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Nutritional status is an important predictor of diaphragm strength in young patients with cystic fibrosis^1,2,3

¹ From the Respiratory Muscle Laboratory, Royal Brompton Hospital, Fulham Road, London (NH and MIP); the Pediatric Pulmonary Department and Research Unit (NH, AC, MB, and BF) and the Pediatric Gastroenterology and Nutrition Department (PT), Armand Trousseau Hospital-AP-HP, Paris; and the Department of Clinical Physiology, Raymond Poincaré Hospital-AP-HP, Garches, France (NH and FL)

Background: The effect of nutritional status and lung disease progression on diaphragm strength in young patients with cystic fibrosis remains unclear.

Objective: The aim of this study was to investigate the effect of nutritional status and airway obstruction on diaphragm strength.

Design: Twitch transdiaphragmatic pressure (Tw Pdi) obtained by bilateral anterior magnetic phrenic nerve stimulation, body mass index (BMI) z score, fat mass, fat-free mass (FFM), arm muscle circumference (AMC), forced expiratory volume in 1 s (FEV₁), and functional residual capacity (FRC) were measured in 20 patients aged 15.1 ± 2.8 y ( ± SD). Values were expressed as a percentage of predicted values.

Results: Mean (±SD) Tw Pdi was 24.3 ± 5.5 cm H₂O. Univariate regression analysis showed positive correlations between Tw Pdi and nutrition scores (BMI z score: r = 0.63, P = 0.003; FFM: r = 0.47, P = 0.04; AMC: r = 0.45, P = 0.04), airway obstruction (FEV₁: r = 0.68, P = 0.001), and arterial oxygen partial pressure (r = 0.68, P = 0.001). Negative correlations were observed between Tw Pdi and dynamic hyperinflation (FRC: r = –0.65, P = 0.005) and arterial carbon dioxide pressure (r = –0.50, P = 0.03). Furthermore, stepwise regression analysis showed that Tw Pdi correlated with BMI z score (r = 0.75, P = 0.0002) and FEV₁ (r = 0.69, P = 0.001).

Conclusions: Diaphragm strength is relatively well preserved in young patients with cystic fibrosis. However, the strength of the diaphragm decreases with the progression of malnutrition and airway obstruction.

Key Words: Diaphragm strength • magnetic stimulation • phrenic nerves • cystic fibrosis • respiratory muscles • body composition • nutritional status • airway obstruction • children

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