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Background: Cardiovascular diseases in the context of renal and liver transplants remain the leading cause of morbidity and mortality. Physical exercise at a moderate intensity is allowed to contrast the risk profile. Echocardiographic evaluation is essential to stratifying potential cardiotoxicity by the standard and, more recently, the deformation and dynamic study of the intracardiac vortex. This study aims to investigate the vortex echo parameters of solid-organ-transplanted subjects who are physically active compared to a control group of healthy subjects. Methods: A group of 33 transplanted subjects (16 kidneys and 17 livers) was studied via a transthoracic echocardiography exam, comprehending the myocardial deformation parameters of global longitudinal strain (GLS), twisting of the left ventricle (LV) chamber, and HyperDoppler image acquisition. Results: The subjects enrolled in this study were 50 in total: there were 33 transplanted and 17 healthy subjects. The transplanted subjects presented higher values of interventricular septum in diastole (IVSd p = 0.001), posterior wall diastolic (PWd p = 0.05), and left ventricle mass index (LVMI p = 0.029); ejection fraction (EF) was found to be higher in athletes (p < 0.001). Transplanted subjects presented mild diastolic dysfunction, emerging only from septal E values (p = 0.001). The 4DStrain (p = 0.018) and GLS2c (p = 0.017) were significantly better in the athletes. All of the geometrical and energetical vortex data were in the normal range and no significant differences were found. An interesting positive correlation was evident for the diastolic parameter, particularly the E/A ratio (p = 0.023) and E’ septal value (p = 0.049), along with the vorticity fluctuation. This behavior was present for all subjects, particularly those that were transplanted (p = 0.005). Conclusions: In the vortex investigation, especially in cases of normal EF, the positive correlation of some diastolic parameters with the flow dynamic patterns corroborates this hypothesis. The HyperDoppler analysis could be helpful to detecting potential damage earlier in the diastolic time before a systolic deficiency.

Background Hemodialysis sessions exert an acute impact on cardiac geometry and mechanics. The recent development of quantitative measurement of intracardiac fluid-dynamics offers a new opportinuty to better understand the fine changes in intracardiac cardiac hemodynamics associated with hemodialysis sessions. Our aim was to assess the impact of an hemodialytic session on intracardiac flow dynamics. Methods We included 26 consecutive patients on chronic hemodialysis in clinically stable phase. They underwent echocardiography including intracardiac fluid-dynamic analysis by Color Vector Flow Mapping (Hyperdoppler) before and after a single dialysis session. Patients with hemodynamically relevant valvular disease were excluded. A complete fluid-dynamics evaluation included the measurement of multiple parameters such asvortex area (VA); vortex length (VL); vortex depth (VD). Bland Altman Plot has been used to assess intra and inter-observer variability. Changes in fluidodynamics after dialysis sessions were tested using the Wilcoxon matched-pairs test. Results Mean Vortex Area (VA) (p=0.034), Vortex Depth (VD) (p=0.024) and Vortex Length (VL) (p=0.037) were significantly reduced after the dialysis session. A similar trend towards the reduction of Direct Flow (DF) parameter after the session was found, which was significantly larger for patients with larger baseline left ventricular (LV) end-diastolic diameter (r=0.446; p=0.037). On the other hand, mean Vortex Intensity (VI) was significanlty increased after dialysis (p=0.046). Among energy parameters, the intradialytic change in Kinetic Energy Fluctuation (KEF) (r=0.4; p=0.058) and Shear Stress Fluctuation (SSF) (r=0.435; p=0.038) were most closely correlated with intradialytic weigth change. Some fluid-dynamic parameters had similar trends of intradialytic change, with stonger correlations among geometric parameters. Delta changes in VA were closely related to changes in VI (p Discussion This is the first study assessing the impact of hemodialytic sessions on intracardiac flow dynamics. Measurement of hyperdoppler indices on hemodialysis chair was feasible and reliable in the whole population. Our results uncovered quantitative chenages of echocardiographic parameters of vortex geometry and energy during hemodialysis.

Background Hyperdoppler is a novel advanced echocardiographic technique for the quantitative assessment of intracardiac fluid-dynamics. Recently, its use has been proven useful to complement traditional echocardiographic evaluation of cardiac function in Heart Failure. However, no specific data are available on its application to differentiate between different types of LV remodeling. Thus, our aim was to investigate whether quantitative changes in fluid dynamics might be able to characterize patients with different ventricular geometry. Methods We enrolled 100 consecutive patients with one of the following: LV concentric hypertrophy (CH), eccentric hypertrophy (EH), concentric remodeling, normal LV geometry (CTRL). They underwent a complete echocardiographic examination with intracardiac fluid-dynamic analysis by Color Vector Flow Mapping (Hyperdoppler, Esaote, Genova, Italy). The following parameters were obtained: vortex area (VA); vortex length (VL); vortex depth (VD). Bland Altman Plot has been used to assess intra and inter-observer variability. Results Mean Vortex Depth was significantly higher in CR (p=0.011), CH (p=0.001) and EH (p=0.025) compared to CTRL (p=0.013, p=0.001 and p=0.022, respectively). In addition, CH had significantly higher Vortex Length (p= 0.008) and larger VA (p=0.012) compared to CTRL. No significative differences in vortices parameters have been observed between CH and EH. Conclusion(s) Quantitative fluid-dynamic assessment was feasible and reliable in the whole population and is a promising tool to complement standard echocardiography for the early characterization of different types of LV remodeling.

Objective To assess the most frequent clinical features of Multisystem Inflammatory Syndrome in children (MIS-C) at presentation to the Emergency Department (ED) in a large multicenter cohort of patients, in order to define useful tools for a timely diagnosis.MethodsClinical and laboratory characteristics were retrospectively reviewed for 210 MIS-C patients from 18 Italian pediatric EDs. We assessed correlations between clinical and laboratory parameters and compared features of patients of different age (≤5 years and >5 years). ResultsFever was the main presenting symptom (100%), followed by conjunctivitis (46%), abdominal pain (44%), vomit (41%) and diarrhea (39%). Forty-nine percent of children presented to the ED in critical or nearly critical conditions. A higher prevalence of mucocutaneous involvement was found in younger children (69% versus 47%, p5 years (62% versus 85%, ppConclusionsApart from fever, most common MIS-C manifestations at presentation to the ED are conjunctivitis, abdominal pain, vomit and diarrhea. Younger children more frequently present with mucocutaneous involvement, while gastrointestinal manifestations are more common in older patients. These findings should be considered when MIS-C is suspected in the ED, in order to achieve a timely recognition of the condition.

Background The pathogenesis of the novel described multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) is still debated as it is not clear if they are the same or different nosological entities. However, for both the diseases a rapid and unequivocal diagnosis is mandatory to start the therapy before the onset of severe complications. In this study, we aimed to evaluate the white cell populations in MIS-C and KD as potential markers to discriminate between the two diseases. Methods We studied white cell populations by flow cytometry in 46 MIS-C and 28 KD patients in comparison to 70 age-matched healthy children. Results MIS-C patients had a significant lymphopenia that involved both B and T populations while KD patients showed a significant neutrophilia and thrombocythemia. Granulocyte/lymphocyte ratio helped to diagnose both MIS-C and KD with a high diagnostic sensitivity, while a multivariate analysis of granulocyte and T lymphocyte number contributed to discriminate between the two diseases. Conclusions The relevant lymphopenia observed in MIS-C patients suggests that the disease would be a post-infectious sequel of COVID-19 immunologically amplified by a massive cytokine release, while the significant neutrophilia and thrombocythemia observed in KD confirmed that the disorder has the genesis of a systemic vasculitis. The analysis of a panel of circulating cells may help to early diagnose and to discriminate between the two diseases.

Aims Over the past decades growing evidence have demonstrated the promising role of intracardiac fluid-dynamics in evaluating cardiac performance. To investigate quantitative changes in vortices parameters in patients with different ventricular geometry. Methods and results We enrolled 50 consecutive patients with one of the following: LV concentric hypertrophy (CH), eccentric hypertrophy (EH), concentric remodelling, and normal LV geometry (CTRL). They underwent a complete echocardiographic examination with intracardiac fluid-dynamic analysis by Color Vector Flow Mapping (Hyperdoppler). The following parameters were obtained: vortex area (VA); vortex length (VL); and vortex depth (VD). Bland Altman Plot has been used to assess intra and inter-observer variability. Mean VD was higher in CR, CH, and EH compared to CTRL (P = 0.013, P = 0.001, and P = 0.022, respectively). Moreover, CH showed higher VL (P = 0.006) and larger VA (P = 0.012) compared to CTRL. A similar trend was noticed in EH patients, despite did not reach statistical significance (P = 0.21 and P = 0.07 for VA and VL, respectively). No significative differences in vortices parameters have been observed between CH and EH. Conclusions This is the first study providing quantitative echocardiographic parameters of vortex location and morphology in different LV geometries. Quantitative fluid-dynamic assessment was feasible and reliable in the whole population.

Aims A new echocardiographic, color Doppler-based technique, named HyperDoppler, has been recently introduced for non-invasive evaluation of intracardiac flow dynamics. The aim of this study was to verify the feasibility and reproducibility of this technique and its capability to differentiate geometrical and energy measures of vortex flow within the left ventricle (LV) in normal subjects, athletes and patients with heart failure. Methods and results Two Italian cardiology centres enrolled each one 100 unselected, consecutive patients presenting at the echocardiography laboratory for a clinical examination, regardless of the indication to echocardiography. In these patients, the feasibility, repeatability, reproducibility, and inter-centre reproducibility of the HyperDoppler technique were tested using the intraclass correlation coefficient (ICC) and Bland–Altman analysis. In addition, 50 normal subjects, 30 professional athletes, and 50 patients with stabilized chronic heart failure and LV ejection fraction Conclusions HyperDoppler is a new ultrasound technique which is feasible, reliable, and practical for assessment of LV flow dynamics. It can quantitate several measures of the LV vortex and may distinguish normal subjects and patients. Future studies are needed to clarify how to implement this technique in cardiology clinical practice.

Clinical presentation of COVID‐19 disease is only in part due to viral infection itself, with the host response playing an important role.1‐3 Despite the mild clinical course during the acute phase of infection in children, latest ongoing researches are pointing the attention towards a hyperinflammatory shock or a Kawasaki‐like disease as a possible consequence to COVID‐19 exposure.4,5 This article is protected by copyright. All rights reserved.

Gastroesophageal reflux (GER) and wheeze are two common conditions in children. GER has been advocated as a causative factor for explaining recurrent to persistent respiratory symptoms at any age. This association very often means that many children with cough, wheezing, or recurrent respiratory infections receive empirical anti-reflux medications. The causal relationship is still largely discussed. Compared to the large number of studies in infants and adolescents, literature on the relationship between GER and wheeze in preschool children is scarce and inconclusive. The aim of the present narrative review was to summarize what is known so far, and what the literature has proposed in the last 20 years, on the relationship between preschool wheezing and GER. In preschool children with respiratory symptoms there is a high rate of positivity of reflux testing, for this reason pH-MII testing and endoscopy are recommended. Flexible bronchoscopy may be useful to exclude anatomical abnormalities as the cause of wheezing in infancy and preschool years. Several biomarkers, as well as empirical anti-reflux therapy, have been proposed for the diagnosis of GER-related airway diseases, but the conclusions of these studies are controversial or even conflicting. There is a great need for future clinical trials to confirm or rule out the association.

The basic knowledge necessary for a European pediatric gastroenterologist/hepatologist/nutritionist is set-out in the training syllabus (TS) of the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN). We retrospectively compared the topics covered in ESPGHAN's training events between 2013 and 2016 with the basic knowledge TS items. Thirty-six initiatives including e-learning were identified. Twelve (33%) courses focused on gastroenterology, 9 (25%) on hepatology, and 10 (28%) on nutrition. Five (14%) courses covered >1 field and were classified "General." The initiatives covered 12 of 57 (21%) TS items; 31 of 57 items (54%) were partially covered; and 14 of 57 (25%) not covered. Five of 9 e-learning courses covered gastroenterology topics, whereas none covered hepatology topics. ESPGHAN's 3-year educational offer partially met the training needs listed in the TS. A coordinated educational program covering all TS items would harmonize training within Europe and would provide trainees with a professional portfolio for employment purposes.

Analysis of intracardiac flows has gained increasing interest in the last years. This analysis has become possible due to the development of technologies for noninvasive cardiovascular imaging, which allow visualization and quantitation of intracardiac flow dynamics. Several studies have shown that abnormalities in cardiac function are related to changes in intracardiac vortical flows. Thus, analysis of cardiac vortex has been used for better understanding of the pathophysiology in many heart diseases and to test initial clinical hypotheses. The aims of this review are to introduce the reader to the topic of intracardiac flow dynamics, to briefly describe current cardiac imaging techniques for analysis of the intracardiac vortex, and to indicate potential clinical applications of a vortex-based approach to the study of cardiac function.

Bronchiectasis, conventionally defined as irreversible dilatation of the bronchial tree, is generally suspected on a clinical basis and confirmed by means of chest high-resolution computed tomography. Clinical manifestations, including chronic productive cough and endobronchial suppuration with persistent chest infection and inflammation, may deeply affect quality of life, both in children/adolescents and adults. Despite many cases being idiopathic or post-infectious, a number of specific aetiologies have been traditionally associated with bronchiectasis, such as cystic fibrosis (CF), primary ciliary dyskinesia or immunodeficiencies. Nevertheless, bronchiectasis may also develop in patients with bronchial asthma; chronic obstructive pulmonary disease; and, less commonly, rheumatological disorders and inflammatory bowel diseases. Available literature on the development of bronchiectasis in these conditions and on its management is limited, particularly in children. However, bronchiectasis may complicate the clinical course of the underlying condition at any age, and appropriate management requires an integration of multiple skills in a team of complementary experts to provide the most appropriate care to affected children and adolescents. The present review aims at summarizing the current knowledge and available evidence on the management of bronchiectasis in the other conditions mentioned and focuses on the new therapeutic strategies that are emerging as promising tools for improving patients' quality of life.

Introduction: primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are increasingly compared. There are no chest magnetic resonance imaging (MRI) comparative studies of PCD and CF. Aims and objectives: we assessed clinical, functional, microbiological and MRI findings in PCD and mild CF patients in order to evaluate different expression of lung disease. Methods: 20 PCD (15.1 years) and 20 CF subjects with mild respiratory impairment (16 years, 70% with pancreatic insufficiency) underwent MRI, spirometry, and sputum cultures when clinically stable. MRI was scored using the modified Helbich system. Results: PCD was diagnosed later than CF (9.9 versus 0.6 years, p=0.03), despite earlier symptoms (0.1 versus 0.6 years, p=0.02). In the year preceding the study, patients from both groups underwent two systemic antibiotic courses (p= 0.48). MRI total scores were 11.6±0.7 and 9.1±1 in PCD and CF, respectively. FEV1 and FVC Z- scores were -1.75 (range, -4.6-0.7) and -0.6 (-3.9-1.8) in PCD, and -0.9 (range, -5.4- 2.3) and -0.3 (-3.4-2.5) in CF, respectively. No difference was found between lung function or structure, despite a higher MRI subscore of collapse/consolidation in PCD versus CF (1.6±0.1 and 0.6±0.2, p Conclusions: MRI is a valuable radiation-free tool for comparative PCD and CF lung disease assessment. Patients with PCD may exhibit similar MRI and lung function changes as CF subjects with mild pulmonary disease. Delay in PCD diagnosis is unlikely the only determinant of similarities.

Nitric oxide plays an important role in several physiological and pathophysiological processes in the respiratory tract. Different ways to measure nasal nitric oxide levels in children are currently available. The possibility of obtaining nasal nitric oxide measurement from relatively young children, combined with the availability of portable devices that can be used even in the office setting, opens new perspectives for nasal nitric oxide analysis in the pediatric daily practice. This review presents a synopsis about the current clinical applications of nasal nitric oxide measurement in the pediatric clinical practice. A total of 3,775 articles on the topic were identified, of which 883 duplicates were removed, and 2,803 were excluded based on review of titles and abstracts. Eighty-nine full text articles were assessed for eligibility and 32 additional articles were obtained from the reference lists of the retrieved studies. Since very low nasal nitric oxide levels are found in the majority of patients with primary ciliary dyskinesia, most publications support a central role for nasal nitric oxide to screen the disease, and indicate that it is a very helpful first-line tool in the real-life work-up in all age groups. Decreased nasal nitric oxide concentration is also typical of cystic fibrosis, even though nasal nitric oxide is not as low as in primary ciliary dyskinesia. In other upper airway disorders such as allergic rhinitis, rhinosinusitis, nasal polyposis, and adenoidal hypertrophy, clinical utility of nasal nitric oxide is still critically questioned and remains to be established. Since nNO determination is flow dependent, a general consensus from the major investigators in this area is highly desirable so that future studies will be performed with the same flow rate. A shared nNO methodology will enable to overcome the challenges that lie ahead in incorporating nNO measurement into the mainstream clinical setting of pediatric airway diseases.

Summary Background No studies longitudinally, simultaneously assessed body mass index (BMI) and spirometry in primary ciliary dyskinesia (PCD). Methods We determined BMI and spirometry in 158 PCD children and adolescents from London, UK (n = 75), Naples, Italy (n = 23) and Copenhagen, Denmark (n = 60) at first presentation and during follow-up. Annual BMI and spirometry were prospectively collected and analyzed over blocks of 2, 4, and 6 consecutive years. Sputum pathogens were recorded. Results Age at first spirometry was 8.7 years (range, 4.2–17.4). Mean Z scores of first measured BMI, FEV1, FVC, and FEF25–75 were 0.01, −1.37, −0.84, and −1.68, respectively. First spirometry was not more frequently impaired in patients referred at age ≥6 years than in those referred at preschool age (P = 0.13). There were no differences in slopes for BMI, FEV1, FVC, or FEF25–75 over any time block. H. influenzae was the most common pathogen, isolated at least once in 65% of patients. P. aeruginosa was found in 58 subjects (37%) of whom 8 (5%) were chronically infected. Neither pathogens was associated with spirometry changes. Conclusions Preschool referral to a PCD center was not associated with better spirometry or BMI. PCD children and adolescents receiving centralized care show steady BMI and spirometry during medium term follow-up. There was a high prevalence of Pseudomonas aeruginosa infection, but the evolution of spirometry or BMI was not affected by this microorganism in medium term. Despite our longitudinal analysis showed no differences between the three centers, the assessment of spirometry and BMI over time represents a quality improvement tool. Future studies are needed to highlight the role of spirometry and BMI in long term PCD management and identify subgroups of patients with a higher risk of early lung failure or nutritional problems. Pediatr Pulmonol. 2014; 49:1243–1250. © 2014 Wiley Periodicals, Inc.

Respiratory syncitial virus (RSV) is the most common cause of lower respiratory tract infections (LRTI) in children worldwide and it is associated with significant childhood morbidity. Acute infection may result in respiratory failure with varying degrees of severity, and increasing evidence supports a role of RSV infection as a key determinant for the development of subsequent chronic respiratory disease. Independent predictors of RSV severity include; prematurity, congenital heart disease, cystic fibrosis, immune defects and neuromuscular disorders. Passive immunization with palivizumab has proven to be safe and effective for preventing RSV hospitalization in infants at higher risk of acquiring severe RSV infection, but its expense and cumbersome monthly intravenous delivery schedule make it inaccessible to many. Furthermore, implementing prophylaxis in 32- to 35-week-gestational age infants and the mode of its administration still represent areas of uncertainty. In this review, we describe several aspects of RSV infection and analyze recent advances in the assessment of cost-effective palivizumab prophylaxis.

A 40-day-old infant presented with pallor and poor food intake, and was found to have normochromic normocytic anaemia and mild neutrophil leucocytosis. As late-onset sepsis was suspected, antibiotic therapy was started, with clinical improvement within 7 days, but haemoglobin levels remained below 8 g/dL. There was no evidence of bleeding or haemolysis, and no response to iron supplementation and a 4-week cow's milk protein elimination diet, and she was referred to our centre at 2.5 months of age. Abdominal MRI showed a vast pseudonodular mass involving and …

Objective Few data exist on natural history of irritable bowel syndrome (IBS) in children; therefore we investigated symptoms evolution over time in a cohort of children with IBS. Study design In this observational, single-center study, we prospectively enrolled newly diagnosed children with IBS and reassessed them after 24 months. At both time points, patients completed a symptoms questionnaire, and a score of stool consistency was obtained. The therapeutic strategy adopted was also recorded. Results Eighty-three children (age 11 years, range, 4-16.6 years; 53 males) completed the study. Forty-seven (56.6%) patients received no medical treatment, whereas polyethylene glycol, probiotics, and trimebutine were prescribed to 9 (10.8%), 24 (28.9%), and 3 (3.6%) subjects, respectively. Twenty-four months after diagnosis, 48 children (57.8%) reported resolution of symptoms ( P .001), without differences between sexes ( P = .35) or among IBS subtypes ( P = .49). Of these, 30 (62.5%) had been only reassured and 18 (37.5%) had been prescribed medical treatment ( P = .26). Despite not being statistically significant, symptoms resolution was more common in patients receiving no medical treatment than in those receiving probiotics (63.8% vs 41.6%, P = .08). Among patients with constipation-IBS, no difference was found in symptoms resolution between patients receiving polyethylene glycol and those receiving no medical treatment (67% and 40%, respectively, P = 1). Conclusions Children with IBS are likely to show spontaneous symptoms resolution over a 24-month follow-up, regardless of sex, age, impact of symptoms on daily activities, and IBS subtypes.

GOALS: We assessed the efficacy of a probiotic mixture of Bifidobacterium infantis M-63, breve M-16V, and longum BB536 in improving abdominal pain (AP) and quality of life (QoL) in children with irritable bowel syndrome (IBS) and functional dyspepsia (FD). BACKGROUND: AP-associated functional gastrointestinal disorders, particularly IBS and FD, are common in pediatrics, and no well-established treatment is currently available. Although probiotics have shown promising results in adults, data in children are heterogeneous. STUDY: Forty-eight children with IBS (median age, 11.2 y; range, 8 to 17.9 y) and 25 with FD (age, 11.6 y; range, 8 to 16.6 y) were randomized to receive either a mixture of 3 Bifidobacteria or a placebo for 6 weeks. After a 2-week "washout" period, each patient was switched to the other group and followed up for further 6 weeks. At baseline and follow-up, patients completed a symptom diary and a QoL questionnaire. AP resolution represented the primary outcome parameter. RESULTS: In IBS, but not in FD, Bifidobacteria determined a complete resolution of AP in a significantly higher proportion of children, when compared with placebo (P=0.006), and significantly improved AP frequency (P=0.02). The proportion of IBS children with an improvement in QoL was significantly higher after probiotics than after placebo (48% vs. 17%, P=0.001), but this finding was not confirmed in FD. CONCLUSIONS: In children with IBS a mixture of Bifidobacterium infantis M-63, breve M-16V, and longum BB536 is associated with improvement in AP and QoL. These findings were not confirmed in FD subjects. Trial identifier: NCT02566876 (http://www.clinicaltrial.gov).

Background and objective: Chest MRI is increasingly used to assess pulmonary diseases, but its utility compared with high-resolution computed tomography (HRCT) has never been evaluated in children using specific performance outcomes. The aim of this study was to assess the accuracy and reliability of MRI compared with HRCT in children with non-cystic fibrosis (CF) chronic lung disease. Methods: Fifty subjects aged 5.9–20 years, with primary ciliary dyskinesia (n = 17), primary immunodeficiency (n = 17) or recurrent pneumonia (n = 16), underwent chest HRCT and MRI. The prevalence of lung abnormalities on HRCT was evaluated, and sensitivity, specificity, accuracy and positive and negative likelihood ratios for MRI versus HRCT were calculated. MRI and HRCT scans were also assessed using a modified Helbich score. Results: Bronchiectasis, mucous plugging, peribronchial wall thickening, consolidation, bullae, abscesses and emphysema were detected by HRCT in 72, 68, 66, 60, 10, 8 and 8% of subjects, respectively. Sensitivity, specificity, accuracy and positive and negative likelihood ratios for MRI were good or excellent for most of the changes that were assessed. Median total Helbich scores for HRCT and MRI were 10 (range 0–20) and 10 (range 0–18), respectively. There was good-to-excellent agreement between the two techniques for all scores (r ≥ 0.8). A Bland–Altman plot confirmed this agreement between total scores (bias value: 0.2 ± 1.18; 95% limits of agreement of mean difference: −2.12–2.52). Conclusions: Chest MRI was equivalent to HRCT to determine the extent of lung disease in children with non-CF lung disease. The findings support the use of chest MRI as an alternative to HRCT in diagnostic pathways for paediatric chronic lung disorders.

Background: Cardiac mechanical efficiency requires that opposing left ventricular regions are coupled both in shortening and lengthening during the same phase of cardiac cycle. Aim of this study was to evaluate whether global measures of mechanical dyssynchrony are able to predict reverse remodeling of the left ventricle in patients receiving cardiac resynchronization therapy (CRT). Methods: Sixty-two patients underwent a clinical examination, including New York Heart Association class evaluation and 6-minute walking distance and both echocardiographic study before and 6 months after CRT. Intraventricular dyssynchrony was evaluated by two-dimensional strain echocardiography, measuring the amount of uncoordinated contraction and relaxation between septum and free wall for both longitudinal and radial function and was presented as the longitudinal global dyssynchrony index (LGDI) and the radial global dyssynchrony index (RGDI). Reverse remodeling was defined by a left ventricular end systolic volume reduction > 15%. Results: After CRT 39 patients showed reverse remodeling. In this group, RGDI (0.74 ± 0.26 vs 0.32 ± 0.30; P = 0.0001) and LGDI (0.52 ± 0.28 vs 0.30 ± 0.24; P = 0.002) were significantly higher than in nonresponders. A receiver-operating characteristic curve analysis showed that RGDI> 0.47 and LGDI>0.34 had a sensitivity and a specificity to predict reverse remodeling of 87% and 74%, 82%, and 74%, respectively. Stepwise forward multiple logistic regression analysis showed that RGDI (O.R.:13.4; 95%C.I.:4.2-120.5; P < 0.0001) was an independent determinant of a positive response to CRT. Conclusion: A radial global dyssynchrony index predicts left ventricular reverse remodeling after CRT. (PACE 2008; 31:819-827).

The aim of this prospective, randomised study was to evaluate the effects of nasal douches with sodium hyaluronate on clinical and endoscopic variables, on parental perception of their child’s health-related quality of life (HR-QoL), and on parental workdays lost in preschool recurrent upper respiratory tract infections (URTIs). Children aged 2-6 years with recurrent or persistent URTIs underwent at baseline the evaluation of upper respiratory tract symptoms in the previous two weeks, and nasal endoscopy. Parents of enrolled children were assessed for self-perception of their children’s HR-QoL using a standardised questionnaire. The same variables were reassessed after a 2-week treatment with either 9 mg sodium hyaluronate plus saline solution or saline alone by nasal douches. Forty of the 48 children enrolled completed the study (22 assigned to the combined treatment). Compared to baseline, the combined treatment resulted in a significant reduction of the prevalence of children with missed daycare days (45% vs 14%, p=0.04) and of parents with workdays lost (36% vs 5%, p=0.02), and in a significant improvement of HR-QoL score (3.7 vs 2.8, p=0.004). At endoscopy, the secretion and mucosal oedema score significantly improved after the combined treatment (6 vs 2, p < 0.001), and there was a trend towards a reduction of the adenoid hypertrophy score (p=0.06). No clinical, HR-QOL or endoscopy changes were found in the saline group. In preschool children with recurrent or persistent URTIs, sodium hyaluronate by nasal douche significantly improves endoscopic features. Additional benefits include the children’s HR-QoL and daycare attendance, and parental work.

Aims To elucidate right ventricular (RV) function in patients with idiopathic pulmonary fibrosis (IPF) with and without pulmonary hypertension (PH) and its relation to other features of the disease. Methods and Results Clinical evaluation, standard Doppler echo, Doppler myocardial imaging (DMI), and 2D strain echocardiography (STE) of RV septal and lateral walls were performed in 52 IPF patients (66.5 ± 8.5 years; 27 males) and in 45 age- and sex-comparable controls using a commercial US system (MyLab Alpha, Esaote). Pulmonary artery mean pressure (mPAP) was estimated by standard echo Doppler. RV global longitudinal strain (RV GLS) was calculated by averaging RV local strains. The IPF patients were divided into 2 groups by noninvasive assessment of PH: no PH (mPAP

PURPOSE: A sensitive imaging technique that assesses ataxia telangiectasia (AT) lung disease without ionizing radiation is highly desirable. We designed a study to evaluate lung changes using magnetic resonance imaging (MRI), and to investigate the relationships among severity and extent of pulmonary abnormalities and clinical, microbiological and functional data in children and young adults with AT. METHODS: Fifteen AT patients (age, 11.3 years; range, 6-31) underwent 3.0-T MRI, spirometry, and deep throat or sputum culture. Images were scored using a modified Helbich score. RESULTS: Although only 8 patients (53 %) had recurrent/chronic respiratory symptoms, MRI identified lung abnormalities in all. Bronchiectasis, peribronchial thickening, mucous plugging, and collapse/consolidation were present in 60 %, 87 %, 67 %, and 13 % of cases, respectively, with no difference between subjects with or without respiratory symptoms. No difference in changes of specific scores was found between the two groups, but the total MRI score was higher in patients with respiratory symptoms (6.5 versus 5, respectively; p = 0.02). Total or specific MRI scores were not associated with patients' age. Of all scores, only mucous plugging subscore appeared significantly related to FEV1 (r = 0.7, p = 0.04) and FEF25-75% (r = 0.9, p = 0.001). MRI scores from patients with positive (n = 5) or negative (n = 10) sputum culture were not significantly different. CONCLUSIONS: MRI is valuable in the assessment of extent and severity of pulmonary changes in children and adults with AT. It represents an helpful tool for the longitudinal evaluation of patients and may be also used as an outcome surrogate to track the effects of medications.