Fakülteler / Faculties
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Item Long-Term Social Outcomes of Pediatric Liver Transplant Recipients: Transition From Childhood to Adulthood(2023) Karakaya, Emre; Erdogan, Ulascan; Saban, Sevval; Teksam, Buse; San, Aydin; Ozcay, Figen; Yildirim, Sedat; Haberal, Mehmet; 38263781Objectives: Chronic disorders may negatively affect people's learning status, marital status, occupational life, and social life. Liver transplant is the only curative treatment for chronic liver diseases. This study was undertaken to evaluate the psychosocial effects of liver transplant in adult patients who had undergone liver transplant during the pediatric period compared with psychosocial facts in the general population. Materials and Methods: We retrospectively reviewed adult patients (>18 years of age) who had received liver transplant as children. We compared sex, age at the time of transplant, current age, type of donor, graft survival status, marital status, age at first delivery, number of children, educational status, and occupational status in the study population versus the general (normal) population. To compare the liver transplant patients included in the study with the general population correctly, we used data from the Turkish Statistical Institute. Results: Among 77 liver transplant patients included in our study, the mean age at transplant was 10.9 years (range, 0.5-16 y) and the mean age at the time of the study was 25.2 years (range, 18-42 y). Of the patients, 61 (79.2%) were single and 16 (20.8%) were married. Patients in the study population married at a younger age than the general population (25.5 vs 28.1 y for men, 24.3 vs 25.4 y for women). Of 16 married patients, 9 (56.2%) had a healthy child or children. The percentage of patients who graduated from higher education or were continuing their higher education process was higher in our study population than in the general population (36.3% vs 22.8%). Among our study population, 37 patients (48%) were workers. Conclusions: Liver transplant had no negative effects on the social, educational, and professional lives among adults in our study who received transplants in the pediatric period.Item Novel Deoxyguanosine Kinase Gene Mutations in the Hepatocerebral Form of Mitochondrial DNA Depletion Syndrome(2015) Sezer, Taner; Ozcay, Figen; Balci, Oya; Alehan, Fusun; 0000-0002-5214-516X; 0000-0002-2278-1827; 0000-0002-8402-8208; 24423689; ABG-5684-2020; AAJ-5931-2021; AAI-9346-2021Deoxyguanosine kinase (DGUOK) gene mutations have been identified in the hepatocerebral form of mitochondrial DNA depletion syndromes. We report here clinical and laboratory features of 3 infants with novel DGUOK gene mutations, c.130G>A (Glu44Lys), c.493G>A (Glu165Lys), and c.707+3_6delTAAG.Item Postoperative Gastrointestinal Bleeding After an Orthotopic Liver Transplant: A Single-Center Experience(2014) Fidan, Cihan; Kirnap, Mahir; Akdur, Aydincan; Ozcay, Figen; Selcuk, Haldun; Arslan, Gulnaz; Moray, Gokhan; Haberal, Mehmet; https://orcid.org/0000-0002-9093-1524; https://orcid.org/0000-0002-8726-3369; https://orcid.org/0000-0002-5214-516X; https://orcid.org/0000-0002-8445-6413; https://orcid.org/0000-0003-2498-7287; https://orcid.org/0000-0002-3462-7632; 24635817; F-5830-2019; AAH-9198-2019; AAA-3068-2021; ABG-5684-2020; AAJ-6976-2021; AAE-1041-2021; AAJ-8097-2021Objectives: The overall incidence, causes, and treatment of posttransplant gastrointestinal bleeding, have been previously described. In this study, we examined the causes and treatment of postoperative gastrointestinal bleeding after orthotopic liver transplant. Materials and Methods: Clinical data of 335 patients who underwent an orthotopic liver transplant at our institution between September 2001 and December 2012 were analyzed retrospectively. The diagnosis and treatment of postoperative gastrointestinal bleeding after an orthotopic liver transplant were reviewed. Results: Gastrointestinal bleeding occurred in 13 patients (3.8%) after an orthotopic liver transplant. Five patients (38.4%) were adult and 8 patients (61.6%) were pediatric. The sites of the bleeding were Roux-en-Y anastomosis bleeding in 5 cases, peptic ulcer in 3 cases, erosive gastritis in 3 cases, gastric and esophageal varices in 1 case, and hemobilia in 1 case. These 13 patients with gastrointestinal bleeding were managed with conservative treatment, endoscopic treatment, radiologic interventional embolism, or exploratory laparotomy. No patients died because of gastrointestinal bleeding. During follow-up, 4 patients died because of sepsis and 1 patient died of recurrence of hepatocellular carcinoma. Conclusions: Gastrointestinal bleeding after liver transplant and its incidence, causes, and treatment are not well-described in the literature. Diagnosis and management of gastrointestinal bleeding requires a multidisciplinary approach involving surgeons, hepatologists, advanced and experienced endoscopists, and interventional radiologists.Item Liver and Kidney Transplant in Primary Hyperoxaluria: A Single Center Experience(2015) Moray, Gokhan; Tezcaner, Tugan; Ozcay, Figen; Baskin, Esra; Akdur, Aydincan; Kirnap, Mahir; Yildirim, Sedat; Arslan, Gulnaz; Haberal, Mehmet; 0000-0002-5735-4315; 0000-0003-2498-7287; 0000-0002-5214-516X; 0000-0002-8726-3369; 0000-0003-4361-8508; 0000-0002-3462-7632; 0000-0002-3641-8674; 25894144; AAF-4610-2019; AAE-1041-2021; ABG-5684-2020; AAA-3068-2021; B-5785-2018; AAJ-8097-2021; AAH-9198-2019; AAD-9865-2021Objectives: Primary hyperoxaluria, especially type 1, is a severe disease with multisystem morbidity and high mortality. We present 3 primary hyperoxaluria type 1 patients who underwent liver transplant, including living-donor liver transplant or combined liver and kidney transplant in our institution. Case Reports: Patients who underwent liver transplant or combined liver/kidney transplant at our institution were evaluated, retrospectively. Between January 2002 and 2013, there were 3 patients who underwent transplant for primary hyperoxaluria. All 3 patients had disease onset in childhood, and the definitive diagnosis was established at age < 1, 6, and 8 years. Although early diagnosis was made, primary hyperoxaluria resulted in end-stage renal disease in 2 patients, and hemodialysis was introduced before liver transplant. All 3 patients underwent living-donor liver transplant. Case 1 was a 10-year-old girl who had an uneventful course after living-donor liver transplant, and she received a living-donor kidney transplant from the same donor 4 months after living-donor liver transplant. Case 2 was a 7-year-old boy who was the younger brother of the first patient; he did not have end-stage renal disease or any renal disorder after successful living-donor liver transplant. Case 3 was a 3-year-old boy who was diagnosed at age 2 months with renal disorders; although he was discharged from the hospital after living-donor liver transplant, he was readmitted because of unconsciousness that developed 1 day after discharge, and he died because of intracranial hemorrhage 2 months after liver transplant, unable to receive a kidney transplant. Conclusions: Primary hyperoxaluria is a rare disorder that is difficult to diagnose until end-organ damage is severe. Outcomes may be improved with early and accurate diagnosis, aggressive supportive treatment, and correction of the enzyme defect by liver transplant before systemic oxalosis develops. However, kidney transplant or combined liver and kidney transplant is required in many primary hyperoxaluria type 1 patients because of the delayed diagnosis or long organ waiting time.Item Large-for-Size Liver Transplant: A Single-Center Experience(2015) Akdur, Aydincan; Kirnap, Mahir; Ozcay, Figen; Sezgin, Atilla; Soy, Hatice Ebru Ayvazoglu; Yarbug, Feza Karakayali; Yildirim, Sedat; Moray, Gokhan; Arslan, Gulnaz; Haberal, Mehmet; 0000-0002-8726-3369; 0000-0002-5735-4315; 0000-0003-2498-7287; 0000-0002-3462-7632; 0000-0002-0993-9917; 0000-0002-5214-516X; 25894137; AAA-3068-2021; AAF-4610-2019; AAE-1041-2021; AAJ-8097-2021; AAH-9198-2019; AAC-5566-2019; ABG-5684-2020Objectives: The ideal ratio between liver transplant graft mass and recipient body weight is unknown, but the graft probably must weigh 0.8% to 2.0% recipient weight. When this ratio > 4%, there may be problems due to large-for-size transplant, especially in recipients < 10 kg. This condition is caused by discrepancy between the small abdominal cavity and large graft and is characterized by decreased blood supply to the liver graft and graft dysfunction. We evaluated our experience with large-for-size grafts. Materials and Methods: We retrospectively evaluated 377 orthotopic liver transplants that were performed from 2001-2014 in our center. We included 188 pediatric transplants in our study. Results: There were 58 patients < 10 kg who had living-donor living transplant with graft-to-body-weight ratio > 4%. In 2 patients, the abdomen was closed with a Bogota bag. In 5 patients, reoperation was performed due to vascular problems and abdominal hypertension, and the abdomen was closed with a Bogota bag. All Bogota bags were closed in 2 weeks. After closing the fascia, 10 patients had vascular problems that were diagnosed in the operating room by Doppler ultrasonography, and only the skin was closed without fascia closure. No graft loss occurred due to large-for-size transplant. There were 8 patients who died early after transplant (sepsis, 6 patients; brain death, 2 patients). There was no major donor morbidity or donor mortality. Conclusions: Large-for-size graft may cause abdominal compartment syndrome due to the small size of the recipient abdominal cavity, size discrepancies in vascular caliber, insufficient portal circulation, and disturbance of tissue oxygenation. Abdominal closure with a Bogota bag in these patients is safe and effective to avoid abdominal compartment syndrome. Early diagnosis by ultrasonography in the operating room after fascia closure and repeated ultrasonography at the clinic may help avoid graft loss.Item Results of Pediatric Liver Transplant: A Single-Center Experience(2015) Moray, Gokhan; Tezcaner, Tugan; Akdur, Aydincan; Ozcay, Figen; Sezgin, Atilla; Kirnap, Mahir; Yildirim, Sedat; Arslan, Gulnaz; Haberal, Mehmet; 0000-0002-3641-8674; 0000-0002-8726-3369; 0000-0002-3462-7632; 0000-0002-5735-4315; 0000-0002-5214-516X; 0000-0003-2498-7287; 25894129; AAH-9198-2019; AAD-9865-2021; AAA-3068-2021; AAJ-8097-2021; AAF-4610-2019; ABG-5684-2020; AAE-1041-2021Objectives: Liver transplant is an established curative therapy for children with chronic end-stage liver disease or acute liver failure. In this study, we aimed to evaluate pediatric liver transplant in terms of outcomes, complications, and long-term follow-up results. Materials and Methods: Pediatric patients who had liver transplant in our institution were included. We retrospectively evaluated demographic features including body weight, Child-Pugh score, etiology of liver disease, graft source, perioperative outcomes, perioperative complications, postoperative complications, and long-term results. Outcomes of treatment of complications and revision transplant were evaluated. Results: Between September 2001 and December 2013, there were 188 pediatric liver transplants performed in our institution. Most grafts (90.9%) were obtained from living-related donors. There were 13 patients (6.9%) who had an intervention because of a hemorrhage postoperatively. Biliary leakage was observed in 33 patients (17.5%) and biliary stricture during follow-up was observed in 32 patients (17%). Thrombosis rates in the hepatic artery and portal vein were 12.3% and 0.5%. Revision transplant was performed in 11 patients (5.8%); reason for revision transplant was rejection in 50% patients. The remaining children were alive with good graft functioning after treatment of complications and revision transplant. The overall 5- and 10-year survival rates were 82.3% and 78.9%. Conclusions: The overall outcomes of pediatric liver transplant at our center are very promising. With improved care of younger children and the combined efforts of the parents and medical team, the number of the children receiving transplants will increase in the future.Item Liver Transplant for Fulminant Hepatic Failure: A Single-Center Experience(2015) Kirnap, Mahir; Akdur, Aydincan; Ozcay, Figen; Soy, Ebru; Yildirim, Sedat; Moray, Gokhan; Haberal, Mehmet; 0000-0002-8726-3369; 0000-0003-2498-7287; 0000-0002-3462-7632; 0000-0002-0993-9917; 0000-0002-5214-516X; 0000-0002-5735-4315; 26029995; AAH-9198-2019; AAA-3068-2021; AAE-1041-2021; AAJ-8097-2021; AAC-5566-2019; ABG-5684-2020; AAF-4610-2019Objectives: Acute liver failure is a life-threatening condition with sudden onset liver injury, decreased liver functions, hepatic encephalopathy, and coagulopathy in patients without preexisting liver disease. In this study, we sought to evaluate the results of liver transplant as a treatment for acute liver failure. Materials and Methods: Between November 1988 and March 2015, we performed 482 liver transplants in 471 patients. We performed 36 liver transplants in 35 patients because of acute liver failure. Only 5 of these were from deceased donors. Thirty of those 34 patients were pediatric (85%) and 5 were adults (15%). Results: Five patients died (4 in early postoperative period and 1 during the 18th month of living-donor liver transplant). We diagnosed 11 acute rejections (32%); 6 biliary leaks (17%); 6 intraabdominal hemorrhage (17%); 5 hepatic arterial thromboses (15%), and 1 venous complication (3%) during the early postoperative period. We have no morbidity or mortality in living-donor liver transplants. Conclusions: Living-donor liver transplants are an efficient and successful treatment for acute liver failure patients. In our center, we mostly consider and prefer living-donor liver transplants to deceased-donor liver transplant because of the paucity of organ donation, especially for pediatric patients. Considering acceptable postoperative complications, living-donor liver transplant is a lifesaving treatment for acute liver failure.Item Is It Necessary To Re-Evaluate Diagnostic Criteria For Wilson Disease in Children?(2014) Sezer, Oya Balci; Perk, Peren; Hosnut, Ferda Ozbay; Kose, Serdar Kenan; Ozcay, Figen; https://orcid.org/0000-0002-8402-8208; https://orcid.org/0000-0002-5214-516X; 25599783; AAI-9346-2021; GSJ-0760-2022; ABG-5684-2020Background/Aims: The differential diagnosis of Wilson Disease (WD) is challenging, especially in children, because liver copper levels may also increase in other chronic liver diseases with bile stasis. The aim of this study is to determine urine and liver copper cut-off values to differentiate WD from other chronic liver diseases (non-WD, NWD) in children. Materials and Methods: Seventy-six patients participated in the study, 35 with WD and 41 with NWD. The two groups were divided into two subgroups according to the presence of cholestasis. At the time of diagnosis, age, sex, biochemical test results, serum ceruloplasmin, baseline 24-h urinary copper levels, liver biopsy histological findings, liver copper levels, and Child-Pugh scores were obtained from medical records. Copper content in liver tissue and copper levels in urine were measured by atomic absorption spectrometry. Cut-off values for differentiation of WD from NWD were determined by receiver operating characteristic (ROC) analysis. Results: A liver copper cut-off value of 98 mu g/g indicated WD with 91% sensitivity and 65.4% specificity (area under the curve =0.838, 95% CI: 0.749-0.927). A 24-h urinary copper cut-off value of 67.5 mu g/24h indicated WD with 85% sensitivity and 71% specificity (area under the curve =0.843, 95% CI: 0.752-0.934). Conclusion: In this study of pediatric chronic liver disease patients, copper cut-off values for distinguishing WD differed substantially from those used for diagnosis. A larger scale study is warranted to re-evaluate liver copper and 24-h urinary copper cut-offs for children with suspected WD.Item 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-9198-2019Diaphragmatic 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.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.
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