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Background: Pediatric inflammatory bowel disease (PIBD) affects different age groups and its incidence is increasing worldwide. However, there is a lack of research focusing on age subgroups in Asian countries. In this nationwide population‐based study, we investigated the epidemiology of PIBD among different age subgroups in Korea. Methods: We analyzed Korean health administration data from 2005 to 2016. Data were divided by age at diagnosis as follows: group 1, 0–1 years; group 2, 2–5 years; group 3, 6–9 years; group 4, 10–16 years. We analyzed the overall incidence, temporal changes, and regional differences by age subgroups, using Poisson regression analysis. Results: From 2005 to 2016, 2734 inflammatory bowel disease (IBD) cases were diagnosed among patients under 17 years of age. In the overall population, the incidence rate of PIBD over the entire study period was 2.248/105 person‐years (PY), significantly increasing from 1.173/105 PY in 2005–2007 to 3.267/105 PY in 2014–2016. The incidence rates in groups 1 and 2 remained unchanged, whereas those of groups 3 and 4 increased significantly. The same trend was observed when analyzed separately for Crohn's disease (CD) and ulcerative colitis (UC). The incidence rates of CD in groups 3 and 4 showed differences between metropolitan and non‐metropolitan areas, whereas those in groups 1 and 2, and UC of all age subgroups showed no difference. Conclusions: The temporal trend and regional differences of PIBD differed among age subgroups, suggesting that genetic and environmental factors have varying impacts on IBD development across different subgroups. [ABSTRACT FROM AUTHOR]

Pediatric inflammatory bowel disease (PIBD) is rising rapidly in many industrialized and affluent areas in the Asia‐Pacific region. Current available guidelines, mainly from Europe and North America, may not be completely applicable to clinicians caring for children with PIBD in this region due to differences in disease characteristics and regional resources constraints. This position paper is an initiative from the Asian Pan‐Pacific Society for Pediatric Gastroenterology, Hepatology and Nutrition (APPSPGHAN) with the aim of providing an up‐to‐date, evidence‐based approach to PIBD in the Asia‐Pacific region, taking into consideration the unique disease characteristics and financial resources available in this region. A group of pediatric gastroenterologists with special interest in PIBD performed an extensive literature search covering epidemiology, disease characteristics and natural history, management, and monitoring. Gastrointestinal infections, including tuberculosis, need to be excluded before diagnosing IBD. In some populations in Asia, the Nudix Hydrolase 15 (NUD15) gene is a better predictor of leukopenia induced by azathioprine than thiopurine‐S‐methyltransferase (TPMT). The main considerations in the use of biologics in the Asia‐Pacific region are high cost, ease of access, and potential infectious risk, especially tuberculosis. This position paper provides a useful guide to clinicians in the medical management of children with PIBD in the Asia‐Pacific region. [ABSTRACT FROM AUTHOR]

Disease phenotype of pediatric inflammatory bowel disease (PIBD) in children from the Asia‐Pacific region differs from that of children from the West. Many parts of Asia are endemic for tuberculosis, making diagnosis and management of pediatric Crohn's disease a challenge. Current available guidelines, mainly from Europe and North America, may not be completely applicable to clinicians caring for children with PIBD in Asia due to differences in disease characteristics and regional resource constraints. This position paper is an initiative from the Asian Pan‐Pacific Society for Pediatric Gastroenterology, Hepatology and Nutrition (APPSPGHAN) that aims to provide an up‐to‐date, evidence‐based approach to PIBD in the Asia‐Pacific region. A group of pediatric gastroenterologists with a special interest in PIBD performed an extensive literature search covering epidemiology, disease characteristics and natural history, management, and monitoring. Attention was paid to publications from the region with special consideration to a resource‐limited setting. This current position paper deals with surgical management, disease monitoring, immunization, bone health, and nutritional issues of PIBD in Asia. A special section on differentiating pediatric Crohn's disease from tuberculosis in children is included. This position paper provides a useful guide to clinicians in the surgical management, disease monitoring, and various health issues in children with IBD in Asia‐Pacific region. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Summary: Spent fuel dry storage cask, which stores the spent fuel from wet storage facility to final processing, has been developed and managed. Even though thermal management mechanisms of dry storage casks secure the thermal integrity of the cask, improvement of thermal management capacity of dry storage cask would enhance thermal margin against unforeseen off‐normal conditions with increasing specific storage capacity. Ulsan National Institute of Science and Technology (UNIST) CANister (UCAN) utilizing the new‐type thermosyphon heat pipe, is under development as an advanced design of spent fuel dry storage cask. Hybrid control rod‐heat pipe, which is an annular thermosyphon by containing neutron absorber, is a key component of UCAN. Heat removal performance of the hybrid control rod‐heat pipe was analyzed bya series of experiment withtest facility simulating single fuel assembly with full height. The UCAN fuel assembly showed the reduced structural and fuel temperatures compared to bare fuel assembly of conventional cask designs, exhibitingimproved thermal margin. According to experimentally observed heat removal rate of the hybrid control rod‐heat pipe, UCAN design could extend manageable thermal capacity from 13.3% to 33.8%. The removeddecay heat through hybrid control rod‐heat pipe could be reused in electricity generation with stirling engine and thermoelectric devices improving energy management efficiency. Intermittent boiling accompanying geyser phenomena was frequently occurred inside the test section, and the boiling behaviors according to fill ratios (FRs) and operating pressures dominated the heat removal performances. The hydrostatic pressure owing to large aspect ratio affected differentiated thermal‐hydraulic behaviors compared to conventional thermosyphons. To develop heat transfer and hydraulic models of the hybrid control rod‐heat pipe, geyser phenomenon inside the test section wasdiscussed physically. This workexhibited the feasibility of UCAN design and provides fundamental physics on intermittent boiling of thermosyphon operating at sub‐atmospheric pressures. [ABSTRACT FROM AUTHOR]

Objectives: To investigate the diagnostic value of transient elastography (TE), 2‐dimensional (2D) shear wave elastography (SWE), and the serologic fibrosis indices aspartate transaminase–to–platelet ratio index (APRI) and Fibrosis‐4 (FIB‐4) score for Wilson disease (WD). Methods: We retrospectively identified patients with a diagnosis of WD who underwent TE and 2D SWE on the same day. Their APRI and FIB‐4 scores were calculated. Hepatic involvement was classified into 5 clinical categories (I–V) based on the laboratory findings, hepatic morphologic characteristics on ultrasound (US) imaging, and clinical symptoms of cirrhosis: I, normal (n = 17); II, only biochemical abnormality (n = 15); III, altered hepatic morphologic characteristics (n = 10); IV, compensated liver cirrhosis (n = 3); and V, decompensated liver cirrhosis (n = 0). We compared the area under the receiver operating characteristic curve (AUROC) data for TE, 2D SWE, the APRI, and the FIB‐4 score. A combined assessment of the serologic markers and US elastography was performed, and the AUROCs of the combinations were compared. Results: Forty‐five patients were included in the study (median age, 16.0 years; range, 3–35 years). Transient elastography, 2D SWE, and APRI were comparable in distinguishing the clinical categories (AUROC, 0.799–0.928). The FIB‐4 score showed lower diagnostic value in distinguishing clinical category I from the other categories (AUROC, 0.647). Combining the serologic markers and US elastography significantly increased the AUROC value of the FIB‐4 score (with TE and 2D SWE, P =.01 and.02). Conclusions: Transient elastography and 2D SWE showed excellent diagnostic accuracy for differentiating the clinical categories of hepatic involvement. The APRI showed better diagnostic performance than the FIB‐4 score. The assessment of hepatic manifestations in WD can be improved by combining US elastography with serologic indices. [ABSTRACT FROM AUTHOR]

This study aimed to investigate the incidence of PTLD in pediatric liver transplant recipients and the risk factors for the development of PTLD. We also determined clinically useful quantitative EBV PCR parameters for aiding in the diagnosis of EBV‐associated PTLD in the pediatric liver transplant recipients at our institute. We reviewed children < 18 years old who had undergone liver transplantations and quantitative analysis of whole blood EBV load at our institute from January 2006 to March 2015. A total of 142 liver transplant recipients were included, and their median age was 1.5 years. Clinically significant high‐level EBV DNAemia ≥ 10 000 copies/mL at least twice was observed in 53.5% and PTLD occurred in 9.9%. Among PTLD group, graft failure and mortality rate were as high as 21.4% and 14.3%, respectively. Deceased donor, presence of high‐level EBV DNAemia, and primary CMV infection following transplant were associated with an increased risk for PTLD in the multivariate analysis. The peak titer at 10 875 copies/mL could be used as a cutoff value with a sensitivity of 92.9% and a specificity of 37.9%; the rate of increase in EBV load suggested a sensitivity of 64.3% and a specificity of 70.9% at the cutoff value of 44 000 copies/mL/week. In conclusion, the incidence of PTLD following liver transplant in children was as high as 10%. PTLD is associated with significant morbidity and mortality. Close monitoring of EBV DNAemia is crucial for the early diagnosis and proper treatment of PTLD in pediatric liver transplant recipients. [ABSTRACT FROM AUTHOR]

Background and Aim: Anti‐tumor necrosis factor (TNF) agents, such as infliximab (IFX), have been increasingly used to induce and maintain disease remission in patients with Crohn's disease (CD). Despite a considerable non‐response rate, little is known about the genetic predictors of response to anti‐TNF therapy in CD. Our aim in this study was to investigate the genetic factors associated with response to anti‐TNF therapy in patients with CD. Methods: We performed a two‐stage genome‐wide association study (GWAS) to identify loci influencing the response to IFX among Korean patients with CD, comprising 42 good responders with mucosal healing and 70 non‐responders. The achievement of mucosal healing was assessed by endoscopy and imaging. The functional significance of TRAP1 (TNF receptor associated protein 1) was examined using dextran sodium sulfate‐induced colitis model in TRAP1 transgenic mice. Results: The GWAS identified rs2158962, an intronic single nucleotide polymorphism (SNP) of TRAP1, significantly associated with mucosal healing (odds ratio = 4.94; Pcombined = 1.35 × 10−7). In the dextran sodium sulfate‐induced acute colitis, TRAP1 transgenic mice showed a better response to IFX than the wild‐type mice. Conclusions: The TRAP1 gene is associated with mucosal healing in CD patients following IFX therapy. Identifying the genetic predictors of mucosal healing to anti‐TNF therapy can prevent patients from exposure to ineffective therapies. [ABSTRACT FROM AUTHOR]

Objectives: To assess the diagnostic performance of ultrasound (US) elastography in evaluating portal hypertension in children and compare the liver and spleen stiffness values between the portal hypertension and control groups. Methods: Studies in the MEDLINE and Embase databases were selected that investigated the diagnostic performance of US elastography in children with portal hypertension up to December 21, 2017. Pooled sensitivity and specificity data were assessed by hierarchical logistic regression modeling. Results: Eleven studies were included in the systematic review, and a meta‐analysis could be conducted in 7 of these publications to evaluate the diagnostic performance of US elastography. The summary sensitivity and specificity of this method for liver stiffness were 90% (95% confidence interval [CI], 83%–94%) and 79% (95% CI, 73%–84%), respectively, and the area under the hierarchical summary receiver operating characteristic curve was 0.92 (95% CI, 0.90–0.94). A subgroup analysis of 5 transient elastographic studies revealed similar diagnostic performance (sensitivity, 90%; specificity, 78%). In 10 of the 11 studies that investigated liver stiffness and 2 of the 3 studies that also measured spleen stiffness, patients in the portal hypertension group had a significantly higher stiffness value than the control group (P < .05). Conclusions: Ultrasound elastography shows good performance in diagnosing portal hypertension and can identify significant differences in liver and spleen stiffness in children with this condition. This method thus has considerable potential as a noninvasive tool for screening portal hypertension–related complications in children with chronic liver disease. [ABSTRACT FROM AUTHOR]

Abstract: Background and Aim: CDKN2A/CDKN2B locus on 9p21 is reported to be associated with various diseases, including cancer and cardiovascular and inflammatory diseases. Significant downregulation of CDKN2B‐AS1 in inflamed colon tissue of inflammatory bowel disease (IBD) cases was reported in Europeans. This study aimed to confirm the suggestive association of CDKN2A/CDKN2B with IBD identified in our recent genome‐wide association study (GWAS). Methods: We examined the association of CDKN2A/CDKN2B locus with IBD in an additional sample of 574 IBD cases and 542 controls, totaling 4068 cases and 8074 controls. In silico study was performed at various levels for functional annotation of the causal variant. Co‐localization of the GWAS association signals and the corresponding expression quantitative trait loci in IBD‐related tissues was evaluated using eCAVIAR. Results: An expanded GWAS showed genome‐wide significant association of rs3731257 at 9p21 with IBD (odds ratio = 1.17, 95% confidence interval = 1.12–1.22, Pcombined = 5.68 × 10−9) and Crohn's disease (odds ratio = 1.22, 95% confidence interval = 1.15–1.28, Pcombined = 8.85 × 10−9) in the Korean population. Co‐localization study suggested that both CDKN2B‐AS1 and CDKN2A might be functionally associated with the locus in the small intestine. Conclusions: rs3731257 in CDKN2A/CDKN2B is an IBD‐susceptible locus in Koreans, with a suggestive role for small intestine‐specific gene regulation. Our findings suggested that alterations of the CDKN2A/CDKN2B locus could affect the pathophysiology of IBD. [ABSTRACT FROM AUTHOR]

The popular textbook image of viruses as noxious and selfish genetic parasites greatly underestimates the beneficial contributions of viruses to the biosphere. Given the crucial dependency of viruses to reproduce in an intracellular environment, viruses that engage in excessive killing (lysis) can drive their cellular hosts to extinction and will not survive. The lytic mode of virus propagation must, therefore, be tempered and balanced by non-lytic modes of virus latency and symbiosis. Here, we review recent bioinformatics and metagenomic studies to argue that viral endogenization and domestication may be more frequent mechanisms of virus persistence than lysis. We use a triangle diagram to explain the three major virus persistence strategies that explain the global scope of virus-cell interactions including lysis, latency and virus-cell symbiosis. This paradigm can help identify novel directions in virology research where scientists could artificially gain control over switching lytic and beneficial viral lifestyles. Also see the Video Abstract: [ABSTRACT FROM AUTHOR]

Background: Genome-wide association studies have identified tumor necrosis factor superfamily member 15 (TNFSF15) as a Crohn's disease (CD)-related gene. The aim of this study was to evaluate the association between five TNFSF15 polymorphisms and CD in Korean children and analyze their genotypes in relation to phenotype. Methods: Five single-nucleotide polymorphisms of TNFSF15 (rs3810936, rs6478108, rs6478109, rs7848647, rs7865494) were genotyped in 108 CD patients and in 599 healthy controls. Risk allele, genotype, and haplotype were analyzed in CD patients and controls, and genotype-phenotype relationships were studied. Results: There were significant associations of rs3810936, rs6478108, rs6478109, rs7848647 with CD in Korean pediatric patients (P = 6.5×10-8, P = 1.3×10-8, P = 3.7×10-8, P = 2.9×10-8, respectively). The adjusted OR (aOR) for the homozygous risk allele genotype was significantly higher than that for the homozygous genotype for the opposite allele: rs3810936, aOR, 5.36 (95%CI: 2.61-10.98, P = 4.6×10-6); rs6478108, aOR, 6.62 (95%CI: 3.03-14.46, P = 2.2×10-6); rs6478109, aOR, 6.24 (95%CI: 2.85-13.66, P = 4.6×10-6); rs7848647, aOR, 6.32 (95%CI: 2.89-13.81, P = 3.8×10-6). The risk allele of rs3810936 was associated with later symptom onset, later diagnosis, and the presence of perianal lesion (P = 0.013, P = 0.016 and P = 0.029). Conclusion: There was a significant association of TNFSF15 with pediatric CD in Korean patients. [ABSTRACT FROM AUTHOR]

Background 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. Methods Between January 1997 and December 2013, 19 children were diagnosed with AGS at Asan Medical Center, Seoul, Korea. Their clinical features, outcomes, and JAG1 and NOTCH2 mutation status were retrospectively analyzed. Results The prevalence of clinical features in the 19 patients was as follows: dysmorphic facial features, 100% ( n = 19); liver symptoms, 89% ( n = 17); cardiac symptoms, 95% ( n = 18); ophthalmologic symptoms, 67% ( n = 10); skeletal deformities, 47% ( n = 9); and renal symptoms, 21% ( n = 4). JAG1 mutations were identified in 14 patients. The 13 different JAG1 mutations, seven of which were novel, included four deletions, three insertions, two missense mutations, three nonsense mutations, and one indel mutation. No NOTCH2 mutations were found. Two patients who received liver transplantation due to liver failure were still alive. Two patients died of comorbidities related to AGS: one of cardiac failure and one of hepatic failure. Conclusion This study describes the clinical characteristics of 19 Korean AGS patients with seven novel JAG1 mutations. Neonatal cholestatic jaundice was the most common initial presenting symptom; thus the presence of neonatal cholestasis warrants screening for syndromic features of AGS. Complex heart anomalies and progressive liver dysfunction resulted in significant morbidity and mortality in AGS. [ABSTRACT FROM AUTHOR]

DNHB is common in countries with high prevalence of hepatitis B, and therefore, contracting hepatitis B after LT with HBcAb+ grafts is a major concern. We studied DNHB in 247 children (aged <18 yr) who underwent LT from 1994 to 2013. Sixty-six of 247 recipients received HBcAb+ donor grafts. The incidence of DNHB was 5.7% (14 of 247 children) and that in HBcAb+ donor grafts was 19.7% (13 of 66 children). The incidence of DNHB without LAM prophylaxis was 31.3% (nine of 29 children), while that with prophylaxis was 10.8% (four of 37 children). LAM prophylaxis negatively correlated with DNHB by Cox regression analysis (p = 0.028, odds ratio = 0.258). Among 13 DNHB patients with HBcAb+ donor grafts, eight recovered from DNHB and four showed the emergence of LAM resistance. There was no DNHB-related graft failure. This study showed that HBcAb+ donor graft was associated with development of DNHB, and use of LAM prophylaxis decreased the incidence of DNHB with HBcAb+ graft. [ABSTRACT FROM AUTHOR]

The origin and evolution of viruses remain mysterious. Here, we focus on the distribution of viral replicons in host organisms, their morphological features, and the evolution of highly conserved protein and nucleic acid structures. The apparent inability of RNA viral replicons to infect contemporary akaryotic species suggests an early origin of RNA viruses and their subsequent loss in akaryotes. A census of virion morphotypes reveals that advanced forms were unique to viruses infecting a specific supergroup, while simpler forms were observed in viruses infecting organisms in all forms of cellular life. Results hint toward an ancient origin of viruses from an ancestral virus harboring either filamentous or spherical virions. Finally, phylogenetic trees built from protein domain and tRNA structures in thousands of genomes suggest that viruses evolved via reductive evolution from ancient cells. The analysis presents a complete account of the evolutionary history of cells and viruses and identifies viruses as crucial agents influencing cellular evolution. [ABSTRACT FROM AUTHOR]

The composition of lichen ecosystems except mycobiont and photobiont has not been evaluated intensively. In addition, recent studies to identify algal genotypes have raised questions about the specific relationship between mycobiont and photobiont. In the current study, we analyzed algal and fungal community structures in lichen species from King George Island, Antarctica, by pyrosequencing of eukaryotic large subunit (LSU) and algal internal transcribed spacer (ITS) domains of the nuclear r RNA gene. The sequencing results of LSU and ITS regions indicated that each lichen thallus contained diverse algal species. The major algal operational taxonomic unit (OTU) defined at a 99% similarity cutoff of LSU sequences accounted for 78.7-100% of the total algal community in each sample. In several cases, the major OTUs defined by LSU sequences were represented by two closely related OTUs defined by 98% sequence similarity of ITS domain. The results of LSU sequences indicated that lichen-associated fungi belonged to the Arthoniomycetes, Eurotiomycetes, Lecanoromycetes, Leotiomycetes, and Sordariomycetes of the Ascomycota, and Tremellomycetes and Cystobasidiomycetes of the Basidiomycota. The composition of major photobiont species and lichen-associated fungal community were mostly related to the mycobiont species. The contribution of growth forms or substrates on composition of photobiont and lichen-associated fungi was not evident. [ABSTRACT FROM AUTHOR]

Background: Helicobacter pylori (H. pylori) infection plays an important role in the early stage of cancer development. However, various bacteria that promote the synthesis of reactive oxygen and nitrogen species may be involved in the later stages. We aimed to determine the microbial composition of gastric mucosa from the patients with chronic gastritis, intestinal metaplasia, and gastric cancer using 454 GS FLX Titanium. Methods: Gastric mucosal biopsy samples were collected from 31 patients during endoscopy. After the extraction of genomic DNA, variable region V5 of the 16S rRNA gene was amplified. PCR products were sequenced using 454 high-throughput sequencer. The composition, diversity, and richness of microbial communities were compared between three groups. Results: The composition of H. pylori-containing Epsilonproteobacteria class appeared to be the most prevalent, but the relative increase in the Bacilli class in the gastric cancer group was noticed, resulting in a significant difference compared with the chronic gastritis group. By analyzing the Helicobacter- dominant group at a family level, the relative abundance of Helicobacteraceae family was significantly lower in the gastric cancer group compared with chronic gastritis and intestinal metaplasia groups, while the relative abundance of Streptococcaceae family significantly increased. In a UPGMA clustering of Helicobacter-dominant group based on UniFrac distance, the chronic gastritis group and gastric cancer group were clearly separated, while the intestinal metaplasia group was distributed in between the two groups. The evenness and diversity of gastric microbiota in the gastric cancer group was increased compared with other groups. Conclusions: In Helicobacter predominant patients, the microbial compositions of gastric mucosa from gastric cancer patients are significantly different to chronic gastritis and intestinal metaplasia patients. These alterations of gastric microbial composition may play an important, as-yet-undetermined role in gastric carcinogenesis of Helicobacter predominant patients. [ABSTRACT FROM AUTHOR]

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 Children's 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. [ABSTRACT FROM AUTHOR]

The purpose of this study was to evaluate retrospectively the results of PTA for late-onset PV complications after pediatric LDLT and to assess whether a meso-Rex shunt is a viable option for treating restenosis of the PV after PTA in selected cases. Seventy-five children who underwent adult-to-child LDLT were included in this study, and there were six late-onset PV complications (8.0%). The initial therapeutic approach was PTA, with or without stent: PTA with balloon dilation for three children, PTA with stent placement for one child, and failure to cannulate the occluded PV for two children. A meso-Rex shunt was performed in the two children after failed PTA: One suffered complete obstruction of the main PV, and the other, restenosis with total thrombosis after PTA with stent. The PTA was a technical and clinical success in four with PV stenosis of the six patients (66.7%), and successful application of a meso-Rex shunt in the other two children resulted in restoration of PV flow. In conclusion, PTA is a safe and effective procedure for treating late-onset PV stenosis after pediatric LDLT. However, in growing pediatric recipients with restenosis of the PV after PTA or chronic PV thrombosis, a meso-Rex shunt may be a better choice for late-onset PV complications. [ABSTRACT FROM AUTHOR]

Between 2004 and 2016, 142 paediatric ALF cases were identified according to the Pediatric Acute Liver Failure Study Group criteria.5 The diagnosis of WD was confirmed by genetic testing. With interest we read Güngör et al's study1 on the reliability of simple biochemical markers for fast and accurate diagnosis of fulminant Wilson's disease (fWD). Instead of ceruloplasmin and urine copper, which require several days, simple 2-hour indexes such as aspartate aminotransferase (AST)/alanine aminotransferase (ALT) ratio >2.2 and alkaline phosphatase (ALP)/total bilirubin (T.bil) ratio < 4.0 are valuable for distinguishing adult fWD from other types of acute liver failure (ALF).4 However, in the aforementioned study,1 age-stratified analysis was not performed, possibly resulting in less clinical applicability despite statistical significance. [Extracted from the article]

Background 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. Methods The clinical features and laboratory findings for eight children with ALF caused by HLH ( ALF-HLH group) and 27 children with ALF of unknown etiology ( ALF-UK group) were retrospectively compared by reviewing medical records. Results The ALF-HLH group had a higher incidence of pleural effusion, C-reactive protein elevation (especially >5 mg/dL), thrombocytopenia, anemia, fever, splenomegaly, and hypoalbuminemia (<2.5 mg/dL), and a higher in-hospital mortality rate. No significant differences were found in the white blood cell count, liver enzymes, coagulation profile, or incidence of hepatomegaly. Conclusions LT should be performed only after it is proven that ALF is not caused by HLH, if a child with ALF shows the differential clinical features of ALF caused by HLH. Further research with larger sample sizes, however, is needed. [ABSTRACT FROM AUTHOR]

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. © 2011 John Wiley & Sons A/S. Abstract: Massive pulmonary hemorrhage and other serious cardiopulmonary diseases in patients with fulminant hepatitis result not only in graft failure but also mortality after LT. ECMO is used to treat children with cardiorespiratory failure refractory to conventional intensive care. We describe a five-yr-old girl with genetically confirmed fulminant Wilson disease and severe pulmonary hemorrhage who underwent successful primary LT following veno-arterial ECMO. To our knowledge, this is the first report of successful primary LT in a patient using veno-arterial ECMO. The present case demonstrates that ECMO, as a bridging modality to LT, may be necessary to manage both massive pulmonary hemorrhage and possible graft loss because of hypoxemia. [ABSTRACT FROM AUTHOR]

Yoon SO, Yu E, Cho YM, Suh C, Kim KM, Han DJ, Lee SG, Huh J. Post-transplant lymphoproliferative disorders: clinicopathological analysis of 43 cases in a single center, 1990-2009. Clin Transplant 2012: 26: 67-73. © 2011 John Wiley & Sons A/S. Abstract: Post-transplant lymphoproliferative disorders (PTLDs) are a heterogeneous set of complications of organ transplantation associated with poor patient prognosis. We analyzed the clinicopathological features of PTLDs in 43 adult and pediatric recipients of solid organ or bone marrow transplantation at a large transplant service in the Republic of Korea between 1990 and 2009. Of 4545 solid organ and 747 bone marrow transplant recipients, 37 (0.81%) and 6 (0.8%), respectively, developed heterogeneous types of PTLDs. The cumulative incidences of PTLDs during this period were 1.79% (4/223) for heart transplant recipients, 0.78% (17/2192) for kidney transplant recipients, and 0.77% (16/2067) for liver transplant recipients. The patterns of disease onset, histology, and patient survival were associated with the types of organs transplanted. There is a trend for shorter overall survival (OS) in recipients with early-onset PTLDs and monomorphic PTLD histology, while kidney transplant recipients showed favorable OS. This study may be the first comprehensive analysis of the characteristics of PTLDs in Korean patients. [ABSTRACT FROM AUTHOR]

Wilson disease (WND), an autosomal recessive disorder of copper transport, is characterized by excessive accumulation of intracellular copper in liver and extrahepatic tissues because of impaired biliary copper excretion and disturbed incorporation of copper into ceruloplasmin. Hepatic cirrhosis and neuronal degeneration are the major symptoms of WND, and mutations in the ATP7B gene are associated with WND. We have identified 28 different mutations in the ATP7B gene, including six novel variations, in 120 unrelated Korean patients with WND. Molecular defects in ATP7B were present in only 75.0% of Korean WND patients, with the most common mutation, p.Arg778Leu, having an allele frequency of 39.2%. To evaluate the functional defects of ATP7B caused by novel mutations, we used a yeast complementation system, and we used confocal microscopy to localize each mutation after transient expression in mammalian cells. Six novel variations were cloned into a yeast expression vector and two into a mammalian expression vector for confocal analysis. We found that c.2785A>G (p.Ile929Val) and c.3316G>A (p.Val1106Ile) were rare polymorphisms, whereas the others were novel variations disturbing ATP7B function. Hum Mutat 28(11), 1108-1113, 2007. © 2007 Wiley-Liss, Inc. [ABSTRACT FROM AUTHOR]

A 12-year-old girl who had a history of liver transplantation due to congenital biliary atresia, presented with a 6-month history of an enlarging erythematous plaque with crust on her right buttock. A diagnosis of EBV-positive cutaneous PTLD of T-cell lineage was made. This case suggests that B-cell and T-cell PTLD, both associated with EBV infection, can co-occur in a liver transplant recipient. [Extracted from the article]

Background and Aims: Biliary disease is one of the most common causes of acute pancreatitis in adults; however, this cause and outcome in children have rarely been described in the literature. Therefore, the present study was conducted to evaluate the role of biliary disease as a cause of acute pancreatitis in children. Methods: The present study included 56 children with acute pancreatitis, of which 16 (29%) cases were associated with biliary disease. The 16 cases consisted of four boys and 12 girls ranging in age from 2 to 13 years. The underlying causes of the biliary disease were evaluated, as well as its clinical presentations, management modalities, and outcomes. Results: The causes of biliary disease in the 16 children included choledochal cyst in seven, biliary sludge in six, gallstone in two, and anomalous pancreaticobiliary junction in one. Acute pancreatitis with biliary disease showed increased presentation of jaundice and abnormalities in a liver-function test. Therapeutic interventions were performed more frequently in acute pancreatitis associated with biliary disease. All seven children with choledochal cysts needed hepaticojejunostomy. Of eight children with biliary sludge or gallstones, five children remained free of and two suffered from repeated attacks of pancreatitis after endoscopic papillotomy. Mortality did not occur. Conclusion: The present study suggests that biliary disease can be one of the causes of acute pancreatitis in children and has the clinical characteristics of jaundice and/or abnormalities in a liver-function test. Appropriate therapeutic interventions should be considered as the treatment modality. [ABSTRACT FROM AUTHOR]