An echogenic lobulated round mass in the head of pancreas was found during abdominal ultrasonography for routine checkup on a 34 year-old man. CT and MRI showed characteristic findings of lipoma. Clinical followup confirmed intrapnacreatic lipoma, which had been reported only once proviously. This lesion should be added to the list of benign connective tissue tumor of the pancreas.
Connective Tissue ; Follow-Up Studies ; Head ; Lipoma* ; Magnetic Resonance Imaging ; Pancreas ; Ultrasonography

CT scans of 23 patients with gallbladder carcinoma were retropectively reviewed to determine the incidence of lymphatic spread and to access the pattern of regional lymph node metastases. The lymphatic spread were noted in 74%(17/23). The involved patterns of regional lymph node metastases were nodes of porta hepatis 65%, portocaval nodes 41%, superior pancreaticoduodenal nodes 47%, posterior pancreaticoduodenal nodes 59%, nodes around the celiac trunk 35%, nodes around the superior mesenteric artery 29% and paraaortic nodes 35%. The findings suggested that the pattern of regional lumph node metastases in gallbladder carcinoma occurred along the normal lymphatic pathway of the gallbladder.
Gallbladder* ; Humans ; Incidence ; Lymph Nodes* ; Mesenteric Artery, Superior ; Neoplasm Metastasis* ; Tomography, X-Ray Computed*

CT scans of 23 patients with gallbladder carcinoma were retropectively reviewed to determine the incidence of lymphatic spread and to access the pattern of regional lymph node metastases. The lymphatic spread were noted in 74%(17/23). The involved patterns of regional lymph node metastases were nodes of porta hepatis 65%, portocaval nodes 41%, superior pancreaticoduodenal nodes 47%, posterior pancreaticoduodenal nodes 59%, nodes around the celiac trunk 35%, nodes around the superior mesenteric artery 29% and paraaortic nodes 35%. The findings suggested that the pattern of regional lumph node metastases in gallbladder carcinoma occurred along the normal lymphatic pathway of the gallbladder.
Gallbladder* ; Humans ; Incidence ; Lymph Nodes* ; Mesenteric Artery, Superior ; Neoplasm Metastasis* ; Tomography, X-Ray Computed*

Castleman disease is a relatively rare disease of differential diagnostic interest in patients with lymphadenophathy. The etiology and pathogenesis of the Castleman disease are still not elucidated and the MRI findings of disease has not yet been reported. Two patients with Castleman disease studied by MRI are presented: one case presented with a localized anterior mediastinal mass and the other case, with a neck mass. The lesions were characterized by relatively high signal intensities on both T1 and T2 weighted images in both cases, and significant degree of enhancement was seen in the cervical Castleman disease.
Giant Lymph Node Hyperplasia* ; Humans ; Lymph Nodes* ; Magnetic Resonance Imaging* ; Neck ; Rare Diseases

We report a case of metastatic signet ring cell carcinoma of the ureter, The patient a 50-year-old man, presented with pain in the left flank. Radiologic examination revealed partial obstruction in the mid-portion of the left ureter. Microscopically, numerous signet ring cells were infiltrated in the muscle and serosa. Although multiple bone metastases were noted, the primary site was not identified after complete clinical examination in this case, which is unique to us and may be of interest to others.
Carcinoma, Signet Ring Cell* ; Humans ; Middle Aged ; Neoplasm Metastasis ; Serous Membrane ; Ureter*

Pancreaticopleural fistula (PPF) a fistulous connection between the pancreas and pleural space due to prolonged chronic pancreatitis (CP). PPF is a very rare complication which presents in 0.4% of chronic pancreatitis cases, especially among children. We report a case involving a 3-year-old boy who presented with pleural effusion caused by a PPF, a complication of hereditary pancreatitis, which was, for the first time in Korea, successfully managed with endoscopic treatment. Chest radiography and computed tomography showed massive pleural effusion. Percutaneous catheter drainage was performed. High amylase levels were observed in the pleural fluid and serum, suggesting PPF. The patient was managed with bowel rest and octreotide infusion. Endoscopic retrograde cholangiopancreatography revealed CP, and pleural effusion was successfully managed with stent placement. PRSS1 genetic screening revealed R122H mutation.
Amylases ; Catheters ; Child ; Child, Preschool ; Cholangiopancreatography, Endoscopic Retrograde ; Drainage ; Fistula ; Genetic Testing ; Humans ; Korea ; Male ; Octreotide ; Pancreas ; Pancreatic Fistula ; Pancreatitis ; Pancreatitis, Chronic ; Pleural Effusion ; Radiography ; Stents ; Thorax

Biliary atresia (BA) is the major cause of cholestasis and the leading indication for liver transplantation (LT). However, the incidence of BA in Korea has not been reported. The aim of this study was to investigate the incidence and clinical outcomes of BA in Korea. We used the Korean universal health insurance database and extracted data regarding BA patients younger than 18 years of age admitted between 2011 and 2015. The incidence of BA was calculated by dividing the number of BA patients by the number of live births. Two hundred forty infants were newly diagnosed with BA. A total of 963 BA patients younger than 18 years of age were followed up for 5 years. The overall incidence of BA was 1.06 cases per 10,000 live births. The incidence of BA was 1.4 times higher for female patients than for male patients. Additionally, significant seasonal variation was observed; in particular, the incidence of BA was 2 times higher from June through August than from December through February. Congenital anomalies were found in 38 of 240 patients (15.8%). Congenital heart diseases were major associated congenital anomalies (6.3%). Several complications developed during the study period, including cholangitis (24.0%), varix (6.2%), and gastrointestinal bleeding (4.4%). Three hundred and one of the 963 BA patients under 18 years of age (31.3%) received LT for BA. The incidence of BA is higher in Korea than that in Western countries. We also report significant gender-associated differences and seasonal variation with respect to the incidence of BA.

PURPOSE: Monogenic inflammatory bowel disease (IBD) patients do not respond to conventional therapy and are associated with a higher morbidity. We summarized the clinical characteristics of monogenic IBD patients and compared their clinical outcomes to that of non-monogenic IBD patients. METHODS: We performed a retrospective cohort study of all children <18 years old who were diagnosed with IBD between 2005 and 2016. A total of 230 children were enrolled. Monogenic IBD was defined as a presentation age less than 6 years old with confirmation of a genetic disorder. We subdivided the groups into monogenic IBD (n=18), non-monogenic very early-onset IBD (defined as patients with a presentation age <6 years old without a confirmed genetic disorder, n=12), non-monogenic IBD (defined as all patients under 18 years old excluding monogenic IBD, n=212), and severe IBD (defined as patients treated with an anti-tumor necrosis factor excluding monogenic IBD, n=92). We compared demographic data, initial pediatric Crohn disease activity index/pediatric ulcerative colitis activity index (PCDAI/PUCAI) score, frequency of hospitalizations, surgical experiences, and height and weight under 3rd percentile among the patients enrolled. RESULTS: The initial PCDAI/PUCAI score (p < 0.05), incidence of surgery per year (p < 0.05), and hospitalization per year (p < 0.05) were higher in the monogenic IBD group than in the other IBD groups. Additionally, the proportion of children whose weight and height were less than the 3rd percentile (p < 0.05 and p < 0.05, respectively) was also higher in the monogenic IBD group. CONCLUSION: Monogenic IBD showed more severe clinical manifestations than the other groups.
Adolescent ; Child ; Cohort Studies ; Colitis, Ulcerative ; Crohn Disease ; Hospitalization ; Humans ; Immunologic Deficiency Syndromes ; Incidence ; Inflammatory Bowel Diseases ; Interleukin-10 ; Necrosis ; Retrospective Studies

PURPOSE: Vitamin D deficiency is common in Crohn disease (CD). The aim of the study was to examine the prevalence of vitamin D deficiency and evaluate the association between vitamin D status and growth outcome in Korean pediatric CD patients. METHODS: In this retrospective study, 17 children younger than 18 years old diagnosed with CD were enrolled and their serum 25-hydroxy vitamin D (25[OH]D) was checked between 2011 and 2015. We categorized the patients into two groups, Group 1 and Group 2. Group 1 included patients with serum 25(OH)D levels below 10 ng/mL, and Group 2 was for patients with a 25(OH)D serum levels between 10 ng/mL and 30 ng/mL. The z-scores for height (Htz), weight (Wtz), and body mass index (BMIz) were measured at baseline, 6 months, and 12 months. RESULTS: The mean serum 25(OH)D levels of the total 65 CD patients and 17 enrolled patients were 15.64±6.9 ng/mL and 13.1±5.1 ng/mL, respectively. There was no correlation at the beginning of the study between vitamin D level and growth parameters (Htz, Wtz, BMIz) or other variables including laboratory data and Pediatric Crohn Disease Activity Index. The Htz, Wtz, and BMIz in Group 1 showed no significant improvement at 6 months and 12 months follow-up. In Group 2, Wtz and BMIz showed significant improvements sustained until 12 months of follow-up. Htz showed no significant improvement at 6 months but there was significant improvement at 12 months. CONCLUSION: It seems that baseline vitamin D status affects growth outcome in pediatric CD.
Body Mass Index ; Child ; Crohn Disease* ; Follow-Up Studies ; Humans ; Prevalence ; Retrospective Studies ; Vitamin D Deficiency ; Vitamin D* ; Vitamins*

PURPOSE: Vitamin D deficiency is a condition widespread throughout the world. Recent studies have suggested that vitamin D deficiency was associated with obesity and metabolic syndrome. The purpose of the study was to examine the relationship between vitamin D deficiency and nonalcoholic fatty liver disease (NAFLD) in adolescents. METHODS: The data were obtained from the Korean National Health and Nutrition Examination Survey from 2008–2014. A total of 3,878 adolescents were included in the study. Vitamin D deficiency was defined as a 25-hydroxyvitamin D concentration <20 ng/mL and suspected NAFLD was defined as an alanine transaminase concentration >30 U/L. RESULTS: Vitamin D deficiency was noted in 78.9% of the studied population. Age, body mass index, waist circumference, and blood pressure, glucose, cholesterol, and triglyceride levels were significantly higher in adolescents with suspected NAFLD than in adolescents without suspected NAFLD, while the mean vitamin D level was significantly lower in adolescents with suspected NAFLD. The multivariate-adjusted odds of suspected NAFLD were higher with increased age, male gender, obesity, and metabolic syndrome. Individuals with vitamin D deficiency were at higher risk of suspected NAFLD (odds ratio, 1.77; 95% confidence interval, 1.07–2.95) after adjusting for age, gender, obesity, and metabolic syndrome. CONCLUSION: Vitamin D deficiency was associated with suspected NAFLD, independent of obesity and metabolic syndrome, in adolescents.
Adolescent ; Alanine Transaminase ; Blood Pressure ; Body Mass Index ; Child ; Cholesterol ; Glucose ; Humans ; Male ; Non-alcoholic Fatty Liver Disease ; Nutrition Surveys ; Obesity ; Triglycerides ; Vitamin D Deficiency ; Vitamin D ; Vitamins ; Waist Circumference