Lung ultrasound evaluation in people with cystic fibrosis: A new approach in the pulmonology outpatient clinic.

BACKGROUND: Cystic fibrosis (CF) is a genetic disease that causes progressive lung disease with major impact on the quality of life. Lung ultrasound (LUS) allows to assess the lung involvement through the artefacts analysis and is increasingly used in children but is not yet used to monitor people with CF(pwCF). The main aim of this study was to describe the LUS pattern of pwCF during their routinary check-up visit. The secondary objective was to correlate the LUS findings with pulmonary function indices. METHODS: We performed a cross-sectional observational study, enrolling adolescents and young adults with CF. Each patient underwent clinical assessment, measurement of SpO2, assessment of lung function by spirometry and LUS. RESULTS: Twenty-nine subjects with CF were included. The most frequent alterations were consolidations (72.4%) located in the left apical anterior and right apical posterior regions followed by interstitial syndrome (65.5%). The 41.4% of cases presented the lingula involvement, characterized by a consolidation with static air bronchogram, and 55.2% showed pleural irregularity mainly in the posterior apical regions. A significant correlation was found between the LUS total score and spirometric indices: FEV1 (p = .003), FVC (p = .002), Tiffenau Index <80% (p = .014), and FEF 25-75 (p = .004). CONCLUSIONS: Our study describes LUS findings in pwCF. It also showed a correlation between LUS score and the patients' lung function measured by spirometric indices. We conclude that LUS may be useful in routine monitoring of pwCF in combination with clinical and spirometric assessment.

Defibrotide impact on the acute GVHD disease incidence in pediatric hematopoietic stem cell transplant recipients.

Despite advances in acute graft-versus-host disease (aGVHD) prophylaxis, current pharmacological approaches fail to prevent aGVHD. The protective effect of defibrotide on GVHD incidence and GVHD-free survival has not been sufficiently studied. 91 pediatric patients included in this retrospective study were divided into two groups based on defibrotide use. We compared the incidence of aGVHD and chronic GVHD-free survival between the defibrotide and control groups. The incidence and severity of aGVHD were significantly lower in patients who received defibrotide prophylactic administration than in the control group. This improvement was observed in the liver and intestinal aGVHD. No defibrotide prophylaxis benefit was observed in the prevention of chronic GVHD. The pro-inflammatory cytokine levels were significantly higher in the control group. Our findings suggest that prophylactic administration of defibrotide in pediatric patients significantly reduces the incidence and severity of aGVHD, with a modification of cytokine pattern, both strongly coherent with the protective drug's action. This evidence adds to pediatric retrospective studies and preclinical data suggesting a possible defibrotide role in this setting.

Invasive Infections Associated with the Use of Probiotics in Children: A Systematic Review.

Although the effectiveness of probiotics has only been proven in specific conditions, their use in children is massively widespread because of their perception as harmless products. Recent evidence raises concerns about probiotics' safety, especially but not only in the paediatric population due to severe opportunistic infections after their use. This review aimed at summarising available case reports on invasive infections related to probiotics' use in children. For this purpose, we assessed three electronic databases to identify papers describing paediatric patients with documented probiotic-derived invasive infections, with no language restrictions. A total of 49 case reports from 1995 to June 2021 were identified. The infections were caused by Lactobacillus spp. (35%), Saccharomyces spp. (29%), Bifidobacterium spp. (31%), Bacillus clausii (4%), and Escherichia coli (2%). Most (80%) patients were younger than 2 years old and sepsis was the most observed condition (69.4%). All the patients except one had at least one condition facilitating the development of invasive infection, with prematurity (55%) and intravenous catheter use (51%) being the most frequent. Three (6%) children died. Given the large use of probiotics, further studies aiming at evaluating the real incidence of probiotic-associated systemic infections are warranted.