Lung Clearance Index Improves in People with Cystic Fibrosis not Achieving a Clinical Important Difference in Forced Expiratory Volume in One Second After Elexacaftor/Tezacaftor/Ivacaftor Therapy.

Purpose: In people with cystic fibrosis (pwCF), elexacaftor/tezacaftor/ivacaftor (ETI) therapy is associated with an average improvement in FEV of 10-14%. However, a subset of individuals fails to achieve a clinically meaningful increase in spirometric indicators. In this study, we aimed to assess whether the lung clearance index (LCI), a more sensitive indicator of lung involvement, improves following ETI initiation in this population.

Effectiveness and safety of elexacaftor/tezacaftor/ivacaftor treatment in children aged 6-11 years with cystic fibrosis in a real-world setting.

Background: Elexacaftor-tezacaftor-ivacaftor (ETI) is a highly effective cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulating therapy for people with CF and at least one F508del variant. However, there is limited data about the safety and efficacy of this therapy in pediatric populations and in real-world settings. This study aimed at evaluating the effectiveness, tolerability, and safety of ETI in children with CF.

Covid-19 in cystic fibrosis patients compared to the general population: Severity and virus-host cell interactions.

Background: People with cystic fibrosis (pwCF) are considered at risk of developing severe forms of respiratory viral infections. We studied the consequences of COVID-19 and virus-host cell interactions in CF vs. non-CF individuals.

Severity of SARS-CoV-2 infection in a hospital population: a clinical comparison across age groups.

Background: Children tend to have milder forms of COVID-19 than adults, however post-acute complications have been observed also in the paediatric population. In this study, we compared COVID-19-related outcomes and long-term complications between paediatric and adult patients infected by SARS-CoV-2.

Methods: The study is based on individuals enrolled from October 2020 to June 2021 in the DECO COVID-19 multicentre prospective study supported by the Italian Ministry of Health (COVID-2020-12371781).

Cepacia syndrome in cystic fibrosis: A systematic review of the literature and possible new perspectives in treatment.

Background: Cepacia syndrome (CS) is an acute, necrotizing pneumonia with a high mortality rate, occurring in patients with cystic fibrosis (CF) infected with Burkholderia cepacia complex (BCC). Due to its low incidence, data on this condition are limited.

Methods: We conducted a systematic review of the reported cases of CS by searching MEDLINE, Embase and the Cochrane Library to improve knowledge of this rare but potentially lethal condition.

Impact of COVID-19 on Lung Disease in People with Cystic Fibrosis: A 6-Month Follow-Up Study on Respiratory Outcomes.

Background: The impact of COVID-19 on respiratory outcomes in people with cystic fibrosis (pwCF) has not been clearly characterized. We evaluated changes in respiratory function indicators derived from spirometry and pulmonary exacerbation rates 6 months after SARS-CoV-2 infection.

Methods: This multicentre prospective study was based on pwCF enrolled between October, 2020 and June, 2021 in the DECO COVID-19 project.

Immunogenicity of BNT162b2 mRNA-Based Vaccine against SARS-CoV-2 in People with Cystic Fibrosis According to Disease Characteristics and Maintenance Therapies.

During the SARS-CoV-2 vaccination campaign, people with CF (pwCF) were considered a clinically vulnerable population. However, data on the immunogenicity of anti-SARS-CoV-2 vaccines in pwCF are lacking. We conducted a prospective study enrolling all patients aged > 12 and who were followed-up in our CF center and received two doses of the BNT162b2 vaccine in the period of March−October 2021.

A successful treatment of a lobar atelectasis in a patient with cystic fibrosis.

Lobar atelectasis may be a complication of pulmonary exacerbations in cystic fibrosis (CF). There are no established guidelines on the management of this condition in patients with CF. Therapeutic bronchoscopy with recombinant human deoxyribonuclease (rhDNase) instillation has been described to be successful in patients not responding to conservative measures.

Immunological profiles of children with recurrent otitis media with or without spontaneous tympanic membrane perforation and of children with recurrent respiratory infections.

Objectives: The primary aim of this study was to compare the immunological profiles of children affected by recurrent acute otitis media (rAOM) with and without spontaneous tympanic membrane perforation (STMP). The secondary aim was to compare immunological features of children without parameters outside the normal range and affected by either rAOM or recurrent respiratory tract infections (rRTI).

Methods: In this study, otherwise healthy children (<10 years of age) with rAOM or rRTI were included.

SARS-CoV-2 antibodies among people with cystic fibrosis prior to the vaccination campaign: A seroprevalence study in two specialized centres in Northern Italy.

The prevalence of anti-SARS-CoV-2 antibodies in people with cystic fibrosis (CF) is largely unknown. We carried out a cross-sectional study between March and June 2021 with the aim of estimating the seroprevalence of anti-SARS-CoV-2 antibodies in two CF centres in Northern Italy. Total serum anti-SARS-CoV-2 (spike) antibodies levels were measured and values ≥0.

Cytokine storm syndrome in a young patient with cystic fibrosis.

We report the case of a patient with cystic fibrosis (CF) presenting with a full-blown cytokine storm syndrome probably triggered by infection. This condition is rare and the diagnosis can be particularly difficult in patients with a complex chronic disease such as CF. However, timely recognition and appropriate treatment in the early stages are key to avoiding a potentially fatal course.

Role of ocular cytology in vernal keratoconjunctivitis.

Background: Children with vernal keratoconjunctivitis (VKC) present symptoms that are similar to other ocular allergies, but more pronounced, and are controlled using topical steroids. To avoid excessive and prolonged use of topical steroid eye drops, over the past 20 years galenic eye drops of cyclosporine with a concentration of 1% to 2% and tacrolimus with a concentration of 0.1% have been introduced as a treatment for the severe and unresponsive forms.

Phenotype Profiling and Allergy in Otitis-Prone Children.

Otitis-prone children can present some distinctive clinical patterns and although a number of known risk factors for recurrent acute otitis media (RAOM) are known, no dedicated epidemiological models have been developed to explain clinical heterogeneity. A preliminary retrospective pilot study was planned to evaluate the possible effect of allergic disease in the development of different disease phenotypes in otitis-prone children aged 3-10 years, particularly the absence (simple RAOM), or presence of episodes of otitis media with effusion between acute infections (RAOM with OME). Analysis was based on the data contained in 153 charts (55.

Impact of adenotonsillectomy on pediatric quality of life: review of the literature.

Adenotonsillectomy (ADT) is one of the most widely used procedures in the treatment of paediatric recurrent acute tonsillitis (RAT) and obstructive sleep apnoea syndrome (OSAS), both of which have significant repercussions on the patients' quality of life (QoL). The purpose of our review of literature was to highlight the great variety of tools that are currently used to evaluate QoL in children, to examine data available on their efficacy and the feasibility of their use in daily clinical practice, and to determine possible limitations related to an indirect and subjective assessment of QoL in children.Although the use of different parameters makes it difficult to compare the published studies, an analysis of the evidence currently available in the literature suggests that ADT has a generally positive impact on the QoL (especially in case of OSAS).

Inhaled Antibiotic Therapy for the Treatment of Upper Respiratory Tract Infections.

Upper respiratory tract infections (URTIs), primarily acute rhinopharyngitis, tonsillitis, adenoiditis, and rhinosinusitis (RS), are major causes of morbidity in both children and adults of any age. In addition to the frequent occurrence of related medical problems and the associated socioeconomic costs, URTIs have a significant impact on the quality of life of patients and their families, and have placed an escalating financial burden on the global healthcare system. Most URTIs are caused by viruses and require only symptomatic treatment.

Genetic Polymorphisms of Functional Candidate Genes and Recurrent Acute Otitis Media With or Without Tympanic Membrane Perforation.

Evaluation of the genetic contribution to the development of recurrent acute otitis media (rAOM) remains challenging. This study aimed to evaluate the potential association between single nucleotide polymorphisms (SNPs) in selected genes and rAOM and to analyze whether genetic variations might predispose to the development of complicated recurrent cases, such as those with tympanic membrane perforation (TMP).A total of 33 candidate genes and 47 SNPs were genotyped in 200 children with rAOM (116 with a history of TMP) and in 200 healthy controls.

Middle ear problems in children hospitalised because of lower respiratory tract infections: a comparison between two cohorts in Burundi and Italy.

Objectives: To compare the prevalence of acute otitis media (AOM) and otitis media with effusion (OME) in children hospitalized for acute lower respiratory infections (LRTIs) in Burundi and Italy.

Methods: The study, which was conducted from 1 February to 30 April 2011 at the hospital of Kiremba (Burundi, Africa) and at Paediatric Clinic 1 of the University of Milan (Italy), enrolled patients aged <5 years who were hospitalised because of LRTIs. Upon admission, the children underwent an otological examination (pneumatic otoscopy and tympanometry), and middle ear diseases were compared between the two groups.