Purpose: The purpose of this study was to evaluate the efficiency of treatment of living-related liver transplantation (LRLT) with the parental heterozygote carrier graft in children with Wilson disease. Methods: We retrospectively evaluated 7 children with Wilson disease who had received liver transplantation from 1994 to 2002 at Asan Medical Center. All the donors were parental. Liver functions, Kayser-Fleischer ring, and other factors regarding to copper metabolism were analyzed. Results: Of the 7 children, 5 had fulminant hepatitis and 2 had decompensated liver cirrhosis irresponsive to medical therapy. All donors being parental, all grafts came to be heterozygote carrier grafts. Survival rate was 100% in those 7 children, 87% in all children with liver transplantation in the same period, and 84% in children with non-metabolic liver disease. After liver transplantation, all 7 children could stop low copper diet and penicillamine therapy and their AST, total bilirubin and prothrombin time were recovered to normal. After liver transplantation, ceruloplasmin and serum copper levels were also recovered to normal. A marked reduction in 24 hr-urinary copper excretion was observed in all recipients after transplantation. During follow-up, Kayser-Fleischer rings resolved completely after LRLT in 5 children and partially in 1 child. Conclusion: We concluded that living-related liver tranplantation in children with Wilson disease with parental heterozygote carrier graft is an effective treatment modality.
Purpose: We compared the therapeutic efficacy of low dose with that of standard dose of interferon (IFN) treatment and also compared the first IFN treatment with retreatment. Methods: We have studied 51 children (age, 2~14) treated for chronic hepatitis B from March 1990 to August 1999. Twenty seven children had been treated with $3\;MU/m^2$ ($2.66{\pm}0.66\;MU/m^2$) of IFN-${\alpha}$ three times a week for 6 months (range, 6~12 months), whereas 24 children with $6\;MU/m^2$ ($4.45{\pm}0.94\;MU/m^2$). There was no significant difference in gender, age, initial ALT and HBV DNA levels between each comparative group. Results: Among the 27 children treated with $3\;MU/m^2$ of IFN, ALT level had normalized in 11 children (41%) and anti-HBe seroconversion occurred in 9 children (33%) one year after the initiation of treatment. In comparison, among the 24 children treated with $6\;MU/m^2$ of IFN, ALT normalized in 12 children (50%) and anti-HBe seroconversion occurred in 7 children (29%). In comparing the first treatment group to retreatment group, ALT level had normalized in 23 children (45%) and anti-HBe seroconversion occurred in 16 children (31%) among the 51 children treated with the first course of IFN treatment. In comparison, ALT normalized and anti-HBe seroconversion occurred in 3 children (25%) among the retreated 12 children. Conclusion: There was no significant difference in the therapeutic efficacies between $3\;MU/m^2$ and $6\;MU/m^2$ dose of IFN treated groups in ALT normalization and anti-HBe seroconversion. The retreatment efficacy of IFN-${\alpha}$ was as effective as the first treatment.
Purpose: The aim of the study was to evaluate the long-term outcome of glycogen storage disease (GSD) type 1 with particular reference to hepatic adenoma and hepatocellular carcinoma, and to analyze risk factors affecting the development of hepatic adenoma in GSD type 1. Methods: Forty-three GSD type 1 patients (31 males and 12 females, mean age $13.9{\pm}6.4$ years) were analyzed retrospectively. Hepatic adenoma was detected on abdominal USG and diagnosed on histologic examination. Clinical profiles were compared between patients with hepatic adenoma (n=16) and age-matched controls without hepatic adenoma (n=16). Results: 1) Of 43 GSD type 1 patients, 16 (37.2%) had hepatic adeoma. Hepatic adenoma was detected at the age of mean $14.2{\pm}4.1$ years (range: 7.9~25.7 years). Fourteen (87.5%) adenomas were multiple at detection. 2) Comparison of the clinical profiles between adenoma group and non-adenoma group revealed that age at first introduction of uncooked cornstarch treatment was significantly late in adenoma group compared with non-adenoma group ($9.1{\pm}5.2$ years vs. $3.0{\pm}1.8$ years, p=0.003). Portocaval shunt surgery was performed in 11 (68.8%) patients in adenoma group and 3 (18.8%) in non-adenoma group (p=0.004). Hepatic adenoma developed mean $5.8{\pm}4.2$ years after shunt operation. 3) One patient was diagnosed as hepatocellular carcinoma at the age of 25.7 years. Conclusion: Hepatic adenoma is an important late complication of GSD type 1 with the risk of malignant transformation. Early introduction of cornstarch therapy with strict metabolic control is needed to prevent the development of hepatic adenoma in GSD type 1.
Purpose: Recently, while the authors were experiencing that the epidemic period of rotaviral infection happened more in the early spring, we tried to find out how the outbreaks of rotaviral infection are changing in detail depending on the weather condition since it has something to do with the climate factors and PM10. Methods: Fourteen hundreds seventy nine patients who were proved to be positive to rotavirus were chosen among children less than 5 years old from January 1995 to June 2003. Among various climate factors, monthly average temperature, humidity, rainfall and PM10 were selected. Results: Rotaviral infection was most active in 2002 as 309 (20.9%) patients. It has been the spring that is the most active period of rotaviral infection since 2000. The temperature (RR=0.9423, CI=0.933424~0.951163), rainfall (RR=1.0024, CI=1.001523~1.003228) and PM10 (RR=1.0123, CI=1.009385~1.015248) were significantly associated with the monthly distribution of rotaviral infection. Conclusion: Through this study we determined that the epidemic period of rotaviral infection is changed to spring, which is different from the usual seasonal periods such as late fall or winter as reported in previous articles. As increased PM10 which could give serious influence to the human body, and changing pattern of climate factors such as monthly average temperature and rainfall have something to do with the rotaviral infection, we suppose that further study concerning this result is required in the aspects of epidemiology, biology and atmospheric science.
Purpose: This study was undertaken to evaluate the gastroduodenal pathology and Helicobacter pylori infection in children with upper gastrointestinal symptoms. Methods: One hundred and seven pediatric patients with upper gastrointestinal symptoms were undergone endoscopy at the Gyeongsang National University Hospital from June 1990 to April 1991. Histopathologic examination was done by H & E staining of gastric antral biopsy specimen and gastritis was defined according to the Sydney System. Tissue H. pylori status was evaluated with the urease test using Christensen's urea broth and H & E or Warthin-Starry silver staining of gastric antral biopsy specimen. IgG Immunoblotting were also performed to detect specific anti-H. pylori antibody in these patients. Results: The reasons for endoscopy were recurrent abdominal pain, acute abdominal pain, sallow face, hunger pain, and frequent nausea. Variable degrees of gastric mucosal hyperemia were found in most of the patients. Gastric hemorrhagic spots, gastric ulcer, duodenal ulcer, duodenal erosion, and hemorrhagic duodenitis were rare endoscopic findings. Histologic chronic gastritis was found in 88% of 107 patients. Histologic chronic duodenitis was observed in all 99 patients whose tissue were available. Gastric tissue H. pylori was positive in 57% of 107 patients by one of the ureasetest, H & E staining and Warthin-Starry silver staining. However, gastric tissue H. pylori detection rate was lower in the younger age groups. Anti-H. pylori IgG antibodies were detectable in 96% of 107 patients. Conclusion: Chronic gastroduodenitis and anti-H. pylori IgG antibody were ubiquitous in children with upper gastrointestinal symptoms.
Purpose: Recently, the incidence of acute hepatitis A has increased nationwide and is related to a low rate of IgG anti-HAV production. To establish effective measures for preventing hepatitis A virus infection, an epidemiologic study on the seroprevalence of anti-HAV is needed. Thus, we investigated the seroprevalence of IgG anti-HAV in children living in Gwangju and Jeonnam. Methods: IgG anti-HAV levels were measured in a total of 1,435 patients who visited Chosun University Hospital between January 2009 and December 2009. Results: The overall seropositve rate was 40.8% (586/1,435). The seropositive rates were 41% among children under the age of 1 year, 49.9% for children 1~5 years old, 51.1% among individuals 5~10 years old, 12.9% for individuals 10~15 years old, and 8.2% for subjects over 15 years old. There was no significant difference between genders in any group. The seropositive rates in Gwangju and Jeonnam were 57.3% and 32.9% for children under the age of 1 year, 52.5% and 44.3% for children 1~5 years old, 60.2% and 33.9% among children 5~10 years old, 14.1% and 9.7% for children 10~15 years old, and 10.8% and 4.2% for individuals over 15 years old. Conclusion: The results demonstrated the low rates of IgG anti-HAV, particularly among subjects over 10 years old, which suggests the possibility of increasing clinical HAV infection rates among adults in the near future. We should actively prevent the spread of hepatitis A virus. Vaccination is the most effective means of preventing hepatitis A virus transmission among persons at risk for infection. Hepatitis A vaccination is recommended for children who have low IgG anti-HAV seropositive rates.
Purpose: We previously reported that concurrent reactivation of latent Epstein-Barr virus (EBV) in children with hepatitis A virus (HAV) infection is common and EBV reactivation with HAV infection adversely affects the clinical features of hepatitis. However, the incidence of concurrent reactivation was not accurate because the detection of EBV reactivation was based on serologic methods. Therefore, we studied the effects of polymerase chain reaction (PCR)-proven EBV reactivation, thus a more precise concurrence, on acute HAV infection in children. Methods: PCR were conducted in 34 patients, who had enrolled previous study and diagnosed with acute HAV infection between January 2008 and June 2010. Their medical records were reviewed. Results: Among 34 patients with acute HAV infection, 12 patients (35.3%) had EBV reactivation which was proven using serologic and molecular biologic techniques. There were significant differences in the peak levels of AST and ALT between the reactivated and non-reactivated groups (p=0.001 and p<0.001, respectively). The duration of full recovery from hepatitis was more prolonged in the reactivated group (p<0.001). Clinical parameters, such as serum protein (p<0.001) and albumin concentrations (p<0.001), atypical lymphocyte count (p=0.001), prothrombin time-international normalized ratio (PT-INR, p<0.001), and splenomegaly (p<0.001), showed significant differences. The clinical features in the reactivated sub-group >10 years of age revealed more liver dysfunction compared to the non-reactivated sub-group. A comparison with a previous study was performed. Conclusion: PCR-proven reactivation of latent EBV in children with HAV infection is common and EBV reactivation with HAV infection adversely affects the clinical features of hepatitis, especially in older children.
Purpose: Mecnoium obstruction in very low birth weight infants (VLBWI), which delays enteral feeding and is one of the major causes of bowel obstruction, can be diagnosed and treated with hyperosmolar water-soluble contrast enema. The purpose of this study was to observe the clinical findings of meconium obstruction, the improvement of small bowel obstruction after contrast enema, and the complications related to the enema. Methods: Hypersolmolar water-soluble contrast enemas were performed in 14 VLBWIs with meconium obstruction. Clinical findings, radiologic findings, feeding intolerance, effectiveness, and complications of enemas were observed. Also, clinical findings related to meconium obstruction were compared with 18 VLBWIs without meconium obstruction. Results: 1) Fourteen VLBWIs with meconium obstruction had significantly lower 5 minutes Apgar scores than 18 VLBWIs without meconium obstruction (p<0.05). Moreover, the day of last meconium passing, and the day of the first trial and full enteral feeding were delayed significantly. 2) A total of 18 enemas were performed in the 14 infants. The contrast medium passed the ileocecal valve and reached the terminal ileus in 12 enemas. Of the 12 enemas, 11 were successful, but 1 infant underwent an ileotomy, even though the contrast medium reached the terminal ileum. 3) Intestinal obstruction was not relieved in three of five infants, in whom the contrast medium failed to pass the ileocecal valve. Obstruction was relieved after repeated enemas in which the contrast medium reached the terminal ileum. 4) No complications associated with water-soluble contrast enemas were observed. Conclusion: Hyperosmolar water-soluble contrast enema is considered to be safe and therapeutic for meconium obstruction in VLBWIs.
Purpose: To assess the relationship between lifestyle and metabolic syndrome in obese children and adolescents. Methods: We retrospectively reviewed the medical records and laboratory results of 109 subjects (7~15 years of age) who visited our pediatric obesity clinic between January 2004 and December 2007. They completed the parent- and self-report questionnaire developed by the Committee on Nutrition of the Korean Pediatric Society to assess lifestyle. The metabolic syndrome was defined as having 3 or more of the following metabolic risk factors: obesity, hypertension, serum triglycerides ${\geq}$110 mg/dL, HDL-cholesterol ${\leq}$40 mg/dL, fasting glucose ${\geq}$110 mg/dL, and insulin ${\geq}20{\mu}IU/mL$. Results: All subjects had at least 1 risk factor (obesity). Sixty-three percent of subjects had 2 or more risk factors, 32% of subjects had 3 or more risk factors, and 10% had 4 or more metabolic risk factors. Hypertriglyceridemia (36%), hypertension (32%), hyperinsulinemia (24%), and HDL-hypocholesterolemia (20%) were observed. Fasting blood glucose levels were normal in all subjects. Hypertension was significantly associated with an unbalanced diet and hyperinsulinemia was significantly associated with parental obesity (p<0.05). Those who ate after 8 PM were at a risk of hypertension (odds ratio, 2.5; 95% CI, 1.0~6.1). Those who did not have a preference for exercise were at a risk of hyperinsulinemia (odds ratio, 10.4; 95% CI, 2~54.1). Those who watched TV for ${\geq}$3 hours/day were at a risk of metabolic syndrome (odds ratio, 4.8; 95% CI, 1.2∼18.8). Conclusion: Lifestyle, such as eating late, no preference for exercise, and TV watching ${\geq}$3 hours/day, were related to metabolic syndrome in obese children and adolescents.
Purpose: This study was designed to characterize the nutritional status and assess obesity to determine the relationship between obesity and serum lipid profiles in 6~7 year old children. Methods: In 2007, we surveyed 483 children (233 boys and 250 girls) aged 6~7 years. The total cholesterol, triglyceride levels and HDL-cholesterol were measured in the fasting state. Dietary information was obtained by a questionnaire. Results: The prevalence of obesity was 9.9%. There was no significant difference between genders. The mean caloric intake was 1,781 kcal in boys and 1,640 kcal in girls. The prevalence of excessive calories was 33% in boys and 30% in girls. The prevalence of a total cholesterol ${\geq}$200 mg/dL was 8.4%, TG ${\geq}$130 mg/dL was 5.0%, LDL-cholesterol ${\geq}$130 mg/dL was 3.1%, and HDL-cholesterol <35 mg/dL was 4.4%. The prevalence of hypertension was 2.1%. There was no significant difference between genders. The systolic blood pressure, triglyceride levels and LDL-cholesterol were significantly related to an increased obesity index (p<0.05). The mean caloric intake and nutritive component were not related to the obesity index. The obesity group was compared to the control group: for triglycerides ${\geq}$130 mg/dL the odds ratio was 4.08; for LDL-cholesterol ${\geq}$130 mg the odds ratio was 2.85; for a TC/HDL-cholesterol ${\geq}$4.0 the odds ratio was 1.16. The BMI and triglyceride levels in the group with hypertension were higher than control group (p<0.05). There were significant positive correlations between the BMI and blood pressure as well as the LDL-cholesterol and triglycerides (p<0.05). The BMI was not correlated with the mean caloric intake or nutrition. Conclusion: The prevalence of hypertension and hyperlipidemia in 6~7 year old children was significantly related to an increased obesity index. The management of obesity in 6~7 year old children should include a reduction in the risk for hyperlipidemia and hypertension.
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