{"title":"Mucolipidosis Type III: A Hidden Challenge in the Differential Diagnosis of Rheumatological Disorders.","authors":"Ezgi Burgac, İrem Kaplan, Fatma Derya Bulut, Esra Kara, Burcu Köseci, Sevcan Tuğ Bozdoğan, İlke Coşkun Benlidayı, Deniz Kor, Neslihan Önenli Mungan","doi":"10.65717/TurkArchPediatr.2026.25402","DOIUrl":"https://doi.org/10.65717/TurkArchPediatr.2026.25402","url":null,"abstract":"<p><strong>Objective: </strong>Mucolipidosis type III (ML-III) alpha/beta and gamma are uncommon lysosomal storage diseases caused by a partial deficiency of the N-acetylglucosaminyl-1-phosphotransferase enzyme. Biallelic mutations in the GNPTAB gene are responsible for ML-III alpha/beta, whereas mutations in GNPTG lead to ML-III gamma. This study aims to thoroughly investigate the clinical manifestations and diagnostic clues of ML-III, with the goal of preventing misdiagnosis and delays in diagnosis.</p><p><strong>Methods: </strong>A retrospective review was conducted of all patients referred to the Pediatric Metabolism Department between 2010 and 2022 due to joint stiffness and/or pain, with a total of 192 patients evaluated during this period. The clinical, laboratory, radiological, and genetic data of patients diagnosed with ML-III were reviewed in detail.</p><p><strong>Results: </strong>Six patients (3.1%) from the cohort of 192 were diagnosed with ML-III. The median age of these patients at symptom onset was 2.75 years (min-max: 1.2-11.5 years). The initial finding of all patients was stiffness of finger joints. Five patients were misdiagnosed before admission, and the median age at diagnosis was 12 years (min-max: 5-35 years). The median delay in diagnosis was 6.5 years (min-max: 3-24 years). Neither ML-III alpha/beta nor ML-III gamma patients had psychomotor retardation. The patients' ages ranged from 8.6 to 38 years.</p><p><strong>Conclusion: </strong>The ML-III's clinical findings can lead to misdiagnoses, such as rheumatological disorders. Heightened awareness could prompt earlier detection, underlining the importance of thorough evaluations for all patients with joint-related presentations.</p>","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"61 5","pages":"399-405"},"PeriodicalIF":1.7,"publicationDate":"2026-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147847421","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}
Ievgeniia Burlaka, Heorhii Liezhentsev, Valeriia Kolodchenko, Inga Mityuryayeva-Korniyko
{"title":"Oxidative Stress in Childhood Nephrotic Syndrome: A Narrative Review From Mechanisms to Clinical Implications.","authors":"Ievgeniia Burlaka, Heorhii Liezhentsev, Valeriia Kolodchenko, Inga Mityuryayeva-Korniyko","doi":"10.65717/TurkArchPediatr.2026.25476","DOIUrl":"https://doi.org/10.65717/TurkArchPediatr.2026.25476","url":null,"abstract":"<p><strong>Unlabelled: </strong>Pediatric nephrotic syndrome (NS) is a complex renal disorder characterized by proteinuria, hypoalbuminemia, edema, hyperlipidemia, and risk of chronic kidney disease. Oxidative stress (OS) is increasingly recognized as a central mechanism driving glomerular and tubular injury, inflammation, and progression of kidney injury. The aim of this review was to summarize the evidence on the role of OS in the pathophysiology and clinical implications of NS. A narrative review was conducted using PubMed, Scopus, Web of Science, and Google Scholar (2000-2025), focusing on recent studies. Keywords included \"nephrotic syndrome,\" \"oxidative stress,\" \"reactive oxygen species,\" and \"antioxidant enzymes.\" Data were synthesized to identify key oxidative mechanisms, biomarkers, and clinical relevance. The NS is characterized by excessive reactive oxygen species production via NADPH oxidase, xanthine oxidase, and mitochondrial pathways, accompanied by impaired antioxidant defenses such as glutathione and superoxide dismutase. Elevated malondialdehyde and advanced oxidation protein products correlate with proteinuria, podocyte injury, and steroid resistance. Reduced antioxidants indicate diminished cellular defense and redox imbalance. The OS contributes to podocyte effacement, tubulointerstitial apoptosis, fibrosis, and progressive nephron loss. Monitoring OS biomarkers can improve assessment of disease activity, predict relapse or steroid resistance, and guide possible antioxidant interventions.</p>","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"61 8","pages":"660-665"},"PeriodicalIF":2.3,"publicationDate":"2026-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148655133","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":"Raising Active Generations: A Pediatric Call to Address Physical Inactivity and Sedentary Lifestyles.","authors":"Ela Tarakcı, Gökçe Leblebici, Bülent Bayraktar","doi":"10.65717/TurkArchPediatr.2026.20264001","DOIUrl":"https://doi.org/10.65717/TurkArchPediatr.2026.20264001","url":null,"abstract":"","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"61 4","pages":"285-289"},"PeriodicalIF":1.7,"publicationDate":"2026-03-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147791762","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":"Musculoskeletal Ultrasound in Juvenile Idiopathic Arthritis: Advances in Imaging and Clinical Practice.","authors":"Şeyma Türkmen, Betül Sözeri","doi":"10.65717/TurkArchPediatr.2026.25415","DOIUrl":"https://doi.org/10.65717/TurkArchPediatr.2026.25415","url":null,"abstract":"<p><p>Musculoskeletal ultrasound (MSUS) has become a cornerstone imaging modality in pediatric rheumatology, providing a safe, non-invasive, and highly sensitive method for assessing musculoskeletal pathology. Reliable interpretation of MSUS requires awareness of developmental anatomy, optimization of technical parameters, and recognition of common artifacts. These considerations are especially important in children, where physiological vascularization and age-related changes in cartilage or bone can mimic pathology. MSUS enables the detailed visualization of synovitis, tenosynovitis, enthesitis, and early structural abnormalities, often detecting both overt and subclinical inflammation that may be missed on clinical examination. These subclinical observations can offer additional context during follow-up and support more informed decisions in disease monitoring and management. In addition to its diagnostic and prognostic roles, MSUS also enhances interventional approaches. Ultrasound-guided intra-articular corticosteroid injections improve precision, safety, and patient comfort compared with landmark-based techniques, while also expanding therapeutic access to joints that are otherwise difficult to inject. Altogether, MSUS is increasingly recognized not only as a complementary tool but as an integral component of juvenile idiopathic arthritis (JIA) management. This review will provide an updated overview of MSUS in the context of JIA, with a particular focus on practical aspects of scanning in children, the differentiation between physiological and pathological findings, the main disease-related sonographic features, and the expanding role of ultrasound in guiding intra-articular procedures. Current challenges, limitations, and future perspectives regarding standardization and integration into clinical care will also be discussed.</p>","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"61 4","pages":"290-297"},"PeriodicalIF":1.7,"publicationDate":"2026-03-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147791700","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":"Brain Abscess Clinical Presentation, Microbiology, Management, and Outcome: Evidence from a Tertiary Pediatric Center.","authors":"Mahmoud Khodabandeh, Roya Haghighi, AmirAli Soltani, Mahsa Soti Khiabani","doi":"10.65717/TurkArchPediatr.2025.25244","DOIUrl":"https://doi.org/10.65717/TurkArchPediatr.2025.25244","url":null,"abstract":"<p><strong>Objective: </strong>Brain abscess (BA) is a life-threatening infection affecting the brain parenchyma. It can be caused by various microorganisms and often occurs due to underlying etiology. This study aimed to assess the clinical profile, etiology, paraclinical data, and outcomes of pediatric BA.</p><p><strong>Materials and methods: </strong>This retrospective descriptive study was conducted at the Children's Medical Center Hospital in Tehran, Iran, from 2010 to 2020. Diagnosis was based on clinical presentation and imaging. Data was collected for each patient from the hospital database.</p><p><strong>Results: </strong>A total of 45 patients were included in the study. The mean age of patients was 4.93 years (range: 2 months old-18 years old (216 months old) (SD: 58.16 months)), with a male-to-female ratio of 1.36:1. The most frequent clinical symptoms were vomiting, headache, and fever. Streptococcus viridans was the most common organism isolated (25%), though causative organisms were identified in only 35.5% of cases. Predisposing factors included cyanotic heart disease (CHD) (17.7%), meningitis (6.6%), and otitis media (6.6%). The most involved anatomical regions were the frontal lobe (35.5%) and then the multiple lobes (26.6%), respectively. Antibiotic treatment resulted in the cure of 23 patients (51.1%), while 22 patients (48.9%) required surgical abscess drainage in addition to antibiotics. The average hospital stay was 39 days and overall mortality was 6.66%.</p><p><strong>Conclusion: </strong>Considering BA in patients with CHD, meningitis, and otitis media helps in earlier diagnosis. Early initiation of antibiotics and selection based on the causative organism can improve BA outcomes in children.</p>","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"61 4","pages":"336-343"},"PeriodicalIF":1.7,"publicationDate":"2026-03-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147791588","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}
Ayfer Gozu Pirinccioglu, Savaş Mert Darakçi, Suleyman Yildiz, Orhan Derman
{"title":"Investigation of the Association of the Attitudes and Beliefs of Adolescents and Their Parents with the Aggression Behaviours of Adolescents.","authors":"Ayfer Gozu Pirinccioglu, Savaş Mert Darakçi, Suleyman Yildiz, Orhan Derman","doi":"10.65717/TurkArchPediatr.2026.24344","DOIUrl":"https://doi.org/10.65717/TurkArchPediatr.2026.24344","url":null,"abstract":"<p><strong>Objective: </strong>Violent behavior is prevalent among adolescents and represents a leading cause of morbidity and mortality. Understanding the factors contributing to adolescent aggression is essential for developing effective prevention strategies and promoting public health. This study aims to examine the association of the beliefs and attitudes of adolescents and their parents with the aggression of adolescents.</p><p><strong>Materials and methods: </strong>This cross-sectional study includes 413 adolescents aged 13-19 and their parents (408 mothers and 383 fathers). The Buss-Perry Aggression (BPA) scale was employed to assess aggression levels in adolescents, while the Attitude Toward Violence (ATV) and Belief in Violence (BIV) scales were used to evaluate the attitudes and beliefs of adolescents and their parents.</p><p><strong>Results: </strong>The mean BPA score of 409 adolescents (aged 13-19 years) was 2.56 ± 0.58, with 41 (10%) classified as low, 321 (78.5%) as medium, and 47 (11.5%) as high. Given that the overall aggression score was at a moderate level, subscale scores were not examined. The BPA scores were significantly associated with adolescents' ATV and BIV scores, as well as mothers' ATV scores. Correlation analysis revealed significant positive relationships among all scales, except between BPA and the BIV scores of both mothers and fathers.</p><p><strong>Conclusion: </strong>The findings suggest that the attitudes and beliefs regarding violence held by adolescents and their parents significantly influence the development of aggressive behaviors in adolescents within their immediate social environment.</p>","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"61 4","pages":"298-303"},"PeriodicalIF":1.7,"publicationDate":"2026-03-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147791657","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":"Fat-Soluble Vitamins and Adiposity: Commentary on \"Effect of Excess Weight on 25-Hydroxyvitamin D Levels\".","authors":"Emre Özer","doi":"10.65717/TurkArchPediatr.2026.25532","DOIUrl":"https://doi.org/10.65717/TurkArchPediatr.2026.25532","url":null,"abstract":"","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"61 4","pages":"366-367"},"PeriodicalIF":1.7,"publicationDate":"2026-03-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147791573","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":"Success Rate of Discontinuation of High-Flow Nasal Cannula at 4 LPM Compared to 2 LPM in Late Preterm and Term Neonates with Respiratory Distress.","authors":"Nattakarn Sookhanaphibarn, Sommon Jindakul, Meera Khorana","doi":"10.65717/TurkArchPediatr.2026.250226","DOIUrl":"https://doi.org/10.65717/TurkArchPediatr.2026.250226","url":null,"abstract":"<p><strong>Objective: </strong>To compare the success rate of discontinuation of high-flow nasal cannula (HFNC) at a predefined flow of 4 liters per minute (LPMs) versus a predefined flow of 2 LPMs.</p><p><strong>Materials and methods: </strong>This prospective randomized clinical trial was conducted in the neonatal units at Queen Sirikit National Institute of Child Health from October 2021 to March 2022. Infants who were receiving HFNC were randomly assigned to discontinuation of HFNC either at predefined flow 4 LPMs or 2 LPMs. The primary outcome was successful discontinuation of HFNC for 48 hours.</p><p><strong>Results: </strong>A total of 79 infants were enrolled in this study, of which 9 were excluded for a final analysis of 70 infants, 35 in each group. The success rate of discontinuation of HFNC was similar in both therapy groups (91.43% in the 4 LPMs group compared with 94.29% in the 2 LPMs group, P = .64). However, the predefined 4 LPMs group had a significantly shorter median HFNC duration (35 hours; interquartile range [IQR]: 23-85) compared with the 2 LPMs group (51 hours; IQR: 36-97) (P=.028).</p><p><strong>Conclusion: </strong>The successful discontinuation of HFNC at predefined flow 4 LPMs was similar to discontinuing at predefined flow 2 LPMs. Moreover, the median duration on HFNC was shorter in the 4 LPMs group. There is currently no evidence available to suggest the best strategy for weaning and discontinuation of HFNC.</p>","PeriodicalId":75267,"journal":{"name":"Turkish archives of pediatrics","volume":"61 4","pages":"320-325"},"PeriodicalIF":1.7,"publicationDate":"2026-03-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147791782","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}