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Objectives: To analyse the prevalence of CECR1 mutations in patients diagnosed with early onset livedo reticularis and/or haemorrhagic/ischaemic strokes in the context of inflammation or polyarteritis nodosa (PAN). Forty-eight patients from 43 families were included in the study.Methods: Direct sequencing of CECR1 was performed by Sanger analysis. Adenosine deaminase 2 (ADA2) enzymatic activity was analysed in monocyte isolated from patients and healthy controls incubated with adenosine and with or without an ADA1 inhibitor.Results: Biallelic homozygous or compound heterozygous CECR1 mutations were detected in 15/48 patients. A heterozygous disease-associated mutation (p.G47V) was observed in two affected brothers. The mean age of onset of the genetically positive patients was 24 months (6 months to 7 years). Ten patients displayed one or more cerebral strokes during their disease course. Low immunoglobulin levels were detected in six patients. Thalidomide and anti-TNF (tumour necrosis factor) blockers were the most effective drugs. Patients without CECR1 mutations had a later age at disease onset, a lower prevalence of neurological and skin manifestations; one of these patients displayed all the clinical features of adenosine deaminase 2deficiency (DADA2) and a defective enzymatic activity suggesting the presence of a missed mutation or a synthesis defect.Conclusions: DADA2 accounts for paediatric patients diagnosed with PAN-like disease and strokes and might explain an unrecognised condition in patients followed by adult rheumatologist. Timely diagnosis and treatment with anti-TNF agents are crucial for the prevention of severe complications of the disease. Functional assay to measure ADA2 activity should complement genetic testing in patients with non-confirming genotypes. [ABSTRACT FROM AUTHOR]

Two letters to the editor are presented in response to the article "Managing Gastro-oeophageal Reflux in Infant," published in the August 27, 2010 issue, including a claim that the evidence for the use of off label drugs in children with gastro-oeosaphageal reflux is poor, and an argument on the non-pharmacologic approach to managing gastro-oeosophageal reflux in infants.

A letter to the editor concerning a previously published article about pneumonia is presented.

A letter to the editor is presented in response to a study by A. Hamden on treatments for whooping cough in the May 21, 2009 issue.

A letter to the editor is presented in response to the article "Update on drugs for gastro-oesophageal reflux disease," by S. Keady in the previous issue.

A letter addressed to Doctor F. Marchetti of the Department of Paediatrics Institute of Child Health in Italy regarding the glucocorticoid treatment of croup is presented. It opposes the single oral dose of dexamethasone for the treatment of croup. It suggests inhaled rather than oral/injectable corticosteriods for mild-moderate croup treatment. It recommends to conduct studies comparing inhaled and oral corticosteriods with regard to their onset of action and security profile.

Presents a letter to the editor regarding management of chronic pain in children and adolescents.

Background: There is little experience on the use of the WHO Standards for improving the quality of care (QOC) for children. We describe the use of four prioritised WHO Standard-based Quality Measures to assess the provision of care for children with pain in emergency departments (EDs)., Methods: In a multicentre observational study in 10 EDs with different characteristics in Italy, we collected data on 3355 children accessing the EDs between January 2019 and December 2020. The association between children and facility characteristics and quality measures was analysed through multivariate analyses., Results: The proportion of children whose pain was measured was 68.7% (n=2305), with extreme variations across different centres (from 0.0% to 99.8%, p<0.001). The proportion of children treated for pain was 28.9% (n=970) again with a wide range (5.3%-56.3%, p<0.001). The difference between the frequency of children with pain measured and pain treated varied widely between the facilities (ranging from -24.3 to 82). Children with moderate and severe pain were more frequently treated (48.9% and 62.9% of cases, respectively), although with large variations across centres (ranges: 0%-74.8% and 0%-100% respectively, p<0.001). After correction for children's characteristics, the variable more strongly associated with analysed outcomes was the facility which the child accessed for care. Being a facility in Northern Italy was associated with a higher rate of pain measurement (67.3%-95% CI: 39.9% to 94.6%, p<0.001) compared with facilities in South Italy (-22.1% lower (95% CI: -41.7% to -2.50%, p=0.03)., Conclusions: The use of few WHO Standard-based measures related to pain can help identifying priority gaps in QOC for children and in monitoring it over time. There is a need for more implementation research to establish which are the most sustainable and effective interventions to improve the QOC for acute pain in children., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Experience is lacking on the implementation of the WHO standards for improving the quality of care (QOC) for children at facility level. We describe the use of 10 prioritised WHO standard-based quality measures to assess provision of care for children with acute respiratory infections (ARI) in Italy., Methods: In a multicentre observational study across 11 emergency departments with different characteristics, we collected 10 WHO standard-based quality measures related to case management of children with ARI and no emergency/priority signs. Univariate and multivariate analyses were conducted., Results: Data from 3145 children were collected. Major differences in QOC across facilities were observed: documentation of saturation level and respiratory rate varied from 34.3% to 100% and from 10.7% to 62.7%, respectively (p<0.001); antibiotic prescription rates ranged from 22.6% to 80.0% (p<0.001), with significant differences in the pattern of prescribed antibiotic; hospitalisations rates ranged between 2.3% and 30.6% (p<0.001). When corrected for children's individual sociodemographic and clinical characteristics, the variable more consistently associated with each analysed outcome was the individual facility where the child was managed. Higher rates of antibiotics prescription (+33.1%, p<0.001) and hospitalisation (+24.7%, p<0.001) were observed for facilities in Southern Italy, while university centres were associated with lower hospitalisation rates (-13.1%, p<0.001), independently from children's characteristics., Conclusions: The use of 10 WHO standard-based measures can help quickly assess QOC for children with ARI. There is an urgent need to invest more in implementation research to identify sustainable and effective interventions to ensure that all children receive high QOC., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: There is little experience in the use of the WHO Standards for improving the quality of care (QOC) for children at the facility level. We describe the use of 75 WHO Standard based Quality Measures to assess paediatric QOC, using service users as a source of data, in Italy., Study Design: In a cross-sectional study including 12 hospitals, parents/caregivers of admitted children completed a validated questionnaire including 75 Quality Measures: 40 pertinent to the domain of experience of care; 25 to physical/structural resources; 10 to COVID-19 reorganisational changes. Univariate and multivariate analyses were conducted., Results: Answers from 1482 service users were analysed. Physical resources was the domain with the higher frequency of reported gaps in QOC, with key gaps (higher rates of responders reporting need for improvement and low variability across centres) being: (1) quality of meals (48.1%; range across facilities: 35.3%-61.7%); (2) presence of cooking areas (50.9%; range: 34.6%-70.0%); (3) spaces for family/friends (51.3%; range: 31.8%-77.4%). For experience of care , the most critical gap was the information on the rights of the child (76.6%; range: 59.9%-90.4%), with most other Quality Measures showing an overall frequency of reported need for improvement ranging between 5% and 35%. For reorganisational changes due to COVID-19 an improvement was felt necessary by <25% of responders in all Quality Measures, with low variability across centres. At the multivariate analyses, factors significantly associated with the QOC Index largely varied by QOC domain., Conclusions: The use of the 75 prioritised Quality Measures, specific to service users' perspective, enabled the identification of both general and facility-specific gaps in QOC. Based on these findings, quality improvement initiatives shall focus on a core list of selected Quality Measures common to all facilities, plus on an additional list of Quality Measures as more relevant in each facility., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

In Italy, reimbursement restrictions regarding palivizumab prophylaxis approved in 2016 have been revoked in 2017, restoring use in infants with Gestational Age (GA) >29 weeks. Respiratory Syncytial Virus (RSV) hospitalisations and prevalence of palivizumab use in infants aged <6 months during five seasons (2014-2019), were considered according to different GA. Although RSV hospitalisations rate showed no significant changes, during different seasons in all GA, lower prevalence of palivizumab use in 2016 (0.8% vs 0.3%), returned to a higher level following the revoke of restrictions. Changes in reimbursement criteria were not associated with neonatal RSV hospitalisations rate but with a significant impact on palivizumab use., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To evaluate the effect of the COVID-19 epidemic on paediatric emergency department (ED) attendance in a region of Northern Italy., Methods: A survey was proposed to six out of nine paediatric EDs in the Emilia Romagna region to evaluate attendance data, distribution by age and gender, triage code score, outcome of clinical course, number of hospitalisations and the distribution of patients by disease. Data were collected during March 2020 and compared with that of March 2019., Results: A drop in paediatric ED attendance of more than 83.8% was observed, with a higher percentage of infants and severe triage scores. The proportion of patients hospitalised was significantly higher in 2020 than in 2019 (p value: <0.001). The effect size for the comparison of proportions of hospitalised patients was 0.379. Looking at the distribution of attendance by type of disease, a significantly different distribution was highlighted (p value: <0.00001, Cramer's V); there was a greater proportion of patients presenting to paediatric EDs with poisonings (effect size=0.07), psychiatric pathologies (effect size=0.110), head injuries (effect size=0.167) and fever (effect size=0.212)., Conclusions: Our survey suggests that in the first month of the COVID-19 epidemic in Italy, there has been an increase in delayed attendance and provision of care of potentially severe diseases in paediatric EDs. Hospital and community paediatricians should be aware of this phenomenon and adopt appropriate strategies to prevent this danger, as it may affect children more seriously than COVID-19 itself., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)