The Turkish journal of pediatrics最新文献

{"title":"Genotype-phenotype divergence in pediatric familial Mediterranean fever across two closely related populations: a Türkiye-Azerbaijan comparison.","authors":"Vafa Guliyeva, Selen Duygu Arık, Pınar Prencuva Akyürek, Bengisu Menentoğlu, Govhar Verdiyeva, Leyla Sahratova, Fatma Gül Demirkan, Özlem Akgün, Nuray Aktay Ayaz","doi":"10.24953/turkjpediatr.2026.8444","DOIUrl":"10.24953/turkjpediatr.2026.8444","url":null,"abstract":"<p><strong>Background: </strong>Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease and has a high prevalence in Türkiye. Despite shared ethnic ties, pediatric FMF in Azerbaijan remains poorly characterized. This study aimed to systematically compare the clinical, laboratory, and genetic characteristics of pediatric FMF patients from Türkiye and Azerbaijan to determine whether common ancestral origins result in uniform disease expression.</p><p><strong>Methods: </strong>This retrospective bicentric study enrolled pediatric FMF patients aged 0-18 years who met the Eurofever/PRINTO criteria and had ≥12 months of follow-up at specialized centers in Türkiye and Azerbaijan. Demographics, clinical features, laboratory findings, Mediterranean FeVer (MEFV) genotypes, severity scores, and treatment data were compared.</p><p><strong>Results: </strong>The cohort comprised 549 patients: 477 (86.9%) Turkish and 72 (13.1%) Azerbaijani. Azerbaijani patients had earlier symptom onset (30 [IQR 16-67] vs. 48 [IQR: 24-72] months, p=0.044) but a longer diagnostic delay (14 [IQR: 11.4-48.7] vs. 12 [IQR: 6-23] months, p<0.001). Turkish patients showed higher rates of chest pain (22.6% vs. 8.3%, p=0.005), arthralgia (52.6% vs. 32 %, p= 0.001), annual attack frequency (12 [IQR: 6-12] vs. 6 [IQR: 4-8], p<0.001), International Severity Score for Familial Mediterranean Fever (ISSF) scores (2 [IQR: 1-3] vs. 2 [IQR: 1-2], p=0.002), and C-reactive protein (CRP) levels (56 [IQR: 34-96] vs. 17.9 [IQR: 3.2-35.6] mg/L, p<0.001). Confirmatory exon 10 genotypes predominated in Turkish patients (66.7%) whereas unconfirmatory genotypes in Azerbaijani patients (59.7%). M694V was the most common variant in both groups. Within exon 10 homozygotes, Turkish patients had higher ISSF scores (median 3 [IQR: 2-4] vs. 2 [IQR: 1-3], p=0.037) and more arthritis (39% vs 6.3%, p=0.032). Colchicine response was comparable (90.6% vs. 93.1%), but anti-IL-1 use was higher in Turkish patients (11.5% vs. 2.9%, p=0.028).</p><p><strong>Conclusion: </strong>This first systematic Türkiye-Azerbaijan comparison reveals distinct genotype-phenotype correlations in pediatric FMF, with Turkish patients showing a higher burden of confirmatory exon 10 genotypes and more severe disease, highlighting the need for center- and population-aware management strategies.</p>","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"68 4","pages":"627-638"},"PeriodicalIF":0.0,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149032513","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":"Psychosocial outcomes and related factors in adolescents using clean intermittent catheterization.","authors":"Tuğba Menteşe Babayiğit, Gökçe Yağmur Efendi, Muammer Babayiğit, Merve Çıkılı Uytun, Aykut Akıncı, Özhan Yalçın, Berk Burgu, Birim Günay Kılıç","doi":"10.24953/turkjpediatr.2026.7636","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2026.7636","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;Clean intermittent catheterization (CIC) is an established treatment for lower urinary tract dysfunction and has been used across age groups with voiding problems due to various etiologies. Although medically safe and straightforward, CIC may create social and emotional challenges for adolescents and their caregivers. During adolescence, when autonomy and peer acceptance become central, the need for continuous catheterization in daily life and, at times, dependence on another person can be difficult to cope with and may affect psychological well-being and peer relations. This study aimed to examine factors associated with potential psychopathology, self-perception, peer relationship characteristics, and quality of life in adolescents performing CIC.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;This prospective cross-sectional study included 50 adolescents aged 12-18 years: 25 performing CIC and 25 age and sex-matched healthy controls. Participants with intellectual disability, low mental capacity preventing comprehension of study procedures, or uncorrected vision/hearing deficits were excluded. Psychiatric diagnoses were assessed using the Schedule for Affective Disorders and Schizophrenia for School-Aged Children-Present and Lifetime Version DSM-5 (K-SADS-PL). Self-perception, quality of life, behavioral difficulties, and peer relationships were evaluated using the Piers-Harris Children's Self-Concept Scale, Pediatric Quality of Life Inventory, Strengths and Difficulties Questionnaire, and Peer Relationship Scale. Parental attitudes and caregivers' psychological symptoms were assessed using the Parental Attitude Research Instrument and the General Health Questionnaire-28. Statistical analyses were performed using SPSS version 23.0. Group comparisons were conducted using the independent samples t-test or Mann-Whitney U test as appropriate; categorical variables were analyzed with the Chi-square or Fisher's exact test. Multiple linear regression analysis was performed to identify independent predictors. A two-tailed p value &lt;0.05 was considered statistically significant.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;The groups were similar in age, sex and sociodemographic characteristics. Adolescents performing CIC showed higher social avoidance and poorer peer relationships. Eight adolescents (32%) in the CIC group met criteria for at least one psychiatric disorder according to DSM-5. Negative parental attitudes, maternal mental disorders, primary etiology requiring CIC, age at self-catheterization initiation, and catheterization performed by another individual were identified as significant predictors of lower self-esteem, reduced quality of life, and poorer peer relationships.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusion: &lt;/strong&gt;CIC may have meaningful psychological and psychosocial effects on adolescents, influenced by clinical variables and parental factors. Early identification of risk indicators and strengthening of protective factors are essential. A m","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"68 4","pages":"603-618"},"PeriodicalIF":0.0,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149032525","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":"Mortality characteristics in individuals with autism spectrum disorder across the lifespan: a narrative review.","authors":"Buket Kılıç, Dilek Ünal, Cihan Aslan, Kevser Nalbant","doi":"10.24953/turkjpediatr.2026.7909","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2026.7909","url":null,"abstract":"<p><p>This narrative review provides a comprehensive synthesis of mortality rates and associated risk factors among individuals with autism spectrum disorder (ASD) across the lifespan. Following a search across PubMed, Scopus, and Google Scholar, 31 peer-reviewed studies were identified for synthesis. The findings consistently demonstrate that individuals with ASD experience significantly higher mortality rates compared to neurotypical controls. Analysis shows a distinct developmental shift: in childhood and adolescence, external causes are predominant, with accidents-particularly drowning-accounting for a substantial portion of fatalities, estimated between 70% and 90% in some reports. In contrast, adult mortality is increasingly driven by natural causes. The evidence indicates a reduced life expectancy, with some studies reporting average ages of death as low as 36 years, depending on comorbid conditions. Furthermore, standardized mortality ratios appear notably higher in individuals with co-occurring intellectual disabilities and epilepsy, with some cohorts showing risks several times higher than the general population. A substantial proportion of ASD-related mortality is potentially preventable through targeted safety training, early metabolic screening, and ASD-friendly healthcare practices. Addressing communication barriers and sensory sensitivities in clinical settings is essential for early diagnosis and effective management of chronic conditions to reduce the mortality gap.</p>","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"68 4","pages":"555-565"},"PeriodicalIF":0.0,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149032509","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":"Spontaneous renal pelvis perforation in an adolescent with a solitary kidney.","authors":"Dilara Beşli Çelik, Songül Yılmaz, Berk Burgu, Ömer Suat Fitöz, Seda Kaynak Şahap, Zeynep Birsin Özçakar","doi":"10.24953/turkjpediatr.2026.7209","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2026.7209","url":null,"abstract":"<p><strong>Background: </strong>Spontaneous renal pelvis perforation is a rare but serious condition, often associated with urinary obstruction, infection, or increased intrapelvic pressure. Here, we present a pediatric patient with a solitary kidney who developed this rare condition.</p><p><strong>Case presentation: </strong>We report the case of a 14-year-old girl with a solitary kidney who presented with abdominal pain, vomiting, and anuria. Laboratory findings revealed acute kidney injury, requiring emergent hemodialysis. Imaging studies demonstrated a spontaneous perforation in the anteromedial renal pelvis. The patient was initially managed with antibiotics and a double-J (DJ) ureteral stent. However, persistent fever and worsening retroperitoneal fluid collection necessitated percutaneous abscess drainage. Despite initial clinical improvement, she later developed recurrent urine leakage due to DJ stent occlusion, which required percutaneous nephrostomy placement. At the three-month follow-up, spontaneous resolution of the perforation was confirmed, and the patient was diagnosed with stage 4 chronic kidney disease.</p><p><strong>Conclusions: </strong>Spontaneous renal pelvis perforation remains a rare entity in the pediatric population, with limited cases documented in the literature. This case emphasizes the importance of early recognition, timely imaging, and appropriate urinary drainage to prevent long-term renal impairment. In pediatric patients with a solitary kidney, close monitoring and a multidisciplinary approach are crucial to optimizing outcomes.</p>","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"68 4","pages":"710-714"},"PeriodicalIF":0.0,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149032203","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":"The face of pediatric and adult brain abscesses, differences and clinical characteristics: a single center 10-year experience.","authors":"Tamer Çelik, Merve Kılıç Çil, Ümit Çelik, Mehmet Can, Ali İhsan Ökten","doi":"10.24953/turkjpediatr.2026.6250","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2026.6250","url":null,"abstract":"<p><strong>Background: </strong>Brain abscesses (BA) are a medical emergency in all age groups and require early diagnosis and treatment. Childhood BA constitute 25% of all abscesses and may have a different clinical course than adults. This article aims to investigate patients followed up for adult and child BA over a 10-year period.</p><p><strong>Materials and methods: </strong>A retrospective analysis was performed on adult and pediatric patients diagnosed with BA. Demographic, clinical, predisposing factors, imaging, treatment modalities, and prognosis of the patients, and clinical differences were examined.</p><p><strong>Results: </strong>Of a total of 46 patients; 54.3% of the patients were children and the mean age was 10.9±6.3 years for the pediatric group and 46.2±16.7 years for the adult group. Fever, vomiting, and seizures were significantly higher in children (p<0.05). When the differences between the groups were examined, headache was the most common finding in the adult group, while the most common finding in children was fever and vomiting. The classic triad of fever, headache, and focal neurological deficits was present in 12 (48%) of the children, compared to only 4 (19.1%) of the adults. Trauma history (21.7%), immunosuppression (19.5%), and local spread (mastoiditis, sinusitis, and otitis) (32.6%) were among the most common predisposing factors. Previous otorhinolaryngogenic infections were the most common cause in children (36%), while immunosuppression was the most common cause in adults (28.5%). The frontal lobe was most frequently involved among the patients (47.8%). BA was more commonly localized in the left hemisphere in both groups. Culture growth was detected in 23.9% of all operated patients, with a positivity rate of 20% in children and 28% in adults. The most common microorganism was Staphylococcus aureus (8.6%). The average hospitalization time was significantly longer in children than in adults (46 ± 18 days vs 34 ± 13 days; p=0.024). 26.1% of the patients recovered with sequelae, and the mortality rate was 8.6%.</p><p><strong>Conclusion: </strong>Our findings suggest that a high index of suspicion should be maintained for BA in children, especially in the presence of fever, vomiting, and seizures. This study highlights significant clinical and etiological differences between pediatric and adult BA, underscoring the importance of age-specific diagnostic and management strategies. The differences observed in clinical presentation and underlying causes between the two groups warrant further investigation to improve outcomes.</p>","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"68 4","pages":"594-602"},"PeriodicalIF":0.0,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149032435","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":"Phenotypic characteristics and clinical management of Alagille syndrome with multiple intracranial aneurysms: a case report and literature review.","authors":"Yu Wang, Yangyang Sun, Zhenxing Yang, Jinlong Chen, Ding Wan, Dejun Huang","doi":"10.24953/turkjpediatr.2026.7715","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2026.7715","url":null,"abstract":"<p><strong>Background: </strong>Alagille syndrome (ALGS) is an autosomal dominant multisystem developmental disorder associated with JAG1 and NOTCH2 mutations. Although vascular involvement is recognized, life-threatening intracranial hemorrhage as the initial presentation is extremely rare.</p><p><strong>Case presentation: </strong>A 5-year-old male presented with a severe headache and was diagnosed with intracerebral and subarachnoid hemorrhage caused by multiple ruptured intracranial aneurysms. Genetic testing confirmed a pathogenic JAG1 mutation, establishing the diagnosis of ALGS. The patient, who had known aortic stenosis, developed infective endocarditis-raising the possibility that infection was driving the aneurysms. After two microsurgical craniotomies, he ultimately died of multi-organ failure.</p><p><strong>Conclusions: </strong>This case highlights a rare presentation of ALGS involving acute rupture of multiple intracranial aneurysms, complicated by cardiac malformation and infective endocarditis, with newly formed aneurysms highly suspected to be infection-driven. ALGS should be considered in young children presenting with early intracranial hemorrhage, particularly when cardiac anomalies coexist. Early multidisciplinary intervention may prevent the catastrophic sequelae of ALGS-associated intracranial aneurysms.</p>","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"68 4","pages":"680-688"},"PeriodicalIF":0.0,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149032456","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":"Frequency of gastroptosis and gastroparesis in children with treatment-resistant dyspepsia and duodenogastric reflux: a prospective study.","authors":"Melike Arslan, Edibe Gözde Başaran, Coşkun Fırat Özkeçeci, Bekir Nihat Doğrul, Necati Balamtekin","doi":"10.24953/turkjpediatr.2026.7467","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2026.7467","url":null,"abstract":"<p><strong>Background: </strong>Studies on gastroptosis and gastroparesis in children are limited. This prospective study aimed to evaluate the frequency of gastroptosis and gastroparesis and their association with duodenogastric reflux (DGR) and treatment-resistant dyspeptic symptoms in children.</p><p><strong>Methods: </strong>The study evaluated pediatric patients aged 2-18 years with dyspepsia who underwent upper gastrointestinal endoscopy between March 2020 and June 2022. Fluoroscopy and gastric emptying scintigraphy were performed in 88 patients (50 with DGR and 38 with treatment-resistant dyspepsia).</p><p><strong>Results: </strong>The mean age of the patients was 13.7±3.6 years, and 62 (70.5%) were girls. Gastroptosis was detected in 22 (25%) and gastroparesis in 24 (27%) of all 88 patients. Gastroptosis was present in 10 (20%) and gastroparesis in 20 (40%) of the 50 patients with DGR. Gastroptosis was present in 12 (32%) and gastroparesis in 4 (11%) of the 38 patients with treatment-resistant dyspepsia. Gastroparesis was more common in those with DGR (40%) than in those with treatment-resistant dyspepsia (11%) (p=0.09). Gastroptosis was also detected in 14 (58.3%) patients with gastroparesis. The median body mass index (BMI) z-score of those with gastroptosis was significantly lower than those without gastroptosis (p=0.002).</p><p><strong>Conclusions: </strong>Gastroptosis and gastroparesis were frequently identified in children with treatment-resistant dyspeptic symptoms or DGR. These conditions should be considered and investigated in this patient population.</p>","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"68 4","pages":"672-679"},"PeriodicalIF":0.0,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149032487","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":"Hirschsprung's disease and a rare mutation of the <i>PIGO</i> gene: Mabry syndrome.","authors":"Tugay Tartar, Serkan Kırık, Muhammet Çalık","doi":"10.24953/turkjpediatr.2026.7661","DOIUrl":"10.24953/turkjpediatr.2026.7661","url":null,"abstract":"<p><strong>Background: </strong>Mabry syndrome is a very rare condition characterized by cognitive impairment, distinctive facial features, hypotonia, elevated alkaline phosphatase (ALP) levels in the blood (hyperphosphatasia), and recurrent generalized tonic-clonic seizures. It can be accompanied by gastrointestinal anomalies such as Hirschsprung's disease (HD). In this study, we aim to present a case of hyperphosphatasia with impaired intellectual development syndrome 2 (HPMRS2) from Türkiye, featuring a novel homozygous p.Arg445Pro variant in the PIGO gene, occurring in association with HD.</p><p><strong>Case presentation: </strong>A two-year-old male patient was referred to the pediatric neurology clinic with decreased responsiveness to the environment, lack of trunk control, and failure to respond to his name. ALP value was 613 IU/L. No pathology was detected in metabolic tests, vitamin D levels, or cranial magnetic resonance imaging, and electroencephalography (EEG). At 11 months of age, he had a generalized tonic-clonic seizure without fever. Since no abnormalities were detected in chromosome analysis and array analysis, whole exome sequencing (WES) was performed. A homozygous mutation was detected in the PIGO gene (NM_032634.4): c.1334G>C (p.Arg445Pro). Sanger analysis revealed heterozygous carrier status in both parents. The patient had constipation that was unresponsive to medical treatment since birth. Physical examination revealed abdominal distension. Rectal examination revealed an explosive discharge of gas and stool. A rectal biopsy was performed. The pathology result was ganglion-negative, and the patient was diagnosed with HD. After definitive surgery, spontaneous defecation has been achieved during follow-up, except for rare episodes of enterocolitis.</p><p><strong>Conclusions: </strong>It should be remembered that Mabry syndrome can be accompanied by gastrointestinal anomalies, primarily HD. This case highlights the increased phenotypic variability associated with PIGO mutations and demonstrates the importance of genetic assessment in atypical neurodevelopmental conditions.</p>","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"68 4","pages":"689-694"},"PeriodicalIF":0.0,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149032548","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":"When non-specific effects matter: methodological considerations in a randomized controlled trial of abdominal massage for pediatric functional constipation.","authors":"Fei-Yi Zhao, Li-Ping Yue, Qiang-Qiang Fu, Jia-Yi Zhu","doi":"10.24953/turkjpediatr.2026.8361","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2026.8361","url":null,"abstract":"","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"68 4","pages":"715-716"},"PeriodicalIF":0.0,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149032364","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":"Long-term outcomes and prognostic factors in pediatric Wilms tumor: a 47-year single-center experience.","authors":"Oğuz Salih Dinçer, Alper Uygun, Ayhan Dağdemir, Merve Ecem Öğretici Çolak, İbrahim Kartal","doi":"10.24953/turkjpediatr.2026.7072","DOIUrl":"https://doi.org/10.24953/turkjpediatr.2026.7072","url":null,"abstract":"<p><strong>Introduction: </strong>Wilms tumor is the most common renal malignancy in children. Survival rates now exceed 90% with multimodal therapy. This study aims to retrospectively analyze 104 pediatric Wilms tumor patients diagnosed over a 47-year period at our institution, examining demographic characteristics, tumor staging, treatment modalities, complications, recurrence rates, and survival outcomes.</p><p><strong>Materials and methods: </strong>This retrospective single-center study included 104 pediatric patients diagnosed with Wilms tumor between January 1, 1978, and January 31, 2025. Patients were stratified into early (1978-2005) and late (2006-2025) periods. Survival analysis was performed using the Kaplan-Meier method and multivariable Cox proportional hazards regression.</p><p><strong>Results: </strong>The 5-year overall survival (OS) and event-free survival rates were 61.1% and 57.3% in the early era, 86.8% and 81.5% in the late era, and 73.6% and 65.8% in the overall cohort. OS was significantly lower in patients with anaplasia (p=0.035), advanced-stage disease (p=0.004), and in those treated in the early era (p=0.009). In multivariable analysis, treatment era and metastatic disease at diagnosis were independently associated with poorer survival, whereas anaplasia did not retain statistical significance after adjustment.</p><p><strong>Conclusion: </strong>Survival outcomes improved substantially over time. Metastatic disease at diagnosis and treatment era were the strongest independent predictors of survival, while advanced stage and anaplasia were associated with outcome in univariable analyses.</p>","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"68 4","pages":"649-661"},"PeriodicalIF":0.0,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149032551","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}