• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.2
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• pp.117-128
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• 2007

Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE mediated hypersensitivity disorder, which is associated with mainly gastrointestinal symptoms and has a delayed onset. The vomiting and/or diarrheal symptoms of FPIES typically begin in the first month of life in association with a failure to thrive, metabolic acidosis, and shock. Therefore, the differential diagnosis of FPIES and neonatal or infantile sepsis-like illnesses or gastroenteritis is difficult. The early recognition of indexes of suspicion for FPIES may help in the diagnosis and treatment of this disorder. The diagnosis of FPIES is generally made through clinical practice and food-specific IgE test findings are typically negative in this condition. Therefore, oral cow's milk challenge (OCC) remains the valid diagnostic standard for FPIES. An investigation of positive OCC outcomes helps to find out a diagnostic algorithm of criteria of a positive challenge in FPIES. Moreover, it has not been clearly determined in infantile FPIES when $1^{st}$ follow up-oral food challenge (FU-OFC) should be performed, with what kind of food protein (e.g., cow's milk, soy), and how much protein should be administered. Hence, to prevent the risk of inappropriate FU-OFC or accidental exposure and achieve appropriate dietary management, it is necessary to identify tolerance rates to major foods under the careful follow up of infantile FPIES patients. On the other hand, small intestinal enteropathy with villous atrophy is observed in FPIES and this enteropathy seems to be in part induced by both of epithelial apoptosis and intercellular junctional complex breakdown. The purpose of this report is to introduce an update on diagnostic and therapeutic approaches in FPIES and suggest the possible histopathological evidences in this disorder.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.20 no.2
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• pp.94-99
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• 2017

Purpose: Abdominal cysts of gastrointestinal origin are rare. Their rarity and varied clinical presentations make their pre-operative diagnosis difficult. Methods: Fourteen patients with histological diagnosis of cysts of gastrointestinal origin admitted between 2009 and 2015 were retrospectively analyzed with respect to age, sex, clinical presentation, diagnostic modality, site and type of cyst, management, outcome and follow-up. Results: The mean age at presentation was 4 years and there were six males and eight females. Abdominal pain was the most common presenting symptom. Five patients had an acute presentation-three had distal ileal mesenteric cysts and two had ileal duplication cyst sharing a common wall with ileum. Six patients presented with chronic abdominal pain and lump-three patients had omental cysts and three had mesenteric cysts-two of these in distal ileum and one in sigmoid colon. Two patients presented with antenatally diagnosed palpable abdominal lump. One had a mesenteric cyst of the ileum and the other had a distal ileal duplication cyst which required excision with resection and anastomosis. One patient had an atypical presentation. He was a known case of sickle cell trait and had presented with vague abdominal pain, recurrent cough and multiple episodes of haemoptysis over a period of one year. At laparotomy, gastric duplication cyst was found which was excised completely. Histopathology confirmed the diagnosis. Conclusion: Cysts of gastrointestinal origin are rare and have varied presentation. Surgical excision is the mainstay of treatment. The results and prognosis are good.

• The Korean Journal of Food And Nutrition
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• v.10 no.2
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• pp.186-192
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• 1997

Endoscopic diagnosis and medical management from K43 and C45 patients, whose sex, occupation, character and life patterns are quite different each other, were discussed. The K43 was patient with erosive gastritis, which may caused by maldietary habits and life stresses. Although the effective drugs such as H2-receptor antagonists with atacids, H+/K+-pump inhibitors, prokinetics, colloidal bismuth, and sucralfates, were administrated for long time, symptomatic relief of abdominal pain was not improved. The tests of clinical phathology and abdominal sonogram were normal, visceral hypersensitivity was appeared from barostat test of stomach. However C45 had taken headache during every day life, and endoscopically confirmed as a gastric·duodenal ulcer patiant, which may caused by chronic use of NSAIDs. The her ulceric symptoms were suppressed from active stage to healing stage by using combination therapy for H. pylori with traditional antipeptic ulcer drugs, where as amitriptyline was administrated to the K43 but not effective. Nonulcer dyspepsia and irritable bowel syndrome is pooly defined, and affect about 15% of the population annually in U. S. A. but don't seek medical care. Author would like to point out that statistics for visceral pains are absent in Korea.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.18 no.4
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• pp.268-275
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• 2015

Purpose: Primary sclerosing cholangitis (PSC) is a rare condition that can be associated with inflammatory bowel disease (IBD). The aim of this study was to evaluate PSC and its association with IBD in children. Methods: We retrospectively enrolled 13 pediatric patients (<18 years) with PSC treated at Asan Medical Center between June 1989 and December 2013. Clinical findings and long-term outcomes were investigated. During the same period, the incidence of PSC among IBD patients was evaluated among 600 Crohn disease (CD) and 210 ulcerative colitis (UC) patients. Results: All 13 study patients diagnosed with PSC also presented with IBD. Eleven boys and two girls with a median age of 15.0 years old (9.0-17.8 years) were included. The cumulative incidence of PSC for UC was 5.7% (12 of 210) and 0.2% for CD (1 of 600), respectively. PSC occurred during follow-up for IBD for five patients (38.5%) whereas, IBD developed during follow-up for PSC for two patients (15.4%), and was diagnosed during the initial work-up for PSC for 6 patients (46.2%). For the 77.3 month median follow-up period, 9/13 patients (69.2%), neither the clinical symptoms nor blood test results worsened. Two cases (15.4%) developed liver cirrhosis and underwent liver transplantation. Among 13 PSC patients with IBD, two (15.4%) developed colorectal cancer, and no one developed cholangiocarcinoma. Conclusion: All patients with PSC in this study had associated IBD. The incidence of PSC was not rare compared to reports in adults. PSC should be considered during the management of IBD and vice versa in children.

• Journal of Korean Clinical Health Science
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• v.4 no.4
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• pp.742-755
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• 2016

Pupose. This study aimed to analyze the relationship between metabolic syndrome and glaucoma in a middle-aged men over the age of 40's by using the data from the 5th Korean National Health and Nutrition Examination Survey(2010~2012). Methods. The subjects for the analysis were comprised of 410 patients who were at least 40 years or more with metabolic syndrome and glaucoma. The comparison between the metabolic syndrome and glaucoma according to the health demographic characteristics, health behavior and health conditions was analyzed with ${\chi}^2$-test. The complex samples logistic regression analysis was used to calculate the odds ratio of glaucoma according to the health demographic characteristics, health behavior and health conditions and the combination of the comprising factors of the metabolic syndrome. Results. The prevalence of glaucoma among the subjects with metabolic syndrome and glaucoma was significantly higher in the case of females, as their age increased, educational level decreased, income level decreased, who are non-smokers, non-drinkers, with exercising habits of less than 3 days a week (p<0.001). However, the glaucoma OR was significantly high in case of increasing age, with exercising habits of less than 3 days a week, and hypertension (p<0.01), while the OR was significantly low in case of the drinking group (p<0.05). The results of analyzing the odds ratio for the occurrence of glaucoma with the components of metabolic syndrome showed that the OR for glaucoma is significantly low (OR 0.63) in case of concurrently high fasting blood glucose, high triglycerides and low HDL cholesterol levels (p=0.05). Conclusions. Therefore the thorough management of metabolic syndrome and components is needed to prevent glaucoma.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.3
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• pp.225-232
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• 2019

Purpose: To evaluate intra- and inter-observer variability and guideline adherence amongst pediatricians in treating children aged between 4 and 18 years referred with recurrent abdominal pain (RAP) without red flags. Methods: The first part of the study is a retrospective single-center cohort study. The diagnostic work-ups of eight pediatricians were compared to the national guidelines. Intra- and inter-observer variability were examined by Cramer's V test. Intra-observer variability was defined as the amount of variation within a pediatrician and inter-observer variability as the amount of variation between pediatricians in the application of diagnostic work-up in children with RAP. Prospectively, the same pediatricians were requested to provide a report on their management strategy with a fictitious case to prove similarities in retrospective diagnostic work-up. Results: A total of 10 patients per pediatrician were analyzed. Retrospectively, a (very) weak association between pediatricians' diagnostic work-ups was found (0.22), which implies high inter-observer variability. The association between intra-observer diagnostic was moderate (range, 0.35-0.46). The Cramer's V of 0.60 in diagnostic work-up between pediatricians in the fictitious case implied the presence of a moderately strong association and lower inter-observer variability than in the retrospective study. Adherence to the guideline was 66.8%. Conclusion: We found a high intra- and inter-observer variability and moderate guideline adherence in daily clinical practice amongst pediatricians in treating children with RAP in a teaching hospital.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.1
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• pp.100-108
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• 2021

Purpose: Percutaneous endoscopic gastrostomy (PEG) tube placements are commonly performed pediatric endoscopic procedures. Because of underlying disease, these patients are at increased risk for airway-related complications. This study compares patient characteristics and complications following initial PEG insertion with general endotracheal anesthesia (GETA) vs. anesthesia-directed deep sedation with a natural airway (ADDS). Methods: All patients 6 months to 18 years undergoing initial PEG insertion within the endoscopy suite were considered for inclusion in this retrospective cohort study. Selection of GETA vs. ADDS was made by the anesthesia attending after discussion with the gastroenterologist. Results: This study included 168 patients (GETA n=38, ADDS n=130). Cohorts had similar characteristics with respect to sex, race, and weight. Compared to ADDS, GETA patients were younger (1.5 years vs. 2.9 years, p=0.04), had higher rates of severe American Society of Anesthesiologists (ASA) disease severity scores (ASA 4-5) (21% vs. 3%, p<0.001), and higher rates of cardiac comorbidities (39.5% vs. 18.5%, p=0.02). Significant associations were not observed between GETA/ADDS status and airway support, 30-day readmission, fever, or pain medication in unadjusted or adjusted models. GETA patients had significantly increased length of stay (eβ=1.55, 95% confidence interval [CI]=1.11-2.18) after adjusting for ASA class, room time, anesthesia time, fever, and cardiac diagnosis. GETA patients also had increased room time (eβ=1.20, 95% CI=1.08-1.33) and anesthesia time (eβ=1.50, 95% CI=1.30-1.74) in adjusted models. Conclusion: Study results indicate that younger and higher risk patients are more likely to undergo GETA. Children selected for GETA experienced longer room times, anesthesia times, and hospital length of stay.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.1
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• pp.80-85
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• 2019

Purpose: Abernethy malformation is a rare condition, which was first described in 1793 as a congenital extrahepatic porto-systemic shunt (CEPS) directing splanchnic blood flow into the inferior vena cava. Eighty cases have been published so far that reported CEPS, while in Korea, very few cases have been reported. Through this study, we present 6 cases of patients diagnosed with CEPS at Samsung Medical Center and compare these with other such cases published in France and China. Methods: We reviewed clinical, laboratory, and imaging data of 6 children with CEPS in our pediatric clinic between 2004 and 2017. Results: A total of 6 children with CEPS was included in this study, namely, one with type 1a, two with type 1b, and three with type 2 CEPS. The most common presenting symptom was gastrointestinal bleeding (50.0%). Therapeutic interventions included shunting vessel ligation (16.7%) in type 2 CEPS and liver transplantation (16.7%) in type 2 CEPS patient with suddenly developed hepatic encephalopathy. Conclusion: There is no consensus guideline for the optimal management of patients with CEPS. Large-sample studies regarding CEPS are needed to evaluate the characteristics of patients with CEPS and determine the treatment guideline for CEPS.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.3
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• pp.282-290
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• 2019

Characterizing inflammation and fibrosis in Crohn's disease (CD) is necessary to guide clinical management, but distinguishing the two remains challenging. Novel ultrasound (US) techniques: contrast-enhanced US (CEUS) and shear wave elastography (SWE) offer great potential in evaluating disease activity in pediatric patients. Three patients ages 16 to 20 with known CD underwent CEUS and SWE to characterize bowel wall inflammation and fibrosis. Magnetic resonance enterography, endoscopy, or surgical pathology findings are also described when available. The patients' disease activity included acute inflammation, chronic inflammation with stricture formation, and a fibrotic surgical anastomosis without inflammation. CEUS was useful in determining the degree of inflammation, and SWE identified bowel wall fibrosis. Used together these techniques allow for better characterization of the degree of fibrosis and inflammation in bowel strictures. With further validation CEUS and SWE may allow for improved characterization of bowel strictures and disease flares in pediatric patients suffering from CD.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.1
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• pp.30-37
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• 2021

Purpose: To investigate the differences in the colon microbiota composition of Hirschsprung's disease (HSCR) patients with and without a history of postoperative Hirschsprung's associated enterocolitis (HAEC). Methods: Colon tissue microbiota was characterized by bacterial deoxyribonucleic acid (DNA) extraction and 16S rDNA sequencing for taxonomic classification and comparison. Results: The sequence diversity richness within samples was significantly higher in samples from patients with a history of postoperative HAEC. We observed an increased relative abundance of the phyla Bacteroidetes, Firmicutes and Cyanobacteria in HAEC patients and Fusobacteria, Actinobacteria and Proteobacteria in HSCR patients and, an increased relative abundance of the genera Dolosigranulum, Roseouria and Streptococcus in HAEC patients and Propionibacterium and Delftia in HSCR patients. Conclusion: Our findings provide evidence that the colon tissue microbiota composition is different in HSCR patients with and without postoperative HAEC.