Filippo Festini

Cystic fibrosis (CF) is a life-threatening and common genetic disorder. Cystic fibrosis newborn screening (CF NBS) has been implemented in many countries over the last 30 years, becoming a widely accepted public health strategy in economically developed countries. False-negative (FN) cases can occur after CF NBS, with the number depending on the method. We evaluated the delayed diagnosis of CF, identifying the patients who had false-negative CF NBS results over 26 years (1992–2018) in Tuscany, Italy. The introduction of DNA analysis to the newborn screening protocol improved the sensitivity of the test and reduced the FNs. Our experience showed that, overall, at least 8.7% of cases of CF received FNs (18 cases) and were diagnosed later, with an average age of 6.6 years (range: 4 months to 22 years). Respiratory symptoms and salt-loss syndrome (metabolic hypochloremic alkalosis) are suggestive symptoms of CF and were commons events in FN patients. In Tuscany, a region with a high CFTR allelic heterogeneity, the salt-loss syndrome was a common event in FNs. Therefore, we provided evidence to support the claim that the FN patients had CFTR mutations rarer compared with the true-positive cases. We underline the importance of vigilance toward clinical manifestations suggestive of CF on the part of the primary care providers and hospital physicians in a region with an efficient newborn screening program.

Vaccination represents one of the most effective means of preventing infections for the population and for the public health in general. Recently there has been a decline in vaccinations, also among healthcare workers (HCWs). The aim of the study is to detect the knowledge, skills, attitudes and barriers of HCWs regarding vaccinations in a tertiary children’s hospital in order to support clinical management in immunisation practices. An observational study was conducted on 255 subjects over a period of 8 months. The 31-item questionnaire considered profession, level of instruction and different ages. It included questions taken from a questionnaire used for a Canadian research and one used by the Bellinzona hospital. A 4-point Likert scale and closed-ended questions were used. A confidence interval of 95%, p value ≤ 0.05, Chi-square, ANOVA and the Kruskal-Wallis test were considered. In the last 5 years less than one third of the sample were vaccinated against flu. 77.8% (n.130) of nurses and 45.8% (n.19) of doctors were not vaccinated (p < 0.0001). As for risk perception, 51.5% of nurses and 90.6% of doctors believe that their risk of contracting influenza is greater than that of the general population. In relation to the injection site, in all the age ranges there was a high level of knowledge except for those aged over 61 who responded incorrectly. Doctors were more prepared (p < 0.0001). 50% of the sample used internet only as a source of information for vaccines. Generally, scientific sources were used infrequently. The higher the education level, the more frequent the utilisation of trustworthy scientific resources and literature. (p = 0.0002). In line with the attitude observed in recent years, nurses are not inclined to get vaccinated themselves although they agree to having their children vaccinated. HCWs have a good level of knowledge about vaccines and immunisation practices. With the nurses we found that the higher the education level, the greater the knowledge about vaccines which leads to the conclusion that low levels of adherence are not due to a lack of knowledge, but rather, to a low perception of risks. Hence the need to strengthen the vaccination strategies inside the companies.

Research from JAMA Pediatrics — Effectiveness of an App for Reducing Preoperative Anxiety in Children — A Randomized Clinical Trial

The Activities Scale for Kids performance (ASKp) is one of the few self-assessment questionnaires in pediatric rehabilitation that measures child perception in performance of daily routine activities. ASKp is composed of 30 questions designed to explore activities and participation in children and teenagers with musculoskeletal disorders. Scores assess level of physical ability, identify appropriate treatment and monitor changes over time. We undertook this cross-cultural validation study to achieve a culturally adapted Italian version of ASKp, to be tested on Italian children. ASKp was converted into Italian by two independent certified translators, supported by two Italian research physiotherapists. Twelve children with musculoskeletal disorders, their parents and eleven expert pediatric physiotherapists independently assessed clarity of language and semantics of each item. Physiotherapists also evaluated content validity of each item. One item out of 30 did not reach inter-rater agreement ≥80% for clarity and was modified according to suggestions given. A panel of three research physiotherapists improved clarity of some other items based on suggestions from study participants. Regarding content validity of ASKp, I-CVI was ≥0.59 for all items and overall ASKp was deemed valid (S-CVI/Ave 0.93). The Italian version of ASKp is linguistically clear and culturally coherent with a high content validity. It can be extremely useful in assessing activity limitation perspectives in Italian children and adolescent ranging from five to 15 affected by musculoskeletal disorders.

Professors Festini and Glasper discuss the significant contribution of Dr Charles West to the development of paediatrics and children’s nursing during the 19^th century and reflect on his legacy to the contemporary care of sick children.

Cervical lymphadenopathy is a common disorder in children due to a wide spectrum of disorders. On the basis of a complete history and physical examination, paediatricians have to select, among the ...

Several charters of rights have been issued in Europe to solemnly proclaim the rights of children during their hospital stay. However, notwithstanding such general declarations, the actual implementation of hospitalized children’s rights is unclear. The purpose of this study was to understand to which extent such rights, as established by the two main existing charters of rights, are actually implemented and respected in Italian pediatric hospitals and the pediatric units of Italian general hospitals, as perceived by the nurses working in them. Cross-sectional study. A 12-item online questionnaire was set up and an invitation was sent by email to Italian pediatric nurses using professional mailing lists and social networks. Responders were asked to score to what extent each right is respected in their hospital using a numeric scale from 1 (never) to 5 (always). 536 questionnaires were returned. The best implemented right is the right of children to have their mothers with them (mean score 4.47). The least respected one is the right of children to express their opinion about care (mean 3.01). Other rights considered were the right to play (4.29), the right to be informed (3.95), the right to the respect of privacy (3.75), the right to be hospitalized with peers (3.39), the right not to experience pain ever (3.41), and the right to school (3.07). According to the majority of nurses, the most important is the right to pain relief. Significant differences in the implementation of rights were found between areas of Italy and between pediatric hospitals and pediatric units of general hospitals. According to the perception of pediatric nurses, the implementation of the rights of hospitalized children in Italian pediatrics units is still limited.

Venipuncture is perhaps the scariest aspect of hospitalization for children as it causes pain and high levels of behavioral distress. Pain is a complex experience which is also influenced by social factors such as cultural attitudes, beliefs and traditions. Studies focusing on ethnic/cultural differences in pain perception and behavioral distress show controversial results, in particular with regards to children. The aim of this paper is to evaluate differences in pain perception and behavioral manifestations between Italian and Chinese children undergoing a venipuncture, through a cross-sectional study. Behavioral distress and self-reported pain were measured in Chinese and Italian outpatient children during a standardized blood-drawing procedure, using the Observational Scale of Behavioral Distress (OSBD) and pain scales. We observed 332 children: 93 Chinese and 239 Italian. Chinese children scored higher than Italians on pain scales − mean scores 5.3 (95%CI 4.78-5.81) vs. 3.2 (95%CI 2.86-3.53) − but lower mean OSBD scores − mean 4.1 (95%CI 3.04-5.15) vs. 8.1 (95%CI 7.06-9.14). Our data suggest that Chinese children experience higher levels of pain than their Italian peers, although they show more self-control in their behavioral reaction to pain when experiencing venipuncture.