Corrigendum: HLH as an additional warning sign of inborn errors of immunity beyond familial-HLH in children: a systematic review.

[This corrects the article DOI: 10.3389/fimmu.2024.1282804.].

HLH as an additional warning sign of inborn errors of immunity beyond familial-HLH in children: a systematic review.

Background: Hemophagocytic Lymphohistiocytosis (HLH) is a rare and life-threatening condition characterized by a severe impairment of the immune homeostasis. While Familial-HLH (FHL) is a known cause, the involvement of other Inborn Errors of Immunity (IEI) in pediatric-HLH remains understudied. Objective: This systematic review aimed to assess the clinical features, triggers, laboratory data, treatment, and outcomes of pediatric HLH patients with IEI other than FHL (IEInotFHL), emphasizing the importance of accurate identification and management. Methods: A systematic search for studies meeting inclusion criteria was conducted in PubMed, EMBASE, MEDLINE, and Cochrane Central. Quality assessment was performed through JBI criteria. Results: A comprehensive search yielded 108 records meeting inclusion criteria, involving 178 patients. We identified 46 different IEI according to IUIS 2022 Classification. Combined immunodeficiencies, immune dysregulation disorders, and phagocyte defects were the IEI most frequently associated with HLH. In 75% of cases, HLH preceded the IEI diagnosis, often with an unrecognized history of severe infections. Triggers reflected the specific infection susceptibilities within IEI groups. Liver and central nervous system involvement were less common than in FHL cases. Treatment approaches and outcomes varied, with limited long-term follow-up data, limiting the assessment of therapeutic efficacy across IEI groups. Conclusion: A comprehensive evaluation encompassing immunological, infectious, and genetic aspects is essential in pediatric-HLH. Relying solely on FHL or EBV susceptibility disorders tests is insufficient, as diverse other IEI can contribute to HLH. Early recognition of HLH as a potential warning sign can guide timely diagnostic investigations and facilitate tailored therapeutic interventions for improved outcomes. Systematic review registration: https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=371425, PROSPERO, CRD42022371425.

Knowledge, attitude and disinformation regarding vaccination and immunization practices among healthcare workers of a third-level paediatric hospital.

BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

[Control of postoperative pain in children undergoing hypospadias surgery: quasi-experimental controlled trial]. / Controllo del dolore post-operatorio nel bambino operato di ipospadia: studio quasi-sperimentale controllato.

BACKGROUND: Hypospadias is one of the most common congenital anomalies occurring in approximately (1/300) male births. If it is not surgically corrected the consequences may negatively impact on quality of life in adolescents. The surgery is very invasive and the post-operative phase very painful. To improve the control of post-operative pain, continuous analgesia via epidural catheter was implemented. AIMS: To compare the effectiveness in controlling pain of two different regimens: continuous epidural catheter infusion vs oral and rectal non-steroidal pain-killers. MATERIALS AND METHODS: Comparative study on children undergoing hypospadias surgery. Group A (catheter) was treated with continuous postoperative analgesia via epidural catheter and Group B (scheduled times) with rectal and oral analgesics at scheduled times and on demand, after caudal block. In both groups nurses measured pain with VAS and FLACC scales (score from 0 to 10) for 72 hours after surgery. RESULTS: 41 children were studied (average age 64.1 months, SD 47.3), with 332 post-operative pain recordings (Group A n = 161, Group B n = 171). Mean pain score of Group A was 0.13 (SD 0.3) and 0.45 (SD 0.8) in group B, p = 0.006. The median duration of the epidural catheter was 65 hours, mean 51.8 hours (SD 24.3). During the 1st post-operative medication, the mean pain score in Group A was 1.2 (SD 1.4), and 3.2 (SD 1.8) in group B, p = 0.003. In group A the number of pain scores indicating pain (> 0) where 3.1% while in group B were 10.5%, p = 0.0007. CONCLUSIONS: In children undergoing hypospadias surgery, post-operative analgesia with continuous epidural catheter infusion seems to offer a better analgesic coverage than the traditional oral/rectal analgesic treatment at scheduled times and on demand.