Affiliation:
1. Department of Pulmonology and Allergology, Medical University of Gdańsk, Poland
2. Department of Clinical Nutrition, Medical University of Gdańsk, Poland
3. Department of Paediatric Cardiology and Congenital Heart Defects, Medical University of Gdańsk, Poland
4. Department of General Nursery, Medical University of Gdańsk, Poland
Abstract
IntroductionRespiratory status is one of the main factors affecting the length of survival in patients with Duchenne muscular dystrophy (DMD) – the most common, severe, progressive muscular dystrophy.The aim: (1) to assess pulmonary function in DMD patients using the z-score method and (2) to identify factors affecting it, irrespective of the disease progress.Material and methodsWe evaluated 55 boys (aged 5 – 18 years) with DMD. The spirometry was performed with: forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), peak expiratory flow (PEF) analysis as absolute values (in litres or litres/min), % predicted value (%pv) and z-scores [z]. The need of ventilation support, ambulatory status, steroid therapy were collected.Results25(45%) subjects were non-ambulatory, 38(69%) used steroid therapy. Mean FVC[z] -2.4±2.2, FEV1[z] -2.0±1.9, PEF[z] -1.5±1.3 value significantly decrease with age (r=-0.62/-0.65/-0.55; p<0.001 respectively), after reaching the peak values between 9-12 or 6-9 years of age depending on analysis method (absolute, %pv or z-score). The results fell below normal range (z-score<-1.64) at the age of 9.8/10.4/11.6 years and below 80%pv at 10.7/13.2/13.2 for FVC/FEV1/PEF, respectively. The pulmonary function test results were significantly lower in non-ambulant (p<0.001) and non-steroid patients (p<0.02).ConclusionsAnalysis of pulmonary function test based on z-score shows that deterioration of pulmonary function in DMD males may appear earlier than we thought measured by %pv and absolute values. Early loss of ambulation, lack of or delayed steroid therapy are risk factors for worse pulmonary outcomes. To confirm these findings cohort longitudinal studies are necessary.
Cited by
4 articles.
订阅此论文施引文献
订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献