Usefulness of early endoscopy for predicting the development of stricture after corrosive esophagitis in children / 소아과

PURPOSE: This study was performed to demonstrate the usefulness of early endoscopy for predicting the development of stricture following corrosive ingestion in children. METHODS: We conducted a retrospective study on 34 children who were brought to Seoul National University Childrens Hospital and Seoul National University Bundang Hospital for corrosive ingestion from 1989 to 2007. RESULTS: The corrosive burns were classified as grade 0 in 8 patients, grade 1 in 2, grade 2a in 7, grade 2b in 13, and grade 3 in 4. There was no significant correlation between the presence of esophageal injury and symptoms including vomiting, dysphagia, and drooling. There was a statistically significant relation between the presence of oropharyngeal injury and esophageal injury (P=0.014). There were no complications including hemorrhage and perforation related to endoscopy. Strictures of the esophagus or the stomach developed in 12 patients (36.4%). Esophageal stricture was observed in 11 patients and pyloric stenosis in 1 patient. The endoscopic grade of mucosal injury was significantly related to the frequency of development of esophageal stricture (P=0.002). Two of eleven patients with esophageal stricture responded to repeated dilation. The remaining seven patients underwent surgery. CONCLUSION: Early esophagogastroduodenoscopy is not only a safe and useful diagnostic tool for children with accidental caustic ingestion but also a necessity for determining the degree and the extent of caustic burns and for predicting the development of late complications.

Pharmacological Treatment for FunctionalAbdominal Pain in Children / 대한소아소화기영양학회지

Functional gastrointestinal disorders are among the most common medical problems in children. Abdominal pain-related functional gastrointestinal disorders can be categorized as functional dyspepsia, irritable bowel syndrome, abdominal migraine and childhood functional abdominal pain according to the Rome III criteria for pediatric functional gastrointestinal disorders. The aim of this paper was to examine the evidence supporting the use of the range of therapeutic options available for functional gastrointestinal disorders.

Pharmacological Treatment for FunctionalAbdominal Pain in Children / 대한소아소화기영양학회지

Functional gastrointestinal disorders are among the most common medical problems in children. Abdominal pain-related functional gastrointestinal disorders can be categorized as functional dyspepsia, irritable bowel syndrome, abdominal migraine and childhood functional abdominal pain according to the Rome III criteria for pediatric functional gastrointestinal disorders. The aim of this paper was to examine the evidence supporting the use of the range of therapeutic options available for functional gastrointestinal disorders.

Trace Elements Deficiency and the Diagnostic Usefulness of Hair Mineral Analysis in Children with Chronic Gastrointestinal Disease / 대한소아소화기영양학회지

PURPOSE: Patients with chronic gastrointestinal disease are at risk for trace element deficiency due to impaired absorption and gastrointestinal loss. The aim of this study was to evaluate the trace element status of patients with gastrointestinal disease by blood and hair analysis, and to determine the usefulness of hair mineral analysis for diagnosing trace element deficiency not detected by a blood test. METHODS: An analysis of hair minerals was performed and compared with blood mineral analysis in 13 patients with chronic gastrointestinal disease. The concentration of each element in the hair and blood was compared in the subgroups based on parenteral nutritional support or clinical symptoms. RESULTS: Almost all patients had trace element deficiency. The trace elements deficient in the blood or hair analysis included zinc, selenium and copper. The hair zinc concentration was significantly lower in the group receiving parenteral nutritional support. The hair selenium concentration was statistically associated with the clinical symptoms of hair loss, brittle hair and loss of hair pigmentation. CONCLUSION: The results of this study suggest that patients with chronic gastrointestinal disease should receive adequate zinc and selenium replacement to avoid trace element deficiency especially when treated with long-term parenteral nutrition. Hair mineral analysis is useful as a complementary tool for the detection of a trace element deficiency.

A Case of Metastatic Signet Ring Cell Carcinoma ofthe Colon in a Child / 대한소아소화기영양학회지

Colorectal carcinomas are extremely rare in childhood and adolescence; however, the colon is the most common site of a gastrointestinal carcinoma. Mucin secreting adenocarcinomas with signet ring formation is the most common type of colon cancer identified in children. An 11-year-old boy had abdominal pain and weight loss for three months, back pain and left thigh pain for two months, and hematochezia for four days. Colonoscopy showed an annular mass in the sigmoid colon and the histopathology revealed a signet ring cell carcinoma. A metastatic signet ring cell carcinoma was suspected from the findings of the bone scan, and confirmed later by a left scalp mass incisional biopsy and a bone marrow biopsy. We report a case of a metastatic signet ring cell carcinoma of the colon in a child.

Clinical manifestation and outcome of alagille syndrome in Korea: a study of 30 patients / 소아과

PURPOSE: The purpose of this study was to examine the clinical courses and long-term outcomes of children with Allagille syndrome in Korea, and to evaluate the prognostic potentials of identified variables. METHODS: We reviewed the clinical manifestations and outcomes of 30 children with Alagille syndrome, investigated from 1984 to 2006 until the end of this study (defined as death or last visit; mean follow-up : 5 years). RESULTS: Cholestasis occurred in 100 percent, cardiovascular abnormalities in 83.3 percent, butterfly vertebrae in 30.0 percent, posterior embryotoxon in 43.3 percent, and a characteristic facial appearance in 100 percent. At study conclusion, of these 30 patients, eight had died (26.7 percent); six related to Alagille syndrome. Five patients died of a liver disease complication. Liver transplantation was carried out in five of the 30 patients (16.7 percent) and one of these died due to hyperacute rejection. At age two, cholestasis improved in 17 of the 30 patients. Those who had severe cholestasis at 2 years of age tended to have a complication, such as liver cirrhosis or liver transplantation, or to have died. CONCLUSION: Hepatic complications account for the most mortalities in patients with Alagille syndrome. Careful and complete assessments should be made in children who have cholestasis at 2 years of age. Further investigations of more cases are required.

A case of hereditary pancreatitis with a N29I mutation in the cationic trypsinogen gene / 소아과

Hereditary pancreatitis is an autosomal dominant disease characterized by recurrent episodes of pancreatitis, often beginning in childhood, with a positive family history involving at least two other affected family members with no known other precipitating factors. Most forms of hereditary pancreatitis are caused by one of two common mutations, i.e., R122H in exon 3 and N29I in exon 2 of the cationic trypsinogen (CT) (PRSS1) gene, located on chromosome 7. The authors describe the case of a 15-year-old boy who had suffered from recurrent attacks of pancreatitis since age three. His mother and grandmother had chronic pancreatitis and diabetes mellitus. Mutation analysis was performed on the family due to the suspicion of hereditary pancreatitis. The CT gene was analyzed in DNA samples extracted from the peripheral blood of three family members, the mother, the proband, and the proband's sister. Two members of the family, the mother and the proband, were found to have a N29I mutation in the CT gene. The authors document the first family with hereditary pancreatitis associated with the N29I mutation in Korea.