Turkish archives of pediatrics最新文献

{"title":"Effectiveness, Safety, and Treatment Satisfaction of 20% Subcutaneous Immunoglobulin Replacement Therapy in Pediatric Patients with Primary and Secondary Immunodeficiencies.","authors":"Asena Pinar Sefer, Mehmet Sirin Kaya","doi":"10.5152/TurkArchPediatr.2025.24305","DOIUrl":"https://doi.org/10.5152/TurkArchPediatr.2025.24305","url":null,"abstract":"<p><p>Objective: Primary immunodeficiencies (PID) and secondary immunodeficiencies (SID) increase the risk of severe and recurrent infections. Immunoglobulin replacement therapy (IGRT) is essential for these patients, administered as intravenous immunoglobulin (IVIG) or subcutaneous immunoglobulin (SCIG). Subcutaneous immunoglobulin therapy, notably the high-concentration 20% immune globulin subcutaneous solution (Ig20Gly), provides significant advantages, including home administration, reduced systemic adverse reaction (SAR), and enhanced patient autonomy through less frequent dosing and lower infusion volumes. This study aims to evaluate the effectiveness, safety, and treatment satisfaction of Ig20Gly in patients with PID and SID, offering insights into optimizing IGRT and the impact of high-concentration SCIG on improving outcomes. Materials and Methods: The authors conducted a retrospective cohort study of 22 pediatric patients with PID or SID who transitioned from IVIG to SCIG. The authors gathered clinical and demographic data, including infection rates, immunoglobulin G (IgG) levels, school absenteeism, and parental workday loss. Treatment satisfaction was assessed using the treatment satisfaction questionnaire for medication-9, and statistical analyses were performed to compare outcomes before and after the transition. Results: Transitioning to SCIG raised median IgG levels from 690 mg/dL during IVIG to 850 mg/dL after 12 months of SCIG (P < .001). Severe bacterial and lower respiratory tract infections decreased significantly, along with parenteral antibiotic use (P = .003, P = .005, P = .009, respectively). School absenteeism and caregiver workday loss also declined (P < .001). While no SARs were observed with SCIG, mild local reactions were reported, which diminished over time. Treatment satisfaction improved significantly, with younger children under 9 reporting lower convenience scores. Patients on IVIG for over 24 months before SCIG had higher satisfaction scores. Conclusion: Twenty percent SCIG therapy has demonstrated a favorable safety and tolerability profile, providing substantial practical advantages for pediatric patients and their families. This therapy not only streamlines the management of pediatric immunodeficiencies but also effectively maintains optimal Ig levels, thereby decreasing infection rates and improving overall patient well-being. These results underscore the potential of SCIG therapy to enhance treatment satisfaction and quality of life. However, additional research is necessary to confirm these findings and evaluate long-term outcomes.</p>","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"60 2","pages":"217-225"},"PeriodicalIF":1.3,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143652500","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Retinoblastoma in Albania: A 25-Year Retrospective Analysis.","authors":"Enkeleda Duka, Eduart Hashorva, Mirzana Kapllanaj, Mirela Xhafa, Anila Godo, Alketa Tandili, Eneda Rustemi, Elizana Petrela, Mattan Arazi, Ido Didi Fabian, Anila Babameto, Mirela Tabaku, Lila Shundi, Donjeta Bali","doi":"10.5152/TurkArchPediatr.2025.24203","DOIUrl":"https://doi.org/10.5152/TurkArchPediatr.2025.24203","url":null,"abstract":"<p><p>Background: Retinoblastoma (RB), the most common intraocular cancer worldwide, has been extensively investigated. To the best of our knowledge, however, no reports exist on RB in Albania. We aimed to present the first case series of RB in Albania, including presentation, treatment, and outcome of patients. Materials and Methods: This was a retrospective case series of patients diagnosed with RB from 1998 to 2023 at a single country Mother Teresa University Hospital Center in Albania. Epidemiologic and clinical data were extracted from follow-up clinical charts. Results: During the 25-year study period, 22 patients were diagnosed with RB, of whom 59% were females. The average age at diagnosis was 21.8 months (SD-18.8 months). In 13 (59%) cases, the disease was diagnosed within the first year of life, and less than 5% were diagnosed after the age of 5 years. Overall, 18% of patients had family history of RB, and 41% had bilateral RB. The time from the first symptom to diagnosis was less than a month in 32% of cases, while 77% of patients were diagnosed within 4 months. The main presenting symptom was leukocoria in 6 (27%) cases, strabismus in 3 (14%) cases, and combination of both in 3 (14%) cases. Treatment was mainly a combination of enucleation and systemic chemotherapy in 15 (71%) cases. Only 13 (59%) patients continued treatment within Albania, with the rest being treated abroad. Conclusion: We present the first cohort of children with RB from Albania, a country with limited diagnostic and treatment resources. The advanced disease states of these children underscore the importance of implementing national pediatric screening programs.</p>","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"60 2","pages":"159-163"},"PeriodicalIF":1.3,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143652512","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Investigation of Trace Element and Toxic Metal Blood Levels in Obese Children.","authors":"Sinan Kılıç","doi":"10.5152/TurkArchPediatr.2025.242762","DOIUrl":"https://doi.org/10.5152/TurkArchPediatr.2025.242762","url":null,"abstract":"","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"60 2","pages":"245-246"},"PeriodicalIF":1.3,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143652506","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cystic Fibrosis Treatment Landscape: Progress, Challenges, and Future Directions.","authors":"Ceren Ayça Yıldız, Yasemin Gökdemir, Ela Erdem Eralp, Pınar Ergenekon, Fazilet Karakoç, Bülent Karadağ","doi":"10.5152/TurkArchPediatr.2025.24257","DOIUrl":"https://doi.org/10.5152/TurkArchPediatr.2025.24257","url":null,"abstract":"<p><p>Cystic fibrosis (CF) is a monogenic autosomal recessive disorder that primarily affects the respiratory and gastrointestinal systems. It results from variants in the CFTR gene, leading to dysfunctional chloride channels, thickened mucus secretion, and subsequent multisystem complications. Significant advances have been made in CF treatment, particularly with the development of CFTR modulators, which are unique to genotypes and have improved clinical outcomes in many people with CF. However, the benefits of these therapies are not universal, with a considerable portion of the CF population-especially those with rare mutations-still without access to effective treatment options. This review provides a comprehensive overview of the pathophysiology and genetic basis of CF, explores current and emerging treatments, and discusses the ongoing challenges in the field.</p>","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"60 2","pages":"117-125"},"PeriodicalIF":1.3,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143652498","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Transient Thyroid Dysfunction in New-Onset Type 1 Diabetes: Reassessing Test Timing for Accurate Results.","authors":"Talha Üstüntaş, Mehmet Emre Atabek, Beray Selver Eklioğlu, Saime Ergen Dibeklioğlu","doi":"10.5152/TurkArchPediatr.2025.24159","DOIUrl":"https://doi.org/10.5152/TurkArchPediatr.2025.24159","url":null,"abstract":"<p><p>Objective: Type 1 diabetes mellitus (T1DM) is a prevalent chronic disease affecting children, necessitating ongoing care from multidisciplinary clinics. This study aimed to determine the prevalence of thyroid dysfunction in newly diagnosed T1DM patients, investigating long-term thyroid function through periodic monitoring. Materials and Methods: A retrospective cohort study utilized the medical records of pediatric patients with newly diagnosed T1DM. Extracted data included age at initial diagnosis, presenting clinical manifestations, standard laboratory findings, and thyroid function panel results. Results: The sample included 208 T1DM patients, with a higher proportion of males (54.8%) than females (45.2%). Initial assessment revealed that 69.2% of patients were euthyroid, 24.5% exhibited euthyroid sick syndrome (ESS), 5.8% compensated hypothyroidism, and 0.5% primary hypothyroidism. About 76.5% of patients initially diagnosed with ESS were euthyroid at the second visit. Analysis confirmed a significant difference in the distribution of euthyroid and ESS patients between the first and second visits (P < .001). Temporal analysis indicated that thyroid function tended to normalize within 1-2 weeks of the initial presentation. Conclusion: Tests should be performed within 1-2 weeks following clinical stabilization to evaluate thyroid function in newly diagnosed T1DM patients. However, patients with a history of thyroid disease, or those with clinical signs indicative of thyroid dysfunction, should undergo immediate testing at the time of diagnosis.</p>","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"60 2","pages":"153-158"},"PeriodicalIF":1.3,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143652558","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Surfactant Therapy in Late Preterm Infants with Respiratory Distress in Türkiye: An Observational, Prospective, Multicenter Study.","authors":"Sumru Kavurt, Nihal Demirel, İstemi Han Çelik, Ahmet Yagmur Bas, Makbule Ercan, Beyza Özcan, Zehra Aslan, Demet Oğuz, Musa Turgut, Melike Kefeli Demirel, Nilay Hakan, Ali Bülbül, Kadir Tekgündüz, Samet Benli, Hüseyin Altunhan, Murat Konak, Ferda Ozlü, Oğuz Tuncer, Ozge Aydemir","doi":"10.5152/TurkArchPediatr.2025.24236","DOIUrl":"https://doi.org/10.5152/TurkArchPediatr.2025.24236","url":null,"abstract":"<p><p>Objective: Surfactant therapy (ST) is commonly used in late preterm (LPT) infants with respiratory distress despite a lack of definitive recommendation for these infants. Our aim was to establish a national prospective database to evaluate the use of surfactants in LPT infants. Materials and Methods: A multicenter, prospective observational cohort study was conducted among LPT infants treated with surfactant between January 1, 2022, and December 31, 2022. Twenty neonatologists from 16 neonatal intensive care units (NICUs) participated in the study. Results: During the study period, a total of 3327 LPT infants were admitted to the participating NICUs. Among them, 1866 infants experienced respiratory distress, and 288 received surfactant treatment. In this study, respiratory distress syndrome (RDS) was the most common indication for surfactant administration, affecting 158 infants (54.8%), followed by congenital pneumonia in 79 infants (27.4%) and transient tachypnea of the newborn (TTN) in 32 infants (11.1%). Conclusion: We demonstrated that ST is administered with significant variability among LPT infants experiencing respiratory distress. Additionally, respiratory issues in LPT infants beyond RDS, such as congenital pneumonia and TTN, are also frequently treated with surfactant.</p>","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"60 2","pages":"164-171"},"PeriodicalIF":1.3,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143652592","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Investigation of Quality of Life Change After Catheter Ablation in Children with Arrhythmia.","authors":"Kardelen Yagmur Isin, Meryem Beyazal, Ilker Ufuk Sayici, Tamer Yoldas, Senem Ozgur","doi":"10.5152/TurkArchPediatr.2025.24184","DOIUrl":"https://doi.org/10.5152/TurkArchPediatr.2025.24184","url":null,"abstract":"<p><p>Objective: Arrhythmia in children is a serious health problem. The role of pharmacological treatment in the treatment of arrhythmia in children is limited. Therefore, ablation has an important role in the treatment of arrhythmia. The aim of this study is to evaluate the quality of life (QoL) after catheter ablation. Materials and Methods: Patients and their parents completed the scales independently but simultaneously, before ablation and 6 months after ablation. Seventy-two children and their parents were included in the study. The QoL Scale was designed as a five-point Likert scale and was divided into 2 age groups, 8-12 years and 13-18 years, with each age group having separate child and parent forms. The scale forms consisted of 4 sub-sections to understand physical, emotional, social, and school functioning. Results: Of the ablations, 65 (90.3%) were successful, 5 (6.9%) were unsuccessful and 2 (2.8%) were suboptimally successful. Accordingly, there was a statistically significant increase in quality of life after successful ablation (P =.001). There was also a significant difference in the magnitude of the increase in QoL according to the type of ablation (P =.025). However, when the sex, age groups, and sub-diagnosis groups of the patients were compared, no significant difference was observed in terms of change in quality of life. Conclusion: This study is the first in Türkiye to investigate the impact of catheter ablation on QoL of children who underwent catheter ablation for various cardiac arrhythmias.</p>","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"60 2","pages":"200-207"},"PeriodicalIF":1.3,"publicationDate":"2025-03-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143652504","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neurodevelopmental Impact of Pesticides: A Silent Threat.","authors":"Kıykım Ertuğrul","doi":"10.5152/TurkArchPediatr.2025.1112252","DOIUrl":"https://doi.org/10.5152/TurkArchPediatr.2025.1112252","url":null,"abstract":"","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"60 2","pages":"114-116"},"PeriodicalIF":1.3,"publicationDate":"2025-02-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143652507","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prevalence and Severity of Gastroesophageal Reflux Disease in Indian Children Presenting with Symptoms of Acid Reflux: A Real-world Evidence Study.","authors":"Dhanasekhar Kesavelu, Nithya Franklyn, C Venkatraghavan, Dev Narayan, Kumar P Ravindra, Akram Wasim","doi":"10.5152/TurkArchPediatr.2025.24140","DOIUrl":"https://doi.org/10.5152/TurkArchPediatr.2025.24140","url":null,"abstract":"","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2025-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143054600","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Element Levels According to Biologic Matrix During Childhood Period.","authors":"Merve Çiçek Kanatlı, Sıddika Songül Yalçın","doi":"10.5152/TurkArchPediatr.2024.24276","DOIUrl":"10.5152/TurkArchPediatr.2024.24276","url":null,"abstract":"","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":" ","pages":"243-244"},"PeriodicalIF":1.3,"publicationDate":"2025-01-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143054584","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}