Browsing by Author "Baris, Zeren"

Now showing 1 - 17 of 17

Clinical Features, Laboratory Findings and Prognosis in Fulminant Wilson's Disease
(2018) Ozcay, Figen; Baris, Zeren; Sezer, Oya Balci; Haberal, Mehmet; 0000-0002-5214-516X; 0000-0002-8402-8208; 0000-0002-3462-7632; ABG-5684-2020; AAB-4153-2020; AAI-9346-2021; AAJ-8097-2021
Clinical Findings and Explant Liver Histology in Crigler Najjar Disease
(2018) Baris, Zeren; Ozgun, Gonca; Sezer, Oya Balci; Haberal, Mehmet; 0000-0002-8402-8208; 0000-0002-3462-7632; AAB-4153-2020; AAI-9346-2021; AAJ-8097-2021
Etiologies, outcomes, and prognostic factors of pediatric acute liver failure: A single center's experience in Turkey
(2016) Ozcay, Figen; Karadag-Oncel, Eda; Baris, Zeren; Canan, Oguz; Moray, Gokhan; Haneral, Mehmet; 0000-0003-2498-7287; 0000-0002-3462-7632; 0000-0002-5214-516X; 0000-0003-0614-4497; 27782894; AAE-1041-2021; AAJ-8097-2021; ABG-5684-2020; AAB-4153-2020; AAI-9386-2021
Background/Aims: Our aim was to determine the etiologies, outcomes, and prognostic indicators in children with acute liver failure. Materials and Methods: Ninety-one patients who were followed for pediatric acute liver failure (PALF) over a 15-year period were included. Patients who survived with supportive therapy were designated as Group 1, while those who died or underwent liver transplantation were designated as Group 2. Results: There were 37 (40.6%) patients in Group 1 (spontaneous recovery) and 54 (59.4%) patients in Group 2. Thirty-two patients (35.2%) underwent liver transplantation. Infectious and indeterminate causes were the most common etiologies (33% each). Among the infectious causes, hepatitis A (76%) was the most frequent. Hepatic encephalopathy grade 3-4 on admission and during follow-up and high Pediatric Risk of Mortality (PRISM) and Pediatric End-Stage Liver Disease (PELD) scores within the first 24 h were related with a poor prognosis. Group 2 had a more prolonged prothrombin time, higher international normalized ratio, more prolonged activated partial thromboplastin time (aPTT), and higher levels of total and direct bilirubin, ammonia, and lactate (for all, p<0.01). Conclusion: Infectious and indeterminate cases constituted the most common etiology of PALF, and the etiology was related to the prognosis in our series. Although high PELD and PRISM scores were related to poor prognoses, no sharp thresholds for individual laboratory tests could be elucidated. Liver transplantation was the only curative treatment for patients with poor prognoses and resulted in high survival rates (1-, 5-, and 10-year survival rates of 81.3%, 81.3%, and 75%, respectively) in our study.
Evaluation of spleen volume with computed tomography after liver transplantation in pediatric recipients
(2019) Baris, Zeren; Haberal, Murat; Sezer, Oya Balci; Ozcay, Figen; Haberal, Mehmet; AAB-4153-2020; ABG-5684-2020
Experience of Post-Transplant Lymphoproliferative Disorder (PTLD) After Pediatric Liver Transplant: Incidence, Outcomes and Association with Food Allergy
(2018) Baris, Zeren; Ozcay, Figen; Ozbek, Ozlem; Haberal, Nihan; Sarialioglu, Faik; Haberal, Mehmet; 0000-0002-5214-516X; 0000-0001-9852-9911; 0000-0002-8257-810X; 0000-0002-3462-7632; AAB-4153-2020; ABG-5684-2020; AAK-4587-2021; AAL-7766-2021; AAJ-8097-2021
Experience with mTOR Inhibitors in Pediatric Liver Transplantation
(2016) Ozcay, Figen; Baris, Zeren; Gulsan, Meltem; Moray, Gokhan; Haberal, Mehmet; https://orcid.org/0000-0002-5214-516X; https://orcid.org/0000-0003-2498-7287; https://orcid.org/0000-0002-3462-7632; ABG-5684-2020; AAB-4153-2020; AAE-1041-2021; AAJ-8097-2021
Incidence, Clinical Features and Prognosis of Food Allergy in Children who Underwent Liver Transplantation
(2018) Koksal, Burcu T.; Baris, Zeren; Ozcay, Figen; Ozbek, Ozlem Yilmaz; Haberal, Mehmet; 0000-0001-9580-7656; 0000-0002-5214-516X; 0000-0002-3462-7632; AAF-2109-2021; AAB-4153-2020; ABG-5684-2020; AAJ-8097-2021
Incidence, clinical features, and outcomes of food allergy in children who underwent liver transplant: 16-year experience
(2019) Baris, Zeren; Koksal, Burcu; Ozbek, Ozlem; Ozcay, Figen; Haberal, Mehmet; 0000-0001-9580-7656; 30884056; ABG-5684-2020; AAB-4153-2020
Food allergies often develop after liver transplant, especially in young children. However, data are scarce on clinical characteristics and patient outcomes. When we evaluated our pediatric liver transplant patients over a 16-year period, food allergy incidence was 8% (19/236 patients). All patients with food allergies were <18 months old, with incidence in this age group of 19.2% (19/99). Two patients had a single food and 17 had multiple food allergies. Five patients showed only non-IgE-mediated food allergies. Eggs, milk, nuts, and wheat were the most common allergens. Presenting symptoms included diarrhea, flushing, angioedema attacks, wheezing/chronic cough, and vomiting. Seven patients had EBV, and two patients had CMV infections at time of food allergy diagnosis. Twelve patients had eosinophilia. Seven patients (36.8%) were able to regain tolerance to all food allergens. However, one patient with single nut allergy and three with multiple food allergies were still on allergen-eliminated diets. Eight patients with multiple food allergies gained tolerance to some of the food allergens. In conclusion, food allergies in our patients were mainly against multiple foods and IgE mediated. Infections like EBV and CMV may play a role in food allergies after liver transplant, especially in pretransplant-naive patients.
Intestinal Failure and Aberrant Lipid Metabolism in Patients With DGAT1 Deficiency
(2018) Ozcay, Figen; Baris, Zeren; van Rijn, Jorik M.; Ardy, Rico Chandra; Kuloglu, Zarife; Haerter, Bettina; van Haaften-Visser, Desiree Y.; van der Doef, Hubert P. J.; van Hoesel, Marliek; Kansu, Aydan; van Vugt, Anke H. M.; Thian, Marini; Kokke, Freddy T. M.; Krolo, Ana; Basaran, Meryem Keceli; Kaya, Neslihan Gurcan; Aksu, Aysel Unlusoy; Dalgic, Buket; Kain, Renate; Stigter, Edwin C. A.; Lichtenbelt, Klaske D.; Massink, Maarten P. G.; Duran, Karen J.; Verheij, Joke B. G. M.; Lugtenberg, Dorien; Nikkels, Peter G. J.; Brouwer, Henricus G. F.; Verkade, Henkjan J.; Scheenstra, Rene; Spee, Bart; Nieuwenhuis, Edward E. S.; Coffer, Paul J.; Janecke, Andreas R.; van Haaften, Gijs; Houwen, Roderick H. J.; Mueller, Thomas; Middendorp, Sabine; Boztug, Kaan
BACKGROUND & AIMS: Congenital diarrheal disorders are rare inherited intestinal disorders characterized by intractable, sometimes life-threatening, diarrhea and nutrient malabsorption; some have been associated with mutations in diacylglycerol-acyltransferase 1 (DGAT1), which catalyzes formation of triacylglycerol from diacylglycerol and acyl-CoA. We investigated the mechanisms by which DGAT1 deficiency contributes to intestinal failure using patient-derived organoids. METHODS: We collected blood samples from 10 patients, from 6 unrelated pedigrees, who presented with early-onset severe diarrhea and/or vomiting, hypoalbuminemia, and/or (fatal) protein-losing enteropathy with intestinal failure; we performed next-generation sequencing analysis of DNA from 8 patients. Organoids were generated from duodenal biopsies from 3 patients and 3 healthy individuals (controls). Caco-2 cells and patient-derived dermal fibroblasts were transfected or transduced with vectors that express full-length or mutant forms of DGAT1 or full-length DGAT2. We performed CRISPR/Cas9-guided disruption of DGAT1 in control intestinal organoids. Cells and organoids were analyzed by immunoblot, immunofluorescence, flow cytometry, chromatography, quantitative real-time polymerase chain reaction, and for the activity of caspases 3 and 7. RESULTS: In the 10 patients, we identified 5 bi-allelic loss-of-function mutations in DGAT1. In patient-derived fibroblasts and organoids, the mutations reduced expression of DGAT1 protein and altered triacylglycerol metabolism, resulting in decreased lipid droplet formation after oleic acid addition. Expression of full-length DGAT2 in patient-derived fibroblasts restored formation of lipid droplets. Organoids derived from patients with DGAT1 mutations were more susceptible to lipid-induced cell death than control organoids. CONCLUSIONS: We identified a large cohort of patients with congenital diarrheal disorders with mutations in DGAT1 that reduced expression of its product; dermal fibroblasts and intestinal organoids derived from these patients had altered lipid metabolism and were susceptible to lipid-induced cell death. Expression of full-length wildtype DGAT1 or DGAT2 restored normal lipid metabolism in these cells. These findings indicate the importance of DGAT1 in fat metabolism and lipotoxicity in the intestinal epithelium. A fat-free diet might serve as the first line of therapy for patients with reduced DGAT1 expression. It is important to identify genetic variants associated with congenital diarrheal disorders for proper diagnosis and selection of treatment strategies.
Liver Cirrhosis in a Patient with Crigler Najjar Syndrome
(2018) Baris, Zeren; Ozcay, Figen; Usta, Yusuf; Ozgun, Gonca; 0000-0002-5214-516X; 30260719; AAB-4153-2020; ABG-5684-2020
Introduction: Crigler Najjar (CN) disease is a genetic disorder which results in increased unconjugated bilirubin level. Liver parenchyma was previously considered structurally normal. Recent reports describe significant fibrosis in the liver parenchyma of patients with CN syndrome. Case report. We present a patient with persistent unconjugated hyperbilirubinemia, clinically diagnosed as CN-2, with a UGT1 A1 p. H39D (c.115C > G) (His -> Asp) mutation. She required hepatic transplantation at the age of 17.5 years for biliary cirrhosis. Explanted liver histopathology revealed regenerative cirrhotic nodules with dilated bile ducts filled with bile plugs. Conclusion: CN can develop significant hepatic fibrosis/cirrhosis requiring liver transplantation.
Liver Transplant in a Patient With Hemophagocytic Lymphohistiocytosis
(2019) Soy, Ebru H. Ayvazoglu; Alam, Humaira; Olcay, Lale; Baris, Zeren; Yildirim, Sedat; Torgay, Adnan; Haberal, Mehmet; https://orcid.org/0000-0002-0993-9917; https://orcid.org/0000-0002-5684-0581; https://orcid.org/0000-0002-5735-4315; https://orcid.org/0000-0002-6829-3300; https://orcid.org/0000-0002-3462-7632; 30777561; AAC-5566-2019; AAK-3548-2021; AAB-4153-2020; AAF-4610-2019; AAJ-5221-2021; AAJ-8097-2021
Hemophagocytic lymphohistiocytosis is a rare and life-threatening systemic disease that can cause hepatic infiltration and present as acute liver failure. Here, we report a case of a 3-year-old pediatric patient who presented with acute liver failure and hepatic encephalopathy secondary to hemophagocytic lymphohistiocytosis. She had left lateral segment liver transplant from her father. After 27 months, she had bone marrow transplant from her sister. At the time of reporting (36 months after liver transplant), she showed normal liver function and blood peripheral counts. We found that liver transplant can be a curative treatment for this type of rare disorder, not only to improve the quality of life but also to prolong survival.
PMM2-CDG and Sensorineural Hearing Loss
(2017) Kasapkara, Cigdem Seher; Baris, Zeren; Kilic, Mustafa; Yuksel, Deniz; Keldermans, Lies; Matthijs, Gert; Jaeken, Jaak; 28762107; AAB-4153-2020
Prevalence of infections in infants within first six months of liver transplantation
(2019) Sezer, Oya Balci; Baris, Zeren; Ecevit, Zafer; Ozcay, Figen; Haberal, Mehmet; ABG-5684-2020; AAB-4153-2020
Report of 3 Patients With Urea Cycle Defects Treated With Related Living-Donor Liver Transplant
(2015) Ozcay, Figen; Baris, Zeren; Moray, Gokhan; Haberal, Nihan; Torgay, Adnan; Haberal, Mehmet; 0000-0003-2498-7287; 0000-0001-9852-9911; 0000-0002-6829-3300; 0000-0002-5214-516X; 0000-0002-3462-7632; 26640932; AAE-1041-2021; AAB-4153-2020; AAK-4587-2021; AAJ-5221-2021; ABG-5684-2020; AAJ-8097-2021
Urea cycle defects are a group of metabolic disorders caused by enzymatic disruption of the urea cycle pathway, transforming nitrogen to urea for excretion from the body. Severe cases present in early infancy with life-threatening metabolic decompensation, and these episodes of hyperammonemia can be fatal or result in permanent neurologic damage. Despite the progress in pharmacologic treatment, long-term survival is poor especially for severe cases. Liver trans plant is an alternative treatment option, providing sufficient enzymatic activity and decreasing the risk of metabolic decompensation. Three patients with urea cycle defects received related living-donor liver transplants at our hospital. Patients presented with late-onset ornithine transcarbamylase deficiency, argininosuccinate lyase deficiency, and citrullinemia. Maximum pretransplant ammonia levels were between 232 and 400 mu mol/L (normal range is 18-72 mu mol/L), and maximum posttransplant values were 52 to 94 mu mol/L. All patients stopped medical treatment and dietary protein restriction for urea cycle defects after transplant. The patient with late-onset ornithine transcarbamylase deficiency already had motor deficits related to recurrent hyperammonemia attacks pretransplant. A major improvement could not be achieved, and he is wheelchair dependent at the age of 6 years. The other 2 patients had normal motor and mental skills before transplant, which have continued 12 and 14 months after transplant. Hepatic artery thrombosis in the patient with the ornithine transcarbamylase deficiency, intra-abdominal infection in the patient with argininosuccinate lyase deficiency, and posterior reversible encephalopathy syndrome in the patient with citrullinemia were early postoperative complications. Histopathologic changes in livers explanted from patients with ornithine transcarbamylase deficiency and citrullinemia were nonspecific. The argininosuccinate lyase-deficient patient had portoportal fibrosis and cirrhotic nodule formation. In conclusion, liver transplant was a lifesaving procedure for our patients. Proper timing for transplant is important because high ammonia levels may result in permanent neurologic damage; however, transplant at younger ages also may increase morbidity.
A single-center experience of post-transplant lymphoproliferative disorder (PTLD) cases after pediatric liver transplantation: Incidence, outcomes, and association with food allergy
(2018) Haberal, Mehmet; Haberal, Nihan; Baris, Zeren; Ozcay, Figen; Ozbek, Ozlem Yilmaz; Sarialioglu, Faik; 29755021; AAB-4153-2020
Background/Aims: We evaluated our 16-year single-center experience of pediatric post-transplant lymphoproliferative disorder (PTLD) cases who underwent liver transplantation between 2001 and 2017. Materials and Methods: Of the 236 pediatric patients who underwent liver transplantation between 2001 and 2017, the clinical and laboratory data of eight patients diagnosed with PTLD were reviewed. The pre-transplant Epstein-Barr virus (EBV) status of 172 patients was also recorded. Results: The total incidence of PTLD was 3.4%. The incidence of PTLD was 10% in pre-transplant EBV immunoglobulin G (IgG)-seronegative patients and 0.8% in pre-transplant EBV IgG-seropositive patients. The mean age of the patients at liver transplantation was 2.71 +/- 3.21 years, and four patients were aged below 1 year at the time of transplantation. PTLD was diagnosed at 21.81 +/- 18.1 months after transplantation. The primary site of involvement was variable among patients: peripheral and mediastinal lymph nodes, stomach and intestine, transplanted graft, bone marrow, and nasopharynx. The eosinophil count varied greatly among patients, with a mean value of 524.62 +/- 679/mm3. Three patients had a food allergy and were administered an elimination diet at the time of PTLD diagnosis. Six patients had PTLD of B-cell origin. One patient died due to neutropenic sepsis during chemotherapy, whereas seven patients were followed up in full remission for 7.75 +/- 4 years. Conclusion: PTLD is a life-threatening complication of solid-organ transplantation with a heterogeneous clinical spectrum. Food allergy had a close association with PTLD. A close follow-up of patients with risk factors and an early diagnosis with appropriate treatment may lead to a better outcome.
Social and psychological impacts of having a child with liver transplant
(2019) Soy, Ebru H. Ayvazoglu; Ozdemir, Aydan; Baris, Zeren; Moray, Gokhan; Haberal, Mehmet; http://orcid.org/0000-0002-0993-9917; AAB-4153-2020; AAC-5566-2019
Solid Liver Lesions in an Infant With Neonatal Cholestasis: Is it Always Malignant?
(2017) Baris, Zeren; Borcek, Pelin; Haberal, Kemal Murat; Ozcay, Figen; 0000-0002-8211-4065; 0000-0002-5214-516X; 28816798; AAB-4153-2020; R-9398-2019; ABG-5684-2020
In this report we describe a patient with neonatal cholestasis who was found to have a liver lesion with suspicious imaging features, although ultimately it was histologically proved to be a pseudotumor. We discuss the characteristic features and imaging findings of macroregenerative nodules of the liver.