Seak Hee Oh

Long-term outcomes of pediatric living donor liver transplantation at a single institution

Oh SH, Kim KM, Kim DY, Lee YJ, Rhee KW, Jang JY, Chang SH, Lee SY, Kim J‐S, Choi BH, Park S‐J, Yoon CH, Ko G‐Y, Sung K‐B, Hwang G‐S, Choi K‐T, Yu E, Song G‐W, Ha T‐Y, Moon D‐B, Ahn C‐S, Kim K‐H, Hwang S, Park K‐M, Lee Y‐J, Lee S‐G. Long‐term outcomes of pediatric living donor liver transplantation at a single institution.
Pediatr Transplantation 2010: 14:870–878.

Infections after living donor liver transplantation in children.

The aim of this study was to evaluate the infectious complications after living donor liver transplantation (LDLT) in children. We enrolled 95 children (38 boys and 57 girls) who underwent LDLT from 1994 to 2004. The median age was 22 months (range, 6 months to 15 yr). We retrospectively investigated the proven episodes of bacterial, viral, and fungal infection. There occurred 150 infections in 67 (70%) of 95 patients (1.49 infections/patient); 74 in 43 patients were bacterial, 2 in 2 were fungal, and 74 in 42 were viral. The most common sites of bacterial infection were the bloodstream (33%) and abdomen (25%). Most of the bacterial infections occurred within the first month after LDLT. Bacterial and fungal infections did not result in any deaths. The most common causes of viral infection were Epstein-Barr virus in 37 patients and cytomegalovirus in 18. Seven of the 14 deaths after LDLT were associated with viral infection. Our study suggests that infection is one of the important causes of morbidity and mortality after LDLT. Especially careful monitoring and management of viral infections is crucial for improving the outcome of LDLT in children.

Improved outcomes in liver transplantation in children with acute liver failure.

Objective: The aim of our study was to review the experiences of a living donor–dominant transplantation program for children with acute liver failure (ALF). Methods: Data were derived from the retrospective chart review of 50 children with ALF in a major liver center in the Republic of Korea. Results: A total of 50 children with ALF underwent 47 (94%) primary living donor liver transplantations and 3 (6%) cadaveric liver transplantations. The cumulative survival rates of the grafts at 1 and 5 years were 81.9% and 79.2%, respectively. The overall retransplantation rate was 12%. The cumulative survival rates of these patients at 1 and 5 years were all 87.9%. Most incidents of mortality followed the failure of the preceding graft. We observed no mortalities among donors. Based on multivariate analysis, children who had pretransplant thrombocytopenia or had to use the molecular adsorbent recycling system preoperatively were related to the graft loss. Age younger than 2 years and a hyperacute onset (within 7 days) of hepatic encephalopathy were associated with pretransplant thrombocytopenia. Conclusions: Living donor–dominant transplantation program in the present study demonstrates tolerable achievements in terms of clinical outcomes of recipients and donors; however, putative factors, such as pretransplant thrombocytopenia, seem to play unclear roles in a poor prognosis following transplantation.

Nutritional Status and Growth in Korean Children with Crohn's Disease: A Single-Center Study

Background/Aims Malnutrition and growth retardation are important issues in treating pediatric Crohn’s disease (CD). Thus, we aimed to investigate the prevalence of various nutritional and growth parameters at the time of diagnosis in Korean children with CD. Methods Seventy-one children (<18 years) were enrolled. We analyzed the Z-scores of height-for-age (HAZ), weight-for-height (WHZ), body mass index for age (BMIZ), bone mineral density for age (BMDZ), and the biochemical markers measured at the time of diagnosis. Results At diagnosis, HAZ <−2 was observed in three patients (4%), WHZ <−2 in 20 patients (28%), BMIZ <−2 in 19 patients (27%), and BMDZ <−2 in 11 patients (18%). The HAZ was significantly lower in females and patients with extraintestinal manifestations, and the WHZ and BMIZ were significantly lower in patients with stricturing and penetrating disease. Subnormal serum levels were highly prevalent for hemoglobin, albumin, iron, ferritin, calcium, magnesium, folate, vitamin B12, and zinc. There was a significant correlation between nutritional status, growth retardation, and disease activity. Conclusions Abnormal nutritional status was highly prevalent in Korean children with CD at the time of diagnosis and was associated with the extent, behavior, and activity of the disease.

Successful liver transplantation following veno-arterial extracorporeal membrane oxygenation in a child with fulminant Wilson disease and severe pulmonary hemorrhage: a case report.

Son SK, Oh SH, Kim KM, Lee YJ, Jhang WK, Park SJ, Shin HJ, Park J‐J, Kim TH, Kim DY, Hwang S, Park K‐M, Lee Y‐J, Lee S‐G. Successful liver transplantation following veno‐arterial extracorporeal membrane oxygenation in a child with fulminant Wilson disease and severe pulmonary hemorrhage: A case report. 
Pediatr Transplantation 2011.

Lack of common NOD2 mutations in Korean pediatric patients with inflammatory bowel disease

Crohn’s disease (CD) and ulcerative colitis (UC) are idiopathic chronic inflammatory disorders of the gastrointestinal tract. Genetic susceptibility of the host is known as a risk factor playing an important role in the pathogenesis. NOD2 gene variants are widely suspected of increasing susceptibility to CD in the Caucasian and Jewish populations. The prevalence of these diseases is increasing around the world and in Korea in particular. Here, we investigated whether the main NOD2 gene mutations are related to CD and UC in Korean children.

Differential clinical characteristics of acute liver failure caused by hemophagocytic lymphohistiocytosis in children

Children with acute liver failure (ALF) caused by hemophagocytic lymphohistiocytosis (HLH) may be at risk of undergoing unnecessary liver transplantation (LT). The aim of this study was to compare the characteristics of ALF caused by HLH with those of ALF of unknown etiology in children.

Risk factors for neurological complications and their correlation with survival following pediatric liver transplantation.

Despite the improved outcomes of LT, post‐operative NCs remain a significant cause of morbidity and mortality. The aim of the study was to identify the incidence of and risk factors for NCs in children who underwent LT. The medical records of pediatric patients who underwent LT at Asan Medical Center Childrens Hospital between January 1994 and December 2010 were retrospectively analyzed. The onset and types of NC and pretransplant variables associated with NC were evaluated. We identified 190 children (85 boys [44.7%], 105 girls [55.3%]) of mean age 4.1 ± 4.7 yr, who underwent LT. Forty‐six NCs occurred in 41 (21.6%) patients after LT, the most common being seizures (n = 13, 28.3%) and encephalopathy (n = 10, 21.7%). Of the 46 NCs, 24 (52.2%) occurred within three months after LT. Multivariate analysis showed that primary liver disease, preoperative neurological problems, preoperatively higher serum creatinine concentration, and graft failure were significant risk factors for NCs. The survival rate was significantly lower for patients with NCs than for those without (p < 0.001). NCs after pediatric LTs were common and associated with a higher mortality rate in our study. Close monitoring and appropriate risk management may improve the long‐term outcomes of pediatric patients who undergo LT.

Clinical features, outcomes, and genetic analysis in Korean children with Alagille syndrome

Alagille syndrome (AGS) is a multisystem autosomal dominant disorder that affects the liver, heart, eyes, face, bone, and other organs. AGS is caused by mutations in one of two genes, JAG1 or NOTCH2. We evaluated clinical features, outcomes, and the presence of JAG1 and NOTCH2 mutations in Korean children with AGS.

Diagnostic Value of Ceruloplasmin in the Diagnosis of Pediatric Wilson's Disease

Purpose Measurement of serum ceruloplasmin level is the first step in screening for Wilsons disease (WD). Despite the rarity of WD in the general population, ceruloplasmin levels are routinely measured through hepatitis screening in both adults and children. Herein, we evaluated the diagnostic value of ceruloplasmin for the diagnosis of WD among children with hepatitis. Methods We retrospectively reviewed data on serum ceruloplasmin levels measured as a serologic marker for patients with hepatitis at Asan Medical Center (Seoul, Korea) between from January 2004 to November 2013. The diagnosis of WD was confirmed by the identification of pathogenic variants in the ATP7B gene. To determine the diagnostic accuracy of ceruloplasmin, receiver operation characteristic (ROC) curves were constructed and the area under curve (AUC) were calculated. Results Measurements of serum ceruloplasmin were performed in 2,834 children who had hepatitis. Among these, 181 (6.4%) children were diagnosed with WD. The sensitivity, specificity, and accuracy of a ceruloplasmin level of <20 mg/dL in the discrimination of WD were 93.4%, 84.2%, and 84.8%, respectively. In this study, 418 (14.7%) false-positive cases and 12 (0.4%) false-negative cases were noted. Using a ROC curve, a ceruloplasmin level of ≤16.6 mg/dL showed the highest AUC value (0.956) with a sensitivity of 91.2%, a specificity of 94.9%, and an accuracy of 94.7%. Conclusion The measurement of serum ceruloplasmin was frequently used for the screening of WD in children, despite a low positive rate. The diagnostic value of ceruloplasmin may be strengthened by adopting a new lower cut-off level.