The Cystic Fibrosis Registry of Türkiye (CFRT) was established by the Turkish Pediatric Respiratory Diseases and Cystic Fibrosis Society and has provided detailed information on demographic, clinical, genetic, and treatment-related aspects of cystic fibrosis (CF) patients since 2017. We aimed to describe the current status of CF in Türkiye using CFRT's 2023 annual data. Demographic, clinical, and treatment data were taken from CFRT's 2023 record. In 2023, 2,258 patients from 34 centers were recorded. The median age of patients was 9.1 years, and 46.9% were female, with a median age at diagnosis of 0.3 years. Only 14.9% of the patients were older than 18 years. Genetic analyses were completed in 97.3% of patients. The most common variant, F508del, had a total variant frequency of 22.1%. The median percent predicted FEV1 and FVC were 88.0 and 94.0 in those aged 6-17 years 71.0 and 84.0 in those aged ≥18 years, respectively. The median values of body mass index z-scores were -0.5, and -0.5 for patients 2-18 and older than 18 years, respectively. Chronic colonization with Pseudomonas aeruginosa was present in 17.2% of the patients. Most patients used inhaled recombinant human DNase (87.1%) and oral pancreatic enzyme replacement treatment (83.0%). CF transmembrane conductance regulator (CFTR) modulators were used by 15.9% of patients. Over the year, 24 patients died, with a median age at death of 13.3 years. The CFRT report provides a valuable resource showing the clinical and laboratory data of patients with CF in the country.
Platelets play a role in hemostasis, thrombosis, and vascular integrity. They also play a major role in the development of inflammation and the activation of immune responses. They have phagocytic activity, stimulate the secretion of immune modulators, and activate other immune cells, which results in platelet-neutrophil aggregation, platelet-induced neutrophil degranulation, and the formation of neutrophil extracellular traps. Data on 124 febrile neutropenia attacks were retrospectively examined. Patients’ age, sex, diagnosis, and relapse history were obtained. The complete blood count levels on the first and last febrile day of the febrile neutropenia attacks, duration of fever, and number, type, and timing of thrombocyte suspension transfusions were recorded. The patients were divided into three groups according to the day of fever when the platelet suspension was administered (1 day, 2–3 days, and >3 days); the duration of fever was compared between the three groups. The fever duration of those who were transfused with platelet suspension on the first day of fever was found to be significantly shorter (p = 0.03 and p < 0.001, respectively). When treating a patient with febrile neutropenia, if thrombocyte suspension transfusion is indicated, transfusing thrombocytes in the first days of fever shortens the fever duration and improves the prognosis of febrile neutropenia attack, supporting the hypothesis that not only neutrophils but also platelets may play a role in fighting against microorganisms.
Tuberculosis (TB) remains a major global health concern, especially for children with rheumatic diseases on biologic therapies. While these agents have improved disease management and quality of life, they also pose new challenges, mainly an increased risk of TB, particularly with tumor necrosis factor‑alpha (TNF‑α) inhibitors like infliximab, adalimumab, and etanercept. Since TNF‑α plays a crucial role in granuloma formation and controlling Mycobacterium TB, its inhibition can allow latent TB infection (LTBI) to develop into active disease. To reduce this risk, screening for LTBI before starting biologic therapy is highly recommended. However, accurately detecting LTBI in children—especially those who are immunosuppressed—remains challenging, as both the tuberculin skin test (TST) and interferon‑gamma release assays (IGRAs) have notable limitations in this context. Using both TST and IGRA together may improve case detection in selected pediatric populations. Preventive treatment for LTBI reduces the risk of developing active TB in patients receiving biologic therapies. Although non-anti-TNF therapies may offer a safer option in this regard, the evidence in pediatric patients is still limited. As the use of biologic and targeted therapies expands in children, further research is needed—especially on non-anti-TNF agents—to create internationally accepted guidelines and to optimize screening, diagnostics, and personalized prevention strategies.