Infective endocarditis (IE) is an infection of the endocardium and/or heart valves that involves thrombus formation (vegetation). This condition might damage the endocardial tissue and/or valves. An indwelling central venous catheter is a major risk factor for bacteremia at-risked pediatric populations such as premature infants; children with cancer and/or connective tissue disorders. Herbaspirillum huttiense is a Gram-negative opportunistic bacillus that may cause bacteremia and pneumonia rarely in this fragile population. Herein we report the very first case of bacteremia and IE in a pediatric oncology patient caused by H. huttiense.

Background Enterococcal bloodstream infections (EBIs) are increasingly concerning in pediatric patients due to high morbidity, mortality, and antimicrobial resistance, especially vancomycin-resistant enterococci (VRE). This study aimed to evaluate clinical characteristics, species distribution, risk factors, and outcomes of pediatric EBSIs, focusing on VRE and mortality predictors. Methods This retrospective study analyzed 368 children with confirmed enterococcal bacteremia. Demographic, clinical, microbiological, and treatment data were collected. Comparative analyses were done between species, VRE vs. vancomycin-susceptible enterococci (VSE), and mortality outcomes. Logistic regression identified independent risk factors for VRE and mortality. Results Mean age was 5.2 ± 3.8 years, with 53.8% male. Common underlying conditions included hematologic/oncologic malignancies (20.9%), necrotizing enterocolitis (18.8%), and neurological disorders (16.8%). E. faecalis (51.9%) and E. faecium (41.6%) were the most frequent isolates, with VRE detected in 10.9%, predominantly among E. faecium isolates. Older age, prior VRE colonization, E. faecium infection, total parenteral nutrition (TPN) use, and persistent bacteremia significantly increased the risk of VRE infection. The overall 30-day mortality rate was 9.0%, while the total mortality during follow-up reached 20.7%. Thirty-day mortality was independently predicted by prior carbapenem use (OR = 3.2), whereas overall mortality was significantly associated with receipt of TPN (OR = 1.9), ICU admission (OR = 6.5), persistent bacteremia (OR = 3.6), and prior vancomycin use (OR = 2.2). Conclusion Pediatric EBSIs are linked with significant comorbidities and mortality. VRE presence, persistent infection, and prior broad-spectrum antibiotic use increase mortality risk, highlighting the need for antimicrobial stewardship and risk-based management.

Background: Port catheters facilitate the administration of chemotherapy, antibiotics, blood products, fluid and parenteral nutritional support to pediatric patients with hematological malignancies. However, as its use has become widespread, local, and systemic catheter-related infections have emerged as important causes of morbidity and mortality. In our study, we aimed to evaluate the success of antibiotic lock therapy in port catheter- related infections of pediatric patients followed up with acute leukemia. Methods: Port catheter cultures taken from a total of 182 pediatric patients with acute lymphoblastic/myeloblastic leukemia who were followed up at Ankara City Hospital Pediatric Hematology Clinic between August 2019 and August 2023 were evaluated retrospectively. Results: Bacterial growth was identified in 739 port catheter culture specimens of 182 patients. Closure or removal of the port was required in 91, and removal of the port catheters in 49 patients due to port catheter-related infections. Antibiotic lock therapy was started in 56 patients with bacterial growth in the port catheter. With antibiotic lock therapy, port catheter-related infections of 42 patients were eradicated, and their catheters began to be used again. As a result, the port catheter-related infections of 42 of 56 (75%) patients whose ports were closed and also received systemic antibiotherapy were eradicated, and no recurrence of infection was observed. Conclusion: Adding antibiotic lock therapy to systemic antibiotics in pediatric patients may be beneficial in terms of catheter salvage.

Objective: Patients with inherited bleeding disorders faced problems in accessing healthcare during coronavirus disease 2019 pandemic. This study aimed to investigate the health problems of patients with inherited bleeding disorders during the coronavirus disease 2019 pandemic. Material and Methods: Children and adult patients with inherited bleeding disorders who had a coronavirus disease 2019 infection between March 2020 and November 2021 were retrospec- tively evaluated. Results: Seven hundred seventy-two patients were reviewed, and 65 patients who had a coro- navirus disease 2019 infection (Male/Female: 58/7, mean age 28.2 ±14.1 years) were analyzed. Sixty patients (92%) had hemophilia A or B or von Willebrand’s disease and 5 (8%) had rare bleeding disorders. Sixteen (24.6%) patients had a comorbid disease and 6 (9.2%) needed hospitalization due to severe coronavirus disease 2019 infection. Seven patients (10.7%) expe- rienced a bleeding episode and were treated with factor concentrates. Totally, 64 (98.46%) patients recovered from the coronavirus disease 2019 infection and 1 died. Conclusion: Patients with inherited bleeding disorders and coronavirus disease 2019 infection mostly had a mild/moderate course of the disease. Cite this article as: Gürlek Gökçebay D, Koç Şenol B, Kurtipek FB, Koşan Çulha V, Özbek NY, Zülfikar B. The effects of coronavirus disease 2019 pandemic on patients with hemophilia and inherited bleeding disorders: Results from 2 centers in Turkey. Turk Arch Pediatr. 2023;58(4):425-428.

Background/Objectives: Inherited platelet disorders (IPDs) are diverse conditions characterized by abnormalities in platelet count and function. Next-Generation Sequencing (NGS) shows promise as a diagnostic tool in the diagnosis of IPDs. This study aims to assess the clinical value and limitations of using a targeted NGS panel in diagnosing children with suspected IPDs. Methods: We conducted a retrospective study of 93 children evaluated for suspected IPDs. A targeted NGS panel of 14 IPD-associated genes (RUNX1, WAS, ADAMTS13, ANKRD26, CYCS, GATA1, GP1BA, GB1BB, GP9, ITGA2B, ITGB3, MASTL, MPL, MYH9) was performed. Results: Genetic variants were identified in 30 patients (32.3% of the cohort). A total of 37 variants, of which 15 (40.5%) were novel, were found across 11 of the 14 genes on the panel (all except MPL, CYCS, and RUNX1). Variants were most frequently found in ITGB3 (18.9% of variants), GP1BA (16.2%), and ADAMTS13 (16.2%) genes. The majority of variants (64.9%) were classified as variants of uncertain significance (VUS), followed by likely pathogenic (LP) (27%) and pathogenic (8.1%) variants. Most variants were in a heterozygous state (73%). Specific cases highlighted complex genetic scenarios, such as co-occurring variants, and the identification of pathogenic and LP variants in patients initially presenting with immune thrombocytopenia. Conclusions: NGS helps to identify genetic causes, assess risk, manage, and provide genetic counseling in the management of IPDs. However, the prevalence of VUS underscores the need for a multidisciplinary approach to evaluate NGS results accurately.