The Prevalence of Maturity Onset Diabetes of the Young(MODY) 3 in Children with Type 2 Diabetes Mellitus / 소아과

PURPOSE: Maturity-onset diabetes of the young(MODY) is a subtype of type 2 diabetes defined by autosomal dominant mode of inheritance, onset of diabetes usually before the age of 25 yrs, and a primary defect in the function of the beta cells of the pancreas. MODY3 is known as the most common form and is caused by mutations in hepatocyte nuclear factor(HNF)-1alpha. We examined the prevalence of MODY3 in children with type 2 diabetes mellitus(DM). METHODS: Children with type 2 DM(N=17) and their family members with type 2 DM(N=5) were enrolled. Inclusion criteria for the children were fasting C-peptide and postprandial C-peptide more than 1.0 ng/mL and 1.5 ng/mL respectively, familial type 2 DM in at least two generations, and body mass index(BMI)(kg/m(2)) less than 95th percentile. Genomic DNA was extracted from blood samples. We analyzed HNF-1alpha for mutation by DNA microarray method and direct sequencing. RESULTS: We found one case with a mutation of the promoter region of HNF-1alpha(5'-ctaGGCTAGTGGGGTTTTGCGGGGGCAGTGGGTGCAAGG-3') in one child's family member among 22 children and adult subjects with type 2 DM. CONCLUSION: Although we found a mutation of HNF-1alpha in an adult family member with type 2 DM, we did not find this mutation in a child with type 2 DM. The further investigation of MODY in children, including other types, is required.

Prevalence of Metabolic Syndrome in Obese Children / 소아과

PURPOSE: Obesity has been known to be a cause of insulin resistance and dyslipidemia, and along with coronary artery disease and diabetes, is associated with metabolic syndrome(MS). This study aimed to ascertain the cause and prevalence of MS in obese children and adolescents. METHODS: Two hundred and seventy-seven school children, who showed more than a 95th percentile of body mass index(BMI) for age and sex, underwent oral glucose tolerance tests; fasting plasma lipid profiles, leptin, and CRP were measured; and ultrasonography was done. RESULTS: Out of 277 obese children, the prevalence of MS was 37.5%, with 38.7% occurring in males and 35.2% in females. The prevalence was 20.8% in primary school children and 50.3% in middle school children. MS was present in 25.2% of mildly obese children, 43.9% of moderate and 71.4% of highly obese children, showing increased occurrence among the severer degrees of obese groups. Increased prevalence was observed in males with high blood pressure, and females with high triglyceride levels. The ratio of children satisfying more than one, more than two, more than three, more than four and all of the five criteria for diagnosis of MS were 90.6%, 63.5%, 37.5%, 8.3%, and 0.4%, respectively. Aside from diagnostic criteria for MS, a statistically significant difference was present between obese patients with or without the syndrome in such items as weight, BMI, degree of obesity, visceral fat thickness, ratio of body fat, leptin, and adiponectin, fasting and 2 hour post prandial insulin concentration. CONCLUSION: The prevalence of MS is currently on the rise among children, due to the rapidly increasing rate of obesity, westernized diet, higher calorie intake and reduced exercise. The prevalence of MS in obese children was 37.5% - higher than US results - after implementation of modified pediatric criteria established by ATP III Asia-Pacific standard in 2003. It would therefore be necessary to establish an acceptable universal standard for the diagnosis of MS, and extend the study to the general pediatric population in order to acquire more accurate data on the prevalence of the disease.

Effect of One- or Two-Week Triple Therapy with Omeprazole, Amoxicillin, and Clarithromycin on Eradication of Helicobacter pylori Infection in Children / 대한소아소화기영양학회지

PURPOSE: The triple therapy with proton pump inhibitor (PPI) has been recognized as the treatment of choice in Helicobacter pylori (H. pylori) infection in adults. However, the effect of triple therapy with omeprazole, amoxicillin and clarithromycin (OAC) on eradication of H. pylori infection in children has not been established yet. This study was performed to evaluate the efficacy of OAC triple therapy and to compare the effect of one-week with two-week therapy on H. pylori eradication. METHODS: From July 1998 to July 2000, 34 children with upper gastrointestinal symptoms, who underwent upper gastrointestinal endoscopy with biopsy at entry and 4 or more weeks after therapy, were enrolled in this study. H. pylori infection was assessed by CLO test and histologic examination (Hematoxylin-Eosin stain or Alcian yellow stain) with biopsy specimens. The regimen consisted of omeprazole (0.7 mg/kg/day), amoxicillin (50 mg/kg/day), and clarithromycin (25 mg/kg/day) for 1 week (n=21) or 2 weeks (n=13). Eradication of H. pylori was determined after the termination of treatment by the CLO test and histologic examination. RESULTS: One-week treatment group consisted of 21 children (11 male, 10 female) with a mean age of 9.5+/-3.0 years. Two-week group consisted of 13 children (4 male, 9 female) with a mean age of 9.9+/-4.0 years. The endoscopic diagnoses included nodular gastritis in 19 cases, superficial gastritis in 7 cases, gastric ulcer in 4 cases, purpuric duodenitis in 2 cases, and normal in 2 cases. H. pylori was eradicated in 28 of total 34 children (82.4%). In 1-week group, H. pylori was eradicated in 17 of 21 children (81%). In 2-week group, H. pylori was eradicated in 11 of 13 children (84.6%). In remaining 6 cases in whom H. pylori had not been eradicated with OAC regimen, H. pylori infection persisted despite of the treatment with additional drugs such as colloidal bismuth subcitrate (Denol(r)) and metronidazole. CONCLUSION: In this study, eradication rate of H. pylori with OAC regimen was 82.4%, and the triple therapy would be highly effective as primary treatment. However, there was no significant difference in the eradication rate between the 1-week and 2-week treatment group (P=0.785).

A Case of Fraley's Syndrome with Benign Hematuria

Fraley's syndrome is a disorder of vascular impression on the renal infundibulum which is associated with unexplained disabling flank pain, painless microscopic hematuria, and urinary tract infection. We experienced one case of Fraley's syndrome in a 12-year-old girl whose chief complaint was persistent microscopic hematuria. We report this case with a brief review of related literatures.