Publications by authors named "Seak Hee Oh"

85 Publications

Pediatric living donor liver transplantation for biliary embryonal rhabdomyosarcoma: a case report of a case showing disease-free survival over 2 years.

Korean J Transplant 2022 Jun 31;36(2):148-153. Epub 2021 Aug 31.

Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Biliary rhabdomyosarcoma is a rare tumor, but it is still the most common tumor of the biliary tract in children. We report a case of a 6-year-old boy with biliary embryonal rhabdomyosarcoma and liver metastasis, which were treated with neoadjuvant and adjuvant chemotherapy combined with living donor liver transplantation (LDLT). Initial imaging studies showed a low-attenuation intraductal mass from the left hepatic duct to the intrapancreatic common bile duct with diffuse upstream dilatation of the intrahepatic duct and liver metastasis. Endoscopic biopsy revealed embryonal rhabdomyosarcoma. After tumor size reduction through neoadjuvant chemotherapy, LDLT was planned to remove the tumor completely. A left lateral section graft weighing 330 g was harvested from his 38-year-old mother and the graft-to-recipient weight ratio was 1.94%. Routine pediatric LDLT operation was performed with deep excavation of intrapancreatic distal bile duct. The explant liver showed minimal residual embryonal rhabdomyosarcoma with no lymph node metastasis. The patient recovered uneventfully from LDLT operation. Scheduled adjuvant chemotherapy was performed for 6 months. The patient is doing well without any evidence of tumor recurrence for 26 months after LDLT. In conclusion, liver transplantation could be an effective treatment for unresectable biliary rhabdomyosarcoma in children according to the location of tumor.
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http://dx.doi.org/10.4285/kjt.21.0008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9296975PMC
June 2022

Living donor liver transplantation in an infant patient with progressive familial intrahepatic cholestasis along with hepatocellular carcinoma: a case report.

Korean J Transplant 2022 Mar;36(1):73-78

Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive inherited disease requiring liver transplantation (LT). Hepatocellular carcinoma (HCC) is very rare in infants. We present a case of living donor LT using a left lateral section graft performed in a 7-month-old female infant diagnosed with PFIC type II and HCC. No mutation on gene was identified. Because of progressive deterioration of liver function, living donor LT with her mother's left lateral section graft was performed. Pretransplant serum alpha-fetoprotein (AFP) level was increased to 2,740 ng/mL, but HCC was not taken into account because of its rarity. The explant liver showed micronodular liver cirrhosis, multiple infantile hemangiomas and two HCCs of 0.7 cm and 0.3 cm in size. The patient recovered uneventfully from the LT operation. This patient has been regularly followed up with abdomen ultrasonography and AFP measurement every 6 months. The patient has been continually doing well for 8 years after the LT. In conclusion, LT is currently the only effective treatment for PFIC-associated end-stage liver diseases. HCC can develop at the cirrhotic liver of any cause, thus elevation of HCC tumor markers in pediatric patients is an important clue to perform further investigation before LT.
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http://dx.doi.org/10.4285/kjt.21.0007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9235533PMC
March 2022

Accuracy of attenuation imaging in the assessment of pediatric hepatic steatosis: correlation with the controlled attenuation parameter.

Ultrasonography 2022 Mar 5. Epub 2022 Mar 5.

Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Purpose: This study evaluated the accuracy of attenuation imaging (ATI) for the assessment of hepatic steatosis in pediatric patients, in comparison with the FibroScan vibration-controlled transient elastography controlled attenuation parameter (CAP).

Methods: Consecutive pediatric patients referred for evaluation of obesity who underwent both ATI and FibroScan between February 2020 and September 2021 were included. The correlation between attenuation coefficient (AC) and CAP values was assessed using the Spearman test. The AC cutoff value for discriminating hepatic steatosis corresponding to a CAP value of 241 dB/m was calculated. Multivariable linear regression analysis was performed to estimate the strength of the association between AC and CAP. The diagnostic accuracy of AC cutoffs was estimated using the imperfect gold-standard methodology based on a two-level Bayesian latent class model.

Results: Seventy patients (median age, 12.5 years; interquartile range, 11.0 to 14.0 years; male:female, 58:12) were included. AC and CAP showed a moderate-to-good correlation (ρ =0.646, P<0.001). Multivariable regression analysis affirmed the significant association between AC and CAP (P<0.001). The correlation was not evident in patients with a body mass index ≥30 kg/m2 (ρ=-0.202, P=0.551). Linear regression revealed that an AC cutoff of 0.66 dB/cm/MHz corresponded to a CAP of 241 dB/m (sensitivity, 0.93; 95% confidence interval [CI], 0.85 to 0.98 and specificity, 0.87; 95% CI, 0.56 to 1.00).

Conclusion: ATI showed an acceptable correlation with CAP values in a pediatric population, especially in patients with a body mass index <30 kg/m2. An AC cutoff of 0.66 dB/cm/MHz, corresponding to a CAP of 241 dB/m, can accurately diagnose hepatic steatosis.
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http://dx.doi.org/10.14366/usg.21246DOI Listing
March 2022

Assessment of pathogens and risk factors associated with bloodstream infection in the year after pediatric liver transplantation.

World J Gastroenterol 2022 Mar;28(11):1159-1171

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul 05505, South Korea.

Background: Bloodstream infection (BSI) is one of the most significantly adverse events that can occur after liver transplantation (LT) in children.

Aim: To analyze the profile of BSI according to the postoperative periods and assess the risk factors after pediatric LT.

Methods: Clinical data, collected from medical charts of children ( = 378) who underwent primary LT, were retrospectively reviewed. The primary outcome considered was BSI in the first year after LT. Univariate and multivariate analyses were performed to identify risk factors for BSI and respective odds ratios (ORs).

Results: Of the examined patients, 106 (28%) experienced 162 episodes of pathogen-confirmed BSI during the first year after LT. There were 1.53 ± 0.95 episodes children (mean ± SD) among BSI-complicated patients with a median onset of 0.4 mo post-LT. The most common pathogenic organisms identified were , followed by and About half (53%) of the BSIs were of unknown origin. Multivariate analysis demonstrated that young age (≤ 1.3 year; OR = 2.1, = 0.011), growth failure (OR = 2.1, = 0.045), liver support system (OR = 4.2, = 0.008), and hospital stay of > 44 d (OR = 2.3, = 0.002) were independently associated with BSI in the year after LT.

Conclusion: BSI was frequently observed in patients after pediatric LT, affecting survival outcomes. The profile of BSI may inform clinical treatment and management in high-risk children after LT.
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http://dx.doi.org/10.3748/wjg.v28.i11.1159DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8985487PMC
March 2022

Clinical characteristics of neonatal cholestasis in a tertiary hospital and the development of a novel prediction model for mortality.

EBioMedicine 2022 Mar 25;77:103890. Epub 2022 Feb 25.

Department of Pediatrics, Asan Medical Center Children's Hospital, University Ulsan College of Medicine, 88, Olympic-ro 43-gil Songpa-Gu, Seoul 05505, South Korea.

Background: Few studies have described the aetiologies of neonatal cholestasis, and the overall neonatal cholestasis-related mortality (NCM) rate is unclear. We investigated the aetiology and outcome of neonatal cholestasis in a tertiary hospital and developed an NCM prediction model for these patients.

Methods: Patients aged <100 days with serum direct bilirubin (DB) levels of >1.0 mg/dL were retrospectively screened. Diagnostic and laboratory data during the 8-week follow-up period after enrolment between 2005 and 2020 were extracted digitally, and medical charts were reviewed manually by clinicians. Logistic regression was used to derive a prediction model for the 1-year mortality outcome of neonatal cholestasis, and performance evaluation and external validation were conducted for the NCM prediction model.

Findings: We enrolled 4028 neonates with DB of >1.0 mg/dL at least once. Prematurity and birth injury (35.4%), complex heart anomalies (18.6%), liver diseases (11.4%), and gastrointestinal anomalies (9.2%) were the most common aetiologies; 398 (9.9%) patients died before one year of age. The peak value of DB was positively correlated to the 1-year mortality rate. In the multivariate analysis, simple laboratory indices, including platelet, prothrombin time, aspartate aminotransferase, albumin, direct bilirubin, creatinine, and C-reactive protein, were independent predictors of 1-year mortality outcome of complete-case subjects. Using these laboratory indices, a logistic regression-based NCM prediction model was constructed. It showed acceptable performances on discrimination (area under the curve, 0.916), calibration (slope, 1.04) and Brier scoring (0.072). The external validation of the sample (n = 920) from two other centres also revealed similar performance profiles of the NCM model.

Interpretation: Various aetiologies of neonatal cholestasis were identified in a tertiary hospital, resulting in unfavourable outcomes of a large proportion. The NCM prediction model may have the potential to help clinicians to be aware of high-risk neonatal cholestasis.

Funding: Ministry of Health & Welfare, Republic of Korea.
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http://dx.doi.org/10.1016/j.ebiom.2022.103890DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8889106PMC
March 2022

Shift to a Younger Age and Regional Differences in Inflammatory Bowel Disease in Korea: Using Healthcare Administrative Data.

Dig Dis Sci 2022 Jan 30. Epub 2022 Jan 30.

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, 88, Olympic-ro 43 gil, Songpa-Gu, Seoul, 05505, Korea.

Background: Research using healthcare administrative data with a validated algorithm can reveal the real-world data of rare diseases.

Aims: We investigated an accurate algorithm for detecting incident cases of inflammatory bowel disease (IBD) from healthcare data and analyzed the nationwide population-based epidemiological features in Korea.

Methods: Healthcare data from Songpa-Kangdong districts in Seoul were extracted from the National Health Insurance Service and analyzed to identify the best algorithm reflecting the cohort data. The most accurate criterion was applied to the entire database for further analysis.

Results: With the selected working criteria, 37,555 incident cases of IBD (Crohn's Disease [CD], 13,130; ulcerative colitis [UC], 24,425) were identified from 2005 to 2016. The male-to-female ratio was 2.5:1 for CD and 1.4:1 for UC. Over 12 years, the annual standardized incidence rate (SIR) per 100,000 people increased from 1.6 to 2.7 and 3.8 to 4.3 for CD and UC, respectively. The peak age at diagnosis of UC shifted from 55-59 years to 20-24 years, whereas that of CD shifted from 19 to 17 years. The SIR of CD was higher in metropolitan areas than in non-metropolitan areas.

Conclusions: This nationwide population-based epidemiologic study of Korean IBD revealed a gradual increase in the incidence rates and a notable shift toward younger age at diagnosis. Males were predominant in both CD and UC. There was an urban-rural difference in the SIR of CD.
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http://dx.doi.org/10.1007/s10620-021-07328-0DOI Listing
January 2022

Gestational alloimmune liver disease treated with exchange transfusion and intravenous immunoglobulin: A case study.

Transfus Apher Sci 2022 Jun 22;61(3):103347. Epub 2021 Dec 22.

Department of Pediatrics, Soonchunhyang University Bucheon Hospital, Soonchunhyang University School of Medicine, Bucheon, South Korea. Electronic address:

Gestational alloimmune liver disease (GALD) is a materno-fetal alloimmune disorder that targets the fetal liver and often causes neonatal liver failure. GALD most commonly presents as neonatal hemochromatosis (NH), which is a severe neonatal liver injury confirmed by extra-hepatic iron accumulation at various sites. With the discovery of the alloimmune mechanism of GALD, exchange transfusion and intravenous immunoglobulin (IVIG) administration are being used as novel treatments. Here, we present a rare case of an 11-day-old female infant who presented with marked hyperbilirubinemia. Laboratory findings showed significantly elevated direct and indirect bilirubin, high ferritin and alpha fetoprotein levels, high transferrin saturation, and severe coagulopathy. Abdominal magnetic resonance imaging revealed markedly reduced T2 signal intensity in the liver and pancreas compared to the spleen, suggesting iron deposition. The infant was diagnosed with NH and successfully treated with exchange transfusion and four doses of IVIG.
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http://dx.doi.org/10.1016/j.transci.2021.103347DOI Listing
June 2022

Liver transplantation in pediatric patients with progressive familial intrahepatic cholestasis: Single center experience of seven cases.

Ann Hepatobiliary Pancreat Surg 2022 Feb;26(1):69-75

Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Backgrounds/aims: Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive inherited disease requiring liver transplantation (LT). The objective of this study was to investigate the clinicopathological features and posttransplant courses of seven LT recipients with PFIC.

Methods: This was a retrospective single-center study of patients with PFIC who underwent LT from January 2013 to June 2020.

Results: Two and five patients were diagnosed with PFIC type 1 and type 2, respectively. For all seven patients, age of PFIC onset was at birth. Jaundice was present in all cases. Mean pretransplant total and direct bilirubin levels were 16.1 ± 8.1 mg/dL and 12.4 ± 6.2 mg/dL, respectively. Median patient age and body weight at LT were 10 months and 7 kg, respectively. Types of donors were mothers of patients in four and deceased donors in three. All five patients with PFIC type 2 recovered uneventfully. One patient each with PFIC type 1 underwent retransplantation due to graft failure or died due to multi-organ failure. Overall graft and patient survival rates at five years were 66.7% and 83.3%, respectively. Bile salt export pump immunohistochemical staining showed normal canalicular expression in two patients with PFIC type 1, focal loss in two patients with PFIC type 2, and total loss in three patients with PFIC type 2.

Conclusions: LT is currently the only effective treatment for PFIC-associated end-stage liver diseases. It is mandatory to perform regular follow-up due to the risk of complications including steatohepatitis, especially for patients with PFIC type 1.
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http://dx.doi.org/10.14701/ahbps.21-114DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8901976PMC
February 2022

Usability of intraoperative cine-portogram during liver transplantation in young pediatric patients with biliary atresia.

Pediatr Transplant 2022 05 9;26(3):e14207. Epub 2021 Dec 9.

Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Background: Pediatric patients with biliary atresia (BA) often present liver cirrhosis-associated portal hypertension and portal vein (PV) hypoplasia. For successful liver transplantation (LT), it is essential to maintain sufficient PV inflow through stenosis-free PV reconstruction with effective ligation of collateral veins. The aim of this study was to assess the clinical usability of intraoperative cine-portogram (IOCP) in young pediatric patients who underwent LT for BA.

Methods: Medical records of pediatric patients younger than 10 years who underwent primary LT for BA from 2018 to 2020 were reviewed.

Results: A total of 31 patients had undergone Kasai portoenterostomy soon after birth. Their median ages at Kasai portoenterostomy and LT were 1 and 11 months, respectively. Types of LT were living-donor LT in 13, deceased-donor split LT in 15, and deceased-donor whole LT in three patients. PV interposition using an iliac vein homograft was performed in 28 patients receiving partial liver grafts. Side-to-side PV unification venoplasty was performed in three patients undergoing whole LT. All patients underwent ligation of collateral veins. IOCP was performed in 6 (19.4%) patients. Four showed no or faint residual venous collaterals. Collateral vein embolization and endovascular stenting were performed in one patient each. PV insufficiency-free survival rate was 100% at 1 year and 93.8% at 3 years. All patients are currently alive with a median follow-up period of 23 months.

Conclusions: Intraoperative cine-portogram can be a useful method for identification and embolization of residual portosystemic collateral veins in young pediatric patients who undergo LT for biliary atresia.
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http://dx.doi.org/10.1111/petr.14207DOI Listing
May 2022

Recent Improvement in Survival Outcomes and Reappraisal of Prognostic Factors in Pediatric Living Donor Liver Transplantation.

Liver Transpl 2022 06 22;28(6):1011-1023. Epub 2021 Oct 22.

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Living donor liver transplantation (LDLT) is a significant advancement for the treatment of children with end-stage liver disease given the shortage of deceased donors. The ultimate goal of pediatric LDLT is to achieve complete donor safety and zero recipient mortality. We conducted a retrospective, single-center assessment of the outcomes as well as the clinical factors that may influence graft and patient survival after primary LDLTs performed between 1994 and 2020. A Cox proportional hazards model was used for multivariate analyses. The trends for independent prognostic factors were analyzed according to the following treatment eras: 1, 1994 to 2002; 2, 2003 to 2011; and 3, 2012 to 2020. Primary LDLTs were performed on 287 children during the study period. Biliary atresia (BA; 52%), acute liver failure (ALF; 26%), and monogenic liver disease (11%) were the leading indications. There were 45 graft losses (16%) and 27 patient deaths (7%) in this population during the study period. During era 1 (n = 81), the cumulative survival rates at 1 and 5 years after LDLT were 90.1% and 81.5% for patients and 86.4% and 77.8% for grafts, respectively. During era 2 (n = 113), the corresponding rates were 92.9% and 92% for patients and 89.4% and 86.7% for grafts, respectively. During era 3 (n = 93), the corresponding rates were 100% and 98.6% for patients and 98.9% and 95.4% for grafts, respectively. In the multivariate analyses, primary diagnosis ALF, bloodstream infection, posttransplant lymphoproliferative disease, and chronic rejection were found to be negative prognostic indicators for patient survival. Based on generalized care guidelines and center-oriented experiences, comprehensive advances in appropriate donor selection, refinement of surgical techniques, and meticulous medical management may eventually realize a zero-mortality rate in pediatric LDLT.
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http://dx.doi.org/10.1002/lt.26308DOI Listing
June 2022

Pediatric deceased donor liver transplantation with size reduction for recipient-graft size matching.

Ann Hepatobiliary Pancreat Surg 2021 Aug;25(3):431-435

Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

We present a case of pediatric deceased donor liver transplantation using a reduced whole liver graft in a 25-month-old boy weighing 12.7 kg. After he had undergone Kasai portoenterostomy for biliary atresia, his general condition deteriorated progressively. He was enrolled on the waiting list for liver transplantation with Pediatric End-stage Liver Disease score of 15. The donor was a 51-monthold boy with body weight of 20 kg. The donor-to-recipient body weight ratio was 158%. The liver graft appeared to be larger than the recipient's abdominal cavity. Thus, we planned to do size reduction. Recipient surgery was performed following standard procedures. We performed graft outflow vein reconstruction using a modified piggyback technique like the double inferior vena cava method. Since the portal vein was hypoplastic, a side-to-side anastomosis technique was used. We also performed intraoperative portogram to embolize venous collaterals. After completing the graft implantation, we found that the liver graft was too large to be accommodated within the abdomen. After resection of the left lateral section parenchyma, we successfully performed primary closure of the abdominal wound. This patient experienced episodes of acute rejection. He has been doing well for four years after the transplantation.
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http://dx.doi.org/10.14701/ahbps.2021.25.3.431DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8382866PMC
August 2021

Dextroplantation of a reduced left lateral section graft in an infant undergoing living donor liver transplantation.

Ann Hepatobiliary Pancreat Surg 2021 Aug;25(3):414-418

Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Graft size matching is essential for successful liver transplantation in infant recipients. We present our technique of graft dextroplantation used in an infant who underwent living donor liver transplantation (LDLT) using a reduced left lateral section (LLS) graft. The patient was an 11-month-old female infant weighing 7.8 kg with hepatoblastoma. She was partially responsive to systemic chemotherapy. Thus, LDLT was performed to treat the tumor. The living donor was a 34-year-old mother of the patient. After non-anatomical size reduction, the weight of the reduced LLS graft was 235 g, with a graft-to-recipient weight ratio of 3.0%. Recipient hepatectomy was performed according to the standard procedures of pediatric LDLT. At the beginning of graft implantation, the graft was temporarily placed at the abdomen to determine the implantation location. The graft portal vein was anastomosed with an interposed external iliac vein homograft. As the liver graft was not too large and it was partially accommodated in the right subphrenic fossa, thus the abdominal wall wound was primarily closed. The patient recovered uneventfully. An imaging study revealed deep accommodation of the graft within the right subphrenic fossa. The patient has been doing well for six months without any vascular complications. This case suggests that dextroplantation of a reduced LLS graft can be a useful technical option for LDLT in infant patients.
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http://dx.doi.org/10.14701/ahbps.2021.25.3.414DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8382867PMC
August 2021

Funneling venoplasty for anomalous graft left hepatic vein in living donor liver transplantation using a split left lateral section graft for an infant patient.

Ann Hepatobiliary Pancreat Surg 2021 Aug;25(3):408-413

Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

The left lateral section (LLS) can have an unusual variant left hepatic vein (LHV) anatomy. We present a case of customized funneling venoplasty of the graft LHV in a 22-month-old girl diagnosed with ornithine transcarbamylase deficiency undergoing deceased donor liver transplantation (LT) using a split LLS graft. The split LLS graft weighed 350 g, yielding a graft-to-recipient weight ratio of 3.2%. Notably, the graft LHV opening was located at the graft liver cut surface, which was only 1 cm in size and 2 cm away from the cephalad apex of the LLS graft. Since such a variant location of the small LHV opening was unsuitable for direct anastomosis, we performed a funneling venoplasty using an inferior vena cava fragment homograft obtained from the same donor. The graft implantation was performed according to standard procedures of infant split LT. Follow-up imaging studies showed no vascular complications. The patient recovered uneventfully from the LT operation. She had normal blood test findings, including normal ammonia level. She has been doing well for 6 months after the transplantation. In conclusion, our surgical technique using a funneling venoplasty enabled successful reconstruction of the anomalous graft LHV. Our results suggest that individualized reconstruction techniques should be applied to infant patients undergoing LT using a LLS graft with variant types of graft LHV anatomy.
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http://dx.doi.org/10.14701/ahbps.2021.25.3.408DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8382873PMC
August 2021

Living donor liver transplantation in a pediatric patient with congenital absence of the portal vein.

Ann Hepatobiliary Pancreat Surg 2021 Aug;25(3):401-407

Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Congenital absence of the portal vein (CAPV) is a rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. We report a case of pediatric living donor liver transplantation (LDLT) for CAPV combined with focal nodular hyperplasia (FNH) and hepatocellular adenoma. A 9-year-old girl who had been diagnosed with multiple FNH had CAPV. Her blood ammonia level was raised to 137 μg/dL. However, she did not complain of any symptoms. To treat CAPV and FNH, we decided to perform LDLT. The graft was a left liver graft from 39-year-old mother of the patient. Recipient hepatectomy was performed according to standard procedures of pediatric LDLT. Portal vein reconstruction was performed using interposition of an iliac vein homograft conduit to the superior mesenteric vein-splenic vein confluence. The CAPV-associated congenital splenorenal shunt was securely ligated. The pathology report of the explant liver showed a 2 cm-sized hepatocellular adenoma and multiple FNH lesions measuring up to 7.1 cm. The patient recovered uneventfully from the LDLT operation. The reconstructed portal vein was maintained well without any hemodynamic abnormalities. In conclusion, as CAPV patients can have various vascular anomalies, combined vascular anomalies should be thoroughly assessed before and during liver transplantation operation. The most effective reconstruction techniques should be used to achieve satisfactory results following liver transplantation.
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http://dx.doi.org/10.14701/ahbps.2021.25.3.401DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8382859PMC
August 2021

Clinical Characteristics and Long-term Outcomes of Pediatric Ulcerative Colitis: A Single-Center Experience in Korea.

Gut Liver 2022 Mar;16(2):236-245

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Background/aims: : Although pediatric ulcerative colitis (UC) has a different phenotype and clinical course than adult UC, its clinical features and outcomes are poorly defined, especially in Asian populations. This study investigated the clinical features and long-term outcomes of pediatric UC in a Korean population.

Methods: We retrospectively analyzed 208 patients aged <18 years diagnosed with UC between 1987 and 2013. The patient characteristics at diagnosis according to the Paris classification and the clinical course were analyzed.

Results: The male-to-female ratio was 1.3:1, and the median patient age was 15.5 years. At diagnosis, 28.8% of patients had proctitis (E1), 27.8%, left-sided colitis (E2); 5.2%, extensive colitis (E3); and 38.2%, pancolitis (E4). The cumulative probabilities of extension after 5, 10, 15, and 20 years were 32.7%, 40.4%, 52.5%, and 65.8%, respectively. Eighteen patients underwent colectomy, and three patients had colorectal cancer. The cumulative probabilities of colectomy after 5, 10, 15, and 20 years were 7.1%, 8.9%, 12.6%, and 15.6%, and those of colorectal cancer after 10, 15, and 20 years were 0%, 2.1%, and 12.0%, respectively. The disease extent, Pediatric Ulcerative Colitis Activity Index severity, and systemic corticosteroid therapy were significant risk factors for colectomy. The development of primary sclerosing cholangitis was significantly associated with colorectal cancer.

Conclusions: This study provides detailed information on the disease phenotype and long-term clinical outcomes in a large cohort of Korean children with UC. They have extensive disease at diagnosis, a high rate of disease extension, and a low rate of cumulative colectomy.
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http://dx.doi.org/10.5009/gnl20337DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8924810PMC
March 2022

Efficacy and safety of autologous adipose tissue-derived stem cell therapy for children with refractory Crohn's complex fistula: a Phase IV clinical study.

Ann Surg Treat Res 2021 Jul 30;101(1):58-64. Epub 2021 Jun 30.

Department of Pediatric Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Purpose: Autologous adipose tissue-derived stem cells (ASCs) have been proposed for patients with refractory Crohn disease, but research is lacking in pediatric patients. This Phase IV study evaluated the efficacy and safety of ASCs in children with refractory Crohn's fistulae.

Methods: Patients with a refractory Crohn's fistula who did not have conventional therapy for more than 3 months or with a recurrent complex Crohn's fistula were included. All patients were at least 14 years old. Patients with infection, poor condition, or active Crohn disease with a disease activity index of 450 and above were excluded. Five patients were treated with ASCs from 2014 to 2015 in Asan Medical Center. ASC administration was adjusted according to fistula size (1 mL per cm). We evaluated the efficacy and safety 8 weeks after injection and followed patients for 6 months.

Results: Fistulae were healed in 4 patients by 8 weeks after ASC injection. Of these 4 patients, 1 had complete fistula closure and sustainability after 6 months. The other 3 with healing effects had less than 50% fistula closure by 6 months. None of these 4 patients have persistent fistulae. One patient had no healing effect, and seton ligation was performed 8 months after ASC injection. There were no adverse effects related to ASC administration.

Conclusion: ASC therapy is a simple and well-tolerated therapeutic option for children with refractory Crohn's complex fistulae. Complete closure was well-sustained. However, more data from a larger number of patients are needed.
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http://dx.doi.org/10.4174/astr.2021.101.1.58DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8255577PMC
July 2021

Third retransplantation using a whole liver graft for late graft failure from hepatic vein stent stenosis in a pediatric patient who underwent split liver retransplantation.

Ann Hepatobiliary Pancreat Surg 2021 May;25(2):299-306

Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

We present a case of third retransplantation using a whole liver graft in a 13-year-old girl who suffered graft failure and hepatopulmonary syndrome following split liver retransplantation with endovascular stenting of the hepatic and portal veins as an infant. She was diagnosed with biliary atresia-polysplenia syndrome, and thus underwent living donor liver transplantation from her mother at 9 months of age. The first liver graft failed due to stenosis of the portal vein. She underwent the second liver transplantation with a split left lateral section graft. Endovascular stenting was performed to the portal vein stenosis 2 months and hepatic vein stenosis 9 months after transplantation. During the next 9 years, 11 sessions of balloon angioplasty for hepatic vein stent stenosis were performed. Ten years after the second transplantation, she underwent third transplantation using a whole liver graft recovered from a 12-year-old-girl. The double inferior vena cava technique was used for outflow vein reconstruction. The graft portal vein was anastomosed with the stent-containing portal vein stump because it was not possible to remove the stent and the inner diameter of the portal vein stent was large enough. An aorto-hepatic jump graft was used for arterial reconstruction. The patient recovered slowly and is doing well for 6 months posttransplant. In conclusion, because stenting of the hepatic vein or portal vein can induce graft failure leading to late retransplantation, we emphasize secure vascular reconstruction to prevent endovascular stenting during LT in infants.
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http://dx.doi.org/10.14701/ahbps.2021.25.2.299DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8180402PMC
May 2021

Efficacy of Living Donor Liver Transplantation in Patients with Methylmalonic Acidemia.

Pediatr Gastroenterol Hepatol Nutr 2021 May 4;24(3):288-294. Epub 2021 May 4.

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Purpose: Despite aggressive medical and nutritional management, patients with methylmalonic acidemia (MMA) often suffer from multi-organ damage. Early deceased donor liver transplantation (DDLT) has emerged as an intervention to prevent disease progression. We investigated the efficacy of living donor LT (LDLT) with a potential carrier of MMA and a small volume of graft in patients with MMA as an alternative to DDLT.

Methods: Of five patients (three male, two female; median age 5.7 years; range, 1.3-13.7 years), four underwent carrier LDLT, while one underwent non-carrier auxiliary LDLT. All patients received pre- and post-LT continuous renal replacement therapy and were provided with minimal restriction diet according to serum MMA level after LT. MMA levels in the serum and urine, the incidence of metabolic crisis, and clinical findings before and after LT were compared.

Results: The survival rate was 100% during 2.2 years of follow up period after LT. In all five cases, MMA titer in the serum after transplantation decreased with less restrictive diet. Metabolic crisis was not observed during the follow-up period. In addition, no patient showed progression of severe renal impairment requiring hemodialysis. Progression of delayed cognitive development was not observed. Social functioning with improved neuropsychiatric development was observed.

Conclusion: This study showed that LDLT achieved improved quality of life with less restrictive diet, therefore it could be a feasible alternative option to DDLT for the treatment of patients with MMA, even with an auxiliary LT.
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http://dx.doi.org/10.5223/pghn.2021.24.3.288DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8128783PMC
May 2021

Recent improvement in survival outcomes and reappraisal of prognostic factors in hepatoblastoma.

Cancer Med 2021 05 3;10(10):3261-3273. Epub 2021 May 3.

Divison of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Background: Prognostic factors in hepatoblastoma need to be reevaluated considering the advances in treatment modalities. The study aimed to evaluate current outcomes of hepatoblastoma and reappraise the association of prognostic factors, including pre-treatment extent of tumor (PRETEXT) stage with annotation factors and Children's Hepatic tumors International Collaboration-Hepatoblastoma Stratification (CHIC-HS) system, with survival outcomes.

Methods: We evaluated 103 consecutive patients with hepatoblastoma retrospectively according to the treatment period based on the introduction of a liver transplantation program.

Results: The 5-year overall survival (OS), event-free survival (EFS), and transplant-free survival rates were 80.2%, 74.2%, and 61.8%, respectively. EFS and OS were improved significantly from 58.6% to 81.6% (P = 0.024) and from 58.6% to 90.8% (P < 0.001), respectively, in the late period (N = 74) compared with the early period (N = 29). The PRETEXT stage was significant or marginally significant for EFS and OS in the early period but not in the late period. The P, F, R, and C factors were significant for OS and EFS in the early period. However, in the late period, only the P factor was significant for OS, and the F and M factors were significant for EFS. The CHIC-HS system was significant or marginally significant for EFS in both the early and late periods; however, it was significant for OS only in the early period.

Conclusion: Survival rates were significantly improved in children with hepatoblastoma, especially in those with advanced PRETEXT stages with positive annotation factors and in a high-risk CHIC-HS group. Prognostic factors had different clinical implications with evolved treatment modalities.
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http://dx.doi.org/10.1002/cam4.3897DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8124118PMC
May 2021

Diagnostic accuracy of simple laboratory tests is not replicable in paediatric fulminant Wilson's disease.

Liver Int 2021 05;41(5):1150-1151

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Republic of Korea.

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http://dx.doi.org/10.1111/liv.14678DOI Listing
May 2021

Risk Factors for Disease Behavior Evolution and Efficacy of Biologics in Reducing Progression in Pediatric Patients with Nonstricturing, Nonpenetrating Crohn's Disease at Diagnosis: A Single-Center Experience in Korea.

Gut Liver 2021 11;15(6):851-857

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Background/aims: : Recently, the treatment of Crohn's disease (CD) has changed to a treat-to-target strategy, in which disease progression is prevented with early intervention. We analyzed the long-term evolution of nonstricturing, nonpenetrating (B1) disease at diagnosis and factors related to disease evolution in pediatric CD.

Methods: We retrospectively analyzed 402 patients between 2000 and 2013 who were younger than 18 years and had B1 disease at CD diagnosis. The median follow-up was 6.1 years (range, 1 to 13 years). The cumulative probabilities of developing stricturing (B2) or penetrating (B3) disease and associations between risk factors and disease behavior evolution were evaluated.

Results: Among the 402 patients, 75 (18.7%) had B2 or B3 disease by the final follow-up. The cumulative probabilities of disease behavior evolution were 18.3%, 34.3%, and 50.9% at 5, 10, and 13 years, respectively. Patients whose disease progressed had an increased risk of intestinal resection (hazard ratio [HR], 3.61; 95% confidence interval [CI], 2.25 to 6.03; p<0.001). First-degree family history of inflammatory bowel disease (HR, 2.38; 95% CI, 1.07 to 5.28; p=0.032), isolated ileal involvement at diagnosis (HR, 7.55; 95% CI, 1.04 to 15.57; p=0.045), and positive anti-Saccharomyces cerevisiae antibody titers (HR, 2.10; 95% CI, 1.03 to 4.25; p=0.040) were associated with disease behavior evolution. Early treatment with biologics significantly reduced disease progression (HR, 0.46; 95% CI, 0.79 to 3.39; p=0.042).

Conclusions: This study suggests that early aggressive therapy should be considered in B1 behavior pediatric CD patients with risk factors of disease evolution to improve long-term outcomes.
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http://dx.doi.org/10.5009/gnl20279DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8593514PMC
November 2021

Pediatric split liver transplantation for congenital factor X deficiency: first 10-year follow-up of a case with portal vein stenting.

Korean J Transplant 2021 Mar 12;35(1):66-70. Epub 2021 Mar 12.

Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Congenital factor X (FX) deficiency is a rare autosomal-recessive disease that induces bleeding disorder. Herein, we present the 10-year posttransplant course of a pediatric patient who underwent liver transplantation (LT) with portal vein (PV) stenting for correction of severe congenital FX deficiency, with focus on long-term maintenance of coagulation function and patency of PV stenting. A 17-month-old infant with recurrent hemorrhagic episodes due to FX deficiency underwent split LT using a left lateral section graft. The graft-recipient weight ratio was 2.2%. The graft implantation procedures were performed by following the standard pediatric split LT procedure. Nevertheless, a wall stent was inserted due to PV anastomotic stenosis on posttransplant day 1. Graft function recovered slowly because of partial parenchyma infarct, and the patient was discharged at 46 days after LT operation. The FX activity started to increase soon after LT and gradually normalized; the coagulation profiles have been maintained well for the past 10 years. The patient has been doing well for the past 10 years after LT without any episodes of abnormal bleeding. Due to the risk of vascular complications owed to PV stenting, life-long follow-up is mandatory with special attention until attainment of complete physical growth to adolescent and adulthood.
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http://dx.doi.org/10.4285/kjt.20.0039DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9235334PMC
March 2021

Pediatric liver transplantation using a hepatitis B surface antigen-positive donor liver graft for congenital absence of the portal vein.

Korean J Transplant 2021 Mar 11;35(1):59-65. Epub 2021 Jan 11.

Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Congenital absence of the portal vein (CAPV) is a rare venous malformation in which mesenteric venous blood drains directly into systemic circulation. Herein, we report a case of pediatric deceased donor liver transplantation (DDLT) for symptomatic CAPV with whole liver graft from a hepatitis B surface antigen (HBsAg)-positive donor. A 4-year-old boy suffered from CAPV and secondary portal hypertension. He was also diagnosed with DiGeorge syndrome and heart anomalies. After waiting for 4 months, a 5-year-old donor weighing 19 kg with positive HBsAg was allocated to this 4-year-old patient weighing 15 kg. Recipient operation was performed according to the standard procedures of pediatric DDLT. Portal vein reconstruction was performed using interposition of a vascular homograft conduit to the superior mesenteric vein-splenic vein confluence. The patient recovered uneventfully from DDLT. He has been administered with lamivudine to prevent hepatitis B virus infection. This patient has been doing well for 5 years after DDLT without growth retardation. In conclusion, CAPV patients can have various vascular anomalies, thus combined vascular anomalies should be thoroughly assessed before and during liver transplantation operation. The most effective reconstruction techniques should be used to achieve satisfactory results following liver transplantation.
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http://dx.doi.org/10.4285/kjt.20.0038DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9235344PMC
March 2021

Interpretation of XIAP Variants of Uncertain Significance in Paediatric Patients with Refractory Crohn's Disease.

J Crohns Colitis 2021 Aug;15(8):1291-1304

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Background And Aims: Mutations in XIAP can lead to the development of treatment-refractory severe paediatric Crohn's disease [CD], for which haematopoietic stem cell transplantation is the primary therapeutic option. The interpretation of variants of uncertain significance [VUSs] in XIAP needs to be scrutinized.

Methods: Targeted next-generation sequencing was performed for 33 male paediatric patients with refractory CD admitted at a tertiary referral hospital. To obtain functional data, biomolecular cell assays and supercomputing molecular dynamics simulations were performed.

Results: Nine unrelated male patients harboured hemizygous XIAP variants. Four known pathogenic variants and one novel pathogenic variant [p.Lys168Serfs*12] were identified in five patients, and two novel VUSs [p.Gly205del and p.Pro260Ser] and one known VUS [p.Glu350del] were identified in the remaining four. Among children with VUSs, only the subject with p.Gly205del exhibited defective NOD2 signalling. Using molecular dynamics simulation, we determined that the altered backbone torsional energy of C203 in XIAP of p.G205del was ~2 kcal/mol, suggesting loss of zinc binding in the mutant XIAP protein and poor coordination between the mutant XIAP and RIP2 proteins. Elevated auto-ubiquitination of zinc-depleted p.G205del XIAP protein resulted in XIAP protein deficiency.

Conclusion: A high prevalence of XIAP deficiency was noted among children with refractory CD. Advanced functional studies decreased the subjectivity in the case-level interpretation of XIAP VUSs and directed consideration of haematopoietic stem cell transplantation.
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http://dx.doi.org/10.1093/ecco-jcc/jjab013DOI Listing
August 2021

Growth, puberty, and bone health in children and adolescents with inflammatory bowel disease.

BMC Pediatr 2021 01 14;21(1):35. Epub 2021 Jan 14.

Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-Ro 43-Gil, Songpa-Gu, 05505, Seoul, Republic of Korea.

Background: Endocrine complications such as impaired growth, delayed puberty, and low bone mineral density (BMD) can be associated with inflammatory bowel disease (IBD) in children and adolescents. This study was performed to investigate the frequency, characteristics, and outcomes of endocrine complications of IBD in children and adolescents.

Methods: This study included 127 patients with IBD diagnosed before 18 years of age [117 with Crohn disease (CD) and 10 with ulcerative colitis (UC)]. Growth profiles, pubertal status, 25-hydroxyvitamin D [25(OH)D] levels, and BMD were reviewed retrospectively.

Results: Short stature was observed in 14 of 127 (11.0 %) with a mean height-SDS of -2.31 ± 0.72. During a 2-year follow-up period, height-SDS did not significantly improve, while weight-SDS significantly improved. Among 109 patients who were older than 13 (girls) or 14 (boys) years of age during the study period, 11 patients (10.1 %) showed delayed puberty, which was associated with low weight-SDS. Vitamin D deficiency was documented in 81.7 % (94/115) with the average 25(OH)D level of 14.5 ± 7.0 ng/mL. Lumbar BMD Z-score was below - 2 SDS in 25 of 119 patients (21.0 %). Height-SDS, weight-SDS, and body mass index (BMI)-SDS were lower in patients with osteoporosis than those without osteoporosis. When pediatric CD activity index scores were high (≥ 30), weight-SDS, BMI-SDS, insulin-like growth factor 1 (IGF-1)-SDS, and testosterone levels were significantly decreased.

Conclusions: Vitamin D deficiency and osteoporosis are common in pediatric IBD patients. As disease severity deteriorates, weight-SDS, IGF-1-SDS, and testosterone levels were decreased. Optimal pubertal development is necessary for bone health.
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http://dx.doi.org/10.1186/s12887-021-02496-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7807425PMC
January 2021

Multiplex gene targeting in the mouse embryo using a Cas9-Cpf1 hybrid guide RNA.

Biochem Biophys Res Commun 2021 02 6;539:48-55. Epub 2021 Jan 6.

Department of Convergence Medicine, Asan Institutes for Life Sciences, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea; Convergence Medicine Research Center, Asan Institute for Life Sciences, Asan Medical Center, Seoul, Republic of Korea. Electronic address:

CRISPR-Cas systems, including Cas9 and Cpf1 (Cas12a), are promising tools for generating gene knockout mouse models. Unlike Cas9, Cpf1 can generate multiple crRNAs from a single concatemeric crRNA precursor, which is favorable for multiplex gene editing. Recently, a hybrid guide RNA (hgRNA) system employing both Cas9 and Cpf1 was developed for multiplex gene editing. As the crRNA of Cpf1 was linked to the 3' end of the sgRNA for Cas9, it can be split into separate guide RNAs by Cpf1. To examine whether this Cas9-Cpf1 hybrid system is suitable for multiplex gene knockouts in the mouse embryo, we generated an hgRNA that simultaneously targets the mouse Il10ra gene by Cas9 and mouse Dr3 (or Tnfrsf25, death receptor3) gene by Cpf1. The expression of hgRNA from a single promoter induced significant indels at each gene in cultured mouse cells upon the co-expression of both Cas9 and Cpf1. Interestingly, the hgRNA exhibited comparable Cas9-mediated indel activity without Cpf1 expression. Similarly, when the hgRNA was co-microinjected with both Cas9 and Cpf1 mRNAs into mouse zygotes at the pronuclear stage, founder mice were generated harboring mutations in both the Il10ra and Dr3 genes. However, when Cas9 mRNA was used alone without Cpf1 mRNA, the mouse Il10ra gene targeting was significantly decreased. These results indicate that the hgRNA system is a possible tool for multiplex gene targeting in the mouse embryo.
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http://dx.doi.org/10.1016/j.bbrc.2020.12.072DOI Listing
February 2021

Ability of Pelvic Magnetic Resonance Imaging to Predict Clinical Course of Perianal Fistula in Paediatric Crohn's Disease Patients.

J Crohns Colitis 2021 Jul;15(7):1152-1160

Department of Pediatrics, University of Ulsan College of Medicine, Seoul, Republic of Korea.

Background And Aims: Evidence is limited regarding clinical course and magnetic resonance imaging [MRI] features of perianal fistula [PAF] in Korean children with Crohn's disease [CD]. We investigated MRI features of PAF and associations with long-term outcomes.

Methods: We retrospectively analysed 243 patients with pelvic MRI for diagnosis of CD. Incidence of clinically evident PAF at diagnosis was determined, as were the proportions of patients with clinical failure [failure to achieve fistula healing within 1 year] and recurrence [new/recurrent PAF after fistula healing within 1 year]. Associations between outcomes and MRI features, specified in modified Van Assche index and MAGNIFI-CD, were evaluated. Associations between later development of clinically evident PAF and subclinical PAF detected on MRI at diagnosis were evaluated.

Results: Among 243 included patients, 108 [44.4%] and 76 [31.3%] had clinically evident and subclinical PAF at diagnosis, respectively; 66.4% of the patients with clinically evident PAF achieved fistula healing within 1 year, and 32.7% of those patients developed recurrence. Fistula length and dominant features of the tracts were associated with clinical failure, and fistula length was associated with recurrence. Clinically evident PAF developed in 17.0% of the patients without clinically evident PAF at diagnosis. We did not find statistically significant association between subclinical PAF and later development of clinically evident PAF [adjusted hazard ratio, 2.438; p = 0.15].

Conclusions: A considerable proportion of Korean paediatric CD patients had clinically evident and subclinical PAF. Fistula length and dominant feature of the tract on MRI are useful predictors of outcomes.
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http://dx.doi.org/10.1093/ecco-jcc/jjab004DOI Listing
July 2021

Unification venoplasty of the outflow hepatic vein for laparoscopically harvested left liver grafts in pediatric living donor liver transplantation.

Korean J Transplant 2020 Dec 16;34(4):293-301. Epub 2020 Dec 16.

Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Laparoscopically harvested left liver (LL) grafts have drawbacks with respect to the size and shape of graft hepatic vein orifices. We present two cases of pediatric living donor liver transplantation (LDLT) using laparoscopically harvested LL grafts and describe refined surgical techniques for graft hepatic vein venoplasty. The first case was a boy aged 4 years and 5 months, with hepatoblastoma. The donor was his 35-year-old mother, and LL graft weighed 315 g. Two separate openings of the graft at the left hepatic vein (LHV) and middle hepatic vein (MHV) were unified through septotomy and septoplasty, and cryopreserved vein homograft patch was attached. Standard procedures of LDLT were performed. This patient recovered uneventfully and has been doing well for 4 years without tumor recurrence. The second case was a 6-year-old girl with ornithine transcarbamylase deficiency. The donor was her 35-year-old mother, and the LL graft weighed 310 g. Two separate openings of graft MHV, with segment III and segment II veins, were unified through septoplasty, and vein patch was attached. Standard procedures of LDLT were performed. This patient has been doing well for 4 years. In conclusion, separate graft hepatic vein openings are a drawback of laparoscopically harvested LL grafts, which thus require unification venoplasty of customized design individually tailored for LL graft and pediatric recipient.
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http://dx.doi.org/10.4285/kjt.20.0027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9186964PMC
December 2020

Pediatric split liver transplantation in a patient with biliary atresia polysplenia syndrome and agenesis of inferior vena cava.

Korean J Transplant 2020 Dec 12;34(4):286-292. Epub 2020 Nov 12.

Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Biliary atresia (BA)-polysplenia syndrome (PS) is diagnosed in a small proportion of BA patients. We present a case of split liver transplantation (LT) successfully performed in a pediatric recipient with BA-PS. The recipient was 29-month-old boy who underwent Kasai procedure. The coexisting malformations included agenesis of the inferior vena cava with azygos vein continuation, polysplenia, intestinal malrotation, truncated pancreas, and preduodenal portal vein and annular pancreas. With pediatric end-stage liver disease score of 33, the patient was allocated for split LT. Under this condition, the left lateral section graft was equivalent to a graft-recipient weight ratio of 2.6%. The recipient surgery was performed according to the standard procedures of pediatric LT. The graft hepatic vein was directly anastomosed with the suprahepatic confluence of the recipient hepatic veins. An external iliac vein homograft was interposed for portal vein reconstruction. Portal collateral veins were embolized intraoperatively to secure portal vein inflow. No surgical complications were developed. Currently, the patient has been doing well for 4 years after transplantation. Our pediatric patient with BA-PS had various anatomical malformations. Thorough preoperative and intraoperative assessment of these anomalies, adoption of customized reconstruction techniques of LT, and careful posttransplant monitoring are necessary for successful LT.
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http://dx.doi.org/10.4285/kjt.20.0023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9186812PMC
December 2020

Whole liver deceased donor liver transplantation for pediatric recipients: single-center experience for 20 years.

Korean J Transplant 2020 Dec 16;34(4):249-256. Epub 2020 Dec 16.

Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Background: We investigated the incidence and outcomes of pediatric deceased donor liver transplantation (DDLT) using whole liver grafts in a high-volume liver transplantation (LT) center.

Methods: The study was a retrospective single-center analysis of whole LT in pediatric recipients. The study period was set as 20 years between January 2000 and December 2019. We defined pediatric recipients and donors to be aged ≤18 years.

Results: During the study period, there were 98 cases of pediatric DDLT, and 34 patients (34.7%) received whole liver grafts. The age range of the deceased donors was 3 months to 56 years and that of pediatric recipients was 7 months to 17 years. Common primary diseases for LT were biliary atresia in 13, acute liver failure in four, Wilson disease in four, congenital portal vein agenesis in three, and genetic metabolic diseases in three. Pediatric-to-pediatric and adult-to-pediatric whole LTs were 22 (64.7%) and 12 (35.3%), respectively. A good correlation was noted between the donor and the recipient's body weight, and the recipient's body weight and allograft's weight. Graft and overall patient survival rates were 91.2% and 91.2% at 1 year, 88.0% and 88.0% at 3 years, and 88.0% and 88.0% at 5 years, respectively.

Conclusions: The results of this study revealed that Korean Network for Organ Sharing (KONOS) regulations with donor-recipient body weight matching exhibited good performance. Considering the reciprocal trades of liver organs among pediatric and adult donors and recipients, it is necessary to establish a policy for pediatric donor liver grafts to pediatric recipients on a priority basis.
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http://dx.doi.org/10.4285/kjt.20.0036DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9186848PMC
December 2020
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