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Öğe Burnout, mental health symptoms, and empathy in healthcare workers who care for children treated in a liver transplant center(Wiley, 2023) Cansel, Neslihan; Varol, Fatma IlknurObjective The healthcare workers in pediatric liver transplantation units are exposed to various stresses; however, their burnout has not been clarified yet. This study aimed to determine burnout, psychological symptoms and empathy levels in healthcare workers, to examine the relationship between these variables and to investigate the predictors of burnout. Method This cross-sectional study was conducted on healthcare workers in Turgut ozal Medical Center Pediatric Liver Transplant Institute. The participants filled out a questionnaire that investigating demographic and occupational characteristics, and including questions from Toronto Empathy Questionnaire, Maslach Burnout Inventory, and Brief Symptom Inventory. Results Fifty healthcare workers participated in the study. Based on moderate and higher severity scores, 48% of the participants were emotionally exhausted, 22% of them were depersonalized. All of them had a high perception of personal accomplishment. There was a correlation between psychological symptoms level and emotional exhaustion and personal accomplishment as well as empathy and personal accomplishment. Not choosing the profession and the department willingly, age, education level, marital status, duration of profession, unit, not receiving in-service training, empathy, depressive symptoms and hostility scores predicted different aspects of burnout. Conclusion Considering that burnout and psychological stress lead to reduced professional abilities and the development of serious complications, the results obtained can guide managers on the measures to be taken.Öğe A case of acute generalised exanthematous pustulosis (AGEP) induced by amoxicillin-clavulanic acid(Bilimsel Tip Yayinevi, 2014) Senbaba, Elif; Catal, Ferhat; Topal, Erdem; Ermistekin, Halime; Varol, Fatma Ilknur; Akpolat, NusretAcute generalised exanthematous pustulosis (AGEP) is a rare skin reaction characterized by sterile, small pustules on the erythematous background. Drugs, especially antibiotics are major caustic factors in etiology. The diagnosis of disease is made by the scoring system which determined by European Severe Cutaneous Adverse Reactions (EuroSCAR)s' working group. The four-year-old patient applied with the complaint of numerous pustular skin eruptions on the erythematous background and had the history of amoxicillin-clavulanic acid treatment which was given two days ago. The biopsy samples were taken from pustular lesions. The diagnosis of AGEP was made according to EuroSCAR score, evaluating clinical feature and the histopatological examination of the biopsy sample. In this presentation, we aimed to emphasize that AGEP can rarely be seen in childhood age and to review the clinical properties, diagnosis and treatment of disease.Öğe Diagnostic delay in children with coeliac disease: a national multicentre study in Turkey(Bmc, 2026) Sahin, Yasin; Sevinc, Eylem; Varol, Fatma Ilknur; Akbulut, Ulas Emre; Bayrak, Nevzat Aykut; Bukulmez, AysegulObjectives There are few studies conducted investigating diagnostic delay in children with coeliac disease (CD). A better understanding of the factors associated with diagnostic delay and the development of strategies to reduce it are important in preventing long-term complications related to the disease. This study aimed to determine the median diagnostic delay in children with CD and to investigate possible underlying factors associated with diagnostic delay. Methods A prospective screening program was performed in children with CD at the seven clinical centers operating in Turkey within the Focus IN CD project between June 2021 and June 2023. Paediatric gastroenterologists were asked to complete a web-based questionnaire about CD. Results The median age of the 188 children with CD (66 male; 35%) was 8.33 years (IQR: 5.02-13.00). The median diagnostic delay was 7.30 months (IQR: 2.73-15.50). Among the 188 patients included in the study, 172 (91.5%) were diagnosed within the first three years, whereas 16 (8.5%) were diagnosed after three years, and 68.7% of these patients had classical CD symptoms. Patients with CD were categorised according to the OSLO criteria. The median delay for the classic CD group, the nonclassic CD group and the silent CD group were found to be 7.63 (IQR: 3.13-16.06), 4.40 (IQR: 1.76-13.20) and 2.21 (IQR: 1.03-16.14) months, respectively.There were no significant differences between the patients with the classic CD group, and the nonclassic CD group, and the silent CD group (p = 0.564). Conclusions The study revealed that the median diagnostic delay was 7.30 months. Furthermore there were no significant differences between clinical subtypes of CD. In order to minimize the delay in diagnosis, it may be a solution to raise community-based awareness of CD, and better inform pediatricians, and family physicians about CD.Öğe Diagnostic Scoring in Biliary Atresia(Aves, 2025) Gungor, Sukru; Varol, Fatma Ilknur; Altundas, Ebubekir; Gok, Emre; Yildiz, Turan; Demiroz, SevgiBackground/Aims: The aim of this study was to develop a diagnostic scoring model to predict the need for intraoperative cholangiography in patients with neonatal cholestasis suspected of having biliary atresia (BA) and to aid in the early diagnosis of BA. Materials and Methods: Data from 70 patients with neonatal cholestasis who underwent intraoperative cholangiography with a preliminary diagnosis of BA between 2019 and 2024 were retrospectively reviewed. Data from patients with and without BA were compared. Thescoring was based on 3 parameters: acholic stool observed clinically on inspection, findings suggestive of BA on ultrasound, and elevated gamma-glutamyl transferase (GGT) levels. The best GGT cut-off point for the diagnosis of BA was determined by receiver operating characteristic analysis. The diagnostic success of the scoring model for BA was statistically evaluated. Results: There were no significant differences in age and gender between BA and non-BA groups. Gamma-glutamyl transferase levels were elevated in all patients. Acholic stools were present in 98% of BA patients. Ultrasound findings suggestive of BA were present in 88.5% of patients with BA. The authors found the best GGT cut-off value for the diagnosis of BA to be >= 366 (73% sensitivity, 77.8% specificity). In the scoring model the authors developed, the presence of 2 parameters provided diagnostic success with high sensitivity (98%) and specificity (83.3%). Conclusion: The study provides a reliable and sensitive diagnostic criterion to determine the need for intraoperative cholangiography in infants with neonatal cholestasis. These data should be validated in larger prospective case series.Öğe Diagnostic value of “t sign” on MRCP-MIP imaging in the evaluation of pancreas divisum(2019) Kivilcim, Meltem; Gumus Dogan, Derya; Kortay Canaloglu, Sinem; Varol, Fatma Ilknur; Selimoglu, Mukadder AyseAim: Feeding difficulties are common in typically developing children and the prevalence ranges from 25% to 45%. Although using an assessment tool is the best approach when planning an intervention, assessment of feeding disorders is mostly accomplished by informal parent interviews in clinical practice. This study was designed to assess the mealtime behaviours of young children with ‘Feeding Difficulties’ (FD) by an instrument and to compare the results with ‘Typically Developing’ (TD) children.Material and Methods: A total of 61 children with FDs and 63 TD children aged 6-42 months were included. The Behavioral Pediatric Feeding Assessment Scale (BPFAS) was completed to describe the child’s feeding behaviors and the parents’ mealtime strategies. Results:FD group had higher scores than TD group in all BPFAS subtests’ scores. The mean ‘Total Frequency Score’ was 104.6 in FD group whereas 72.9 in TD group (p=0.0001). The FD group had more problematic feeding behaviours in comparison to TD group. The mean ‘Total Problem Score’ of TD group was 6.1 whereas 20.1 in children with FD (p=0.0001).Conclusion: This study supports the BPFAS to be a useful and practical feeding assessment tool and also has the advantage of incorporating parents’ feelings about child’s feeding behaviors.Öğe The effect of anxiety and depression levels of children with celiac disease on quality of life(Elsevier Science Inc, 2023) Belpinar, Ayse; Dag, Yeliz Suna; Sulun, Ayse Aricioglu; Yayan, Emriye Hilal; Varol, Fatma IlknurPurpose: This study was conducted to examine the effect of anxiety and depression levels on quality of life in children with celiac disease.Design and method: This descriptive and correlational study was conducted with 98 children diagnosed with celiac disease who applied to the pediatric outpatient clinic of a university hospital in Eastern Anatolia between September 2021 and August 2022. Data were collected using the State-Trait Anxiety Scale, Depression Scale, and Quality of Life Scale through face-to-face interviews conducted by the researchers. Percentage distribution, mean, independent samples t-test, Pearson correlation analysis and regression analysis were used to analyze the data.Results: It was found that 62% of the participant children were female and their mean age was 11.69 +/- 4.15 years. The mean scores of state anxiety, trait anxiety, depression and quality of life of children with celiac disease were 42.46 +/- 5.42 (high), 43.83 +/- 7.08 (high), 23.37 +/- 4.79 (high) and 43.67 +/- 19.67 (low), respectively. Regression analysis revealed that anxiety had a statistically significant relationship with quality of life.Conclusions: It was found that children with celiac disease experienced high levels of depression and anxiety along with physical functionality and psychosocial health problems and this negatively affected their quality of life. It is recommended that children with celiac disease should be followed up and supported psychosocially.Practice implications: That healthcare professionals can contribute to reducing the depression and axienty and improving the quality of life by strengthening the social support systems of childrens with celiac disease.Öğe Effect of Malnutrition on Femoral Cartilage Thickness in Pediatric Patients(Mdpi, 2025) Gungor, Sukru; Buyukavci, Raikan; Varol, Fatma Ilknur; Gok, Emre; Akturk, SemraBackground/Objectives: Malnutrition is an imbalance of nutrients required for growth, development, and organ function. Its impact on bone development is known, but its effects on cartilage remain unclear. This study aimed to evaluate the femoral cartilage thickness in children with primary malnutrition. Methods: In this cross-sectional observational study, 83 children with primary malnutrition and 62 age- and sex-matched healthy controls were included. Patients with primary malnutrition were classified as mild, moderate and severe. Femoral cartilage thickness measurements of all children were taken by ultrasound from the femoral lateral condyle, femoral medial condyle and intercondylar area for both knees with the patient in a supine position with the knees flexed 90 degrees. Results: The right lateral, right medial, left lateral, and left medial femoral cartilages were significantly thicker in patients with malnutrition compared to those without malnutrition (p = 0.002, 0.004, <0.001, and 0.001, respectively). A significant negative correlation was found between age, weight Z-score, and height Z-score and triceps skinfold thickness. Conclusions: Distal femoral cartilage thickness is significantly greater in children with primary malnutrition. This demonstrates the effect of nutritional factors on cartilage tissue and suggests that children with chronic malnutrition are at risk for both knee joint problems and short stature later in life.Öğe The effects of iron and zinc status on prognosis in pediatric Wilson's disease(Elsevier Gmbh, 2019) Gungor, Sukru; Selimoglu, Mukadder Ayse; Varol, Fatma Ilknur; Gungor, Serdal; Uremis, Muhammed MehdiObjectives: Wilson's disease (WD) is a metabolic disorder leading to hepatic and extrahepatic copper deposition. Several studies have reported that besides copper (Cu), iron (Fe) and zinc (Zn) are also accumulated at varying levels in various tissues in WD. However, there is not an adequate number of studies investigating the effects of Fe and Zn status on WD presentation and prognosis. We aimed to evaluate serum levels of ferritin (SFr), copper (SCu), and zinc (SZn) in WD and determine their role in disease presentation and prognosis. Materials-Method: We retrospectively reviewed the medical records of 97 pediatric patients with WD who were diagnosed and followed at Inonii University Pediatric Gastroenterology, Hepatology and Nutrition Department between January 2006 and May 2017. Serum Cu and Zn levels were analyzed by using flame atomic absorption spectrophotometer. Ferritin was analyzed by chemiluminescence immunoassay method. Results: Analysis of serum levels of the elements according to the type of presentation, there was no significant difference between the groups for ceruloplasmin. However, SCU, FSCu, SFr and 24 h urinary copper levels were significantly higher (p = 0.002, p = 0.003, p = 0.023 and p < 0.001, respectively) and SZn and CSZn levels were significantly lower (fulminant WD). p < 0.001, p < 0.001). There was a positive correlation between SFr, SCu serum levels and mortality scores (respectively, r: 0.501, 0.564 for PELD, r:0.490, 0.504 for MELD, r: 0.345, 0.374 for Dhwan), and a negative correlation between SZn level and mortality scores. (r:-0.650 for PELD, r:0.703 for MELD, r:-0.642 for Dhwan) We used the ROC curves to determine the worst prognosis for fulminant Wilson disease. According to these limit values, we found that the sensitivity and specificity of FWD development was significantly higher. (for SZn sensitivity of 91.5%, a specificity of 100%, p= < 0,001, for SCu predicted FWD development with a sensitivity of 100%, a specificity of 73.7%, p = < 0,001, for SFr predicted FWD development with a sensitivity of 92.9%, a specificity of 66.2%, p < 0,001) Conclusion: Our study suggests that SFr, SCu, SZn levels might have prognostic importance for WD.Öğe Evaluation of the Relationship Between Neurologic Manifestations and Genetic Mutations in Wilson's Disease with Next-Generation Sequencing(Mdpi, 2025) Akbulut, Sami; Is, Seyma; Koprulu, Tugba Kul; Varol, Fatma Ilknur; Kucukakcali, Zeynep; Colak, Cemil; Koc, AhmetBackground: Wilson's disease (WD) is a rare autosomal recessive disorder caused by mutations in the ATP7B gene, leading to copper accumulation in the liver and brain. Given the clinical heterogeneity of the disease, this study aimed to characterize the mutational spectrum of ATP7B and explore genotype-phenotype correlations in Turkish patients. Methods: Whole-exome sequencing (WES) was performed in 17 Turkish patients clinically diagnosed with WD. Variants were annotated and evaluated using five in silico prediction tools (REVEL, CADD, PolyPhen, SIFT, MutationTaster). Copy number variation (CNV) analysis was conducted using the CLC Genomics Server (Version 22.0.2). Results: A total of 14 distinct ATP7B variants were identified, comprising 12 missense, 1 nonsense, and 1 frameshift mutation. Variant distribution showed some phenotype-specific patterns: four variants were found more frequently in hepatic cases and three in neurological cases, although no statistically significant or consistent correlation between genotype and clinical presentation could be established. The most frequent mutation was p.His1069Gln, present in both phenotypes. All missense variants were predicted to be pathogenic by at least three computational tools, with high concordance among platforms. No pathogenic CNVs were detected. Conclusions: This study expands the mutational landscape of ATP7B in Turkish patients with WD and supports the utility of WES combined with in silico tools for accurate variant classification. The results emphasize the genetic heterogeneity of WD and suggest possible associations between certain mutations and clinical phenotypes.Öğe Expanding the genotypic spectrum of 3β-hydroxy-δ5-C27-steroid dehydrogenase (HSD3B7) deficiency: novel mutations and clinical outcomes(Walter De Gruyter Gmbh, 2025) Celik, Merve Yoldas; Koseci, Burcu; Burgac, Ezgi; Garip, Sevinc; Varol, Fatma Ilknur; Gungor, Suekrue; Taskin, Didem GulcuObjectives: HSD3B7 deficiency is a genetic disorder caused by mutations in the HSD3B7 gene, leading to impaired bile acid synthesis and the accumulation of toxic intermediates. Affected patients typically present with cholestatic liver disease, including jaundice and progressive liver dysfunction. Case presentation: This case series describes three pediatric patients from two families diagnosed with HSD3B7 deficiency, each demonstrating varying clinical severity and outcomes. All cases exhibited cholestasis with normal GGT levels and elevated AST/ALT. Case 1, a male infant, also presented with craniosynostosis and failure to thrive, responding well to cholic acid therapy. Case 2, a female infant and first cousin of Case 1, had mild cardiac abnormalities and showed slight improvement with ursodeoxycholic acid and vitamin supplementation. Case 3, a male infant with a compound HSD3B7 and ATP8B1 mutation, progressed to fulminant liver failure, ultimately requiring a liver transplant. A novel c.531 + 1G>C variant was identified in Cases 1 and 2, contributing to understanding genotype-phenotype correlations in bile acid synthesis disorders. Conclusions: Early diagnosis and treatment with bile acid therapy are crucial for improving outcomes, although some cases may necessitate liver transplantation. This series emphasizes the need to consider bile acid synthesis disorders in the differential diagnosis of cholestasis.Öğe Factors Associated with Low Bone Mineral Density at the Time of Diagnosis in Children with Celiac Disease(Galenos Publ House, 2023) camtosun, Emine; Varol, Fatma Ilknur; Gungor, Suekrue; Selimoglu, Mukadder AyseObjective: It has been reported that bone mineral density (BMD) is decreased in children with Celiac disease (CD) compared to their healthy peers. The aim of this study was to reveal possible risk factors for low BMD in Turkish children newly diagnosed with CD. Methods: Eighty-six patients (2-18 years old) with CD were included in this retrospective study. The relationship between their lumbar BMD z-scores calculated according to their chronological age (CA) and height age (HA) and their clinical, laboratory [biochemical parameters, tissue transglutaminase antibody-IgA (TTGA) levels, human leukocyte antigen (HLA) types] and histopathological parameters were evaluated.Results: The mean age of the patients at diagnosis was 8.06 +/- 4.08 years. The BMD z-score CA was <=-2 standard deviation (SD) in 26.7% of the patients. The BMD z-score HA was <=-2 SD in 12.8% of the patients. The BMD z-score HA only correlated with their age at diagnosis of CD (rs value 0.269). However, there was no statistically difference between the BMD z-score HA >-2 SD and <=-2 SD subgroups regarding their clinical, laboratory and histopathological parameters. Conclusion: Low BMD is common in children with newly diagnosed CD. Age at diagnosis, gender, body size, Celiac symptoms, biochemical parameters, TTGA level, HLA type, and histopathological stage had no predictive values in terms of low BMD in this patient group.Öğe Infection risk after paediatric liver transplantation(Turkish J Pediatrics, 2020) Selimoglu, Mukadder Ayse; Kaya, Samime; Gungor, Sukru; Varol, Fatma Ilknur; Bag, Harika Gozde Gozukara; Yilmaz, SezaiInfections after liver transplantation (LT), despite prophylactic therapy, are still important causes of morbidity and mortality in children. Although underlying disease and immunosuppression along with the complexity of LT procedure are the major predispositions to infections, there still might be under recognised factors predisposing infections in paediatric LT. In this study, we retrospectively analysed the risk factors of bacterial, viral, and fungal infections after LT in a series of 167 children (median =5 yr.). Of all children, 112 (67%) experienced infections: 93 (55.7%) bacterial, 56 (33.5%) viral and 15 (9%) fungal. Multilogistic regression analysis showed that the need of immunosuppressive switch increased total, bacterial, and viral infection risk 5.3, 2.5, and 2.5 times, respectively, (p=0.001, p=0.021, and p=0,019, respectively). Re-LT increased bacterial infection risk 4.2 times (p=0.040). Viral infection risk was 10 times higher in children who had more than two re-laparotomies (p=0,002). Children who had post-LT, cytomegalovirus (CMV) infection had 5.6 times increased risk for fungal infection (p=0.035). In conclusion, infection is still an important morbidity in paediatric LT and is in close relationship with other morbidities such as surgical complications. CMV infection, itself, is an independent risk factor for fungal infection.Öğe An intrahepatic Portal Vein Aneurysm Presenting with Esophageal Variceal Bleeding in a Pediatric Patient: A Rare Clinical Entity(Galenos Publ House, 2018) Gungor, Sukru; Varol, Fatma Ilknur; Kutlu, Ramazan; Yilmaz, Sezai; Selimoglu, Mukadder Ayse[Abstract Not Available]Öğe Is There a Predictive Factor for an Association with Autoimmune Glandular Disease in Children Diagnosed with Celiac Disease?(Galenos Publ House, 2022) Varol, Fatma Ilknur; Camtosun, Emine; Selimoglu, Mukadder Ayse; Gungor, SukruObjective: A close relationship has been suggested between Celiac disease (CD) and glandular autoimmunity. The aim of this study was to determine the predictive factors for autoimmune glandular disease (AGD) in children with CD.Methods: The study included 228 pediatric patients, diagnosed with CD between 2010 and 2019. The cases with AGD (Group 1) and those without AGD (Group 2) and the patients with type 1 diabetes mellitus (T1DM) (Group A) and those without T1DM (Group B) were retrospectively reviewed and compared in terms of clinical and laboratory features.Results: AGD was detected in 8.8% (n=20) of the patients: T1DM in 13 (65%), T1DM and Hashimoto's thyroiditis (HT) in 3 (15%), HT only in 2 (10%), T1DM and Graves disease (GD) in 1 (5%), and GD only in 1(5%). The mean age at the diagnosis of CD was significantly higher in Group 1 (10.93 +/- 4.15 years) compared to Group 2 (8.10 +/- 4.19 years) (p<0.05) and also was significantly higher in Group A compared to Group B (p<0.05). Most of the diagnoses of AGD were made before the diagnosis of CD and age was an effective factor. There was no difference between Group 1 and Group 2 and Group A and Group B in terms of gender, typical/atypical CD ratio, tissue transglutaminase IgA (TTGA) level, human leucocyte antigen (HLA)-DQ2 and/or HLA-DQ8 positivity rate, and histopathological stage.Conclusion: Although patients with a diagnosis of co-existent CD and AGD were significantly older than patients with isolated CD, gender, celiac symptoms, TTGA level, HLA type, and histopathological stage had no predictive value for the coexistence of AGD in patients with CD.Öğe Isotretinoin Hepatotoxicity or Isotretinoin Induced Autoimmune Hepatitis?(Wolters Kluwer Medknow Publications, 2021) Varol, Fatma Ilknur; Selimoglu, Mukadder Ayse; Karadag, Nese; Gungor, SukruWhile isotretinoin, a drug used for the treatment of severe nodulocystic acne, is known to be hepatotoxic, an association with autoimmune hepatitis (AIH) has not been suggested so far. A 17-year-old girl diagnosed with AIH following isotretinoin use for acne vulgaris is presented, and the differences between isotretinoin hepatotoxicity and isotretinoin triggered AIH were discussed. To conclude this case, we want to underline that induction of an AIH by isotretinoin, even though so far unreported, is possible and thus the drug should be used with care, especially in patients with another autoimmune disease.Öğe Liver Transplantation for Hepatocellular Carcinoma in Patients with Inherited Metabolic Liver Diseases: A Single-Center Analysis(Aves, 2023) Garzali, Ibrahim Umar; Hargura, Abdirahman Sakulen; Ince, Volkan; Varol, Fatma Ilknur; Carr, Brian I.; Yilmaz, SezaiBackground/Aims: Liver transplantation is an acceptable treatment for some selected hepatocellular carcinoma. We report our experi-ence of 6 patients with liver transplantation for hepatocellular carcinoma with background inherited metabolic disease. Materials and Methods: This is a single-center retrospective, descriptive study. Consecutive patients who underwent liver transplanta-tion for hepatocellular carcinoma with background inherited metabolic disease were included in the study. The record of the patients was accessed, and the following data were extracted: sociodemographic variables, type of metabolic disease, date of liver transplan-tation, tumor characteristics, laboratory parameters, Model for End-Stage Liver Disease score, immediate-and long-term outcome after transplantation, disease-free survival, and overall survival. Data were analyzed using Statistical Package for the Social Sciences version 25.0. Results: Six patients received liver transplantation for hepatocellular carcinoma with background inherited metabolic liver disease. The median age was 4.5 years. The median Model for End-Stage Liver Disease score was 29.30. The median maximum tumor diameter was 2.15 cm. Three patients had multiple tumor nodules. Half of the patients had microvascular invasion. Four of the patients had a mod-erately differentiated tumor. Progressive familial intrahepatic cholestasis type II is the commonest inherited metabolic disease seen in 3 patients. Median follow-up is 46.1 months. Half of the patients are currently more than 5 years post liver transplantation with no features of recurrence. The estimated survival rates at 1, 3, and 5 years are 100%, 83.3%, and 83.3%, respectively. Conclusion: Liver transplant for these categories of patients is associated with good disease-free and overall survival, even in the pres-ence of some seemingly poor prognostic features.Öğe Liver transplantation in pediatric monogenic metabolic diseases(Galenos Publ House, 2023) Varol, Fatma IlknurMonogenic diseases are the results of a single gene mutation leading to alterations or defects in a single enzyme causing severe metabolic derangements causing multi -systemic systemic disease and even death Current management strategies include diet to reduce the accumulation of metabolic waste products and treatment to increase the excretion of the toxic metabolites and to induce the activity of the mutant enzyme. However, liver transplantation the only therapeutic strategy that offers a chance of cure to children with certain genetic diseases.Öğe Pediatric Hepatocellular Carcinoma(Springer, 2020) Varol, Fatma IlknurPurpose Pediatric hepatocellular carcinoma is rarely seen in childhood. It constitutes approximately 1% of childhood solid organ malignancies. Pediatric hepatocellular carcinoma is the second most common malignant liver tumor after hepatoblastoma in children. In this review, we aimed to review the diagnosis and treatment of pediatric hepatocellular carcinoma in the light of the latest literature. Methods We reviewed the literature in terms of the diagnosis and treatment of pediatric hepatocellular carcinoma. Results Hepatocellular carcinoma (HCC) and hepatoblastoma constitute 0.5-1.5% of all childhood malignant tumors. HCC is responsible for 27% of all liver tumors and 4% of all pediatric liver transplantations. While 99.6% of HCC is seen in adults, only 0.4% of it is seen in pediatric patients. Etiological predisposition and biological behavior are different from adults. In a child with cirrhosis or liver disease, HCC should be suspected in the presence of a high level of AFP and an abnormal nodule on ultrasonography. Hepatoblastoma should be considered first in the differential diagnosis. Conclusion Treatment of pediatric HCC is challenging. Complete surgical resection is essential for the cure. To this end, different neoadjuvant chemotherapy protocols have been designed to convert non-resectable tumors into resectable tumors. For tumors that cannot be resected, liver transplantation for each patient with childhood HCC should be decided individually.Öğe Pediatric Wilson's disease: findings in different presentations. A cross-sectional study(Associacao Paulista Medicina, 2018) Gungor, Sukru; Selimoglu, Mukadder Ayse; Varol, Fatma Ilknur; Gungor, SerdalBACKGROUND: Wilson's disease (WD) may present with different manifestations: from an asymptomatic state to liver cirrhosis. Here, we aimed to evaluate clinical presentations and laboratory findings and prognoses among WD cases. DESIGN AND SETTING: Cross-sectional study based on patients' records from the university hospital, Inonu University, Malatya, Turkey. METHODS: The medical records of 64 children with WD were evaluated focusing on the clinical, laboratory and liver biopsy findings in different clinical presentations. RESULTS: The mean age at diagnosis was 8.6 +/- 3.26 years (range 3.5-17) and mean length of follow-up was 2.49 years (range 0-9). There were 18 cases (28.1%), 12 (18.8%), 9 (14.1%) and 6 (9.4%) of chronic liver disease, fulminant liver failure, neurological WD and acute hepatitis, respectively. Nineteen (29.7%) were asymptomatic. The most common sign and laboratory finding were jaundice (45.3%) and hypertransaminasemia (85.9%), respectively. The lowest serum zinc level was found in the fulminant liver failure group (P = 0.035). Hepatosteatosis was detected in 35% of the 20 patients who underwent liver biopsy. Among those with hepatosteatosis, 57.1% were asymptomatic. While 35% had copper staining, 25% presented iron accumulation in liver biopsies. Nine cases underwent liver transplantation and seven of these presented fulminant liver failure (77.8%). CONCLUSION: The presentation, symptoms and signs of our cases were similar to those in previously reported series, except for the high proportion of fulminant WD cases. Further studies are needed to clarify the relationship between zinc levels and development of a fulminant course and between iron status and WD.Öğe Pediatric wilson's disease: findings in different presentations. A cross-sectional study(Assocıacao paulısta medıcına, av brıg luıs antonıo, 278-7 andar, sao paulo, cep01318-901, brazıl, 2018) Gungor, Sukru; Selimoglu, Mukadder Ayse; Varol, Fatma Ilknur; Gungor, SerdalBACKGROUND: Wilson's disease (WD) may present with different manifestations: from an asymptomatic state to liver cirrhosis. Here, we aimed to evaluate clinical presentations and laboratory findings and prognoses among WD cases. DESIGN AND SETTING: Cross-sectional study based on patients' records from the university hospital, Inonu University, Malatya, Turkey. METHODS: The medical records of 64 children with WD were evaluated focusing on the clinical, laboratory and liver biopsy findings in different clinical presentations. RESULTS: The mean age at diagnosis was 8.6 +/- 3.26 years (range 3.5-17) and mean length of follow-up was 2.49 years (range 0-9). There were 18 cases (28.1%), 12 (18.8%), 9 (14.1%) and 6 (9.4%) of chronic liver disease, fulminant liver failure, neurological WD and acute hepatitis, respectively. Nineteen (29.7%) were asymptomatic. The most common sign and laboratory finding were jaundice (45.3%) and hypertransaminasemia (85.9%), respectively. The lowest serum zinc level was found in the fulminant liver failure group (P = 0.035). Hepatosteatosis was detected in 35% of the 20 patients who underwent liver biopsy. Among those with hepatosteatosis, 57.1% were asymptomatic. While 35% had copper staining, 25% presented iron accumulation in liver biopsies. Nine cases underwent liver transplantation and seven of these presented fulminant liver failure (77.8%). CONCLUSION: The presentation, symptoms and signs of our cases were similar to those in previously reported series, except for the high proportion of fulminant WD cases. Further studies are needed to clarify the relationship between zinc levels and development of a fulminant course and between iron status and WD.
