Browsing by Author "Ozcay, Figen"

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17 Years Of Pediatric Liver Transplantation Experience For Cirrhosis And Hepatocellular Carcinoma
(2022) Ozcay, Figen; Sezer, Oya Balci; Sarialioglu, Faik; Boyvat, Fatih; Coskun, Mehmet; Reyhan, Nihan Haberal; Haberal, Mehmet; https://orcid.org/0000-0002-3462-7632; AAJ-8097-2021
Assessment Of Long-Term Outcomes Of Pediatric Liver Transplant Recipients
(2022) Karakaya, Emre; Akdur, Aydincan; Soy, Ebru H. Ayvazoglu; Ozcay, Figen; Moray, Gokhan; Haberal, Mehmet; https://orcid.org/0000-0002-0993-9917; https://orcid.org/0000-0002-3462-7632; AAC-5566-2019; AAJ-8097-2021
Broadly effective metabolic and immune recovery with C5 inhibition in CHAPLE disease
(2021) Ozen, Ahmet; Kasap, Nurhan; Vujkovic-Cvijin, Ivan; Apps, Richard; Cheung, Foo; Karakoc-Aydiner, Elif; Akkelle, Bilge; Sari, Sinan; Tutar, Engin; Uygun, Dilara Kocacik; Islek, Ali; Akgun, Gamze; Selcuk, Merve; Sezer, Oya Balci; Ozcay, Figen; Zhang, Yu; Kutluk, Gunsel; Topal, Erdem; Sayar, Ersin; Celikel, Cigdem; Houwen, Roderick H.J.; Bingol, Aysen; Ogulur, Ismail; Eltan, Sevgi Bilgic; Snow, Andrew L.; Lake, Camille; Fantoni, Giovanna; Alba, Camille; Sellers, Brian; Chauvin, Samuel D.; Dalgard, Clifton L.; Harari, Olivier; Ni, Yan G.; Wang, Ming-Dauh; Devalaraja-Narashimha, Kishor; Subramanian, Poorani; Ergelen, Rabia; Artan, Reha; Guner, Sukru Nail; Dalgic, Buket; Tsang, John; Belkaid, Yasmine; Ertem, Deniz; Baris, Safa; Lenardo, Michael J.; https://orcid.org/0000-0002-8402-8208; https://orcid.org/0000-0002-5214-516X; 33398182; AAI-9346-2021; ABG-5684-2020
CHAPLE disease is a lethal syndrome caused by genetic loss of the complement regulatory protein CD55. Lenardo, Ozen and their colleagues report that blockade of C5 complement activation in a small cohort of pediatric patients with CHAPLE disease reduced gastrointestinal pathology and restored their immunity and growth. Complement hyperactivation, angiopathic thrombosis and protein-losing enteropathy (CHAPLE disease) is a lethal disease caused by genetic loss of the complement regulatory protein CD55, leading to overactivation of complement and innate immunity together with immunodeficiency due to immunoglobulin wasting in the intestine. We report in vivo human data accumulated using the complement C5 inhibitor eculizumab for the medical treatment of patients with CHAPLE disease. We observed cessation of gastrointestinal pathology together with restoration of normal immunity and metabolism. We found that patients rapidly renormalized immunoglobulin concentrations and other serum proteins as revealed by aptamer profiling, re-established a healthy gut microbiome, discontinued immunoglobulin replacement and other treatments and exhibited catch-up growth. Thus, we show that blockade of C5 by eculizumab effectively re-establishes regulation of the innate immune complement system to substantially reduce the pathophysiological manifestations of CD55 deficiency in humans.
Can platelet count/spleen diameter ratio be used for cirrhotic children to predict esophageal varices?
(2016) Sezer, Oya Balci; Celik, Deniz; Tutar, Nihal; Ozcay, Figen; 27957245
AIM To determine the laboratory and radiologic parameters, including the platelet count (PC)-to-spleen diameter (SD) ratio as a non-invasive marker that may predict the presence of esophageal varices (EV) in children with cirrhosis. METHODS Eighty-nine patients with cirrhosis, but without a history of variceal bleeding were prospectively included. The children were grouped into 6-12 and 12-18 years of age groups. These groups were also divided into 2 subgroups (presence and absence of EV). All of the patients underwent a complete biochemical and radiologic evaluation. The PC (n/mm(3))-to-SD (mm) ratio was calculated for each patient. RESULTS Sixty-nine of 98 (70.4%) patients had EV. The presence of ascites in all age groups was significantly associated with the presence of EV. There were no differences in serum albumin levels, PC, SD and the PC-to-SD ratio between the presence and absence of EV groups in both age groups (P > 0.05). CONCLUSION Laboratory and radiologic parameters, including the PC-to-SD ratio as a non-invasive marker (except for the presence of ascites), was inappropriate for detecting EV in children with cirrhosis.
Chronic Enteropathy Associated with SLCO2A1 Gene and Hereditary Fructose Intolerance: A Coincidence of Two Rare Diseases
(2022) Donger, Utku; Warasnhe, Khaled; Ozcay, Figen; Haskologlu, Zehra Sule; Aydin, Halil Ibrahim; Ceylaner, Serdar; https://orcid.org/0000-0002-5214-516X; https://orcid.org/0000-0001-7994-4394; 36384942; ABG-5684-2020; AHD-1839-2022
Chronic enteropathy associated with SLCO2A1 gene (CEAS) is a rare disorder characterized by multiple small intestine ulcers. Patients with CEAS typically present with chronic anemia and gastrointestinal bleeding. Besides CEAS, SLCO2A1 mutations cause primary hypertrophic osteoarthropathy (PHO) which is considered as an extraintestinal manifestation in CEAS patients. Since CEAS and Crohn's disease are clinically indistinguishable, patients are often misdiagnosed with Crohn's disease. Herein, we describe a 4-year-old Turkish girl with CEAS due to homozygous pathogenic variant (c.656C > T) in SLCO2A1 with concomitant hereditary fructose intolerance (HFI) caused by homozygous pathogenic variant (c.1005C > G) in ALDOB. Prompt restriction of fructose, sucrose and sorbitol resulted in hepatomegaly regression and mild amelioration of patient's symptoms. Despite budesonide and azathioprine treatments, patient's protein losing enteropathy and chronic anemia did not improve. Although previous CEAS cases were reported from East Asian countries, it is likely to occur in people from other geographic areas. CEAS seems to be underdiagnosed and high index of suspicion is required for the diagnosis of this rare entity. Patients with prior diagnosis of Crohn's disease with no response to immunosuppressive treatment or anti-TNF therapy should be re-evaluated for possible CEAS diagnosis.
Clinical Features and Outcomes Following SARS-cov-2 Infection in Pediatric Liver Transplant Patients
(2022) Siddiqui, Meraj Alam; Bakirci, Oguz; Donger, Utku; Warasnhe, Khaled; Ozcay, Figen; 0000-0002-5739-6590; 0000-0002-5214-516X; ABF-7609-2022; ABG-5684-2020
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
Diaphragmatic Hernia After Pediatric Liver Transplant
(2015) Kirnap, Mahir; Akdur, Aydincan; Ozcay, Figen; Soy, Ebru; Coskun, Mehmet; Moray, Gokhan; Haberal, Mehmet; 0000-0002-3462-7632; 0000-0002-8726-3369; 0000-0003-2498-7287; 0000-0002-5214-516X; 0000-0001-5630-022X; 0000-0002-0993-9917; 26450470; AAJ-8097-2021; AAA-3068-2021; AAE-1041-2021; ABG-5684-2020; AAM-4120-2021; AAC-5566-2019; AAH-9198-2019
Diaphragmatic hernia is an unusual complication after pediatric liver transplant. Nearly half of bowel obstruction cases, which require surgical intervention in liver transplant patients, are caused by diaphragmatic hernia. The smaller patients are at risk for higher rates of diaphragmatic complication after pediatric liver transplant, but diaphragmatic hernia has not been reported as a unique occurrence. Here, we report 3 cases of diaphragmatic hernia after liver transplant and discuss the possible contributing factors. Diaphragmatic hernia should nevertheless be added to the list of potential complications after liver transplant in the pediatric population. Pediatric transplant physicians and surgeons should be aware of this complication so that it is recognized promptly in both acute and nonacute settings and appropriate action is taken.
Disease Burden And Management Of Crigler-Najjar Syndrome: Report Of A World Registry
(2022) Aronson, Sem J.; Junge, Norman; Trabelsi, Mediha; Kelmemi, Wided; Hubert, Aurelie; Brigatti, Karlla W.; Fox, Michael D.; de Knegt, Robert J.; Escher, Johanna C.; Ginocchio, Virginia M.; Iorio, Raffaele; Zhu, Yan; Ozcay, Figen; Rahim, Fakher; El-Shabrawi, Mortada H. F.; Shteyer, Eyal; Di Giorgio, Angelo; D'Antiga, Lorenzo; Mingozzi, Federico; Brunetti-Pierri, Nicola; Strauss, Kevin A.; Labrune, Philippe; Mrad, Ridha; Baumann, Ulrich; Beuers, Ulrich; Bosma, Piter J.; 35274801
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
Expanded Criteria for Hcc in Liver Transplantation
(2017) Haberal, Mehmet; Akdur, Aydincan; Moray, Gokhan; Arslan, Gulnaz; Ozcay, Figen; Selcuk, Haldun; Ozdemir, Handan; 0000-0002-3462-7632; 0000-0002-8726-3369; 0000-0003-2498-7287; 0000-0002-5214-516X; 0000-0002-8445-6413; 0000-0002-7528-3557; AAJ-8097-2021; AAA-3068-2021; AAE-1041-2021; ABG-5684-2020; AAJ-6976-2021; X-8540-2019
Expanded Criteria for Hepatocellular Carcinoma in Liver Transplant
(2017) Haberal, Mehmet; Akdur, Aydincan; Moray, Gokhan; Arslan, Gulnaz; Ozcay, Figen; Selcuk, Haldun; Ozdemir, Handan; 0000-0002-7528-3557; 0000-0003-2498-7287; 0000-0002-8726-3369; 0000-0002-3462-7632; 0000-0002-8445-6413; 0000-0002-5214-516X; 28302000; X-8540-2019; AAE-1041-2021; AAA-3068-2021; AAJ-8097-2021; AAJ-6976-2021; ABG-5684-2020
Objectives: Hepatocellular carcinoma is the sixth most common cancer worldwide and is the third highest cause of malignancy-related death. Because of its typically late diagnosis, median survival is ap proximately 6 to 20 months, with 5-year survival of < 12%. Hepatocellular carcinoma typically arises in the background of cirrhosis, with liver transplant regarded as the optimal therapy for selected patients. Initially, orthotopic liver transplant was limited to patients with extensive unresectable tumors, resulting in uniformly dismal outcomes due to high tumor recurrence rates. Here, we evaluated our long-term results with expanded-criteria liver transplant. Materials and Methods: From December 1988 to January 2017, we performed 552 liver transplants at Baskent University. In candidates with hepatocellular carcinoma, our expanded criteria for liver transplant is applied regardless of tumor size and number, includes those without major vascular invasion and without distant metastasis, and those with negative cytology (if the patient has ascites). Since 1994, of 61 liver transplants for hepatocellular carcinoma, 36 patients received transplants according to our expanded criteria. Results: Of 36 expanded-criteria patients, 11 were children and 25 were adults. Sixteen patients (4 pediatric, 12 adult) were within our expanded criteria both radiologically and pathologically before trans plant. The other 20 patients (7 pediatric, 13 adult) were within Milan criteria radiologically before transplant; however, after liver transplant, when pathologic specimens were evaluated, patients were found to be within our center's expanded criteria. During follow-up, 9/36 patients (25%) had hepatocellular carcinoma recurrence. In pediatric patients, 5-year and 10-year survival rates were 90%; in adults, 5-year survival was 58.7% and 10-year survival was 49.7%. Overall 5-year and 10-year survival rates were 71.7% and 62.7%. Conclusions: Liver transplant is safe and effective in patients with hepatocellular carcinoma in com bination with interventional radiology procedures, regardless of tumor size and number, without major vascular invasion and distant metastasis.
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
Extensive Indications for HCC in Living Donor Liver Transplantation
(2016) Haberal, Mehmet; Akdur, Aydincan; Moray, Gokhan; Arslan, Gulnaz; Ozcay, Figen; Selcuk, Haldun; Ozdemir, Handan; https://orcid.org/0000-0002-3462-7632; https://orcid.org/0000-0002-8726-3369; https://orcid.org/0000-0003-2498-7287; https://orcid.org/0000-0002-5214-516X; https://orcid.org/0000-0002-8445-6413; AAJ-8097-2021; AAA-3068-2021; AAE-1041-2021; ABG-5684-2020; AAJ-6976-2021
Fecal calprotectin levels in Helicobacter pylori gastritis in children
(2020) Aksoy, Ozlem Yuksel; Canan, Oguz; Hosnut, Ferda Ozbay; Akcay, EdaYilmaz; Ozcay, Figen; 0000-0003-0614-4497; 0000-0002-5214-516X; 33372437; AAI-9386-2021; ABG-5684-2020
Background. Fecal calprotectin is an important inflammatory marker in intestinal diseases and is not routinely used in the upper gastrointestinal system disorders. The aim of this study was to show whether there is a relationship between fecal calprotectin levels and Helicobacter pylori (H pylori) gastritis in children and to determine the association of fecal calprotectin levels with gastric biopsy results in terms of chronic inflammation and neutrophil activity. Methods. Patients with the complaints of the upper gastrointestinal system (epigastric pain, heartburn, nausea and vomiting) who were planned to undergo endoscopy were enrolled prospectively. The presence of H pylori was defined according to the gastric antrum biopsy results. Fecal calprotectin level was tested in the stool sample of the patients. The fecal calprotectin levels, upper gastrointestinal endoscopy and gastric biopsy results of 89 patients were evaluated. Results. H pylori was found to be positive in the gastric biopsies of 51 (57.3%) patients. In the H pylori positive group mean fecal calprotectin level was 74.8 +/- 67 mu g/g, and in the H pylori negative group mean fecal calprotectin level was 52.7 +/- 46 mu g/g and the difference was significant (p= 0.039). We also found a significant relationship between fecal calprotectin levels and gastric neutrophil activity grades (p= 0.034). Conclusions. Mean fecal calprotectin levels were found to be higher in H pylori positive subjects in our study. Fecal calprotectin levels were correlated with gastric neutrophil activity grades. Fecal calprotectin represents gastric neutrophilic inflammation. When interpreting a high fecal calprotectin level, H pylori infection should be kept in mind.
The Frequency of Lysosomal Acid Lipase Deficiency in Children With Unexplained Liver Disease
(2019) Ozcay, Figen; Canan, Oguz; 0000-0002-5214-516X; 30540705; ABG-5684-2020
Objectives: Evidence suggests that lysosomal acid lipase deficiency (LAL-D) is often underdiagnosed because symptoms may be nonspecific. We aimed to investigate the prevalence of LAL-D in children with unexplained liver disease and to identify demographic and clinical features with a prospective, multicenter, cross-sectional study. Methods: Patients (aged 3 months-18 years) who had unexplained transaminase elevation, unexplained hepatomegaly or hepatosplenomegaly, obesity-unrelated liver steatosis, biopsy-proven cryptogenic fibrosis and cirrhosis, or liver transplantation for cryptogenic cirrhosis were enrolled. A Web-based electronic data collection system was used. LAL activity (nmol/punch/h) was measured using the dried blood spot method and classified as LAL-D(<0.02), intermediate (0.02-0.37) or normal (>0.37). Asecond dried blood spot sample was obtained from patients with intermediate LAL activity for confirmation of the result. Results: A total of 810 children (median age 5.6 years) from 795 families were enrolled. The reasons for enrollment were unexplained transaminase elevation (62%), unexplained organomegaly (45%), obesity-unrelated liver steatosis (26%), cryptogenic fibrosis and cirrhosis (6%), and liver transplantation for cryptogenic cirrhosis (<1%). LAL activity was normal in 634 (78%) and intermediate in 174 (21%) patients. LAL-D was identified in 2 siblings aged 15 and 6 years born to unrelated parents. Dyslipidemia, liver steatosis, and mild increase in aminotransferases were common features in these patients. Moreover, the 15-year-old patient showed growth failure and microvesicular steatosis, portal inflammation, and bridging fibrosis in the liver biopsy. Based on 795 families, 2 siblings in the same family were identified as LAL-D cases, making the prevalence of LAL-D in this study population, 0.1% (0.125%-0.606%). In the repeated measurement (76/174), LAL activity remained at the intermediate level in 38 patients. Conclusions: Overall, the frequency of LAL-D patients in this study (0.1%) suggests that LAL-D seems to be rare even in the selected high-risk population.
The Impact of Histopathological Features of Primary Tumor to the Long-Term Outcome of Liver Transplants for Hepatocellular Carcinoma: A 10-Year Follow-Up
(2017) Ozgun, Gonca; Ozdemir, B. Handan; Kirnap, Mahir; Ozcay, Figen; Haberal, Mehmet; 0000-0002-7528-3557; 0000-0002-5214-516X; 0000-0002-3462-7632; X-8540-2019; AAH-9198-2019; ABG-5684-2020; 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