Tıp Fakültesi / Faculty of Medicine
Permanent URI for this collectionhttps://hdl.handle.net/11727/1403
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Item 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-8097-2021Urea 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.Item 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-8097-2021Hemophagocytic 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.Item 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-8097-2021Item PMM2-CDG and Sensorineural Hearing Loss(2017) Kasapkara, Cigdem Seher; Baris, Zeren; Kilic, Mustafa; Yuksel, Deniz; Keldermans, Lies; Matthijs, Gert; Jaeken, Jaak; 28762107; AAB-4153-2020Item 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-5684-2020In 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.Item 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-8097-2021Item 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-8097-2021Item 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-8097-2021Item 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-8097-2021Item 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-5684-2020Introduction: 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.