Fakülteler / Faculties
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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 Risk Factors for Postoperative Prolonged Mechanical Ventilation After Pediatric Liver Transplantation(2021) Sahinturk, Helin; Ozdemirkan, Aycan; Zeyneloglu, Pinar; Torgay, Adnan; Pirat, Arash; Haberal, Mehmet; 0000-0002-3462-7632; 0000-0003-0159-4771; 31084587; AAJ-8097-2021; AAJ-1419-2021Objectives: Duration of postoperative mechanical ventilation after pediatric liver transplant may influence pulmonary functions, and postoperative prolonged mechanical ventilation is associated with higher morbidity and mortality. Here, we determined its incidence and risk factors after pediatric liver transplant at our center. Materials and Methods: We retrospectively analyzed the records of 121 children who underwent liver transplant between April 2007 and April 2017 ( 305 total liver transplant procedures were performed during this period). Prolonged mechanical ventilation was defined as postoperative tracheal extubation after 24 hours. Results: Mean age at transplant was 6.2 +/- 5.4 years and 71/121 children (58.7%) were male. Immediate tracheal extubation was achieved in 68 children (56.2%). Postoperative prolonged mechanical ventilation was needed in 12 children (9.9%), with mean extubation time of 78.0 +/- 83.4 hours. Reintubation was required in 13.4%. Logistic regression analysis revealed that presence of preoperative hepatic encephalopathy (odds ratio of 0.130; 95% confidence interval, 0.027-0.615; P =.01), high aspartate amino transferase levels (odds ratio of 1.001; 95% confidence interval, 1.000-1.002; P =.02), intraoperative usage of more packed red blood cells (odds ratio of 1.001; 95% confidence interval, 1.000-1.002; P =.04), and longer surgery duration (odds ratio of 0.723; 95% confidence interval, 0.555-0.940, P =.01) were independent risk factors for postoperative prolonged mechanical ventilation. Although mean length of intensive care unit stay was significantly longer (12.6 +/- 13.6 vs 6.0 +/- 0.6 days; P =.001), mortality was similar in children with and without postoperative prolonged mechanical ventilation. Conclusions: Our results indicate that postoperative prolonged mechanical ventilation was needed in 9.9% of our children. Predictors of postoperative prolonged mechanical ventilation after pediatric liver transplant at our center were preoperative presence of hepatic encephalopathy, high aspartate amino transferase levels, intraoperative usage of more packed red blood cells, and longer surgery duration.