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Öğe Differences in parents of pediatric liver transplantation and chronic liver disease patients(Baishideng Publishing Group Inc, 2020) Akbulut, Sami; Gunes, Gulsen; Saritas, Hasan; Aslan, Bahar; Karipkiz, Yunus; Demyati, Khaled; Gungor, SukruBACKGROUND With advancements in the treatment of chronic liver disease (CLD), including liver transplantation (LT), quality of life and satisfaction after LT have become an important issue for pediatric patients and their parents. More evidence-based information is needed to describe and assess the impact of pediatric CLD on parents and the satisfaction of parents with treatment to better understand their needs. AIM To assess the satisfaction of parents of pediatric LT patients and that of parents of pediatric CLD patients METHODS During this survey, data were collected from parents of pediatric patients who underwent LT between January 2010 and April 2017 (LT group; n = 91) and parents of pediatric patients with chronic liver disease (CLD group; n = 94). Group comparisons were made based on the pediatric health-related quality of life (PedsQL) health care parent satisfaction scale, impact on family scale (IFS) and demographic characteristics. The PedsQL was administered to parents during a phone interview and the results were used to assess the health care-related satisfaction of parents. The IFS was used to assess the impact of the child's CLD status on the family. Demographic variables such as education level (elementary vs middle vs high vs university), monthly income (low vs middle vs high), and place of residence (village vs town vs city) were compared between CLD and LT parent groups. Finally, PedsQL and IFS results were also analyzed according to demographic variables. RESULTS A total of 185 parents aged 19 to 65 years were included. There were statistically significant differences between the LT and CLD groups in terms of career (P < 0.001), monthly income (P = 0.016), and education level (P = 0.041). According to the PedsQL results, family inclusion, communication, technical skills, emotional needs, and overall satisfaction were significantly different between the groups; the LT group had consistently higher scores (P < 0.001). Additionally, scores for the IFS parameters of financial impact, familial-social impact, personal strain, and total impact were consistently higher for the LT group (P < 0.001). There were statistically significant relationships between education level, monthly income, and place of residence according to the IFS results but not the PedsQL results. There were inverse relationships between the difficulties that parents experience because of their child's health and education levels, monthly income, and place of residence. However, no relationship was found between education level, monthly income, or place of residence and satisfaction with health care services provided in the hospital according to the PedsQL results. CONCLUSION Parents of children who underwent LT were very satisfied with the health care services provided to their children. However, they had more difficulties than parents of children with CLD.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 Effects of parent- and child-related behavioral feeding problems in early childhood on malnutrition(Elsevier France-Editions Scientifiques Medicales Elsevier, 2023) Gungor, Sukru; Buyukavci, Mehmet Akif; Acipayam, CanObjective: Children's responses to food and their caregivers during normal developmental periods are known as feeding behavior. For the healthy development of these behaviors, parent and child relationships must also be healthy. Therefore, we aimed to investigate the effect of behavioral feeding problems on primary malnutrition (PM).Method: The Behavioral Pediatric Nutrition Assessment Scale (BPFAS) was administered to 300 malnourished and 300 control pediatric patients aged from 9 months to 4 years who were referred to our pediatric gastroenterology outpatient clinic. Pre-and posttreatment data were compared between the two groups.Results: There was no statistically significant difference between patients with and without malnutrition in terms of gender and age (p = 0.191, p = 0.128, respectively). Total behavioral frequency (TBF) and total behavioral problem (TBP) scores were significantly higher in the malnutrition group (p < 0.001). In the logistic regression analysis of risk factors that may affect malnutrition we found that a total TBF score of >85 increases the risk of developing malnutrition 3.731 times, a child TBF score of >62 increases it 2.644 times, and a parental TBF score of >21 increases it 4.82 times (p < 0.001). When anthropometric measurements and BPFAS scores of 127 PM patients who received behavioral therapy with enteral products and who attended follow-up were compared with their pretreatment data, there was a significant improvement (p < 0.05).Conclusion: Our study showed that behavioral feeding problems may increase the risk of PM and that behavioral therapy together with enteral products has a positive effect on treatment. Therefore, in addition to nutritional support in patients with PM, offering behavioral feeding therapy to parents will positively affect both the child's physical development and the relationship between the parents and their child.& COPY; 2022 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.Öğe Evaluation of epicardial adipose tissue in children with type 1 diabetes(Springernature, 2024) Trabzon, Guel; Gungor, Sukru; Gullu, Seyma Demiray; Caliskan, Osman Firat; Gullu, Ufuk UtkuIntroduction Epicardial adipose tissue (EAT), the visceral fat surrounding the heart between the myocardium and visceral pericardium, intersects with Type 1 diabetes (T1D). This review aims to elucidate the intricate association between EAT and childhood T1D.Materials and methods In this retrospective study, two pediatric groups were involved children with type 1 diabetes, and healthy children. Epicardial fat thickness was measured appropriately, and the study documented HbA1c levels and time to diabetes diagnosis for comprehensive analysis.Results Encompassing 51 children with T1D and 69 healthy controls, revealed that children with type 1 diabetes had a mean HbA1c level of 9.4 +/- 0.2, and a mean insulin dose of 0.94 units/kg/day. Epicardial adipose tissue (EAT) values were significantly higher in the Type 1 DM group. It has been shown that epicardial fat thickness may have a specific and sensitive value in type 1 diabetics.Discussion The increased presence of epicardial fat tissue in children with type 1 diabetes is highlighted, prompting the consideration of various mechanisms. However, the complexity of this relationship underscores the need for further studies to provide a more comprehensive understanding of the underlying factors. Ongoing research in this area is crucial for advancing our knowledge and potential therapeutic interventions.Impacts Cardiac complications are one of the most important causes of morbidity and mortality in people with type 1 diabetes. Being able to detect cardiological complications of diabetes at an early stage contributes to morbidity. We found that epicardial fat tissue thickness was thicker in children with type 1 diabetes than in healthy children. Epicardial fat tissue thickness may be associated with poor control in children with type 1 diabetes and maybe a guide in terms of cardiac risks.Öğe Evaluation of growth after liver transplantation in a group of Turkish pediatric patients(2021) varol, Fatma İlknur; Selimoğlu, Mukadder Ayşe; Gungor, Sukru; Yılmaz, SezaiAbstract: Aim: 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 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 Evaluation of neurologically symptomatic and asymptomatic patients in childhood Wilson’s disease with central nervous system involvement: A retrospective observational study(2023) Yücel, Gül; Gungor, SukruAim: The aim was to evaluate the clinical and laboratory characteristics of children with Wilson’s disease (WD) with and without neurological presentation with abnormalities on brain magnetic resonance imaging (MRI) and to describe the relationship of these observations with disease severity and functional outcome and their impact on prognosis. Materials and Methods: Demographic, neuropsychiatric findings, laboratory, disease severity and functional results of 48 children with neurologically symptomatic and asymptomatic WD were evaluated retrospectively. Results: A significant positive correlation was found between the neurologic symptom score and functional outcome in WD with neurological presentation (p<0.001). A significant positive correlation was found between disease severity scores (PELD, MELD, Child Pugh and Dhwin score) and modified Rankin scores (mRS) in WD without neurological presentation (p <0.001, 0.004, <0.001, 0.001, respectively). In addition, a significant positive correlation was found between total bilirubin, direct bilirubin and International Normalized Ratio (INR) values and mRS scores (p= 0.006, 0.012, 0.004, respectively). Kayser-Fleischer Ring sign in the eye was higher in the group with neurological symtomatic WD (p<0.001). The number of patients presenting with clinic of fulminant hepatitis and hepatic encephalopathy was significantly higher in the neurological asymptomatic group (p<0.001). Conclusion: Brain MRI changes may occur even in hepatic WD and presymptomatic cases, although infrequently, regardless of the presence of neurological symptoms. It should be known that neurological symptoms in children and adolescents may occur without significant liver disease. Even without neurological symptoms, all children with WD should have a brain MRI before treatment.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 it possible to diagnose fulminant Wilson's disease with simple laboratory tests?(Wiley, 2020) Gungor, Sukru; Selimoglu, Mukadder A.; Bag, Harika G. G.; Varol, Fatma I.Background Wilson's disease is a rare cause of acute liver failure and is highly fatal without liver transplantation. Fast and accurate diagnostic methods are needed for fulminant Wilson's disease (FWD). In this study, we aimed to develop an early, simple and accurate diagnostic method to differentiate FWD from nonwilsonian acute liver failure (NWALF) causes using routine biochemical data. Methods The medical records of 24 paediatric FWD and 120 paediatric NWALF cases diagnosed at the Department of Pediatric Gastroenterology, Hepatology, and Nutrition between January 2007 and February 2017 were retrospectively reviewed. Results Using receiver operator characteristics curve (ROC) analysis, we have determined the best cut-off point for laboratory findings in FWD. Patients meeting these cut-off points were assigned one point and others were assigned zero point. We then formed a new variable consisting of the combination of 14 variables and performed a new ROC analysis. We obtained a cut-off point of >= 4.5 for FWD. The diagnostic performance of the score was characterized by a sensitivity of 0.889, a specificity of 0.879 (P < .001). A scoring system based only on aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase, AST/ALT ratio, uric acid and haemoglobin had a best cut-off point of >= 2.5 for FWD, which had a sensitivity of 0.875, a specificity of 0.867 (P < .001). Conclusions Our study demonstrated that biochemical markers offer almost as reliable, fast and accurate diagnosis of FWD as offered by ceruloplasmin and 24-hour urinary copper.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 A newborn with gastric hemangioma treated using propranolol(Korean soc pedıatrıc gastroenterology & nutrıtıon, 301 dongsung-dong 25-ı, jongno-gu, seoul, 301 dongsung, south korea, 2018) Kaya, Huseyin; Gokce, Ismail Kursad; Gungor, Sukru; Turgut, Hatice; Ozdemir, RamazanGastric hemangiomas are rare benign vascular tumors that can cause severe gastrointestinal system bleeding. We presented the case of a neonate with fresh bleeding and melena from the orogastric tube and detected gastric hemangioma in esophagogastroduodenoscopic examination. Propranolol is widely used in treatment of cutaneous hemangiomas and non-gastric gastrointestinal system hemangiomas. However, the surgical approach is preferred for treating gastric hemangiomas, and there are few reports of gastric hemangiomas associated with non-surgical treatment. Gastric hemorrhage decreased with antacid and somatostatin treatment. Propranolol treatment was initiated before the surgery decision. After three weeks of treatment, we observed regression in the hemangioma with endoscopic evaluation. During the course of treatment, the patient's gastrointestinal system bleeding did not recur, and there were no side effects associated with propranolol.Öğe A Newborn with Gastric Hemangioma Treated Using Propranolol(Korean Soc Pediatric Gastroenterology & Nutrition, 2018) Kaya, Huseyin; Gokce, Ismail Kursad; Gungor, Sukru; Turgut, Hatice; Ozdemir, RamazanGastric hemangiomas are rare benign vascular tumors that can cause severe gastrointestinal system bleeding. We presented the case of a neonate with fresh bleeding and melena from the orogastric tube and detected gastric hemangioma in esophagogastroduodenoscopic examination. Propranolol is widely used in treatment of cutaneous hemangiomas and non-gastric gastrointestinal system hemangiomas. However, the surgical approach is preferred for treating gastric hemangiomas, and there are few reports of gastric hemangiomas associated with non-surgical treatment. Gastric hemorrhage decreased with antacid and somatostatin treatment. Propranolol treatment was initiated before the surgery decision. After three weeks of treatment, we observed regression in the hemangioma with endoscopic evaluation. During the course of treatment, the patient's gastrointestinal system bleeding did not recur, and there were no side effects associated with propranolol.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 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.Öğe Torsion of a wandering spleen in an adolescent with Gaucher disease(Aves, 2017) Gungor, Sukru; Ozturk, Mehmet; Varol, Fatma Ilknur; Sigirci, Ahmet; Selimoglu, Mukadder AyseA wandering spleen is a rare condition characterized by the malposition of the spleen due to laxity or absence of its supporting ligaments. Although Gaucher disease generally presents with massive splenomegaly, which one of the predisposing causes of a wandering spleen, literature shows only one report of a wandering spleen in a child with Gaucher disease. In this case presentation, a 13-year-oldadolescent with Gaucher disease on enzyme replacement treatment was presented, who was detected having an abdominal mass on a routine visit and diagnosed with partial torsion of a wandering spleen associated with left lobe hypoplasia of the liver.Öğe The Value of Electroencephalogram (EEG) Findings in the Evaluation and Treatment Management of Pediatric Acute Liver Failure(Springernature, 2024) Ozgor, Bilge; Gungor, Sukru; Aladag, Merve; Varol, Fatma I.; Aslan, Mahmut; Yilmaz, Sezai; Gungor, SerdalBackground Pediatric acute liver failure (PALF) is still life -threatening and requires urgent care. The presence of encephalopathy is a clinical diagnosis, but it is more difficult to diagnose in children than in adults, and an electroencephalogram (EEG) can be invaluable. The role of EEG in managing the treatment of patients with PALF, other than the identification of encephalopathy, is unknown. This study aimed to investigate patients' EEGs, which may guide in choosing the most appropriate treatment in encephalopathy children. A further aim was to investigate a new score method, based on the laboratory results, which might indicate the presence of encephalopathy in cases with PALF. Methods Medical data of 33 PALF patients followed in our clinic were reviewed retrospectively. This study included 33 patients, whose EEG recording was taken on the first day of supportive treatment due to liver failure in the pediatric intensive care unit (PICU). The EEG findings were categorized into three classes: normal, epileptic and non -epileptic paroxysmal, and background encephalopathic patterns including widespread slowing and voltage suppression. Result This retrospective study included 13 male and 20 female patients with a mean age at presentation of 4.82 +/- 4.81 months whose EEG was performed on the first day of supportive therapy for liver failure in the PICU. The EEG findings were categorized into three groups: normal, epileptic and non -epileptic paroxysms, and encephalopathic patterns including diffuse background slowing and voltage suppression. Comparing EEG findings and treatments, we found that the normal EEG group responded well to liver -supporting therapy and the rate of plasmapheresis treatment was significantly higher in the diffuse slowing group. Patients with diffuse slowing of the EEG were 9.6 times more likely to receive plasmapheresis. We found that above a cut-off of >= 7.5 for the TAI (total bilirubin, albumin, and international normalized ratio (INR)) score used in our study, the risk of developing encephalopathy increased 14.4 -fold. Conclusions In PALF, EEG findings can provide findings that will help clinicians in determining treatment selection and prognosis, as well as detecting epileptic focus and encephalopathy. The TAI score can be used to assess the risk of encephalopathy in cases of PALF, when it is challenging to identify encephalopathy or when an EEG is not possible.
