Non-cystic fibrosis bronchiectasis in children and adolescents: Neglected and emerging issues.

Pediatric non-cystic fibrosis (CF) bronchiectasis is characterized by endobronchial suppuration, airway neutrophilic inflammation and poor mucus clearance and is associated with persistent productive cough due to recurrent airway infections. Most recommendations are based on expert opinion or extrapolated from CF practice. The present narrative review aims to address some issues on the management of children or adolescents with non CF-bronchiectasis that still require attention, and analyze what available literature offers to reply to open questions. We focused on the potential offered by technological advances on lung disease assessment through novel chest imaging techniques and new or old pulmonary function tests. We also summarized the main novelties in the disease prevention and treatment. Finally, a novel diagnostic algorithm is proposed, that might help physicians in the daily clinical decision-making process. Future directions for research on pediatric non-CF bronchiectasis should include larger study populations and longer prospective clinical trials, as well as new clinical and laboratory endpoints to determine the underlying mechanisms of lung disease progression and support the role of new and existing treatments

Towards a global assessment of pediatric non-cystic fibrosis chronic pulmonary disorders: new insights in disease diagnosis and monitoring

Classification of chronic lung disease (CLD) in children has traditionally distinguished cystic fibrosis (CF), the most common lethal inherited genetic disorder worldwide, from less common non-CF disorders, a heterogeneous group of conditions with different etiologies, but overlapping clinical features. While in CF the multi-organ involvement has traditionally required a systemic approach to patients’ care, not limited to respiratory issues, in non-CF CLD this aspect has often been neglected. However, an increasing awareness is emerging regarding the need to adequately address issues such as growth, nutrition and psychological aspects, sometimes underestimated by pediatric respiratory physicians. In particular, nutritional status and growth in pediatric non-CF CLD are strongly related to lung impairment and these two elements may deeply influence each other. Furthermore, in non-CF CLD poor attention has been dedicated to the impact of the disease on patients’ life, whereas quality of life represents a crucial parameter to assess disease severity, efficacy of treatments and to highlight areas of possible improvement in patients’ care. Given these premises the present phD thesis aimed at (1) evaluating currently used and emerging tools in the diagnosis and monitoring of pediatric non-CF CLD and at (2) assessing the role of nutritional status assessment in the management of non-CF CLD childre

New Drugs for Pediatric Asthma

Asthma is the most common chronic disease in children. As suggested by international guidelines, the main goals of asthma treatment are symptoms control and lung function preservation, through a stepwise and control-based approach. The first line therapy based on inhaled corticosteroids may fail to reach control in more than one third of patients, especially adolescents, and in these lung function and quality of life may progressively worsen. Treatment with omalizumab, the first anti-immunoglobulin E recombinant humanized monoclonal antibody, has been definitely approved in pediatric uncontrolled asthma. In this review, we discuss the mechanisms and potential roles of emerging therapies for pediatric severe asthma. Novel biologic drugs (i.e., dupilumab, mepolizumab, reslizumab, and benralizumab) seem to be promising in reducing annual exacerbation rates and steroid-use in glucocorticoid-dependent cases, but available data are few and limited to adolescents and adults. Evidences on the use of the muscarinic antagonist tiotropium as controller medication in pediatric settings are progressively growing, sustaining an application as asthma maintenance treatment in children aged >6 years and in preschool children with persistent asthmatic symptoms, but well powered trials are needed to confirm its safety and efficacy. New inhaled corticosteroids (i.e., ciclesonide and mometasone) are effective as once-daily controller therapy, but long-term studies in the different pediatric ages are needed to compare effectiveness and safety to usual treatments. At present, the role of macrolides in pediatric severe asthma is controversial and their administration is not recommended routinely, but may be considered in children with neutrophilic asthma for reducing daily oral steroids administration and improving lung function. Despite the availability of several novel therapeutic strategies for uncontrolled asthma, future trials targeted at specific pediatric age subgroups are needed to support evidences of safety and efficacy also in children

Non-cystic fibrosis bronchiectasis in children and adolescents: Neglected and emerging issues.

Pediatric non-cystic fibrosis (CF) bronchiectasis is characterized by endobronchial suppuration, airway neutrophilic inflammation and poor mucus clearance and is associated with persistent productive cough due to recurrent airway infections. Most recommendations are based on expert opinion or extrapolated from CF practice. The present narrative review aims to address some issues on the management of children or adolescents with non CF-bronchiectasis that still require attention, and analyze what available literature offers to reply to open questions. We focused on the potential offered by technological advances on lung disease assessment through novel chest imaging techniques and new or old pulmonary function tests. We also summarized the main novelties in the disease prevention and treatment. Finally, a novel diagnostic algorithm is proposed, that might help physicians in the daily clinical decision-making process. Future directions for research on pediatric non-CF bronchiectasis should include larger study populations and longer prospective clinical trials, as well as new clinical and laboratory endpoints to determine the underlying mechanisms of lung disease progression and support the role of new and existing treatments

Lung disease assessment in primary ciliary dyskinesia: a comparison between chest high-field magnetic resonance imaging and high-resolution computed tomography findings

Abstract Background Primary ciliary dyskinesia (PCD) is associated with pulmonary involvement that requires periodical assessment. Chest high-resolution computed tomography (HRCT) has become the method of choice to evaluate chronic lung disease, but entails exposure to ionizing radiation. Magnetic resonance imaging (MRI) has been proposed as a potential radiation-free technique in several chest disorders. Aim of our study is to evaluate whether high-field MRI is as effective as HRCT in identifying PCD pulmonary abnormalities. We also analyzed the relationships between the severity and extension of lung disease, and functional data. Methods Thirteen PCD patients (8 children/5 adults; median age, 15.2 yrs) underwent chest HRCT and high-field 3T MRI, spirometry, and deep throat or sputum culture. Images were scored using a modified version of the Helbich system. Results HRCT and MRI total scores were 12 (range, 6–20) and 12 (range, 5–17), respectively. Agreement between HRCT and MRI scores was good or excellent (r > 0.8). HRCT and MRI total scores were significantly related to forced vital capacity (r = -0.5, p = 0.05; and r = -0.7, p = 0.009, respectively) and forced expiratory volume at 1 second (r = -0.6, p = 0.03; and r = -0.7, p = 0.009, respectively). Conclusion Chest high-field 3T MRI appears to be as effective as HRCT in assessing the extent and severity of lung abnormalities in PCD. MRI scores might be used for longitudinal assessment and be an outcome surrogate in future studies.</p

Neurophysiological Responses to Different Product Experiences

It is well known that the evaluation of a product from the shelf considers the simultaneous cerebral and emotional evaluation of the different qualities of the product such as its colour, the eventual images shown, and the envelope’s texture (hereafter all included in the term “product experience”). However, the measurement of cerebral and emotional reactions during the interaction with food products has not been investigated in depth in specialized literature. (e aim of this paper was to investigate such reactions by the EEG and the autonomic activities, as elicited by the cross-sensory interaction (sight and touch) across several different products. In addition, we investigated whether (i) the brand (Major Brand or Private Label), (ii) the familiarity (Foreign or Local Brand), and (iii) the hedonic value of products (Comfort Food or Daily Food) influenced the reaction of a group of volunteers during their interaction with the products. Results showed statistically significantly higher tendency of cerebral approach (as indexed by EEG frontal alpha asymmetry) in response to comfort food during the visual exploration and the visual and tactile exploration phases. Furthermore, for the same index, a higher tendency of approach has been found toward foreign food products in comparison with local food products during the visual and tactile exploration phase. Finally, the same comparison performed on a different index (EEG frontal theta) showed higher mental effort during the interaction with foreign products during the visual exploration and the visual and tactile exploration phases. Results from the present study could deepen the knowledge on the neurophysiological response to food products characterized by different nature in terms of hedonic value familiarity; moreover, they could have implications for food marketers and finally lead to further study on how people make food choices through the interactions with their commercial envelope

Fertilizer type influences tomato yield and soil N2O emissions

Improvements in crop management for a more sustainable agriculture are fundamental to reduce environmental impacts of cropland and to mitigate effects on global climate change. In this study three fertilization types – ammonium nitrate (control); mineral fertilizer added with a nitrification inhibitor (3,4-dimethylpyrazole phosphate (DMPP)), and an organo-mineral fertilizer (OM) – were tested on a tomato crop in order to evaluate effects both on crop production and soil N2O emissions. Plants grown under OM fertilization had a greater relative growth rate compared to mineral fertilization, due to a higher net assimilation rate, which was related to a greater light interception rather than to a higher photosynthetic efficiency. OM fertilization determined the highest fruit production and lower soil N2O fluxes compared to NH4NO3, although the lowest soil N2O fluxes were found in response to mineral fertilizer added with a nitrification inhibitor. It can be concluded that organo-mineral fertilizer is a better nutrient source compared to mineral fertilizers able to improve crop yield and to mitigate soil N2O emission

Quantitative assessment of dimensional evolution of solitary osteoma of the mandible through 14 years of radiographic follow-up analysis: A unique case report

Abstract Head & Neck Osteomas are extremely rare osteogenic benign tumors, with unclear pathogenesis and heterogeneous clinical behavior. There are no studies in literature showing the characteristics of dimensional growth of such lesions. We report a case of a peripheral solitary osteoma of the sigmoid notch of the mandibular ramus, describing its radiographic growth in fourteen years. Measurements from OPTs (2003–6x7mm, 2008–8x12mm, 2014–17 × 24mm, 2016–19 × 25mm and 2017–21 × 28mm) and 3d cone-beam computed tomography scan (2016–19 × 15 × 21mm, 2017–21,4 × 19,2 × 22,7) were obtained. The growth of the lesion measured on OPTs was in mean 1.1 × 1.5 mm per year. This is the first case report in literature quantitatively assessing the growth of a solitary osteoma of the mandible through radiographic imaging, which seems to be more than 1 mm per dimension per year

Hypovitaminosis D: a novel finding in primary ciliary dyskinesia

BACKGROUND: A relationship between low levels of serum vitamin D and respiratory infections has been established. No study has examined the frequency and clinical relevance of vitamin D deficiency in patients with primary ciliary dyskinesia (PCD). METHODS: Vitamin D levels were measured in 22 PCD patients (7 females, 10.5 years, range, 2-34 years). In PCD, pulmonary function tests (PFTs), sputum microbiology, self-reported physical activity (PA) level, and quality of life (QoL) by means of the Saint George's Respiratory Questionnaire (SGRQ), were also assessed. RESULTS: Seventy-two percent of PCD patients were vitamin-D deficient-to-insufficient and 28% were sufficient. No differences in PFTs parameters were found between vitamin D deficiency-to-insufficiency and sufficiency groups. Patients with vitamin D deficiency-to-insufficiency had significantly higher SGRQ total scores, and thus poorer QoL (p = 0.03). Seventy-nine percent of PCD subjects had limitations in performing vigorous activities, and 53% performed less than 3 hours of PA per week. Vitamin D deficiency-to-insufficiency and sufficiency groups did not show any differences in age at PCD diagnosis or at onset of respiratory symptoms, BMI, atopy, current asthma or bronchiectasis. However, 79% of patients with bronchiectasis had vitamin D deficiency-to-insufficiency. No differences were found in the rate of positive sputum cultures and in the number of antibiotic courses between the two groups. CONCLUSIONS: Hypovitaminosis D is common in PCD patients, and is associated with poorer QoL. We recommend the assessment and treatment of hypovitaminosis D to be included in the routine management of PCD

Lung structure and function similarities between primary ciliary dyskinesia and mild cystic fibrosis: a pilot study

BACKGROUND: 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. We assessed clinical, functional, microbiological and MRI findings in PCD and mild CF patients in order to evaluate different expression of lung disease. METHODS: Twenty 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 < 0.001). These findings were confirmed after data-control for diagnostic delay. Pseudomonas aeruginosa and Staphylococcus aureus were more frequent in CF than in PCD (p = 0.05 and p = 0.003, respectively). 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