Long-term effects of high-dose systemic corticosteroids on growth and bone mineral density in patients treated for childhood interstitial lung disease (chILD).

BACKGROUND: Children's interstitial lung disease (chILD) is a rare and potentially life-threatening condition. For many chILD conditions, systemic corticosteroids (sCCS) are considered the primary treatment despite a broad spectrum of potential side effects. AIM: We aimed to determine the long-term effects of sCCS treatment on growth, bone mineral density (BMD), and body composition after chILD. MATERIALS AND METHODS: This descriptive cross-sectional single-center study included patients diagnosed with chILD before the age of 18 years treated with sCCS in the period 1998-2020. Dual-energy X-ray absorptiometry, anthropometric measurements, bone age determination, and blood tests were performed in 53 (55% males) of 89 eligible patients. RESULTS: Median (range) age was 19.3 (6.4;30.7 years). Participants received a median (range) cumulative sCCS dose of 1144 (135; 6178) mg over a 2.0 (0.1; 13.8) years period and latest dose was administered 11.7 (1.2; 19.6) years before follow-up. Mean delta height (height standard deviation scores [SDS] - target height SDS) was reduced at sCCS treatment initiation (mean: -0.55, 95% confidence interval [CI]: -0.91; -0.20, p < .005) and at sCCS treatment cessation (mean: -0.86, 95% CI:-1.22; -0.51, p < .001), but normalized in the majority at follow-up (mean: -0.29, 95% CI:-0.61; 0.03, p = .07). Mean (SD) BMD z-score for the spine and whole body was -0.34 (1.06) and 0.52 (1.13), with no significant correlation to sCCS dose. Excess body fat (>30% in females, >25% in males) was found in 58% of patients. CONCLUSION: Long-term treatment with sCCS did not cause significant long-term reduction of height but showed subtle effects on fat mass percentage and BMD. Given the severity of chILD, the observed long-term effects of sCCS on growth and BMD appear acceptable.

Exercise-induced inspiratory symptoms in school children.

BACKGROUND AND AIMS: Exercise-induced inspiratory symptoms (EIIS) have multiple causes, one of which is exercise-induced laryngeal obstruction (EILO). There is limited knowledge regarding EIIS in children, both in primary care practices and in pediatric asthma clinics. The aim of this study was to describe the feasibility of a diagnostic methodology and its results in a cohort of children with EIIS referred to our tertiary pediatric pulmonary center. METHODS: This study analyzed consecutively collected data in children from East Denmark and Greater Copenhagen referred during a 3½ years period. The continuous laryngoscopy exercise (CLE) test directly visualizes the larynx using a flexible laryngoscope during a maximal exercise test. A post-test questionnaire evaluated the subjective impact of the examination. RESULTS: The study included 60 children (37 girls/23 boys) with a mean age of 14 years (range 9-18). The feasibility of the CLE test was 98%; 18 children (35%) had EILO, while 33 children (61%) showed no abnormalities. Other laryngeal abnormalities were observed in three children before the exercise test. Asthma medication was successfully discontinued in 13 (72%) children with EILO. A significantly greater proportion of children shown to have EILO in the CLE test reported coping better with their EIIS than children with negative test (85% vs. 45%; P = 0.03). CONCLUSIONS: Continuous laryngoscopy during exercise is feasible and useful for identifying children with EILO. A correct diagnosis of EILO can help relieve patient anxiety, improve their coping with symptoms, and prevent unnecessary long-term and potentially harmful asthma treatments involving high-dose inhaled steroids. Pediatr Pulmonol. 2016;51:1200-1205. © 2016 Wiley Periodicals, Inc.

Improved air trapping evaluation in chest computed tomography in children with cystic fibrosis using real-time spirometric monitoring and biofeedback.

BACKGROUND: The quality of chest Computed Tomography (CT) images in children is dependent upon a sufficient breath hold during CT scanning. This study evaluates the influence of spirometric breath hold monitoring with biofeedback software on inspiratory and expiratory chest CT lung density measures, and on trapped air (TA) scoring in children with cystic fibrosis (CF). This is important because TA is an important component of early and progressive CF lung disease. METHODS: A cross sectional comparison study was completed for chest CT imaging in two cohorts of CF children with comparable disease severity, using spirometric breath hold monitoring and biofeedback software (Copenhagen (COP)) or unmonitored breath hold manoeuvres (Gothenburg (GOT)). Inspiratory-expiratory lung density differences were calculated, and TA was scored to assess the difference between the two cohorts. RESULTS: Eighty-four chest CTs were evaluated. Mean (95%CI) change in inspiratory-expiratory lung density differences was 436 Hounsfield Units (HU) (408 to 464) in the COP cohort with spirometric breath hold monitoring versus 229 HU (188 to 269) in the GOT cohort with unmonitored breath hold manoeuvres (p<0.0001). The Mean TA (95%CI) score was 6.93 (6.05 to 7.82) in COP patients and 3.81 (2.89 to 4.73) in GOT (p<0.0001) patients. CONCLUSIONS: In children with comparable CF lung disease, spirometric breath hold monitoring during examination yielded a large difference in lung volume between inhalation and exhalation, and allowed for a significantly greater measured change in lung density and TA score, compared to unmonitored breath hold maneuvers. This has implications to the clinical use of chest CT, especially in children with early CF lung disease.