07±121.74	109.77±27.22	362.46±159.74	176.23±87.26
ResF (1/s)	21.46±6.62	23.73±6.15	17.65±5.60	30.16±4.84	19.73±5.47
ResF%	132.98±51.79	151.78±55.17	101.50±22.65	201.31±65.32	119.45±35.96
AX (kPa/L)	2.58±2.25	3.35±2.36	1.28±1.26	5.89±2.45	1.92±1.51
AX%	372.52±572.74	521.40±680.06	123.22±83.19	1096.33±10.55	229.26±239.1

p-value	R²	Adjusted R²		Model
SLR - Age (years old)	26.2%	25.6%	<0.001	Log(R5)=0.123−0.03×Age
SLR - Height (cm)	39.2%	38.7%	<0.001	Log(R5)=0.708−0.64×Height
SLR - Mass (kg)	34.9%	34.3%	<0.001	Log(R5)=0.131−0.10×Mass
SLR - FEV₁	60.7%	60.3%	<0.001	Log(R5)=0.102−0.193×FEV₁
SLR - FEF_25-75%	43.0%	42.5%	<0.001	Log(R5)=-0.042−0.100×FEF_25-75%
MLR*		68.2%	<0.001	Log(R5)=0.506−0.334×Height−0.154×FEV₁

p-value	R²	Adjusted R²		Model
SLR - Height(cm)	5.8%	5.0%	0.009	Exp(X5)=1.644+0.346×Height
SLR - Mass(kg)	9.1%	8.3%	0.001	Exp(X5)=1.901+0.007×Mass
SLR - SDS	14.6%	13.8%	<0.001	Exp(X5)=1.548−0.007×SDS
SLR - FEV₁	65.2%	64.9%	<0.001	Exp(X5)=1.716+0.280×FEV₁
SLR - FEF_25-75%	50.2%	49.8%	<0.001	Exp(X5)=1.918−0.151×FEF_25-75%
MLR*		69.2%	<0.001	Exp(X5)=2.062−0.338.Height+0.433× FEV₁ 0.076×FEF_25-75%

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[TFN1] (L): liters; kPa: kilopascal; %: percentage of predicted values; FEV₁: forced expiratory volume in one second; FEF_25-75%: forced expiratory flow from 25% to 75% of the forced vital capacity; Z5: impedance at 5 Hz; R5: total airway resistance; R20: central airway resistance; X5: reactance at 5 Hz; ResF: resonance frequency; AX: reactance area expressed in kPa/L.