Background/Aims: A liver transplant is the preferred treatment for patients with end-stage liver disease, as it usually results in long-term survival. However, due to the use of chronic immunosuppressive therapy, which is necessary to prevent rejection, de novo cancer is a major risk after transplantation. The aim of this study was to assess the incidence of post-transplant malignancies in children after liver transplantations. Materials and Methods: The study group consisted of 206 liver transplant recipients, with no history of cancer, including hepatocellular carcinoma, in two liver transplantation centers in Turkey between 1997 and 2015. Data were obtained from patient’s data chart. Results: In the study group, de novo cancer was diagnosed in 13 of the 206 patients. Post-transplant lymphoproliferative disease (PTLD) occurred in seven (53.8%) patients and other malignancies in six of the 13 patients. The types of PTLD were as follows: B-cell origin (n=2), Epstein-Barr virus (EBV)-related (n=2), T-cell origin (n=1), and Hodgkin’s lymphoma (n=2). EBV DNA was isolated from seven patients, three of whom developed PTLD. The others developed Kaposi’s sarcomas, Burkitt’s lymphomas, cutaneous large-cell lymphomas, Hodgkin’s lymphomas, and liver sarcomas. Conclusion: After transplantation, immunosuppressive treatment is unavoidable, increasing the risk of malignancies. However, a close follow-up and periodic screening can reduce cancer-related mortality and morbidity. Cite this article as: Karakoyun M, Önen Ş, Baran M, et al. Post-transplant malignancies in pediatric liver transplant recipients: Experience of two centers in Turkey. Turk J Gastroenterol 2018; 29: 85-9.
15 November 2018 in Turkish Journal of Gastroenterology
Cite this article as: Altın Z, Atabay Y, Özer S, et al. Primary intestinal lymphangiectasia and a review of the current literature. Turk J Gastroenterol 2018; 29: 714-6.
25 July 2019 in Turkish Journal of Gastroenterology
Background/Aims: Pediatric intestinal pseudo-obstruction (PIPO) is a severe disorder of gut motility. In this rare and difficult-to-manage disease, complex treatment method, such as intestinal transplantation, is sometimes needed. This study evaluated the management and follow-up results of patients with PIPO who received treatment at our center. Materials and Methods: The cases of 13 patients with PIPO were reviewed retrospectively. Demographic data, clinical features, etiologies, pharmacological and surgical treatments, nutritional support, anthropometric findings, small bowel transplantation (SBT), and survival rates were assessed. Results: Two of the patients were diagnosed at 1 and 5 years of age, while other patients were diagnosed during neonatal period. The etiological cause could not be identified for 5 patients. Pharmacological treatment response was observed in 38.4% of patients. Post-pyloric feeding was applied in 4 patients, but no response was observed. Gastrostomy decreased the clinical symptoms in 3 patients during the abdominal distension period. Total oral nutrition was achieved in 38.4% of the total-parenteral-nutrition (TPN)-dependent patients. It was observed that anthropometric findings improved in patients with total oral nutrition. Liver cirrhosis developed in 1 patient. Venous thrombosis developed in 4 patients. The SBT was performed on 3 patients. One of these patients has been followed up for the last 4 years. Conclusion: Pediatric intestinal pseudo-obstruction is a rare disease that can present with a wide range of clinical symptoms. While some patients require intestinal transplantation, supportive care may be sufficient in others. For this reason, patients with PIPO should be managed individually. Cite this article as: Çağan Appak Y, Baran M, Öztan MO, et al. Assessment and outcome of pediatric intestinal pseudo-obstruction: A tertiary-care-center experience from Turkey. Turk J Gastroenterol 2019; 30(4): 357-63.
Background/Aims: Biliary atresia (BA) is the main cause of neonatal cholestasis and the primary reason for infant liver transplants worldwide. It is an obliterative cholangiopathy observed only in children and caused by progressive inflammation and fibrosis of the bile duct. We collaborated with a liver transplantation center to investigate the effects of follow-up in patients with BA. Materials and Methods: Medical records of 99 patients who were diagnosed with BA and monitored at our center from 1990 to 2002 (27 patients) and from 2003 to 2015 (72 patients) were analyzed retrospectively. Patients were evaluated for birth weight; age at jaundice onset; age at alcoholic stool detection; age at the time of Kasai portoenterostomy (KPE), if performed; age at admission to our center; age at liver transplantation; duration between KPE and transplantation; pediatric end-stage liver disease (PELD) scores during transplantation; and growth and developmental status. The periods 1990-2002 and 2003-2015 were defined as phases I and II, respectively. Results: The median age of the patients at presentation to our hospital was 149 (range: 20-730) days during phase I and 61 (range: 28-720) days during phase II. The median age at jaundice onset was 7 days, and the median age at alcoholic stool detection was 15 days. There was no significant difference between phases I and II in terms of age at jaundice onset, age at alcoholic stool detection, or birth weight. Twenty-five (92.5%) of the 27 patients in the phase I group were admitted to our center after undergoing KPE. Forty-four (61.1%) of the 72 patients in the phase II group (median age at the time of KPE: 47 days) were operated at our center. Median ages of the patients at the time of KPE at our center were 67.5 (range: 25-220) and 47 (range: 28-139) days during phases I and II, respectively. The median age of the 28 patients who were transferred from another center was 70 (range: 45-105) days during phase II. Liver transplantation was performed in 55 of 99 patients (55.5%). Significant differences were observed in the age at transplantation, duration between KPE and transplantation, and PELD scores between patients with BA who underwent KPE at our center and who underwent KPE at other institutes from other institutes. Conclusion: These findings demonstrate the importance of a timely diagnosis of BA and undergoing KPE before malnutrition and/or cirrhosis deteriorate the patient’s health. Furthermore, follow-up of patients with BA at a liver transplantation center increased the success of KPE and improved survival rates. Cite this article as: Karakoyun M, Baran M, Turan C, Kılıç M, Ergun O, Aydoğdu S. Infants with extrahepatic biliary atresia: Effect of follow-up on the survival rate at Ege University Medical School transplantation center. Turk J Gastroenterol 2017; 28: 298-302.