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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 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 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 growth after liver transplantation in a group of Turkish pediatric patients(2021) Varol, Fatma Ilknur; Selimoglu, Mukadder Ayse; Gungor, Sukru; Yilmaz, SezaiAim: Growth failure is considered as an important predictor of negative outcomes after liver transplantation (LT). In our study we aimed to evaluate the growth of liver transplanted children both at the time of LT and on follow-up and to determine factors which are effective on growth. Materials and Methods: Seventy nine children were included in the study. Evaluation of growth just before the LT and post-LT 6th month,1st,2nd and 3rd years was done by using weight for age Z (WAZ) and height for age Z scores (HAZ). Results: Sixteen (20.3%) patients had HAZ score-2 SD (standard deviation) and 13 (16.5%) had WAZ score -2 SD. Stunting was detected in 17.8% and 23.5% of children with acute liver failure and chronic liver disease, underweight was present in 8.9% and26.5%of them, respectively (p=0.52 and p=0.037, respectively). Both HAZ and WAZ scores increased after LT, especially in the first year. Not mean pre-LT WAZ but mean HAZ score was lower in children who died on follow-up (p=0.023).Conclusion: Malnutrition before LT is a common problem in children. As stunting is a factor that reduces the chance of survival after LT, prevention and correction is very important.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.Öğe The prevalence and severity of reaction during the oral food challenge(Bilimsel Tip Yayinevi, 2014) Topal, Erdem; Catal, Ferhat; Senbaba, Elif; Varol, Fatma Ilknur; Sinanoglu, Muhammed Selcuk; Yildirim, Nurdan; Ermistekin, HalimeObjective: The diagnostic gold standard of food allergy is the oral food challenge. Oral food challenge may induce allergic reactions, ranging from mild cutaneous symptoms to severe, potentially life-threatening reaction. Our aim was to investigate the prevalence and severity of reactions during oral food challenge. Materials and Methods: A retrospective charts review of children undergoing oral food challenge at the our Allergy Clinic between September 2012 and September 2013 was performed. Results: A total of 63 oral food challenges were included the study. Most frequently involved foods were cow's milk, pistachio nut and egg. Oral food challenge were carried out to confirm the diagnosis in 50 (79.4%) patients and were carried out to demonstrate the development of food tolerance in 13 (20.6%). 13/63 (20.6%) were positive. Twelve patients were defined mild reactions and one patient was anaphylaxis. There were no significant differences between the oral food challenge negative group and oral food challenge positive group in terms of sex, age at the time of oral food challenge, concomitant atopic disease, history of IgE mediated food allergy, peripheral eosinophils per cent, serum specific gE and total IgE (p>0.05). But there was a positive correlation between the size of skin prick test and oral food challenge positivity (rho: 0.307, p=0.019). Conclusion: The majority of reactions during oral food challenge were mild. The size of skin prick test was the risk factor for the development of reaction during oral food challenge. Therefore, oral food challenge should be done by the experts and size of skin prick test should be taken into consideration before oral food challenge.Öğe Prevalence of Familial Mediterranean Fever in Children with Cryptogenic Cirrhosis(Oxford Univ Press, 2021) Varol, Fatma Ilknur; Tabel, Yilmaz; Yologlu, Saim; Yesilada, ElifBackground: Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by abdominal and chest pain and recurrent fever due to inflammation in the serosal membranes such as peritoneum, pleura and synovia. In FMF, recurrent inflammatory cytokine production may lead to cirrhosis. The aim of this study was to determine the prevalence of FMF in children with cryptogenic cirrhosis and it was found to be high, to add FMF among the etiological causes of cirrhosis. Materials and methods: This prospective cohort study conducted at the Hospital of Inonu University, Malatya, Turkey. In this study, 44 patients diagnosed with cryptogenic cirrhosis by biopsy, in the Pediatric Gastroenterology, Hepatology and Nutrition Clinic, were included, after the other reasons that may cause chronic liver disease were excluded. MEVF gene analysis was performed for all patients with cryptogenic cirrhosis. Results: FMF genetic mutation was detected in 9 (20%) of 44 patients. M694V mutation was detected in one patient (2.27%) and E148Q homozygous mutation was detected in one patient (2.27%). Various other heterozygous mutations were detected in seven other patients. Homozygous and heterozygous R202Q mutations were detected in one patient. Conclusion: We suggest that FMF plays a role in the etiologic differential diagnosis of cryptogenic cirrhosis.Öğe Rare Cause of Hematochezia in Children: Solitary Rectal Ulcer, Single Center Experience(Korean Soc Gastroenterology, 2024) Varol, Fatma Ilknur; Gungor, Sukru; Selimoglu, Mukadder Ayse; Samdanci, EmineBackground/Aims: Solitary rectal ulcer syndrome (SRUS) can be overlooked, diagnosed late, or misdiagnosed, particularly in childhood. This study reviewed the 13-year experience of the authors' institution to increase clinicians' awareness of SRUS in the presence of symptoms. This paper reports the endoscopic and histopathological findings in children presenting with hematochezia. Methods: The clinical and laboratory findings of 22 patients diagnosed with biopsy-proven SRUS in the authors' clinic between 2007 and 2020 were evaluated retrospectively. Results: The mean age at diagnosis was 12.5 +/- 2.6 years, and 59.1% of the patients were male. The median time of diagnosis was 24 months. A single ulcer lesion was found by colonoscopy in 18 patients (81.8%), two ulcers in two patients (9%), and more than two ulcers in two patients (9%). The pathology reports of all biopsies taken from the lesions were consistent with a solitary rectal ulcer. In the first stage, the treatment was started with toilet training, a high-fiber diet, and laxatives. In 11 patients (50%) who did not respond to the initial treatment, a 5-ASA enema was added. A glucocorticoid enema was added to treatment in five patients (22%) whose complaints did not regress despite this treatment. Clinical remission was achieved in five of the patients (18.1%). The time to diagnosis was significantly shorter in those in remission than those not in remission (p=0.04). Conclusions: This study is the first large series on Turkish children. An increased awareness of SRUS in children will increase the rate of early diagnosis and treatment, allowing remission in more patients.Öğe Single-center experience in management of progressive familial intrahepatic cholestasis(Elsevier, 2021) Varol, Fatma Ilknur; Selimoglu, Mukadder Ayse; Gungor, Sukru; Yilmaz, Sezai; Tekedereli, IbrahimBackground and study aims: Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessively inherited disease that causes intrahepatic-hepatocellular cholestasis. PFIC constitutes approximately 10-15% of cholestatic liver diseases in children. The aim of this study is to draw attention to this group of diseases, which pose a higher risk, in societies where consanguineous marriage is more common, and to share our experiences since the studies in the literature, regarding this group of diseases are case series with small number of patients. Patients and methods: This cross-sectional study was conducted on 34 patients who were admitted with jaundice and diagnosed by genetic analysis, between January 2015 and July 2020. Results: We found 17.6% of patients with PFIC type 1, 55.9% patients had PFIC type 2, 14.7% patients had PFIC type 3, 8.8% patients had PFIC type 4 and 2.9% patients had PFIC type 5. Partial internal biliary diversion was performed in 5 (14.7%) patients, who had severe itching during follow-up, did not respond to medical treatment, and did not have significant fibrosis in liver biopsy yet. The degree of itching before PIBD was rated as +4 (cutaneous erosion, bleeding and scarring), in 5 patients and the rates were 0 (absent) in two patients, and +1 (mild itching) in 3 patients, 6 months after PIBD, these differences were statistically significant(p = 0.027). The mean weight z score was-1.43 (-3.72-+0.73), before PIBD, while it was 0.39(-1.86 -+2.45), six months after PIBD; the diference was statistically significant(p = 0.043). Liver transplantation was performed in 12 (35.3%) patients with significant fibrosis in liver biopsy and developing signs of portal hypertension. Conclusion: The PFIC disease group is a heterogeneous disease group that is difficult to diagnose and treat. It should be considered in patients with cholestasis and/or pruritus and those with a history of consanguineous marriage between parents and death of a sibling with similar clinical symptoms. (c) 2021 Pan-Arab Association of Gastroenterology. Published by Elsevier B.V. All rights reserved.
