Significance of Follow-Up Ultrasonography 24 Hours Post-Reduction in Detecting Intussusception Recurrence / 대한소아소화기영양학회지

Purpose@#The objective of this study was to identify the significance of 24-hour postreduction ultrasonography (US) in pediatric patients with intussusception. @*Methods@#A total of 229 patients with intussusception who were treated with saline reduction at Severance Children’s Hospital between January 2014 and September 2020 were retrospectively reviewed. The 229 patients with successful saline reduction were divided into two groups: a recurrence at 24 hours group (R, n=41) and a non-recurrence group (NR, n=188). The full patient sample was divided into two groups: follow-up US (FU) or no followup US (NFU); the recurrence group was divided into follow-up (R-FU) and non-follow-up (R-NFU) subgroups, and stratified analyses were performed. @*Results@#There were no significant differences in age, sex, laboratory findings, symptoms, and sonographic findings between the NR and R groups. In the R group, 24 patients underwent follow-up US, and 17 patients did not. Specific sonographic findings were statistically significant in the R-FU group compared to the R-NFU group (p=0.002). The R-FU group had fewer admissions (p=0.012) and longer mean hospitalization times (p<0.001) than the R-NFU group. The NFU group had a 12.2% recurrence rate, while the R-FU group recurrence rate was 25.8% (p=0.0099), suggesting that the omission of some recurrent events and follow-up US was a significant variable in the recurrence of intussusception. The median time to recurrence was 21 hours which supports the 24-hour follow-up protocol. @*Conclusion@#Twenty-four-hour follow-up US was shown to be valuable for detecting early recurrence of intussusception.

Effectiveness of High-Volume Therapeutic Plasma Exchange for Acute and Acute-on-Chronic Liver Failure in Korean Pediatric Patients / 대한소아소화기영양학회지

Purpose@#Liver transplantation (LT) is the only curative treatment for acute liver failure (ALF) and acute-on-chronic liver failure (ACLF). In high-volume therapeutic plasma exchange (HV-TPE), extracorporeal liver support filters accumulate toxins and improve the coagulation factor by replacing them. In this study, we aimed to evaluate the effectiveness of HV-TPE in pediatric patients with ALF and ACLF. @*Methods@#We reviewed the records of children waiting for LT at Severance Hospital who underwent HV-TPE between 2017 and 2021. Aspartate aminotransferase (AST), alanine aminotransferase (ALT), total and direct bilirubin (TB and DB), gamma-glutamyl transferase (GGT), ammonia, and coagulation parameter-international normalized ratio (INR) were all measured before and after HV-TPE to analyze the liver function. The statistical analysis was performed using IBM SPSS Statistics for Windows, version 26.0 (IBM Co., Armonk, NY, USA). @*Results@#Nine patients underwent HV-TPE with standard medical therapy while waiting for LT. One had neonatal hemochromatosis, four had biliary atresia, and the other four had ALF of unknown etiology. Significant decreases in AST, ALT, TB, DB, GGT, and INR were noted after performing HV-TPE (930.38–331.75 IU/L, 282.62–63.00 IU/L, 11.75–5.59 mg/dL, 8.10– 3.66 mg/dL, 205.62–51.75 IU/L, and 3.57–1.50, respectively, p<0.05). All patients underwent LT, and two expired due to acute complications. @*Conclusion@#HV-TPE could remove accumulated toxins and improve coagulation. Therefore, we conclude that HV-TPE can be regarded as a representative bridging therapy before LT.

Association between Fecal Calprotectin and Mucosal Healing in Pediatric Patients with Crohn’s Disease Who Have Achieved Sustained Clinical Remission with Anti-Tumor Necrosis Factor Agents

Background/Aims@#Although mucosal healing (MH) is acknowledged as the treatment target in the treat-to-target era, there are limitations on repeated endoscopic examinations, especially in pediatric patients. We aimed to investigate whether fecal calprotectin (FC) could serve as a surrogate marker for the assessment of MH in pediatric patients with Crohn’s disease (CD) who have achieved sustained clinical remission (CR) while treated with anti-tumor necrosis factor (TNF) agents. @*Methods@#This multicenter retrospective cross-sectional study included pediatric CD patients who had sustained a CR for at least 6 months with anti-TNF agents and who simultaneously underwent ileocolonoscopy and FC tests during follow-up. MH was defined as the absence of any ulcer on ileocolonoscopy. @*Results@#A total of 131 patients were included in this study. MH was observed in 87 patients (66.7%). The FC level was significantly lower in patients with MH than in those without MH (median 49.0 mg/kg vs 599.0 mg/kg; p<0.001). According to the multivariate logistic regression analysis, FC was the only factor associated with MH (odds ratio, 0.62; 95% confidence interval [CI], 0.52 to 0.73; p<0.001). According to the receiver operating characteristic curve analysis, the optimal cutoff value for FC for the association with MH was <140 mg/kg (area under the curve 0.890, 95% CI 0.829 to 0.951, sensitivity 78.2%, specificity 88.6%, p<0.001). @*Conclusions@#FC was associated with MH in pediatric patients with CD who had achieved a sustained CR for at least 6 months with anti-TNF agents. In these patients, FC can be used to stratify patients and guide decisions regarding ileocolonoscopy in the treat-to-target era.

Initial Abdominal CT and Laboratory Findings Prior to Diagnosis of Crohn’s Disease in Children

Purpose@#To identify initial abdominal computed tomography (CT) and laboratory findings prior to a diagnosis of Crohn’s disease (CD) in children. @*Materials and Methods@#In this retrospective study, patients (≤18 year-old) who were diagnosed with CD from 2004 to 2019 and had abdominal CT just prior to being diagnosed with CD were included in the CD group. Patients (≤18 years old) who were diagnosed with infectious enterocolitis from 2018 to 2019 and had undergone CT prior to being diagnosed with enterocolitis were included as a control group. We assessed the diagnostic performances of initial CT and laboratory findings for the diagnosis of CD using logistic regression and the area under the curve (AUC). @*Results@#In total, 107 patients (50 CD patients, 57 control patients) were included, without an age difference between groups (median 13 years old vs. 11 years old, p=0.119). On univariate logistic regression analysis, multisegmental bowel involvement, mesenteric vessel engorgement, higher portal vein/aorta diameter ratio, longer liver longitudinal diameter, lower hemoglobin (≤12.5 g/ dL), lower albumin (≤4 g/dL), and higher platelet (>320×103 /μL) levels were significant factors for CD. On multivariate analysis, multisegmental bowel involvement [odds ratio (OR) 111.6, 95% confidence interval (CI) 4.778–2605.925] and lower albumin levels (OR 0.9, 95% CI 0.891–0.993) were significant factors. When these two features were combined, the AUC value was 0.985 with a sensitivity of 96% and specificity of 100% for differentiating CD. @*Conclusion@#Multisegmental bowel involvement on CT and decreased albumin levels can help differentiate CD from infectious enterocolitis in children prior to a definite diagnosis of CD.

Quantitative MRI Assessment of Pancreatic Steatosis Using Proton Density Fat Fraction in Pediatric Obesity

Objective@#To assess the feasibility of quantitatively assessing pancreatic steatosis using magnetic resonance imaging (MRI) and its correlation with obesity and metabolic risk factors in pediatric patients. @*Materials and Methods@#Pediatric patients (≤ 18 years) who underwent liver fat quantification MRI between January 2016 and June 2019 were retrospectively included and divided into the obesity and control groups. Pancreatic proton density fat fraction (P-PDFF) was measured as the average value for three circular regions of interest (ROIs) drawn in the pancreatic head, body, and tail. Age, weight, laboratory results, and mean liver MRI values including liver PDFF (L-PDFF), stiffness on MR elastography, and T2* values were assessed for their correlation with P-PDFF using linear regression analysis. The associations between P-PDFF and metabolic risk factors, including obesity, hypertension, diabetes mellitus (DM), and dyslipidemia, were assessed using logistic regression analysis. @*Results@#A total of 172 patients (male:female = 125:47; mean ± standard deviation [SD], 13.2 ± 3.1 years) were included. The mean P-PDFF was significantly higher in the obesity group than in the control group (mean ± SD, 4.2 ± 2.5% vs. 3.4 ± 2.4%; p = 0.037). L-PDFF and liver stiffness values showed no significant correlation with P-PDFF (p = 0.235 and p = 0.567, respectively). P-PDFF was significantly associated with obesity (odds ratio 1.146, 95% confidence interval 1.006–1.307, p = 0.041), but there was no significant association with hypertension, DM, and dyslipidemia. @*Conclusion@#MRI can be used to quantitatively measure pancreatic steatosis in children. P-PDFF is significantly associated with obesity in pediatric patients.

The Effect of Formula-based Nutritional Treatment on Colitis in a Murine Model

Background@#Exclusive enteral nutrition (EEN) induces remission in pediatric Crohn's disease (CD). The exact mechanism of EEN therapy in CD is unknown, but alteration of the intestinal microflora after EEN is thought to affect mucosal healing. To determine the link between EEN therapy and therapeutic efficacy in CD, we established a murine model of dextran sulfate sodium (DSS)-induced colitis and applied EEN therapy. @*Methods@#Eight-week-old C57BL/6 mice were administered DSS for 4 days to induce colitis, and either normal chow (NC) or EEN was administered for the following 4 days. The mice were grouped according to the feeding pattern after DSS administration: DSS/NC and DSS/ EEN groups. The clinical course was confirmed via daily observation of the weight and stool. Fecal samples were collected and 16sRNA sequencing was used. The mice were sacrificed to confirm colonic histopathology. @*Results@#Weight reduction and increase in disease activity were observed as the day progressed for 4 days after DSS administration. There was significant weight recovery and improvement in disease activity in the EEN group compared to that in the NC group. Verrucomicrobia and Proteobacteria abundances tended to increase and Bacteroidetes abundance decreased in the EEN group. In the EEN group, significant changes in the β-diversity of the microbiota were observed. In the analysis of microbiome species, abundances of Akkermansia muciniphila, Clostridium cocleatum, mucin-degrading bacteria, Flintibacter butyricus, and Parabacteroides goldsteinii, which are beneficial microbiota, were significantly increased in the EEN group compared to those in the NC group. More abundant mucins were confirmed in the colonic histopathology of the EEN group. These microbial and histopathological differences suggested that EEN might improve colitis symptoms in a murine colitis model by promoting mucin recycling and subsequently inducing the healing effect of the gut barrier. @*Conclusion@#EEN showed clinical efficacy in a murine model of colitis. Based on the increase in mucin-degrading bacteria and the pathological increase in mucin production after EEN administration, it can be observed that mucin plays an important role in the therapeutic effect of EEN.

Diffusion-Weighted Imaging for Differentiation of Biliary Atresia and Grading of Hepatic Fibrosis in Infants with Cholestasis

Objective@#To determine whether the values of hepatic apparent diffusion coefficient (ADC) can differentiate biliary atresia (BA) from non-BA or be correlated with the grade of hepatic fibrosis in infants with cholestasis. @*Materials and Methods@#This retrospective cohort study included infants who received liver MRI examinations to evaluate cholestasis from July 2009 to October 2017. Liver ADC, ADC ratio of liver/spleen, aspartate aminotransferase to platelet ratio index (APRI), and spleen size were compared between the BA and non-BA groups. The diagnostic performances of all parameters for significant fibrosis (F3–4) were obtained by receiver-operating characteristics (ROCs) curve analysis. @*Results@#Altogether, 227 infants (98 males and 129 females, mean age = 57.2 ± 36.3 days) including 125 BA patients were analyzed. The absolute ADC difference between two reviewers was 0.10 mm2 /s for both liver and spleen. Liver ADC value was specific (80.4%) and ADC ratio was sensitive (88.0%) for the diagnosis of BA with comparable performance. There were 33 patients with F0, 15 with F1, 71 with F2, 35 with F3, and 11 with F4. All four parameters of APRI (τ = 0.296), spleen size (τ = 0.312), liver ADC (τ = -0.206), and ADC ratio (τ = -0.288) showed significant correlation with fibrosis grade (all, p < 0.001).The cutoff values for significant fibrosis (F3–4) were 0.783 for APRI (area under the ROC curve [AUC], 0.721), 5.9 cm for spleen size (AUC, 0.719), 1.044 x 10-3 mm2 /s for liver ADC (AUC, 0.673), and 1.22 for ADC ratio (AUC, 0.651). @*Conclusion@#Liver ADC values and ADC ratio of liver/spleen showed limited additional diagnostic performance for differentiating BA from non-BA and predicting significant hepatic fibrosis in infants with cholestasis.

Key imaging features for differentiating cystic biliary atresia from choledochal cyst: prenatal ultrasonography and postnatal ultrasonography and MRI

Purpose@#This study compared clinical and radiologic differences between cystic biliary atresia (cBA) and choledochal cyst (CC) type Ia/b. @*Methods@#Infants (≤12 months old) who were diagnosed with cBA or CC type Ia/b from 2005 to 2019 were retrospectively reviewed. Imaging features on preoperative ultrasonography (US) and magnetic resonance imaging (MRI) were compared between the cBA and CC groups. Logistic regression and area under the receiver operating characteristic curve (AUC) analyses were performed for the diagnosis of cBA. Changes in cyst size were also evaluated when prenatal US exams were available. @*Results@#Ten patients (5.5% of biliary atresia cases) with cBA (median age, 48 days) and 11 infants with CC type Ia/b (Ia:Ib=10:1; median age, 20 days) were included. Triangular cord thickness on US (cutoff, 4 mm) showed 100% sensitivity and 90.9% specificity (AUC, 0.964; 95% confidence interval [CI], 0.779 to 1.000) and cyst size on MRI (cutoff, 2.2 cm) had 70% sensitivity and 100% specificity (AUC, 0.900; 95% CI, 0.690 to 0.987) for diagnosing cBA. Gallbladder mucosal irregularity on US and an invisible distal common bile duct on MRI were only seen in the cBA group (10 of 10). Only the CC group showed prenatal cysts exceeding 1 cm with postnatal enlargement. @*Conclusion@#Small cyst size (<1 cm) on prenatal US, triangular cord thickening (≥4 mm) and gallbladder mucosal irregularity on postnatal US, and small cyst size (≤2.2 cm) and an invisible distal common bile duct on MRI can discriminate cBA from CC type Ia/b in infancy.

Korean Children and Adolescents with Crohn's Disease Are More Likely to Present with Perianal Fistulizing Disease at Diagnosis Compared to Their European Counterparts / 대한소아소화기영양학회지

Clinical Practice Guideline for the Diagnosis and Treatment of Pediatric Obesity: Recommendations from the Committee on Pediatric Obesity of the Korean Society of Pediatric Gastroenterology Hepatology and Nutrition / 대한소아소화기영양학회지

The Committee on Pediatric Obesity of the Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition newly developed the first Korean Guideline on the Diagnosis and Treatment of Obesity in Children and Adolescents to deliver an evidence-based systematic approach to childhood obesity in South Korea. The following areas were systematically reviewed, especially on the basis of all available references published in South Korea and worldwide, and new guidelines were established in each area with the strength of recommendations based on the levels of evidence: 1) definition and diagnosis of overweight and obesity in children and adolescents; 2) principles of treatment of pediatric obesity; 3) behavioral interventions for children and adolescents with obesity, including diet, exercise, lifestyle, and mental health; 4) pharmacotherapy; and 5) bariatric surgery.

Therapeutic Efficacy of Exclusive Enteral Nutrition with Specific Polymeric Diet in Pediatric Crohn's Disease / 대한소아소화기영양학회지

PURPOSE: The efficacy of exclusive enteral nutrition (EEN) with a polymeric diet has not been confirmed in Korean pediatric patients with Crohn's disease (CD). This study aimed to compare the effectiveness of EEN with a specific polymeric diet (Encover®) and corticosteroids (CSs) for the induction of remission in Korean pediatric CD patients. METHODS: We retrospectively compared data from 51 pediatric CD patients who underwent induction therapy with EEN (n=19) or CSs (n=32) at Severance Children's Hospital or Incheon St. Mary's Hospital in Korea. The primary endpoint of this study was the rate of clinical remission, defined as a Pediatric Crohn's Disease Activity Index (PCDAI) score < 10, after 8 weeks of induction treatment. Clinical, laboratory, and growth data at post-induction as well as their changes from baseline were also compared between groups. RESULTS: After 8 weeks of induction therapy, clinical remission rates were 78.9% (15/19) and 65.6% (21/32) in the EEN and CS groups, respectively (p=0.313). No significant differences in PCDAI scores, laboratory variables, and growth parameters were noted between the two groups at post-induction. However, significant changes in albumin levels at post-induction were observed in the EEN group compared to the CS group (p=0.038). CONCLUSION: Our results suggest that the effectiveness of EEN with a polymeric diet and CSs for induction therapy did not differ in Korean pediatric CD patients. EEN with a polymeric diet is a good first-line treatment option for the induction of remission in these patients.

Clinical practice guideline for the diagnosis and treatment of pediatric obesity: recommendations from the Committee on Pediatric Obesity of the Korean Society of Pediatric Gastroenterology Hepatology and Nutrition / 소아과

The Committee on Pediatric Obesity of the Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition newly developed the first Korean Guideline on the Diagnosis and Treatment of Obesity in Children and Adolescents to deliver an evidence-based systematic approach to childhood obesity in South Korea. The following areas were systematically reviewed, especially on the basis of all available references published in South Korea and worldwide, and new guidelines were established in each area with the strength of recommendations based on the levels of evidence: (1) definition and diagnosis of overweight and obesity in children and adolescents; (2) principles of treatment of pediatric obesity; (3) behavioral interventions for children and adolescents with obesity, including diet, exercise, lifestyle, and mental health; (4) pharmacotherapy; and (5) bariatric surgery.

A case of gastroduodenal ulcer complicating Kawasaki disease

Kawasaki disease (KD) is a systemic vasculitis associated with various clinical manifestations and complications, such as gastrointestinal abnormalities. We report a 3-year-old boy who presented with hematemesis and diffuse gastroduodenal ulcerations complicating KD. He received standard medical therapy for the disease and gastric ulcer, which showed effect after a few days. Although rare, peptic ulcers should be considered a complication of KD to ensure early diagnosis and treatment as it may cause severe morbidity.

Micronutrients Are Not Deficient in Children with Nonorganic Failure to Thrive / 대한소아소화기영양학회지

PURPOSE: Inadequate calorie intake is one of the most important causes of nonorganic failure to thrive (NOFTT) and is thought to lead to multiple micronutrient deficiencies. However, there have been few studies on NOFTT and micronutrients. The aim of this study was to evaluate the micronutrient status of children with NOFTT. METHODS: We conducted a retrospective cohort study in 161 children (106 with NOFTT and 55 health controls) at a single institution. Data on weight for age, height for age, body mass index, and biochemical parameters, indicating the children's nutritional and micronutrient status were reviewed via electronic medical records, and the two groups were compared. RESULTS: Except inorganic phosphate levels, no statistically significant differences were seen in the laboratory findings indicating the children's nutritional and micronutrient status; notably, the inorganic phosphate levels were within the normal range in both groups. We then compared the severe NOFTT (weight for age below the first percentile) and control groups; however, no statistically significant differences were seen for any of the measured parameters. CONCLUSION: Most children with NOFTT in this study had normal micronutrient levels and other laboratory findings. Therefore, element deficiencies should not be considered a natural consequence of NOFTT or in healthy children. Close monitoring and additional evaluations are needed.

Association of Vitamin D with Inflammatory Bowel Disease Activity in Pediatric Patients

BACKGROUND: It has been known that vitamin D level (serum 25[OH]D) has correlation with inflammatory bowel disease (IBD). The purpose of this study is to investigate changes of serum 25[OH]D in pediatric IBD patients according to the disease activity. METHODS: A total of 96 children and adolescent with IBD were enrolled in this retrospective study. Serologic inflammatory markers and clinical disease activity scores of the patients were collected, and their correlations with serum 25[OH]D were analyzed. Seasonal variations of serum 25[OH]D were also investigated both in active disease state and remission state. RESULTS: Of the 96 patients, 41 (43%) were women and patients with a vitamin D deficiency (< 20 ng/mL) at diagnosis were 77 (80.2%). There was no significant difference between Crohn's disease and ulcerative colitis for serum 25[OH]D. Serum 25[OH]D was higher in remission group than in active disease group (12.4 [8.8–29] ng/mL vs. 17.9 [12.3–34.4] ng/mL; P < 0.001) and the difference was more significant than other micronutrients. There was no significant difference in serum 25[OH]D concentration between patients with ileal involvement and patients without ileal involvement. There were seasonal variations in the active phase, but there was no significant difference by season in the remission phase. CONCLUSION: Serum 25[OH]D is inversely correlated with disease activity in IBD. Monitoring and supplementation is required especially for active disease status and in winter and spring season.

Feasibility of Spin-Echo Echo-Planar Imaging MR Elastography in Livers of Children and Young Adults

PURPOSE: To assess the feasibility of the use of spin-echo echo-planar imaging (SE-EPI) magnetic resonance elastography (MRE) in livers of children and young adults. MATERIALS AND METHODS: Patients (≤ 20 years old) who underwent 3T SE-EPI MRE were included retrospectively. Subjects were divided into three groups according to the purpose of the liver MRI: suspicion of fatty liver or focal fat deposition in the liver (FAT group), liver fibrosis after receiving a Kasai operation from biliary atresia (BA group), and hepatic iron deposition after receiving chemotherapy or transfusions (IRON group). Technical failure of MRE was defined when a stiffness map showed no pixel value with a confidence index higher than 95%, and the patients were divided as success and failure groups accordingly. Clinical findings including age, gender, weight, height, and body mass index and magnetic resonance imaging results including proton density fat fraction (PDFF), T2*, and MRE values were assessed. Factors affecting failure of MRE were evaluated and the image quality in wave propagation image and stiffness map was evaluated using the appropriate scores. RESULTS: Among total 240 patients (median 15 years, 211 patients in the FAT, 21 patients in the BA, and 8 patients in the IRON groups), technical failure was noted in six patients in the IRON group (6/8 patients, 75%), while there were no failures noted in the FAT and BA groups. These six patients had T2* values ranging from 0.9 to 3.8 ms. The image quality scores were not significantly different between the FAT and BA groups (P > 0.999), while the scores were significantly lower in the IRON group (P < 0.001). CONCLUSION: The 3T SE-EPI MRE in children and young adults had a high technical success rate. The technical failure was occurred in children with decreased T2* value (≤ 3.8 ms) from iron deposition.

Correlation between the occurrence of acute gastroenteritis in children and regional temperature at a tertiary hospital emergency department in Korea

PURPOSE: Several studies have investigated the association between acute gastroenteritis (AGE) and regional temperatures. We aimed to investigate the correlation between the occurrence of AGE and regional temperature in Korean children. METHODS: We reviewed the clinical characteristics of children aged 18 years or younger with AGE, defined according to the International Statistical Classification of Diseases, 10th Revision codes, who visited a Korean tertiary hospital emergency department (ED) between 2006 and 2016, and compared the children who were discharged from the ED and those who were hospitalized. Data on temperature in Seoul, Korea during the period were obtained from the Korea Meteorological Administration. Subsequently, we assessed correlations between the monthly mean numbers of the children with AGE and monthly mean temperature using Pearson's correlation analysis.

Estimated Prevalence of Adolescents with Nonalcoholic Fatty Liver Disease in Korea

BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) has become prevalent worldwide in the last decade. However, the recent prevalence of NAFLD in adolescents has not yet been investigated in Korea. METHODS: Data were obtained from 1,416 participants aged 10–18 years from the Korea National Health and Nutrition Examination Survey conducted in 2010 and 2015. Systolic blood pressure (SBP), diastolic blood pressure (DBP), height, weight, waist circumference (WC), body mass index (BMI), fasting glucose, total cholesterol, high-density lipoprotein (HDL), aspartate aminotransferase (AST), alanine aminotransferase (ALT) level, waist-to-height ratio (WHtR), and pediatric NAFLD fibrosis index (PNFI) were analyzed. RESULTS: SBP, weight, WC, BMI, WHtR, and total cholesterol level were significantly higher in 2015 than in 2010. Prevalence of NAFLD (BMI ≥ 85th percentile plus ALT > 30 U/L for boys and ALT > 19 U/L for girls) were 4.7% in 2010 and 5.9% in 2015 (P = 0.360). Using various cutoffs for the ALT level (> 40, > 30, > 25.8 U/L for boys and >22.1 U/L for girls) NAFLD prevalence rates were 3.0%, 4.1%, and 5.5% in 2010; 2.9%, 5.0%, and 7.1% in 2015, respectively (P = 0.899, 0.469, and 0.289). Boys had a higher SBP, DBP, height, weight, WC, BMI, WHtR, fasting glucose, total cholesterol, ALT, and lower HDL level than girls. The probability of liver fibrosis using the PNFI varies between 21.3% and 24.5% among NAFLD participants (P < 0.001). CONCLUSION: The Korean society needs to quickly control the increasing prevalence of NAFLD in adolescents and reduce its complications.

Gastroesophageal Reflux in Neurologically Impaired Children: What Are the Risk Factors?

BACKGROUND/AIMS: Neurologically impaired patients frequently suffer from gastrointestinal tract problems, such as gastroesophageal reflux disease (GERD). In this study, we aimed to define the risk factors for GERD in neurologically impaired children. METHODS: From May 2006 to March 2014, 101 neurologically impaired children who received 24-hour esophageal pH monitoring at Severance Children’s Hospital were enrolled in the study. The esophageal pH finding and the clinical characteristics of the patients were analyzed. RESULTS: The reflux index was higher in patients with abnormal electroencephalography (EEG) results than in those with normal EEG results (p=0.027). Mitochondrial disease was associated with a higher reflux index than were epileptic disorders or cerebral palsy (p=0.009). Patient gender, feeding method, scoliosis, tracheostomy, and baclofen use did not lead to statistical differences in reflux index. Age of onset of neurological impairment was inversely correlated with DeMeester score and reflux index. Age at the time of examination, the duration of the disease, and the number of antiepileptic drugs were not correlated with GER severity. CONCLUSIONS: Early-onset neurological impairment, abnormal EEG results, and mitochondrial disease are risk factors for severe GERD.

Alpers-Huttenlocher Syndrome First Presented with Hepatic Failure: Can Liver Transplantation Be Considered as Treatment Option? / 대한소아소화기영양학회지

Mitochondria play essential role in eukaryotic cells including in the oxidative phosphorylation and generation of adenosine triphosphate via the electron-transport chain. Therefore, defects in mitochondrial DNA (mtDNA) can result in mitochondrial dysfunction which leads to various mitochondrial disorders that may present with various neurologic and non-neurologic manifestations. Mutations in the nuclear gene polymerase gamma (POLG) are associated with mtDNA depletions, and Alpers-Huttenlocher syndrome is one of the most severe manifestations of POLG mutation characterized by the clinical triad of intractable seizures, psychomotor regression, and liver failure. The hepatic manifestation usually occurs late in the disease's course, but in some references, hepatitis was reportedly the first manifestation. Liver transplantation was considered contraindicated in Alpers-Huttenlocher syndrome due to its poor prognosis. We acknowledged a patient with the first manifestation of the disease being hepatic failure who eventually underwent liver transplantation, and whose neurological outcome improved after cocktail therapy.