Fotis Kirvassilis

Pediatric inflammatory myofibroblastic tumor of the trachea: A case report and review of the literature

Inflammatory myofibroblastic tumors (IMTs) are relatively rare entities of a distinct histologic appearance and benign clinical course. Although these lesions have been described in virtually every anatomic location, there are few documented reports with tracheal localization. We add to the short list of pediatric IMTs of the trachea, a 13‐year‐old boy that was referred to our pediatric respiratory unit for evaluation of his respiratory distress. In particular, we describe the clinical and pathologic features of this patient and present a review of all reported lesions in the available literature. Pediatr Pulmonol. 2008; 43:831–835.

WS13.3 Lung clearance index: A tool to assess the response to intravenous treatment among children with cystic fibrosis

Lung Clearance Index (LCI) is a promising endpoint for use in CF clinical trials. Since correlation with validated clinical endpoints has not yet been established, we investigated the association between baseline LCI and risk of respiratory tract exacerbations (RTE) in children with CF. Methods: During a prospective observational study, baseline LCI (N2 washout), FEV1 and CFQR respiratory domain (CFQRres) were measured. RTE, defined as an increase in respiratory symptoms treated with IV antibiotics, were recorded during one year. Whether basline LCI predicted RTE was assessed with a Poisson regression model and Kaplan–Meier plots. LCI z-scores were calculated from values in 57 healthy children. Results: In 63 children with CF (median age 12.4 years, range 5−19), mean LCI z-score was 5.3 (SD 4.6) and mean FEV1 z-score −0.9 (SD 1.3). CFQRres correlated with LCI (R = −0.43, p< 0.001), but not with FEV1 (R=0.24, p = 0.051). In the 53 patients with a normal FEV1, CFQRres and LCI were still correlated (R = −0.44, p = 0.002). During the 12 months follow up, 25 patients (40%) experienced 47 RTE. LCI and FEV1 were predictors of RTE. Time to first RTE decreased with worsening LCI quartiles (Log Rank test, p< 0.001). Similarly, compared to the quartile with the lowest LCI, yearly RTE rate ratio in increasing LCI quartiles was 2.8 (95%CI 0.6–13.9, p = 0.205), 4.7 (95%CI 1.0–21.4, p = 0.046) and 13.6 (95%CI 3.2–57.0, p< 0.001). In the group with normal FEV1, LCI but not FEV1 z-score was still a predictor of RTE. Conclusion: Baseline LCI predicts the risk of RTE in children with CF, even in the subgroup with normal FEV1. These data further support the use of LCI as surrogate outcome in CF clinical trials.

Validation study of the pediatric allergic rhinitis quality of life questionnaire.

BACKGROUND The Paediatric Allergic Rhinitis Quality of Life Questionnaire (Ped-AR-QoL) is the first tool developed for the assessment of health-related quality of life (QoL) in Greek children with allergic rhinitis (AR). OBJECTIVE The aim of the current study was to validate the child and parent forms of the Ped-AR-QoL in children aged 6-14 years-old who suffered from AR and were followed in a pediatric allergy clinic. METHODS The Ped-AR-QoL, which was completed by 112 children and their parents, was correlated to the generic QoL questionnaire (Disabkids), which is already valid in Greece for children with chronic disorders, as well as with expert opinions on the severity of disease. RESULTS The Ped-AR-QoL child and parent forms had very good internal consistency (α values of 0.797 and 0.872, respectively), while there was a moderate positive correlation of the disease-specific questionnaire with most of the subscales of the generic questionnaire. There has been a statistically significant association between the Ped-AR-QoL and the expert perception of disease severity. CONCLUSIONS The Ped-AR-QoL had very good reliability and convergent validity when compared with the generic Disabkids QoL. The significance of the association between the disease-specific questionnaire and the expert opinion is an important finding validating the questionnaire. The Ped-AR-QoL may become a helpful tool which can be used in everyday clinical practice by clinicians and it may also be used for assessing therapeutic interventions in clinical trials.

270 Home antibiotic treatment in cystic fibrosis: An effective, cost saving and preferred choice among patients with CF

Aim A prospective clinical study was carried out to compare safety, efficacy and cost benefits of home and hospital iv antibiotic treatment among CF children and adolescents. Method The clinical, quality of life outcome and cost benefits of Home vs. Hospital antibiotic treatment were assessed. Evaluation of clinical outcome was assessed by measuring forced expiratory volume in one second (FEV 1 ) and weight before and one months after antibiotic treatment. Quality of life was also assessed using the DISABKIDS questionnaire. Cost analysis was performed to assess cost benefits from home iv treatment. Results 35 patients with cystic fibrosis (mean age: 12.6±7 years, 56% male) colonized with Pseudomonas aeruginosa were treated with iv antibiotics for two weeks either at hospital (15 patients), or at home (20 patients). Mean FEV1% was 80.9 (±26), mean weight was 37.7 (±17) kg; mean height was 142.9 (±26) cm. The clinical outcome was beneficial in 92% of the studied population. Lung function (FEV1) and weight improved significantly in both groups (p Conclusions Home iv therapy, monitored by regular visits by a home care nurse, is a safe, effective and cost saving therapeutic option in CF.

Lung clearance index (LCI) as a predictor of exercise limitation among CF patients

FEV1 is often considered the gold standard to monitor lung disease in cystic fibrosis (CF). Recently, there has been increasing interest in multiple breath washout (MBW) and cardiopulmonary exercise testing (CPET) as alternative or even more sensitive techniques. However, limited data exist on associations among the above methods.

A “real-life” study on height in prepubertal asthmatic children receiving inhaled steroids

ABSTRACT Introduction: Asthma is the most common chronic respiratory disease in children and inhaled corticosteroids (ICS) constitute the first line of treatment for these patients. However, the potential growth-inhibiting effect of ICS has often been a cause of concern for both caregivers as well as physicians, and there still remains conflict regarding their safety profile. Objective: To assess whether the administration of ICS in low or medium doses is associated with height reduction in prepubertal children. Methods: We performed a retrospective study to examine the association between ICS treatment and growth deceleration in children with mild persistent asthma. The comparison of height measurements every 6 months from 3 to 8 years of age was conducted among three groups of patients: patients not receiving ICS, patients being treated with low dose of ICS and patients being treated with medium dose of ICS (GINA Guidelines 2015). Results: This study included 284 patients (198 male, 86 female) aged 3–8 years; 75 patients were not receiving ICS, 63 patients were on low-dose ICS and 146 patients were on medium-dose ICS. The measured height every 6 months did not differ significantly (p > 0.05) among the three groups while the difference remained stable (p > 0.05), even when we evaluated males and females separately. Conclusions: In this “real-life” study we found that long-term treatment with ICS in low or medium doses is not associated with height reduction in prepubertal children with asthma.

Telephone monitoring and home visits significantly improved the quality of life, treatment adherence and lung function in children with cystic fibrosis

Cystic Fibrosis (CF) is a chronic and systemic disease with a progressive course. As survival rates continue to improve, there is a growing demand for new therapeutic options that improve treatment adherence, disease management and quality of life (QoL) (1). The aim of this study was to evaluate the safety and effectiveness of a home care programme for children with CF and to assess the value of regular telephone contact with the CF team based at Hippokration Hospital, Thessaloniki, Greece. We recruited 60 children with a mean (SD) age of 13.25 (2.62) over a 2-year period. The home care team was coordinated by the CF centre and consisted of two nurses and two physiotherapists. The study population was divided into two groups: Group A consisted of 26 patients living within 60 km of the hospital, in Thessaloniki and its outskirts, and Group B consisted of 34 patients living 60–400 km from the hospital. Group A was offered home visits by a nurse while they were receiving intravenous antibiotic treatment and weekly visits from a physiotherapist during the study period. Group B was offered telephone communication with the home care team twice a week and whenever they needed it. The telephone calls included questions on exacerbations, respiratory infections or symptoms, weight gain, medication and treatment adherence. We evaluated the effectiveness of the home care programme by assessing the forced expiratory volume in the first second (FEV1), the days and cost of hospitalisation and QoL. All the QoL parameters and the total QoL score improved significantly after the programme started, with a mean (SD) baseline QOL score of 39.88 (8.48) and mean (SD) delta QOL score of 43.44 (14.50) (p < 0.001). FEV1% predicted showed a significant improvement from a mean baseline (SD) of 74.74 (31.50) to a mean delta (SD) of 8.00 (5.2) (p = 0.001). The days of hospitalisation decreased significantly, from a mean (SD) baseline of 5.40 (6.06) days to a mean delta (SD) of 1.65 (2.14) (p = 0.02) and the costs also decreased significantly from a mean baseline (SD) of €2053 (1429) to a mean delta (SD) of 431.00 (565.4) (p = 0.005). The two study groups had comparable baseline FEV1. No significant differences were found between the two groups in FEV1 and the days and cost of hospitalisation after the implementation of the home care programme (Table 1). Our study showed that home care was a highly effective option that improved QoL, treatment adherence and lung function among patients with CF. As a result of our findings, we recommend the use of regular telephone communication to increase treatment adherence, especially among patients with CF living in a long distance from the CF centre. New methods of care and communication between healthcare professionals and patients have been developing in the last few years. Regularly collecting medical information using telephone calls allows clinical support teams to comprehensively monitor patients with chronic conditions and adjust their treatment. It also enables them to identify if their health starts to deteriorate, which often leads to emergency department attendances and, or, hospital admissions if they are not detected quickly enough (2,3). Based on the results of this study, both the home care programme and telephone communication initiative seemed to provide promising therapeutic approaches that significantly improved the QoL, treatment adherence and lung function of children with CF.