Comparison of endoscopic healing and durability between infliximab originator and CT-P13 in pediatric patients with inflammatory bowel disease.

Background and aims: Favourable clinical data were published on the efficacy of CT-P13, the first biosimilar of infliximab (IFX), in pediatric inflammatory bowel disease (IBD); however, few studies have compared the effect on endoscopic healing (EH) and drug retention rate between the IFX originator and CT-P13. Therefore, we aimed to compare EH and the drug retention rate between the IFX originator and CT-P13. Methods: Children with Crohn's disease (CD) and ulcerative colitis (UC)/IBD-unclassified (IBD-U) at 22 medical centers were enrolled, with a retrospective review conducted at 1-year and last follow-up. Clinical remission, EH and drug retention rate were evaluated. Results: We studied 416 pediatric patients with IBD: 77.4% had CD and 22.6% had UC/IBD-U. Among them, 255 (61.3%) received the IFX originator and 161 (38.7%) received CT-P13. No statistically significant differences were found between the IFX originator and CT-P13 in terms of corticosteroid-free remission and adverse events. At 1-year follow-up, EH rates were comparable between them (CD: P=0.902, UC: P=0.860). The estimated cumulative cessation rates were not significantly different between the two groups. In patients with CD, the drug retention rates were 66.1% in the IFX originator and 71.6% in the CT-P13 group at the maximum follow-up period (P >0.05). In patients with UC, the drug retention rates were 49.8% in the IFX originator and 56.3% in the CT-P13 group at the maximum follow-up period (P >0.05). Conclusions: The IFX originator and CT-P13 demonstrated comparable therapeutic response including EH, clinical remission, drug retention rate and safety in pediatric IBD.

Factors Associated with Reaching Mid-Parental Height in Patients Diagnosed with Inflammatory Bowel Disease in Childhood and Adolescent Period.

Background/Aims: The recent update on the Selecting Therapeutic Targets in Inflammatory Bowel Disease initiative has added normal growth in children as an intermediate target in Crohn's disease and ulcerative colitis. We aimed to investigate factors associated with reaching mid-parental height (MPH) in patients diagnosed with inflammatory bowel disease in childhood and the adolescent period. Methods: This multicenter retrospective observational study included pediatric patients with inflammatory bowel disease that had reached adult height. Factors associated with reaching MPH were investigated by logistic regression analyses. Results: A total of 166 patients were included in this study (128 Crohn's disease and 38 ulcerative colitis). Among them, 54.2% (90/166) had reached their MPH. Multivariable logistic regression analysis revealed that height Z-score at diagnosis and MPH Z-score were independently associated with reaching MPH (odds ratio [OR], 8.45; 95% confidence interval [CI], 4.44 to 17.90; p<0.001 and OR, 0.11; 95% CI, 0.04 to 0.24; p<0.001, respectively). According to the receiver operating characteristic curve analysis, the optimal cutoff level of "height Z-score at diagnosis minus MPH Z-score" that was associated with reaching MPH was -0.01 with an area under the curve of 0.889 (95% CI [0.835 to 0.944], sensitivity 88.9%, specificity 84.2%, positive predictive value 87.0%, negative predictive value 86.5%, p<0.001). Conclusions: Height Z-score at diagnosis and MPH Z-score were the only factors associated with reaching MPH. Efforts should be made to restore growth in pediatric patients who present with a negative "height Z-score at diagnosis minus MPH Z-score."

A Report on a Nationwide Surveillance System for Pediatric Acute Hepatitis of Unknown Etiology in Korea.

BACKGROUND: Several cases of pediatric acute hepatitis of unknown etiology related to adenoviral infections have been reported in Europe since January 2022. The aim of this study was to compare the incidence, severity, possible etiology, and prognosis of the disease with those in the past in Korea. METHODS: The surveillance group collected data between May and November 2022 using a surveillance system. Acute hepatitis of unknown etiology was defined in patients aged < 16 years with a serum transaminase level > 500 IU/L, not due to hepatitis A-E or other underlying causes. For comparison, data from 18 university hospitals were retrospectively collected as a control group between January 2021 and April 2022. RESULTS: We enrolled 270 patients (mean age, 5 years). The most common symptom was fever. However, the incidence was similar between 2021 and 2022. Liver function test results, number of patients with acute liver failure (ALF), liver transplantation (LT), death, and adenovirus detection rates did not differ between the two groups. None of the adenovirus-positive patients in either group experienced ALF, LT, or death. In the surveillance group, adenovirus-associated virus-2 was detected in four patients, one of whom underwent LT. Patients with an unknown etiology showed significantly higher bilirubin levels, a lower platelet count, and a higher LT rate than patients with a possible etiology. CONCLUSION: The incidence of pediatric acute hepatitis of unknown etiology and adenovirus detection rate have not increased in Korea.

Clean mucosal area detection of gastroenterologists versus artificial intelligence in small bowel capsule endoscopy.

Studies comparing the detection of clean mucosal areas in capsule endoscopy (CE) using human judgment versus artificial intelligence (AI) are rare. This study statistically analyzed gastroenterologist judgments and AI results. Three hundred CE video clips (100 patients) were prepared. Five gastroenterologists classified the video clips into 3 groups (≥75% [high], 50%-75% [middle], and < 50% [low]) according to their subjective judgment of cleanliness. Visualization scores were calculated using an AI algorithm based on the predicted visible area, and the 5 gastroenterologists' judgments and AI results were compared. The 5 gastroenterologists evaluated CE clip video quality as "high" in 10.7% to 36.7% and as "low" in 28.7% to 60.3% and 29.7% of cases, respectively. The AI evaluated CE clip video quality as "high" in 27.7% and as "low" in 29.7% of cases. Repeated-measures analysis of variance (ANOVA) revealed significant differences in the 6 evaluation indicators (5 gastroenterologists and 1 AI) (P < .001). Among the 300 judgments, 90 (30%) were consistent with 5 gastroenterologists' judgments, and 82 (91.1%) agreed with the AI judgments. The "high" and "low" judgments of the gastroenterologists and AI agreed in 95.0% and 94.9% of cases, respectively. Bonferroni's multiple comparison test showed no significant difference between 3 gastroenterologists and AI (P = .0961, P = 1.0000, and P = .0676, respectively) but a significant difference between the other 2 with AI (P < .0001). When evaluating CE images for cleanliness, the judgments of 5 gastroenterologists were relatively diverse. The AI produced a relatively universal judgment that was consistent with the gastroenterologists' judgements.

Clinical Experiences and Selection of Accessory Devices for Pediatric Endoscopic Foreign Body Removal: A Retrospective Multicenter Study in Korea.

BACKGROUND: Swallowing of foreign bodies (FBs) is the most common indication of therapeutic endoscopy in children. Endoscopic removal may be necessary depending on the type of FB, age of the child, and location of the FB. We attempted to analyze the characteristics of each device used for the endoscopic removal of FBs in children. METHODS: Medical records of the patient's age, sex, weight, type, location, size, shape, type of device used for endoscopic removal, and endoscopic time were retrospectively collected. RESULTS: A total of 424 FB removal procedures were analyzed. The average age of the patients at the time of FB removal was 4.1 ± 3.7 years. Coins were the most common FBs (192, 45.3%). The most common locations of the FBs were the esophagus (45.7%) and the stomach (48.3%), respectively. For a total of 371 cases, forceps were used in 96 cases (25.9%) for esophageal FBs and in 25 cases for gastric FBs (6.7%), while nets were used in 250 cases (67.4%) for gastric FBs retrieval; the average durations of the procedures were 7.2 ± 7.4 minutes, 8.5 ± 7.2 minutes, and 5.7 ± 7.3 minutes, respectively (P = 0.003). The procedure time was significantly shorter, in the group of patients with low body weights, when nets were used than when forceps were used to remove gastric FBs (P = 0.001). CONCLUSION: The endoscopic procedure duration, in low-weight children, was shorter when retrieval nets were used than that with forceps.

The long-term clinical course of protein-losing enteropathy combined with iron deficiency anemia in Korean toddlers: Possible association with cow's milk protein.

BACKGROUND: Protein-losing enteropathy (PLE), a rare condition with excessive gastrointestinal protein loss, presents with hypoalbuminemia, edema, or ascites. Several cases of PLE combined with severe iron deficiency anemia (IDA) have been reported in infants and toddlers that were considered to result from excessive cow's milk consumption, although the mechanism has not been clearly established. METHODS: We retrospectively reviewed the clinical, laboratory, endoscopic, and radiologic characteristics of patients diagnosed and treated for PLE with IDA between 2015 and 2021. Long-term outcomes were analyzed according to dietary intervention during the follow-up period. RESULTS: A total of 10 patients aged 7.0-26.7 months were enrolled in the study and the median follow-up duration of them was 9.4 months (range, 1.3-18.0). Six of them were fed powdered formula, while two were fed whole cow's milk, and their median daily intake was 700 mL (range, 300-900). The times to normalization of hemoglobin, albumin, and eosinophil count were shorter in patients with dietary elimination of cow's milk protein immediately after diagnosis compared to those with reduced intake or no dietary change. CONCLUSION: Early complete elimination of cow's milk protein should be considered, especially if the laboratory parameters are not normalized with adequate iron supplementation even though the clinical symptoms show improvement. We would like to draw attention to the possibility of the cow's milk protein in the pathogenesis of the condition through the non-IgE-mediated immune reactions.

Comparison between Pediatric Crohn's Disease and Ulcerative Colitis at Diagnosis in Korea: Results from a Multicenter, Registry-Based, Inception Cohort Study.

Background/Aims: We aimed to compare the differences in pediatric Crohn's disease (CD) and ulcerative colitis (UC) at diagnosis in Korea. Methods: This was a multicenter, registry-based, inception cohort study conducted at five centers in Korea between 2013 and 2017. Baseline demographics, clinical characteristics, and results from laboratory, endoscopic, radiologic examinations were compared between pediatric CD and UC patients who were <19 years old at diagnosis. Results: A total 307 patients were included (227 CD [73.9%] and 80 UC [26.1%]). The male to female ratio was 2.49:1 for CD, and 1.49:1 for UC (p=0.019). Median age at diagnosis was 14.4 years (interquartile range, 12.4 to 16.2) for CD, and 14.4 years (interquartile range, 11.7 to 16.5) for UC (p=0.962). Hematochezia was the only dominant symptom in UC patients compared to CD patients (86.2% vs 30.8%, p<0.001). White blood cell counts, platelet counts, erythrocyte sedimentation rate, and C-reactive protein levels were significantly higher, and serum albumin level was significantly lower in CD patients than in UC patient. Anti-Saccharomyces cerevisiae antibody was positive in 44.5% and 16.2% of CD and UC patients, respectively (p<0.001), and antineutrophil cytoplasmic antibody was positive in 15.0% and 58.8% of CD and UC patients, respectively (p<0.001). Terminal ileal involvement was prominent in CD, while rectal involvement was more prominent in UC. Small bowel involvement and perianal perforating diseases were also more prominent in CD. Conclusions: This is the first a multicenter study in Korea to compare the differences between pediatric CD and UC at diagnosis in Korea. A large-scale, national study is expected to better clarify these findings in the future.

Fecal Calprotectin Assay at an Early Stage of Treatment Can Be Used as a Surrogate Marker to Predict Clinical Remission and Mucosal Healing in Pediatric Crohn's Disease.

Purpose: This study evaluated the predictive role of fecal calprotectin (FC) measured at an early stage of treatment for monitoring clinical remission (CR) after six months and endoscopic remission (ER) after one year of treatment in pediatric Crohn's disease (CD). Methods: This retrospective study included 45 patients who simultaneously underwent ileocolonoscopy and FC testing during follow-up. FC levels were measured before and after six weeks of treatment. CR was assessed after six months of treatment using Pediatric Crohn' s Disease Activity Index and acute-phase reactants. ER was assessed after one year using the Simple Endoscopic Score for Crohn's Disease. Results: Twenty-nine (64.4%) patients used oral prednisolone for remission induction and 16 (35.6%) patients used anti-tumor necrosis factor-alpha. Thirty (66.7%) patients achieved CR, while 24 (53.3%) achieved ER. The FC level measured after six weeks of treatment could predict CR (χ2=9.15, p=0.0025) and ER (χ2=12.31, p=0.0004). The δFC could predict CR (χ2=7.91, p=0.0049), but not ER (χ2=1.85, p=0.1738). With a threshold of ≤950.4 µg/g, FC at week six could predict CR with 76.7% sensitivity and 73.3% specificity. The area under the curve (AUC) was 0.769 (standard error 0.0773, p=0.0005). The same threshold predicted ER with 87.5% sensitivity and 71.4% specificity. The AUC was 0.774 (standard error 0.074, p=0.0002). Conclusion: FC assay at an early stage of treatment can be used as a surrogate marker to predict CR and mucosal healing in pediatric CD.

Epidemiological Trends of Pediatric Inflammatory Bowel Disease in Korea: A Multicenter Study of the Last 3 Years Including the COVID-19 Era.

BACKGROUND: Studies on how the coronavirus pandemic has affected pediatric inflammatory bowel disease (PIBD) are lacking. We aimed to investigate the trends in epidemiology, characteristics, initial management, and short-term outcomes of PIBD in South Korea over the recent three years including the era of coronavirus disease 2019 (COVID-19). METHODS: This multicenter study retrospectively investigated temporal trends in the epidemiology of PIBD in Korea. Annual occurrences, disease phenotypes, and initial management at diagnosis were analyzed from January 2018 to June 2021. RESULTS: A total of 486 patients from 17 institutions were included in this epidemiological evaluation. Analysis of the occurrence trend confirmed a significant increase in PIBD, regardless of the COVID-19 pandemic. In Crohn's disease, patients with post-coronavirus outbreaks had significantly higher fecal calprotectin levels than those with previous onset (1,339.4 ± 717.04 vs. 1,595.5 ± 703.94, P = 0.001). Patients with post-coronavirus-onset ulcerative colitis had significantly higher Pediatric Ulcerative Colitis Activity Index scores than those with previous outbreaks (48 ± 17 vs. 36 ± 15, P = 0.004). In the initial treatment of Crohn's disease, the use of 5-aminosalicylic acid (5-ASA) and steroids significantly decreased (P = 0.006 and 0.001, respectively), and enteral nutrition and the use of infliximab increased significantly (P = 0.045 and 0.009, respectively). There was a significant increase in azathioprine use during the initial treatment of ulcerative colitis (P = 0.020). CONCLUSION: Regardless of the COVID-19 pandemic, the number of patients with PIBD is increasing significantly annually in Korea. The initial management trends for PIBD have also changed. More research is needed to establish appropriate treatment guidelines considering the epidemiological and clinical characteristics of Korean PIBD.

Capsule Endoscopy versus Magnetic Resonance Enterography for Evaluation of Pediatric Small Bowel Crohn's Disease: Prospective Study.

BACKGROUND: Magnetic resonance enterography (MRE) and capsule endoscopy (CE) are currently used for the evaluation of small bowel involvement in pediatric Crohn's disease (CD). Several studies have been conducted to investigate the usefulness and diagnostic accuracy of each test. However, only a few studies have been conducted to compare the performance of both tests in the assessment of pediatric small bowel CD upon diagnosis and during follow-up. Therefore, the purpose of this study was to assess the diagnostic accuracy and diagnostic consistency of CE and MRE for the evaluation of pediatric small bowel CD at the time of diagnosis and during follow-up. METHODS: Fifteen patients with pediatric CD were recruited for this study. They underwent MRE and CE concomitantly at the time of diagnosis and 10-12 weeks and one year after induction therapy for CD. MRE was interpreted using MRE global score (MEGS) and bowel wall inflammation severity diffusion-weighted imaging score (BWI-DWIS), whereas CE was interpreted using Lewis's score (LS). The two diagnostic modalities were then compared. RESULTS: Eleven patients completed MRE and CE at the time of diagnosis. Analysis of the results showed that LS had a strong correlation with MEGS and BIS-DWIS (ρ = 0.633, p = 0.037, and ρ = 0.629, p = 0.038, respectively). Nine patients completed three MREs and three CEs. LS significantly decreased throughout the sessions (p = 0.044), whereas MEGS and BIS-DWIS did not show any statistically significant changes. When LS was compared with MEGS and BIS-DWIS, both MRE indicators showed statistically significant differences throughout the sessions. CONCLUSIONS: At the time of diagnosis, the severity indexes of MRE and CE showed very good agreement. However, throughout management, MRE and CE did not show consistent changes.

Semantic Segmentation Dataset for AI-Based Quantification of Clean Mucosa in Capsule Endoscopy.

Background and Objectives: Capsule endoscopy (CE) for bowel cleanliness evaluation primarily depends on subjective methods. To objectively evaluate bowel cleanliness, we focused on artificial intelligence (AI)-based assessments. We aimed to generate a large segmentation dataset from CE images and verify its quality using a convolutional neural network (CNN)-based algorithm. Materials and Methods: Images were extracted and divided into 10 stages according to the clean regions in a CE video. Each image was classified into three classes (clean, dark, and floats/bubbles) or two classes (clean and non-clean). Using this semantic segmentation dataset, a CNN training was performed with 169 videos, and a clean region (visualization scale (VS)) formula was developed. Then, measuring mean intersection over union (mIoU), Dice index, and clean mucosal predictions were performed. The VS performance was tested using 10 videos. Results: A total of 10,033 frames of the semantic segmentation dataset were constructed from 179 patients. The 3-class and 2-class semantic segmentation's testing performance was 0.7716 mIoU (range: 0.7031-0.8071), 0.8627 Dice index (range: 0.7846-0.8891), and 0.8927 mIoU (range: 0.8562-0.9330), 0.9457 Dice index (range: 0.9225-0.9654), respectively. In addition, the 3-class and 2-class clean mucosal prediction accuracy was 94.4% and 95.7%, respectively. The VS prediction performance for both 3-class and 2-class segmentation was almost identical to the ground truth. Conclusions: We established a semantic segmentation dataset spanning 10 stages uniformly from 179 patients. The prediction accuracy for clean mucosa was significantly high (above 94%). Our VS equation can approximately measure the region of clean mucosa. These results confirmed our dataset to be ideal for an accurate and quantitative assessment of AI-based bowel cleanliness.

Image quality and diagnostic accuracy of reduced-dose computed tomography enterography with model-based iterative reconstruction in pediatric Crohn's disease patients.

This study assessed the image quality and diagnostic accuracy in determining disease activity of the terminal ileum of the reduced-dose computed tomography enterography using model-based iterative reconstruction in pediatric patients with Crohn's disease (CD). Eighteen patients were prospectively enrolled and allocated to the standard-dose (SD) and reduced-dose (RD) computed tomography enterography (CTE) groups (n = 9 per group). Image quality, reader confidence in interpreting bowel findings, accuracy in determining active CD in the terminal ileum, and radiation dose were evaluated. Objective image quality did not show intergroup differences, except for image sharpness. Although reader confidence in detecting mural stratification, ulcer, and perienteric fat stranding of the RD-CTE were inferior to SD-CTE, RD-CTE correctly diagnosed active disease in all patients. The mean values of radiation dose metrics (SD-CTE vs. RD-CTE) were 4.3 versus 0.74 mGy, 6.1 versus 1.1 mGy, 211.9 versus 34.5 mGy∙cm, and 4.4 versus 0.7 mSv mGy∙cm for CTDIvol, size-specific dose estimation, dose-length product, and effective dose, respectively. RD-CTE showed comparable diagnostic accuracy to SD-CTE in determining active disease of the terminal ileum in pediatric CD patients. However, image quality and reader confidence in detecting ulcer and perienteric fat stranding was compromised.

Diagnosis of Nonmigrating Metallic Foreign Bodies in the Abdomen Using Ultrasound: An Alternative Approach Using a Traditional Method.

Ingestion of foreign bodies (FBs) is a common phenomenon among young children. Plain radiography is the first step diagnostic modality to detect the radio-opaque FBs. And computed tomography has been recommended by several guidelines as useful modalities for diagnosing ingested FBs. However, there is a risk of radiation exposure, making it burdensome to use in asymptomatic patients. Ultrasound (US) is not a commonly used technique for diagnosing ingested foreign bodies. However, US can provide real-time imaging with good resolutions without radiation exposure in pediatric patients. Herein, we report two pediatric cases of metallic foreign body ingestion that were successfully diagnosed using US for localizing foreign bodies. This study indicates that US may be used as an alternative method for detecting the localization of metallic foreign bodies in the gastrointestinal tract without exposure of radiation, particularly in pediatric patients.

Experience with a Hybrid Procedure Involving Laparoscopic Fundoplication with Percutaneous Endoscopic Gastrostomy in Chronically Ill Children.

Gastrostomy with concurrent laparoscopic Nissen fundoplication (LNF) is often performed as a laparoscopic gastrostomy (LG) by surgeons. Since 2014, we started performing percutaneous endoscopic gastrostomy (PEG) as gastrostomy with LNF. This study aims to compare the outcomes of LG and PEG with LNF. Patients were recruited into two groups: LNF with LG (historical control) or PEG. Demographic data, operation time, time to start feeding, time to full feeding, length of hospital stay (LOS), and complications were compared between the groups. Fourteen patients underwent LNF with LG and 49 underwent LNF with PEG. The median age and body weight of patients were 4.25 years and 14.15 kg in the LG group and 2.58 years and 10.60 kg in the PEG group, respectively. Operation times were significantly shorter in the PEG group (1.81 vs. 2.61 h). The times to start feeding and full feeding as well as LOS were shorter in the PEG group. Nevertheless, complications were similar in both groups. In conclusion, PEG with LNF was associated with significantly shorter operation times, times to start feeding and reach full feeding, and LOS. PEG is a suitable method for LNF in chronically ill children.

Delineation of the genetic and clinical spectrum, including candidate genes, of monogenic diabetes: a multicenter study in South Korea.

OBJECTIVES: Monogenic diabetes includes a group of heterogeneous diabetes types. We aimed to identify the frequency, clinical and molecular features of monogenic diabetes in a Korean pediatric cohort. METHODS: A retrospective cohort and multicenter study of Korean children suspected to have monogenic diabetes, managed by four pediatric endocrine centers in the southeast region of South Korea, from February 2016 to February 2020. We recruited 27 pediatric Korean patients suspected to have monogenic diabetes who had at least two of the following three criteria (age at diagnosis, family history, and clinical presentation). Targeted exome sequencing was conducted in these patients. The functional consequences of the variants were predicted by bioinformatics and protein structure analysis. RESULTS: Molecular genetic analysis identified 16 patients (59.3%) with monogenic diabetes. We identified a total of eight unique variants, including five novel variants (HNF4A c.1088C>T, CEL c.1627C>T and c.1421C>T, PAX4 c.538+8G>C, INS c.71C>T). We also identified two potential candidate gene variants for monogenic diabetes, namely c.650T>C in the SLC2A2 gene and c.629G>A in the PTF1A gene. Other variants were identified in the WFS1and NPHP3 genes in two rare genetic disorders. Variant-positive individuals had a lower presence of autoantibody positivity at the time of diagnosis and higher glycosylated hemoglobin levels at last follow-up when compared to variant-negative patients (p<0.001 and p=0.029, respectively). CONCLUSIONS: These results further expand the spectrum of known variants as well as potential candidate gene variants associated with monogenic diabetes in Korea.

Cholecystectomy is Feasible in Children with Small-Sized or Large Numbers of Gallstones and in Those with Persistent Symptoms Despite Medical Treatment.

PURPOSE: We investigated the clinical features and factors affecting the choice of treatment modality and the course of pediatric gallstone (GS) disease. METHODS: We retrospectively analyzed the medical records of 65 patients diagnosed with GS using imaging studies between January 2009 and December 2017 were included. RESULTS: This study included 65 patients (33 boys and 32 girls; mean age, 8.5±5.3 years; range, 0.2-18 years) who primarily presented with abdominal pain (34%), jaundice (18%), and vomiting (8%). Idiopathic GS occurred in 36 patients (55.4%). The risk factors for GS included antibiotic use, obesity, hemolytic disease, and chemotherapy in 8 (12.3%), 7 (10.8%), 6 (9.2%), and 4 patients (6.2%), respectively. We observed multiple stones (including sandy stones) in 31 patients (47.7%), a single stone in 17 (26.2%), and several stones in 17 (26.2%). GS with a diameter of <5 mm occurred in 45 patients (69.2%). Comorbidities included hepatitis, choledocholithiasis, cholecystitis, and acute pancreatitis in 20 (30.8%), 11 (16.9%), 11 (16.9%), and 4 patients (6.2%), respectively. Ursodeoxycholic acid (UDCA) was administered to 54 patients (83.1%), leading to stone dissolution in 22 patients (33.8%) within 6 months. Cholecystectomy was performed in 18 patients (27.7%) (mean age, 11.9±5.1 years). Most patients treated surgically had multiple stones (83%) and stones measuring <5 mm in size (89%), and 66.7% of patients had cholesterol stones. CONCLUSION: Cholecystectomy is feasible in patients with small-sized or large numbers of GS and those with persistent abdominal pain and/or jaundice. UDCA administration with close follow-up is recommended in patients with uncomplicated GS.

Medication-Related Knowledge and Medication Adherence in Pediatric and Adolescent Patients with Inflammatory Bowel Disease.

BACKGROUND: Non-adherence to oral maintenance therapy in adolescents with inflammatory bowel disease (IBD) is a significant healthcare problem. Knowledge of the prescribed medication can increase medication adherence. We aimed to investigate the relationship between medication adherence and disease-related knowledge of pediatric and adolescent patients with IBD. METHODS: We conducted a "pop quiz" to investigate the disease-related knowledge of pediatric patients with IBD who were followed-up at our institution and reviewed their medical records, including data on sex, diagnosis, age at diagnosis, and disease duration. Medication adherence was evaluated by the doctor in charge, and ≥ 80% of patients constituted the "good adherence group." RESULTS: Of 93 patients, 59 (63.4%) were males, and 78 (83.9%) had Crohn's disease. The mean age at diagnosis was 13.8 ± 2.8 years; mean follow-up duration, 4.8 ± 3.4 years; and mean patient age, 18.6 ± 3.7 years. Only 65 patients (69.9%) knew the exact name of the medication they were currently taking, and 34 (36.6%) knew the correct dose. Only 15 patients (16.1%) knew the name of the medications they were previously taking. A total of 64 patients (66.8%) showed an adherence rate of 80% for the weekly prescribed oral medication. The patients in the poor adherence group were significantly older than those in the good adherence group (P = 0.035). The number of hospitalizations per year was statistically higher in the good adherence group (P = 0.024). The proportion of patients who knew the names of the medications they were previously taking and were aware of the side effects of the medications was significantly higher in the good adherence group (P = 0.008 and P = 0.020, respectively). CONCLUSION: Adolescent IBD patients have lower oral medication adherence. Knowledge of the prescribed medications taken previously, and the adverse effects of these medications are associated with good adherence. IBD specialists should educate the patients regarding the disease and about their medication.

Successful rapid weight reduction and the use of liraglutide for morbid obesity in adolescent Prader-Willi syndrome.

Prader-Willi syndrome (PWS), an imprinting disorder, results from the loss of expression of a paternal gene on chromosome 15q11-q13. Progressive obesity and its associated complications lead to increased morbidity and early death in PWS patients. The management techniques available for morbid obesity in adolescents and adults with PWS are limited. Herein, we report successful weight reduction in an adolescent PWS case showing morbid obesity and respiratory failure. An 18-year-old girl with PWS presented with diffuse cellulitis and dyspnea due to severe obesity. Her body weight had increased from 146 to 161 kg despite dietary restriction to 800 kcal/day, and a mechanical ventilator was required for dyspnea. During mechanical ventilation, the patient was managed using diuretics and by restricting fluid intake; her daily calorie intake was reduced to 200 kcal. This aggressive calorie and water restriction continued for 3 weeks and reduced her body weight to 118.6 kg. After transfer to the general ward, the patient was provided with growth hormone therapy and intensive aquatic rehabilitation and was administered liraglutide; as a result, her weight further decreased to 104 kg (body mass index [BMI], 50.8 kg/m2), and she was discharged. Following discharge, she maintained her BMI and adapted to 1,000 kcal/day for 1 year. Aggressive water and calorie restriction were observed as an effective method for rapid weight reduction in PWS patients, and liraglutide appeared useful in maintaining weight reduction in adolescent and adult PWS.

Newly Diagnosed Klippel-Trenaunay Syndrome Presenting with Rectal Polyposis in a Male Pediatric Patient: A Case Report.

Klippel-Trenaunay syndrome (KTS) is a rare disorder characterized by a triad of abnormal bone and soft tissue growth, the presence of a port-wine stain, and venous malformations. Gastrointestinal (GI) manifestations of KTS are relatively common and generally do not cause significant problems. However, persistence can lead to chronic GI blood loss or even massive bleeding in rare cases. The majority of the severe GI manifestations associated with KTS present as vascular malformations around the GI tract and exposed vessels can lead to serious bleeding into the GI tract. Herein, we report a case of a 16-year-old boy with severe iron deficiency anemia who was previously misdiagnosed as hemorrhoid due to small amount of chronic bleeding. The actual cause of chronic GI bleeding was from an uncommon GI manifestation of KTS as rectal polyposis.

Clinical and Imaging Findings of Neonatal Seizures Presenting as Diffuse Cerebral White Matter Abnormality on Diffusion-Weighted Imaging without any Structural or Metabolic Etiology.

Purpose: Some patients with neonatal seizures show diffuse, symmetric diffusion-restricted lesions in the cerebral white matter. The aim of this study was to describe clinical and imaging findings of patients with neonatal seizures who had diffuse, symmetric diffusion-restricted lesions without any structural or metabolic etiology. Materials and Methods: A total of 56 neonates aged less than 1 week underwent brain magnetic resonance imaging (MRI) for evaluation of seizures from November 2008 to February 2017. After excluding 43 patients, 13 patients showed diffuse white matter abnormality on diffusion-weighted imaging. Initial and follow-up clinical and MRI findings were analyzed retrospectively. Results: All 13 patients were born at full term. Among the ten patients who underwent a stool test for viruses, six were positive for rotavirus and one for astrovirus. MRI revealed diffuse, symmetric diffusion-restricted lesions distributed along the cerebral white matter, thalami, and midbrain variably. Conclusion: Diffuse, symmetric diffusion-restricted lesions involving the cerebral white matter can be seen in patients with neonatal seizures without any structural or metabolic etiology. Rotavirus is commonly but not exclusively detected in these patients. Nevertheless, viral infection-associated encephalopathy should be considered for patients with characteristic clinical and MRI findings.