• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.1
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• pp.24-31
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• 2003

Purpose: As diagnostic tools for Hirschsprung's disease (HD), barium enema and rectal biopsy have radiation exposure and invasiveness respectively; however anorectal manometry does not have these disadvantages. We therefore performed this study to evaluate the diagnostic efficacy of anorectal manometry. Methods: We reviewed medical records of infants with one or two symptoms of vomiting, abdominal distension, chronic diarrhea or constipation who had a anorectal manometry followed by barium enema and/or biopsy from July 1995 to May 2002. We evaluated the sensitivity, specificity and predictive value of anorectal manometry and barium enema for diagnosis of HD. We also measured sphincter length, median value of balloon volume at which rectoanal inhibitory reflex (RAIR) occurred. Results: All 61 patients received anorectal manometry, 33 of 61 received barium enema. 18 of 61 were diagnosed as HD according to histology and 43 of 61 were evaluated as a control. The sensitivity, specificity, positive predictive value, negative predictive value of anorectal manometry and barium enema for diagnosis of HD were 1.00, 0.91, 0.82, 1.00 and 0.93, 0.67, 0.70, 0.92 respectively. The mean value of sphincter length in control was $1.68{\pm}0.67$ cm and correlated with age, weight and significantly longitudinal length. The median value of balloon volume at which RAIR occurred was 10 mL and did not correlated with age, weight and longitudinal length. Conclusion: This study suggests that anorectal manometry is an excellent initial screening test for Hirschsprung's disease because of its safety and accuracy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.1
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• pp.32-38
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• 2003

Purpose: Hepatic allografts from donors with hepatitis B core antibody have been demonstrated to transmit hepatitis B virus (HBV) infection to recipients after liver transplantation (LT). The efficacy of hepatitis B immune globulin (HBIg) to prevent de novo hepatitis B was investigated by comparing active immunization in the early phase to HBIg monotherapy in the late phase of pediatric liver transplants at Samsung Medical Center. Methods: Among pediatric liver transplants, from May, 1996 to June, 2002, 15 recipients who were hepatitis B surface antigen (HBsAg) (-) received an allograft from a donor with hepatitis B core antibody (HBcAb) (+). Except two who died from unrelated causes, eleven of 13 recipients were HBsAb (+), and 2 were naive (HBsAb(-), HBcAb(-)). All patients were vaccinated for HBV before LT. In the early phase (January, 1997~November, 1997, 3 patients), HBsAb (+) recipients received booster vaccination after LT. In the late phase (December, 1997~, 10 patients), all recipients were given booster vaccination and received HBIg therapy in order to maintain HBsAb titer greater than 200 IU/L. Lamivudine was given in one case because of severe side effect of HBIg. We retrospectively analyzed the effect of the preventive therapy for de novo hepatitis B through medical records. Results: De novo hepatitis B developed in three of 13 recipients (23.1%). All of 3 patients who received active immunization in the early phase became HBsAg (+) at 7~19 months after transplantation. One of them was naive before LT and the other two were HBsAb (+). All of 10 recipients who were given HBIg in the late phase remained HBsAg (-) at 7~55 months' follow-up. Conclusion: Passive immunization with HBIg was effective for prevention of de novo hepatitis B in HBsAg (-) recipients of hepatic allografts from HBcAb (+) donors.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.1
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• pp.39-46
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• 2003

Purpose: In a retrospective study for the pediatric patients who underwent liver transplantation in the past 6 years at Samsung Medical Center, the clinical features of 5 patients with posttransplant lymphoproliferative disorder (PTLD) were analyzed. Methods: Between June 1996 and June 2002, 41 pediatric patients underwent liver transplantation. Seven of them died in the postoperative period. Thirty-five including one patient who died of PTLD were finally reviewed. Patients were divided into two groups: high risk group, EBV naive recipients of EBV-positive grafts; low risk group, the patients other than those in high risk group. The authors reviewed age at operation, immunosuppressive agent, postoperative duration until diagnosis, postoperative duration until EBV seroconversion, presence of treatment against rejection, and presenting symptoms of PTLD. Results: Five of 41 patients (12.2%) developed PTLD. All of them belonged to high risk group, and the incidence of PTLD in high risk group was 31.3% (5/16). The mean age at operation was 10.8 months old and the mean duration between operation and diagnosis for PTLD was 9.8 months. Primary EBV infection developed after a median of 6 months after transplantation. One patient was diagnosed as laryngeal and gastrointestinal PTLD and the other four, gastrointestinal PTLD. The following symptoms and signs were seen in the patients: anemia (100%), hypoalbuminemia (100%), fever (80%), diarrhea (80%), gastrointestinal bleeding (80%), and anorexia (60%). Conclusion: PTLD is one of the major complications after pediatric liver transplantation, especially in the group of high-risk recipients. Anemia, hypoalbuminemia, fever, diarrhea and gastrointestinal bleeding were features that are characteristic of PTLD. The common features of PTLD development were: (i) EBV-positive donors placed into EBV naive recipients, (ii) primary EBV infection about 6 months after transplantation, (iii) young age, about 1 year old at operation, and (iv) the requirement for intensive posttransplant immunosuppression.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.153-162
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• 2004

Purpose: The Helicobacter pylori stool antigen (HpSA) enzyme immunoassay is a non-invasive test for the diagnosis and monitoring of H. pylori infection. But, there are few validation studies on the HpSA test after eradication in children. The aim of this study was to assess the diagnostic accuracy of HpSA enzyme immunoassay for the detection of H. pylori to confirm eradication in children. Methods: From January 2001 to October 2003, 164 tests were performed in 146 children aged 1 to 17.5 years (mean $9.3{\pm}4.3$ years). H. pylori infection was confirmed by endoscopy-based tests (rapid urease test, histology, and culture). All H. pylori infected children were treated with quadruple regimens (Omeprazole, amoxicillin, metronidazole and bismuth subcitrate for 7 days). Stool specimens were collected from all patients for the HpSA enzyme immunoassay (Primier platinum HpSA). The results of HpSA tests were interpreted as positive for $OD{\geq}0.160$, unresolved for $$0.140{\leq_-}OD$$<0.160, and negative for OD<0.140 at 450 nm on spectrophotometer. Results: 1) One hundred thirty-one HpSA tests were performed before treatment. The result of HpSA enzyme immunoassay showed three false positive cases and one false negative case. The sensitivity, specificity, positive predictive value, and negative predictive value of HpSA enzyme immunoassay before treatment were 96.4%, 97.1%, 90%, and 99%, respectively. 2) Thirty-three HpSA enzyme immunoassay were performed at least 4 weeks after eradication therapy. The results of HpSA enzyme immunoassay showed two false positive cases and one false negative case. The sensitivity, specificity, positive predictive value, and negative predictive value after treatment were 88.9%, 91.7%, 80%, and 95.7%, respectively. Conclusion: Diagnostic accuracy of the HpSA enzyme immunoassay after eradication therapy was as high as that of the HpSA test before eradication therapy. The HpSA enzyme immunoassay was found to be a useful non-invasive method to confirm H. pylori eradication in children.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.228-238
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• 2004

Purpose: Obesity is rapidly increasing in Korean children. Obesity is a risk factor for cardiovascular morbidity and is frequently associated with hypertension, diabetes mellitus and coronary artery disease. This study was designed to evaluate risk factors of the metabolic syndrome in obese children. Methods: From February 2000 to June 2004, eighty eight obese (body mass index ${\geq}95th$ percentile) children aged 4 to 15 years were included. We measured serum lipid levels (total cholesterol, triglyceride, HDL cholesterol, LDL cholesterol), fasting sugar levels and insulin levels. Insulin resistance was determined by homeostasis model assessment, fasting insulin/glucose ratio and quantitative insulin sensitivity check index. Results: Clustering of risk factors for the metabolic syndrome in obese children demonstrated that 60.2% had more than one risk factors. Hypertension (14.8%), hypertriglyceridemia (14.8%), HDL-hypocholesterolemia (14.8%), LDL-hypercholesterolemia (12.5%) and hyperinsulinemia (12.5%) were observed. As BMI increased, there was statistically significant increase in systolic blood pressure, insulin and insulin resistance values. Insulin resistance was correlated to systolic blood pressure, serum lipid and insulin levels. The more risk factors for the metabolic syndrome obese children had, the higher was their insulin resistance. Conclusion: The increase in insulin resistance and clustering of risk factors for the metabolic syndrome are already apparent in obese children. Monitoring these risk factors for the metabolic syndrome should become a part of routine medical care for obese children.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.179-185
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• 2004

Purpose: Perinatal mortality rates have been used as a summary statistic for evaluating child health and medical status. Neonatal mortality rates have decreased over the past 30 years in Korea. To understand the current status of neonatal surgical gastrointestinal diseases in Daegu Busan area, we have studied about neonatal gastrointestinal diseases with their clinical features, postoperative outcome, and mortality rates. Methods: A clinical analysis on 202 neonates who underwent neonatal surgery from January 1996 to July 2003 at Pusan National University, Kyungpook National University, Youngnam University, and Daegu Catholic University was carried out. Results: The main diseases of surgical conditions were anorectal malformation (23.8%), atresia/stenosis of midgut (13.4%) and pyloric stenosis (13.4%). The male to female ratio was 2.8 : 1. Thirty-five cases (17.0%) had one or more associated anomalies including congenital heart disease, cryptoorchidism, hydronephrosis, and chromosomal anomaly. Twenty cases (10.0%) were diagnosed by antenatal ultrasound. Patients with esophageal atresia had the longest hospitalization for 54.6 days. Postoperative complications occurred in 18 cases (8.9%). The main postoperative complications were wound infection (3.5%) and anastomotic leakage (2.5%). Overall mortality was 5.9%. Diaphragmatic hernia showed the highest mortality rate (37.5%), and esophageal atresia (28.6%) and omphalocele (20.0%) were followed. Conclusion: The current status of neonatal surgical gastrointestinal diseases in Daegu Busan area has improved because the disease categories are various, postoperative complications and mortality rates are decreased.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.186-196
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• 2004

Purpose: Autoimmune hepatitis is a chronic inflammatory liver disease with unknown cause that is characterized by liver histology, circulating autoantibodies and increased levels of immunoglobulin G. Only sporadic reports are available on autoimmune hepatitis in children. The aim of this study was to evaluate the clinical, biochemical, and histological features, and the long-term outcome of autoimmune hepatitis in Korean children. Methods: We reviewed the medical records of 14 children diagnosed as having autoimmune hepatitis at Seoul National University Children's Hospital from 1990 to 2004, and analyzed clinical, biochemical, and histological features, and clinical outcomes. Results: Mean age at diagnosis was 9 years and 11 of the 14 children were female. Six children presented with acute hepatitis-like manifestations. Jaundice and fatigue were the most common symptoms. Other autoimmune diseases accompanied in 6 children. Anti-nuclear antibody was detected in 13 patients and anti-smooth muscle antibody was positive in 8. All 14 patients were type 1 autoimmune hepatitis. The main histologic findings were interface hepatitis, rosette formation, and cirrhosis. Clinical and biochemical features were improved in six patients treated with ursodeoxycholic acid. Eight patients were treated with corticosteroid alone or in combination with azathioprine and five of them are in biochemical remission. Conclusion: Autoimmune hepatitis is an inflammatory liver disease, which has a favorable long-term outcome if it is diagnosed and treated promptly. Therefore, autoimmune hepatitis should be suspected in children with chronic hepatitis of unknown etiology, especially in female patients who show hypergammaglobulinemia or some clinical features of autoimmune disease.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.197-207
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• 2004

Purpose: To evaluate the long-term therapeutic efficacy and durability of lamivudine in Korean children with chronic hepatitis B. Methods: A total of 48 children (31 male and 17 female; age, 1~18 years, mean, 8 years) with chronic hepatitis B who received lamivudine for at least six months from March 1999 to September 2004 were followed for a mean period of 29 months (8~66 months) at Department of Pediatrics, Kyungpook National University Hospital in Korea. Response to treatment was defined as the normalization of ALT and HBV DNA levels, and HBeAg seroconversion after the initiation of treatment. Results: Twenty nine (60%) among the 48 children treated with lamivudine responded and nine (19%) children lost HBsAg during therapy. ALT and HBV DNA level had normalized in 94% one year after the initiation of treatment. Kaplan-Meier estimates of cumulative HBeAg seroconversion rates over the years were 13% (0.5 year), 34% (1 year), 50% (1.5 years), 68% (2 years), 79% (2.5 years) and 90% at 3 years respectively. Above all, among the 22 children treated before the age of seven, loss of HBsAg occurred in eight (36%), which showed superior rate of HBsAg loss (p=0.002 vs age >7). Conclusion: Long-term treatment of lamivudine improved the rate of HBeAg seroconversion in Korean children with chronic hepatitis B. After three years' observation, most of treated children have sustained HBeAg clearance. We believe that lamivudine should be tried as the first therapeutic option for children with chronic hepatitis B in immune clearance phase.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.163-169
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• 2004

Purpose: Many diagnostic modalities have been used for diagnosis of gastroesophageal reflux disease (GERD). Feeding materials during esophageal pH monitoring may interfere the result of examination and esophageal pH monitoring can not diagnose GER in case of alkaline reflux. The purpose of our study is to evaluate whether scintigraphy can substitiute 24 hr pH monitoring in children with GERD. Methods: From March 2002 to June 2003, 23 patients (12 boys and 11 girls, mean age 27 months) who have been admitted to Hanyang University Hospital presented with GER symptoms (recurrent vomiting, cough, chest pain, irritability) were included in the study. Scintigraphy and 24 hr pH monitoring were performed in all patients. Results: Six out of the 23 patients (26.1%) had evidence of GER on 24 hr pH monitoring, whereas nine of 23 patients (39.2%) exhibited GER by scintigraphy. Two out of the 23 patients could not be tested because of irritability. Three (14.3%) patients had evidence of GER on both 24 hr pH monitoring and scintigraphy, three (14.3%) patients on only 24 hr pH monitoring, six (28.6%) patients on only scintigraphy, and nine (42.9%) patients had no evidence of GER on both methods. No correlation was observed between 24 hr pH monitoring and scintigraphic results by Fisher's exact test (p=0.523) or Kendal's tau (t=0.678). Conclusion: The results of this study demonstrated that there was no correlation between 24 hr pH monitoring and scintigraphy. Therefore these modalities could be used as complementary tests to diagnose GERD.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.208-214
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• 2004

Purpose: The prognosis of neonates with cholestasis is not clear. Some factors, such as high peak bilirubin levels and liver histologic findings have been claimed to affect the prognosis adversely. Our study aims to define which factors influence the prognosis of neonatal intrahepatic cholestasis. Methods: Retrospective reviews of the medical records were performed in 32 cases with neonatal intrahepatic cholestasis, who were admitted to Department of Pediatrics, Pusan National University Hospital from July 1995 to July 2002. Neonates were classified into 2 groups according to the duration of elevated serum alanine aminotransferase (ALT) levels more or less than 6 months. The data, such as biochemical, histopathologic and radiologic findings, were compared in both groups. Biochemical data included mean peak level of serum ALT, total bilirubin, direct bilirubin, and alkaline phosphatase. Histologic parameters related to lobular architecture, fibrosis, inflammatory infiltration and degenerative features of hepatocytes were arbitrary estimated on a scale of 1 to 3. Results: There were 19 males and 13 females, whose mean age was 48 days (14~77 days). The peak serum ALT levels were higher in the poor outcome group. Ductular proliferation and portoportal bridging were more severe in the poor outcome group. But the degree of multinucleated hepatocytes, hepatocellular swelling and canalicular plug did not appear to be significantly related to the long-term outcome. The DISIDA scintigraphy by visualization time of gall bladder and intestine was not useful in predicting outcome of neonatal intrahepatic cholestasis. Conclusion: Neonates who have intrahepatic cholestasis with high serum ALT levels, severe ductular proliferation and portoportal bridging in the liver biopsy specimen should be carefully followed up because they may have a poor prognosis.