• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.2
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• pp.119-126
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• 2021

The aim of our paper was to present current knowledge, review literature and available practice guidelines of international hepatological associations regarding the effect of severe acute respiratory syndrome coronavirus 2 coronavirus on the liver, patients with underline liver disease, awaiting on liver transplantation (LTx) or being after LTx in the pandemic coronavirus disease 2019 area.

• Korean Journal of Radiology
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• v.24 no.5
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• pp.465-475
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• 2023

Objective: To evaluate the feasibility of ultrasound shear wave elastography (SWE) for predicting hepatic fibrosis and native liver outcomes in patients with biliary atresia. Materials and Methods: This prospective study included 33 consecutive patients with biliary atresia (median age, 8 weeks [interquartile range, 6-10 weeks]; male:female ratio, 15:18) from Severance Children's Hospital between May 2019 and February 2022. Preoperative (within 1 week from surgery) and immediate postoperative (on postoperative days [PODs] 3, 5, and 7) ultrasonographic findings were obtained and analyzed, including the SWE of the liver and spleen. Hepatic fibrosis, according to the METAVIR score at the time of Kasai portoenterostomy and native liver outcomes during postsurgical follow-up, were compared and correlated with imaging and laboratory findings. Poor outcomes were defined as intractable cholangitis or liver transplantation. The diagnostic performance of SWE in predicting METAVIR F3-F4 and poor hepatic outcomes was analyzed using receiver operating characteristic (ROC) analyses. Results: All patients were analyzed without exclusion. Perioperative advanced hepatic fibrosis (F3-F4) was associated with older age and higher preoperative direct bilirubin and SWE values in the liver and spleen. Preoperative liver SWE showed a ROC area of 0.806 and 63.6% (7/11) sensitivity and 86.4% (19/22) specificity at a cutoff of 17.5 kPa for diagnosing F3-F4. The poor outcome group included five patients with intractable cholangitis and three undergoing liver transplantation who showed high postoperative liver SWE values. Liver SWE on PODs 3-7 showed ROC areas of 0.783-0.891 for predicting poor outcomes, and a cutoff value of 10.3 kPa for SWE on POD 3 had 100% (8/8) sensitivity and 73.9% (17/23) specificity. Conclusion: Preoperative liver SWE can predict advanced hepatic fibrosis, and immediate postoperative liver SWE can predict poor native liver outcomes in patients with biliary atresia.

• Anatomy and Cell Biology
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• v.51 no.4
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• pp.232-235
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• 2018

Portal vein provides about three-fourths of liver's blood supply. Portal vein is formed behind the neck of pancreas, at the level of the second lumbar vertebra and formed from the convergence of superior mesenteric and splenic veins. The purpose of this study is to review the normal distribution and variation, morphometry of portal vein and its branches for their implication in liver surgery and preoperative portal vein embolization. It is also helpful for radiologists while performing radiological procedures. A total of fresh 40 livers with intact splenic and superior mesenteric vein were collected from the mortuary of Forensic Department, JSS Medical College and Mysuru Medical College. The silicone gel was injected into the portal vein and different segments were identified and portal vein variants were noted. The morphometry of portal vein was measured by using digital sliding calipers. The different types of portal vein segmental variants were observed. The present study showed predominant type I in 90% cases, type II 7.5% cases, and type III 2.5% cases. Mean and standard deviation (SD) of length of right portal vein among males and females were $2.096{\pm}0.602cm$ and $1.706{\pm}0.297cm$, respectively. Mean and SD of length of left portal vein among males and females were $3.450{\pm}0.661cm$ and $3.075{\pm}0.632cm$, respectively. The difference in the Mean among the males and females with respect to length of right portal vein and left portal vein was found to be statistically significant (P=0.010). Prior knowledge of variations regarding the formation, termination and tributaries of portal vein are very helpful and important for surgeons to perform liver surgeries like liver transplantation, segmentectomy and for Interventional Radiologists.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.27 no.1
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• pp.43-52
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• 2024

Purpose: In this study, we investigated the clinical profile, survival at discharge, and proportion of children with acute liver failure (ALF) meeting the criteria for, yet surviving without, liver transplantation (LT). Methods: Medical case records of children aged >28 days to ≤15 years over a period of 7 years, identified from pediatric admission and discharge registers, were screened. Children satisfying the criteria for ALF were included in this study. Results: A total of 71 records meeting the pediatric ALF (PALF) criteria were included. The survival rate at discharge was 61% (n=44). A considerable proportion of children satisfied the King's College Criteria (KCC) (56.3%) and the European Association for the Study of the Liver (EASL) criteria (7%) for LT at admission. Nonetheless, the survival rate in the absence of LT was 42.5% in children who satisfied the KCC and 20% in those who met the EASL criteria. Infection (29.5%) and paracetamol overdose (19.7%) were the major identifiable causes of PALF. Hepatitis A was the most common infection identified. No significant predictors of poor outcomes were identified in multivariable analysis. Conclusion: Our study highlights the changing survival rates and the clinical and etiological profiles of patients with PALF. In areas with poor access to LT services, survival in these children could be improved through early referral to centers with adequate intensive care facilities. Preventing ALF and referring patients to LT services are paramount to reducing mortality.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.4
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• pp.365-368
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• 2018

Glycogen storage disease (GSD) IV is a rare autosomal recessive inherited disorder caused by mutations in the gene coding for glycogen branching enzyme leading to progressive liver disease. GSD IV is associated with mutations in GBE1, which encodes the glycogen branching enzyme. We report a case of GSD IV with rare homozygous mutations in the GBE1 gene (c.791G>A (p.Gly264Glu), which was successfully treated by liver transplantation.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.192-198
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• 2002

Alagille syndrome is characterized by paucity of interlobular bile ducts, chronic cholestasis, characteristic facial abnormalities, cardiovascular abnormalities, posterior embryotoxon, vertebral arch defects, skeletal abnormalities, and glomerular renal involvement. We experienced a case of Alagille syndrome in a 10 month-old male presenting with jaundice. He had chronic cholestasis, characteristic face, cardiovascular abnormalities (aortic stenosis, dextrocardia, double chamber of left ventricle), and situs inversus. Histological examination of liver biopsy specimen revealed paucity of interlobular bile ducts with septal fibrosis, cirrhotic transformation and severe cholestasis. He underwent liver transplantation, but died of cardiopulmonary arrest associated with cardiac anomaly.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.20 no.3
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• pp.178-185
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• 2017

Purpose: To evaluate the outcomes of a hybrid prophylactic strategy to prevent cytomegalovirus (CMV) disease in pediatric liver transplantation (LT) patients. Methods: CMV DNAemia was regularly monitored by quantitative nucleic acid amplification test (QNAT) and was quantified in all children. CMV infection and disease were defined according to the International Consensus Guidelines. The hybrid strategy against CMV infection consisted of universal 3-week prophylaxis and preemptive treatment of intravenous ganciclovir regardless of the recipient's serostatus. Results: A total of 143 children who underwent living donor LT were managed using the hybrid strategy. The overall incidence of CMV infection by QNAT was 48.3% (n=69/143). The highest CMV DNAemia positivity was observed in 49.2% (n=60/122) of children in the D+/R+ group, followed by 46.7% (n=7/15) in the D+/R- group. CMV disease was noted in 26.1% (n=18/69) patients. Forty-three (62.3%) children had undergone preemptive therapy consisting of intravenous ganciclovir. No symptomatic patients developed tissue-invasive disease, resulting in no CMV-associated mortality. Conclusion: The incidence of CMV infection was high in pediatric LT patients despite the hybrid strategy. However, tissue-invasive disease in pediatric LT did not occur.

• Korean Journal of Clinical Pharmacy
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• v.22 no.4
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• pp.324-329
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• 2012

The most common complication after liver transplantation (LT) is bacterial infection. The incidence of surgical site infections (SSIs) after LT was variable from 8.8%~37%. However, there has been no confirmed guideline in use of prophylactic antibiotics after LT. Ampicillin/sulbactam has been used as main prophylactic antibiotics after LT in Seoul National University Hospital (SNUH) according to the center protocol. The purpose of this study was to determine the incidence and risk factors for SSI after LT and to evaluate the appropriateness of prophylactic antibiotics. A total of 211 patients who underwent LT between July 2008 and June 2010 at SUNH were included. During study period, ampicillin/sulbactam was selected as prophylactic antibiotics in 140 patients (66.4%). A total of 43 patients (20.4%) developed infections and the incidence of SSI was noted in 28 patients (13.2%); 10.0% in ampicillin/sulbactam group and 19.4% in other antibiotics (p=0.049). The most common pathogen of SSI was MRSA (13 episodes, 49.4%). In multivariate analysis, choledochojejunostomy (OR: 7.0; 95% CI, 2.4-20.0) and lower serum albumin (OR: 3.7; 95% CI, 1.1-12.9) were found to be risk factors of SSIs. In conclusion, the incidence of SSIs after LT in this population was similar to those in other studies. Therefore, the prophylactic antibiotics protocol in LT at SNUH seems to be appropriate.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.18 no.3
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• pp.175-179
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• 2015

Purpose: Infants with Alagille syndrome (AGS) are occasionally misdiagnosed as biliary atresia and subsequently undergo Kasai operation. The purpose of this study was to investigate the outcome of patients with AGS who had previously received Kasai operation during infancy. Methods: This retrospective study was conducted at the Department of Pediatrics, Samsung Medical Center. We compared the prognosis and mortality between those who had undergone Kasai operation during infancy (Kasai group) and those who had not (non-Kasai group). Results: Among the 15 children with AGS, five had received Kasai operation, while 10 had not. All subjects in the Kasai group revealed neonatal cholestasis, while 70% of the non-Kasai group showed neonatal cholestasis. Liver transplantation was performed in 100% (5/5) among the Kasai group, and 20.0% (2/10) among the non-Kasai group (p=0.007). Mortality was observed in 60.0% (3/5) among the Kasai group, and 10.0% (1/10) among the non-Kasai group (p=0.077). Conclusion: Although overall mortality rate did not significantly differ between the two groups, the proportion of patients receiving liver transplantation was significantly higher in the non-Kasai group. The relatively worse outcome in AGS patients who had received Kasai operation may be due to the unfavorable influences of Kasai operation on the clinical course of AGS, or maybe due to neonatal cholestasis, irrespective of the Kasai operation.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.27 no.1
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• pp.37-42
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• 2024

Purpose: Limited data exist regarding outcome and morbidity associated with portosystemic shunts in the pediatric transplant population. Our study assesses the outcomes of pediatric patients who underwent a portosystemic shunt procedure, both with and without liver transplantation (LT). Methods: This study retrospectively reviewed the medical records of pediatric patients aged 0-19 years who underwent shunt placement between 2003 and 2017 at a tertiary care center. The analysis included cases of shunt placement with or without LT. Results: A total of 13 pediatric patients were included in the study with median age of 8.8 years. Among the cases, 11 out of 13 (84.6%) underwent splenorenal shunt, 1 (7.7%) underwent a mesocaval shunt, and another 1 (7.7%) underwent a Modified Rex (mesoportal) shunt. Additionally, 5 out of 13 (38.5%) patients had LT, with 4 out of 5 (80.0%) receiving the transplant before shunt placement, and 1 out of 5 (20.0%) receiving it after shunt placement. Gastrointestinal bleeding resulting from portal hypertension was the indication in all cases. A total of 10 complications were reported in 5 patients; the most common complication was anemia in 3 (23.1%) patients. At the most recent follow-up visit, the shunts were functional without encephalopathy, and no deaths were reported. Conclusion: Shunt placement plays a crucial role in the management of patients with portal hypertension. Our study demonstrates favorable long-term outcomes in pediatric patients who underwent shunt placement. Long term shunt outcomes were similar and unremarkable in patients with LT and without LT.