Staphylococcical scalded skin syndrome: a case series description of a rare and critical disease in a tertiary pediatric center.

Background: Staphylococcal-scalded skin syndrome (SSSS) is a potentially life-threatening disorder characterized by superficial skin blistering caused by exfoliative toxins produced by Staphylococcus aureus. This study aimed to investigate SSSS in a cohort of children admitted at a tertiary pediatric hospital in Italy.

Methods: Patients discharged with the diagnosis of staphylococcal infection and of SSSS between January 2010 and March 2023 were retrospectively identified using ICD-9-CM codes (695.

in hospitalised children and adolescents: the impact of vaccination delay, Tuscany, Italy, 2016 to 2024.

BACKGROUNDPertussis is a highly contagious respiratory infection caused by . Vaccination against pertussis is included in the Italian vaccination programme with three doses administered at 3, 5 and 11 months, booster doses at 6 and 12-18 years, and every 10 years. Vaccination coverage in Tuscany is high among infants (97.

Effectiveness of a targeted infant RSV immunization strategy (2024-2025): A multicenter matched case-control study in a high-surveillance setting.

Background: Nirsevimab, a long-acting monoclonal antibody against respiratory syncytial virus (RSV), was recently introduced to prevent infant RSV-related hospitalizations. Although efficacy has been demonstrated in clinical trials, real-world data on targeted immunization strategies remain limited. We aimed to evaluate the effectiveness of nirsevimab in preventing RSV-associated hospitalizations in infants under 12 months, within a seasonal program prioritizing infants born from April onwards.

Evolving treatment strategies for invasive Streptococcus pyogenes in children in the postpandemic era.

Background: : Streptococcus pyogenes (group A Streptococcus [GAS]) is a common cause of bacterial pharyngitis and skin infections in children that can lead to severe and invasive GAS (iGAS) infections. The sudden acute respiratory syndrome coronavirus 2 pandemic coincided with an increase in iGAS cases, with emerging serotypes and risk factors like age, reduced postpandemic immunity, and viral coinfections. The treatment of iGAS with clindamycin and intravenous immunoglobulins (IVIG) is not well standardized, and pediatric data are limited.

Phenotypic Divergence of JAG1- and NOTCH2-Associated Alagille Syndrome & Disease-Specific NOTCH2 Variant Classification Guidelines.

Background & Aims: Alagille syndrome (ALGS) is a rare, autosomal dominant disorder with high phenotypic heterogeneity. Disease-causing variants are primarily identified in Jagged1 (JAG1), with fewer reported in NOTCH2. JAG1 variants cause disease through a mechanism of haploinsufficiency, but the mechanism for NOTCH2 variants is not completely understood, making classification of variants more challenging.

Primary and Secondary Prophylaxis of Gastrointestinal Bleeding in Children with Portal Hypertension: A Multicenter National Study by SIGENP.

: Portal hypertension (PH) is a common complication in children with chronic liver diseases. Primary and secondary prophylaxis of variceal bleeding in these patients remains controversial. Our study aims to evaluate the management of gastrointestinal (GI) varices in children with PH in Italy.

The impact of nirsevimab prophylaxis on RSV hospitalizations: a real-world cost-benefit analysis in Tuscany, Italy.

Background: Respiratory Syncytial Virus (RSV) is the leading cause of hospitalizations in infants. The approval of nirsevimab, a long-acting monoclonal antibody, has extended the potential for RSV prophylaxis to all infants. This study assesses the cost-benefit of various Nirsevimab prophylaxis strategies for infants during their first RSV season in preventing RSV-associated hospitalization in the Tuscany region, Italy.

Blood And Pharyngeal Swab Polymerase Chain Reaction Testing: Diagnostic and Therapeutic Implications in Children With Acute Nonbacterial Encephalitis.

Background: Children with acute nonbacterial encephalitis are often treated with extended antimicrobial therapies. Negative results from cerebrospinal fluid (CSF) examinations frequently lead to unnecessary prolongation of empirical treatments due to uncertain etiology. To enhance the identification rate of infectious causative agents, additional tests are increasingly performed on non-CSF specimens, including viral polymerase chain reaction (PCR) testing on blood or pharyngeal swabs.

Optimizing antimicrobial routes: risk factor analysis for intravenous therapy in children with preseptal orbital cellulitis.

Background: The optimal route of antimicrobial administration for preseptal orbital cellulitis (OC) in children remains uncertain. While mild cases may be managed with oral therapy, distinguishing between mild and severe presentations is challenging. The recently proposed ASSET score offers a tool for assessing the severity of skin and soft tissue infections, but prospective validation in large cohorts of preseptal OC is lacking.

Hip Involvement in Pediatric Scurvy: Early Magnetic Imaging Signs.

Scurvy is an uncommon and often underrecognized disease. However, conditions associated with a restrictive and/or selective diet and inadequate absorption still pose a high risk for developing vitamin C deficiency. Musculoskeletal symptoms are among the most characteristic manifestations of scurvy, often requiring radiological investigations.

Update on pediatric liver transplantation in Europe 2022: An ELITA-ESPGHAN report.

Objectives: The European Liver Transplant Registry (ELTR) has been collecting data on liver transplantation (LT) in Europe since 1968. The aim of this report is to outline the number, techniques utilized, indications for, and outcomes of pediatric LT (pLT) in Europe, focusing on the Year 2022 in comparison to the preceding 5 years.

Methods: Data were obtained from ELTR and Eurotransplant (ET).

Updated recommendations for the management of hepatitis B, C, and E virus infections in patients with haematological malignancies and those undergoing haematopoietic cell transplantation: recommendations from the 9th European Conference on Infections in Leukaemia (ECIL-9).

Viral hepatitis remains a global health challenge and immune status affects outcomes. In patients with haematological malignancies, including haematopoietic stem-cell transplantation recipients, viral hepatitis can be life-threatening due to the direct effects of the virus or the need to modify or delay chemotherapy. Additionally, haematopoietic stem-cell donors with past or current viral hepatitis infections might transmit the virus to recipients.

Changes in liver stiffness and steatosis in children after successful treatment with sofosbuvir/velpatasvir: Results of the PANDAA-PED study.

Aim Of The Study: The aim of this study was to analyze changes in liver stiffness and steatosis using noninvasive methods in children aged 6 to 18 years up to one year after successful treatment with sofosbuvir/velpatasvir (SOF/VEL).

Material And Methods: Evaluation of liver stiffness and steatosis was performed in 49 patients at 12 weeks and one year after treatment using noninvasive methods. Liver stiffness measurement (LSM) and the controlled attenuation parameter (CAP) were obtained by transient elastography (FibroScan 530, Echosens).

Real-world experience with odevixibat in children with progressive familial intrahepatic cholestasis.

Background & Aims: A previously published trial demonstrated that odevixibat is effective in the treatment of cholestatic pruritus of children with progressive familial intrahepatic cholestasis (PFIC). Real-world experience is necessary to confirm the results of registration trials with selective eligibility criteria. We present our 'real-life experience' of the effectiveness and safety of odevixibat in patients with different PFIC subtypes.

Long-term efficacy and safety of sofosbuvir-based direct-acting antiviral regimens in paediatric patients with hepatitis C virus infection: an international registry study.

Background: Direct-acting antiviral treatment is associated with high rates of sustained virological response and has a favourable safety profile in children aged 3-17 years with chronic hepatitis C virus (HCV) infection, but data describing the durability of sustained virological response and its effects on growth and sexual development are needed. This study aimed to assess the efficacy and long-term effects of sofosbuvir-based direct-acting antiviral regimens on growth and development in the paediatric population.

Methods: In this international, multicentre, observational registry study, children aged 3-17 years with HCV who received sofosbuvir plus ribavirin, ledipasvir plus sofosbuvir with or without ribavirin, sofosbuvir plus velpatasvir, or sofosbuvir plus velpatasvir plus voxilaprevir in previous clinical trials were eligible for follow-up of growth and sexual development parameters for up to 5 years.

Modelling the potential clinical and economic impact of universal antenatal hepatitis C (HCV) screening and providing treatment for pregnant women with HCV and their infants in Egypt: a cost-effectiveness study.

Backgrounds And Aims: Pregnant women and children are not included in Egypt's hepatitis C virus (HCV) elimination programmes. This study assesses the cost-effectiveness of several screening and treatment strategies for pregnant women and infants in Egypt.

Design: A Markov model was developed to simulate the cascade of care and HCV disease progression among pregnant women and their infants according to different screening and treatment strategies, which included: targeted versus universal antenatal screening; treatment of women in pregnancy or deferred till after breast feeding; treatment of infected children at 3 years vs 12 years.

Attitudes and perspectives of healthcare workers on treating chronic hepatitis C infection in children and adolescents.

Background And Aims: There are gaps in knowledge about the values and preferences of healthcare workers (HCW) with respect to treatment of children and adolescents living with chronic hepatitis C (HCV) infection. This study was carried out to identify these values and preferences as part of the evidence required to update World Health Organization (WHO) hepatitis C guidelines.

Methods: An online survey was designed and conducted during August/September 2021.

Clinical characteristics and predictive factors of thrombotic complications in children with acute mastoiditis: a single center retrospective study.

Among acute mastoiditis (AM) complications, cerebral venous sinus thrombosis (CVST) is particularly severe, leading to increased intracranial pressure and potential neurological sequelae. Predicting the development of such complications is challenging. The aims of the present study were to evaluate the incidence, clinical characteristics, and risk factors for the development of CVST in AM.

Effect of elexacaftor-tezacaftor-ivacaftor on liver transient elastography, fibrosis indices and blood tests in children with cystic fibrosis.

Background: Elexacaftor-tezacaftor-ivacaftor (ETI) has significantly improved the clinical course of people with cystic fibrosis (pwCF) and eligible CFTR variants. In this study, we prospectively evaluated liver elastography, liver fibrosis indices and liver tests in children with CF aged 6-12 years started on ETI therapy.

Methods: Body mass index, sweat test, percent predicted forced expiratory volume in one second, serum markers of liver injury or portal hypertension, liver fibrosis indices, controlled attenuation parameter and liver stiffness were assessed before starting ETI and three and twelve months post-ETI, according to new international guidelines.

Health-related Quality of Life in Children and Adolescents After Successful Treatment of Chronic Hepatitis C With Sofosbuvir/Velpatasvir: One-year Outcomes.

Background And Aims: The aim of this study was to assess the health-related quality of life (HRQL) of children with chronic hepatitis C (CHC) at 1 year after the effective treatment with sofosbuvir/velpatasvir (SOF/VEL).

Methods: All 50 patients treated for CHC with a fixed dose SOF/VEL in the noncommercial, nonrandomized, open-label PANDAA-PED study achieved sustained virologic response at 12 weeks after the end of treatment. Evaluation of HRQL at 1-year posttreatment was compared with the baseline (before the treatment) assessment.

Primary sclerosing cholangitis in children with inflammatory bowel disease: An ESPGHAN position paper from the Hepatology Committee and the IBD Porto group.

Objective: We aimed to provide an evidence-supported approach to diagnose, monitor, and treat children with inflammatory bowel disease (IBD) and primary sclerosing cholangitis (PSC).

Methods: The core group formulated seven PICO-structured clinical questions. A systematic literature search from inception to December 2022 was conducted by a medical librarian using MEDLINE and EMBASE.

Effectiveness and Safety of Glecaprevir/Pibrentasvir in Italian Children and Adolescents With Chronic Hepatitis C: A Real-Word, Multicenter Study.

Background & Aims: Glecaprevir/Pibrentasvir (GLE/PIB) has been approved by the European Medicine Agency (EMA) and by the US Food and Drug Administration (US-FDA) for the treatment of children and adolescents from 3 years of age with chronic hepatitis C virus (CHC) infection. The aim of this study was to confirm the real-world effectiveness and safety of GLE/PIB in children and adolescents (3 to < 18 years old) with CHC.

Methods: This prospective, multicentre study involved 11 Italian centres.

Exploring Risk Factors Associated With Intensive Care Unit Admission in a Retrospective Cohort of 631 Children With Bronchiolitis.

Aim: Examine the trends and epidemiological features of acute hospitalized bronchiolitis cases among children aged 28 days to 2 years, both before and after the COVID-19 pandemic. Furthermore, evaluate their need for intensive care unit (ICU) admission and identify primary risk factors associated with severe bronchiolitis.

Methods: A retrospective analysis was conducted on the medical records of paediatric patients admitted to Meyer Children's Hospital in Florence (Italy) from September 2019 to March 2023, with the diagnosis of bronchiolitis.