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Purpose: To describe the clinical presentation, treatment preference, and relevant complications of infantile hepatic hemangioma (IHH) in propranolol era., Methods: The National Taiwan University Hospital integrated Medical Database (NTUH-iMD) was used to enroll twenty-one cases of IHH diagnosed from 2006 to 2020. Medical charts were retrospectively reviewed., Results: In nine patients (42.9%), IHH was found incidentally, and in seven patients (33%), it was detected during postnatal self-paid ultrasonography. Focal disease was determined in 17 patients, multifocal disease in 1 patient, and diffuse disease in 3 patients. Patients with diffuse disease had a lower hemoglobulin level than patients with focal IHH (9.38 vs. 12.6 mg/dL, p = 0.045). Two patients had Kasabach-Merritt phenomenon (KMP), one had hypothyroidism, and one had both. All patients with KMP had focal hepatic hemangiomas. Among the 17 patients with focal IHH, nine were prescribed propranolol, one was treated by surgical resection of the tumor, and the others had expectant management. All patients with multifocal and diffuse IHH were administered propranolol. One infant (7.7%) treated with propranolol had bradycardia initially but it subsided after dose adjustment., Conclusions: Most IHH is found incidentally or detected during postnatal ultrasonography screening. Patients with large focal lesions should also be screened for associated complications. Propranolol is the drug of choice and a safe therapeutic option for IHH, especially for focal tumors >5 cm as well as multifocal and diffuse lesions., (Copyright © 2024 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background: The incidence of Clostridium difficile infection (CDI) is increasing around the world, and patients with inflammatory bowel disease (IBD) have a higher risk of obtaining CDI. The data on the incidence rate of CDI in the Asian pediatric IBD population was lacking., Methods: We retrospectively collected data from a tertiary medical center in Taipei, Taiwan. All patients aged 1-18 years old who visited the outpatient department or were admitted to our hospital between 2006 and 2019 were included. CDI was defined as positive stool C. difficile toxin or C. difficile culture results with appropriate antibiotic use within the range of 7 days prior or 14 days after the result., Results: We compared the average annual incidence of CDI before and after 2013. The average incidence of community-acquired CDI (CA-CDI) increased from 0.063 to 0.564 cases per 1,000 visits, with a rate ratio (RR) of 8.82 (95% CI 5.74-14.38). In patients with IBD, the rate increased from 26.738 to 278.873 cases per 1,000 visits (RR=10.12, 95% CI: 4.57-29.02). The average incidence rate increased from 0.685 to 1.874 cases per 1,000 admissions in pediatric general patients (RR = 2.72, 95% CI 1.82-4.20) and from 14.706 to 62.500 cases per 1,000 admissions in pediatric IBD patients (RR = 3.77, 95% CI 0.71-93.53)., Conclusions: Both CA-CDI and healthcare facility-onset CDI (HO-CDI) were increasing substantially in the pediatric population over the past decade in Taiwan. Compared to the general pediatric population, pediatric IBD patients had a much higher incidence of CDI., Competing Interests: Declaration of competing interest The authors declare that they have no conflict of interest., (Copyright © 2024 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background/purpose: The status of nonalcoholic fatty liver disease (NAFLD) can wax and wane over time in children. However, the factors affecting its incidence and remission remain elusive. We aimed to investigate NAFLD incidence, remission and predicting factors in obese children., Methods: Obese children aged 9-10 and 12-13 years were recruited from schools and followed up for 2 years. Liver ultrasonography was performed at baseline and Year 1. Alanine aminotransferase (ALT) concentrations were measured at baseline, Year 1 and Year 2. Elevated ALT was defined as above 26 U/L for boys and 22 U/L for girls. Four NAFLD susceptible genes, including PNPLA3, GCKR, TM6SF2 and MBOAT7, were genotyped. We analyzed the effects of these risk factors on the incidence and remission of NAFLD and elevated ALT., Results: At baseline, 86 of 440 (19.5%) subjects had ultrasonography-diagnosed NAFLD. At Year 1, of 264 subjects without NAFLD at baseline, 20 (7.6%) developed NAFLD. The baseline BMI z-score and increment in BMI z-score independently predicted incident NAFLD. Of the 68 subjects with NAFLD at baseline, 36 (52.9%) had NAFLD remission. Decrement in BMI z-score independently predicted NAFLD remission. The four studied NAFLD susceptible genes were not significantly associated with either the incidence or remission of NAFLD. In addition, changes in BMI z-score predicted the incidence and remission of elevated ALT from Year 1 to Year 2., Conclusion: Obese children with increasing BMI are more likely to develop NAFLD and those with decreasing BMI are more likely to have NAFLD remission., Competing Interests: Declaration of conflicts of interest The authors have no conflicts of interest relevant to this article., (Copyright © 2021 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background/purpose: Helicobacter pylori infection is one of the most common causes of peptic ulcer disease among children. This study is aimed to investigate the eradication rate of 14-day sequential therapy and the antibiotic resistance of H. pylori in children., Methods: Eighty-seven treatment-naïve children (55 males; median age, 13.5 years) with H. pylori infection from January 2009 to August 2019 were recruited in this study. The status of H. pylori infection was confirmed by culture or histology with the aid of urea rapid test or C-13 urea breathe test. Patients treated with either triple therapy for 7 days or 14 days, or sequential therapy for 14 days was analyzed retrospectively., Results: Thirty-eight (43.7%) patients received 14-day sequential therapy, 24 (27.6%) patients received 14-day triple therapy and the remaining 25 (28.7%) patients received 7-day triple therapy. The eradication rate of 14-day sequential therapy was significantly superior to 7-day triple therapy (97.4% vs. 80%, p = 0.032), and tended to be better than 14-day triple therapy (83%, p = 0.07). Of the 54 patients with available antibiotic resistance data, the resistant rate of clarithromycin, metronidazole, levofloxacin, and amoxicillin were 22.2%, 16.7%, 9.1% and 2.2%, respectively. Clarithromycin resistance demonstrated an inverse association with eradication success (Odds ratio = 0.017, p < 0.001)., Conclusion: In treatment-naïve children with H. pylori infection, 14-day sequential therapy is superior to triple therapy, and achieve a high eradication rate (above 90%) in an area of high clarithromycin resistance., Competing Interests: Declaration of competing interest The authors have no conflicts of interest relevant to this article., (Copyright © 2021 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background/purpose: Anastomotic stricture (AS) is a major morbidity of patients with esophageal atresia (EA) after surgical reconstruction. Our study determined the risk factors of AS after EA reconstruction. The therapeutic efficacy and complications of esophageal dilatation for children with AS were also evaluated., Methods: Forty children treated for EA between January 2008 and December 2018 were included in this retrospective analysis. Esophageal dilatation was performed when AS was diagnosed. The therapeutic effect of esophageal dilatation was determined based on nutritional status, as assessed by the weight-for-age z-score., Results: Sixteen EA patients developed AS. A gap >1.5 cm between the esophageal pouches (P = 0.02) in patients with EA and type A EA was a risk factor for developing AS. A mean of 7.7 sessions of esophageal dilatation were performed per patient, and no complications occurred. The nutritional status of EA children with AS after dilatation was not inferior to that of the children without AS at the 6-month follow-up., Conclusion: A gap >1.5 cm between the esophageal pouches and type A EA are risk factors for AS after esophageal reconstruction. Esophageal dilatation is both safe and effective for managing strictures and improves nutritional status in EA children with AS., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest relevant to this article., (Copyright © 2020. Published by Elsevier B.V.)

Background/purpose: To investigate the role of microRNA (miRNA) dysregulation in liver cancer by assessing the miRNA profiles of human hepatic stem cells (HpSCs), marker-carrying human hepatoblastoma (HB) cells, and hepatocellular carcinoma (HCC) cells vs. those of fetal hepatocytes., Methods: We subjected human HCC and HB tumor specimens to immunohistochemical (IHC) staining for markers of HpSCs. We analyzed the miRNA patterns of HpSCs, HCC cells, HB cells, and fetal hepatocytes using microarray analysis, with confirmation via quantitative real-time polymerase chain reaction. The roles of the miRNAs in liver cancer stem cells (CSCs) were also elucidated., Results: The epithelial cell adhesion molecule (EpCAM) was the most prevalent HpSCs marker in human HB and HCC tumor cells and hepatoma cells. EpCAM-positive HB and HCC cells exhibited greater self-renewal and tumorigenicity than their EpCAM-negative counterparts or EpCAM-positive fetal hepatocytes. In EpCAM-positive fetal hepatocytes, miR-126 expression level increased with gestational age. The EpCAM-positive HB cells exhibited downregulation of miR-126 in comparison to EpCAM-positive fetal hepatocytes. An miR-126 mimic reduced sphere and colony formation in, and induced apoptosis of, HB cells. In comparison to EpCAM-positive fetal hepatocytes, EpCAM-positive HCC cells exhibited downregulation of miR-126, miR-144, and miR-451. Transfection of miR-126, miR-144, and miR-451 induced apoptosis of, and reduced sphere and colony formation in, HCC cells., Conclusion: Dysregulation of liver developmental miRNAs, which exert a tumor suppressant effect, in EpCAM-positive HpSCs may contribute to liver carcinogenesis by promoting the transformation of HpSCs to CSCs of HB and HCC., Competing Interests: Declaration of Competing Interest The authors have no potential, perceived, or real conflict of interest. We declare that we have no conflict of interest. The sponsor had no role in the study design, data collection, analysis, and interpretation., (Copyright © 2019 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background: Post-transplantation lymphoproliferative disorder (PTLD) is a heterogeneous, potentially life-threatening complication after liver transplantation in children. In this study, the disease characteristics, outcomes, and prognostic factors of PTLD were investigated., Methods: A retrospective, observational study was conducted on 16 pediatric liver transplant (LT) recipients who developed PTLD between February 2001 and December 2013 at a tertiary referral center in Taiwan. The disease characteristics and treatment outcomes of the patients were analyzed., Results: The median time from liver transplantation to the development of PTLD was 8 months. Early onset (<1 year post-transplantation) PTLD constituted 62.5% of the cases. PTLD location was frequently extranodal (81.3%) and mostly involved the gastrointestinal tract (68.8%). Histopathologic findings showed polymorphic PTLD in six cases (37.5%), diffuse large B-cell lymphoma in five cases (31.3%), and Burkitt's PTLD in two cases (12.5%). Early lesions in PTLD, T-cell lymphoma, and Hodgkin's lymphoma were observed in one case each (6.3%). Overall survival at 1- and 5-years post-PTLD diagnosis was 87.5% and 79.5%, respectively. St Jude's classification stage IV disease was associated with poor survival (hazard ratio [HR] = 13.37, P = 0.037). Two patients (12.5%) had chronic rejection after the treatment for PTLD and one patient (6.3%) developed graft failure., Conclusion: PTLD is a major complication in pediatric LT recipients, but long-term survival is possible in most cases with an adequate treatment strategy. Stage IV disease is a major risk factor for poor survival in pediatric PTLD patients. During the management of PTLD, the possibility of chronic rejection and even graft failure should be considered., (Copyright © 2018 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background/purpose: The study aim to investigate the correlation between diffusion-weighted magnetic resonance imaging (DW-MRI) and transient elastography (TE) liver fibrosis findings in children with cholestatic liver diseases, and the utility of TE findings to predict cholestatic complications in children., Methods: This cross-sectional study enrolled 36 cholestatic children (21 boys and 15 girls). All study subjects underwent TE and DW-MRI studies to assess liver stiffness. All study subjects were followed prospectively, and their cholestatic complications were analyzed. The optimum cut-off TE value for the prediction of cholestatic complications was determined by receiver operating characteristic (ROC) analysis., Results: A significant negative correlation between liver stiffness measurements (LSMs) and right-liver-to-psoas apparent diffusion coefficient ratios (LTPARs) was found in the study cohort (correlation coefficient = -0.52, p = 0.001). An LSM cut-off > 8.6 kPa was optimal for predicting complications of cholestasis in 6 months of this cohort (p < 0.001). Survival analysis revealed that an LSM of >8.6 kPa was significantly predictive of cholestatic complications in 6 months (hazard ratio = 4.89; 95% CI = 1.41-16.97; p = 0.01)., Conclusion: TE and DW-MRI findings showed a similar ability to predict liver fibrosis in cholestatic children. The LSMs measured by TE are predictive of the occurrence of cholestatic complications in 6 months in children with cholestatic liver diseases., (Copyright © 2018 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background/purpose: This study is aimed to investigate the risk factors and clinical characteristics of posttransplant lymphoproliferative disorder (PTLD) after conducting Epstein-Barr virus (EBV) viral load monitoring in pediatric liver transplant (LT) patients in Taiwan, where EBV infection is endemic., Methods: From 2007 to 2013, pediatric LT recipients who underwent EBV viral load monitoring within 3 months after LT were recruited in this study. The impact of clinical parameters-including age at LT, sex, peak EBV viral load and immunosuppressant levels after LT-on the risk of PTLD were assessed., Results: A total 39 patients underwent LT at a median age of 1.3 years (range: 0.6-14.0 years), and 5 patients developed PTLD during follow-up. Cox's proportional-hazards model identified two predictors of PTLD: peak EBV viral load within 3 months of LT >4100 copies/μg peripheral blood mononuclear cells (PBMC) DNA and peak tacrolimus level within 3 months of LT >14.8 ng/mL (Hazard ratio = 17.14 and 11.54, P = 0.02 and 0.03, respectively). Kaplan-Meier survival analysis revealed significant higher cumulative incidence rates of PTLD (27.3% and 41.8% at 0.3 and 1.2 years after LT) in subjects with peak EBV viral load >4100 copies/μg PBMC DNA within 3 months after LT. (P = 0.001, log-rank test)., Conclusion: Close monitoring of EBV viral load within 3 months after LT is helpful to predict a high risk of PTLD. Tapering of immunosuppressants is suggested if the EBV viral load is >4100 copies/μg PBMC DNA in LT children., (Copyright © 2018 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background/purpose: Biliary complications remain a major morbidity after liver transplantation in children. Inadequate arterial supply to the bile duct after transplantation plays an important role in developing anastomotic biliary complications. We aimed to elucidate the relationship between the resistance index (RI) of hepatic artery and the anastomotic biliary complications after liver transplantation in children., Methods: This is a retrospective, case-control design study. We enrolled 11 pediatric patients under 18 years of age with anastomotic biliary complication after liver transplantation and another 26 matched pediatric transplanted patients without biliary complication as the control group. All patients received liver Doppler ultrasonography within 15 months after liver transplantation. We focused on the ultrasound parameters including waveforms, RI and acceleration time (AT) of hepatic artery., Results: We used the receiver operating characteristic curve to analyze the RI in these 37 patients and yielded a cutoff of RI ≦ 0.57 (63.6% sensitivity and 92.3% specificity) for the best prediction of anastomotic biliary complications. Patients with RI ≦ 0.57 have a higher chance of having biliary complications, with an Odds ratio of 21 (95% CI = 3.16-139.66). The Cox's proportional hazard analysis also confirmed the significance of RI ≦ 0.57 in predicting biliary complications (Hazard ratio = 8.11, 95% CI = 2.35-28.01, P = 0.001) after liver transplantation in children. Three (27.3%) patients with biliary complications received hepatojejunostomy reconstruction, and another 8 patients (72.7%) were successfully managed by percutaneous biliary intervention alone., Conclusion: The RI of hepatic artery ≦0.57 may serve as an important non-invasive predictor for anastomotic biliary complications after liver transplantation in children., (Copyright © 2018. Published by Elsevier B.V.)

Background/purpose: This study aimed to investigate the epidemiology, the preference of medication, and the potential outcome of Wilson disease in Taiwan. We aimed to provide better therapeutic options for patients with Wilson disease based on the data generated from this study., Methods: We utilized the National Health Insurance Research Database (NHIRD), which stores clinical records of nearly 99% of Taiwan's residents. The database used is a random sample of two-million out of 23-million beneficiaries in Taiwan's NHIRD in 2005. The integrated medical records of these two-million cases were collected from 2000 to 2011. Subjects of Wilson disease were identified as those with International Classification of Diseases, Ninth Revision (ICD-9) code 275.1 and the specific prescription drugs (including d-penicillamine, zinc, and trientine) in either outpatient clinic or inpatient records., Results: During the study period, 66 cases of Wilson disease were identified. The male to female ratio was 1.75. The average prevalence rate was 1.81 per 100,000 and the average annual diagnosis rate was 0.22 per 100,000. The diagnosis was mostly established at 20-24 and 10-14 years of age, followed by 25-29 years. Fifty four of all subjects (81.8%) started the treatment with d-penicillamine, compared with zinc (12.1%) and trientine (6.1%). Among these 66 cases, 27 (40.9%) had liver cirrhosis and three (4.5%) underwent liver transplantation due to liver failure., Conclusion: d-penicillamine is still the most popular prescription of Wilson disease, followed by zinc monotherapy. Although chronic liver injury cannot be avoided, a favorable potential outcome is well demonstrated in this population-based study., (Copyright © 2017. Published by Elsevier B.V.)

Primary intestinal lymphangiectasia is a rare disease of children, which is characterized by chronic diarrhea and complicated with malnutrition, including fat-soluble vitamin deficiency. We report a girl aged 4 years and 8 months who was diagnosed with the disease by endoscopic duodenal biopsy at 8 months of age. She presented initially with chronic diarrhea at 4 months of age. Generalized edema with hypoalbuminemia frequently occurred despite regular albumin supplements. Multiple vitamins initially were not supplied regularly. Episodes of tetany caused by hypocalcemia developed 4 years after the diagnosis of intestinal lymphangiectasia. Imaging study (long-bone X-ray and dual-energy X-ray absorptiometry) revealed low bone density. Complicated vitamin D deficiency [low serum 25-hydroxy vitamin D concentration (< 12.48 mmol/L, the detection limit)] and secondary hyperparathyroidism were confirmed via blood testing. Vitamin D supplementation for 3 months improved her bone density, secondary hyperparathyroidism and frequent tetany. Vitamin D status should be monitored in patients with intestinal lymphangiectasia.

Background/purpose: Rotavirus infection is the most common etiology of acute gastroenteritis in young children worldwide. The first rotavirus vaccine was licensed by the United States Food and Drug Administration in 1998 but was suspended soon after in 1999 because of the possibility of induced intussusception. This study evaluated the safety and immunogenicity of a newly developed pentavalent rotavirus vaccine in Taiwanese children., Methods: This was a phase III global trial designed to evaluate the safety and immunogenicity of an oral, live pentavalent rotavirus vaccine (RotaTeq). Taiwan was the only site in Asia enrolled in this trial. Normal healthy infants aged 6-12 weeks were enrolled, and each of the subjects received either three oral doses of the vaccine or placebo solution. The safety of the vaccine, particularly the risk of intussusception, and immunogenicity were studied., Results: A total of 189 infants were enrolled. No increased risk of intussusception or other adverse reactions were noted following the vaccination. RotaTeq was immunogenic among subjects enrolled in Taiwan. At least a three-fold rise in serum antirotavirus IgA antibody was found among 93% of the vaccine group. The immunogenicity of RotaTeq in Taiwan was comparable to that in other areas., Conclusion: The pentavalent human-bovine vaccine, RotaTeq, was safe, generally well-tolerated, and immunogenic among Taiwanese infants.

Background/purpose: To explore the etiology, age and gender distribution, complications, and prognosis of recurrent pediatric pancreatitis., Methods: Between 1993 and 2005, 92 children were hospitalized at the National Taiwan University Hospital with pancreatitis. Only 25 diagnosed with recurrent pancreatitis, based on two or more episodes of pancreatitis, elevated serum amylase and/or lipase levels > or = 3 times the upper limit of normal, radiographic evidence, and clinical symptoms, were enrolled., Results: A total of 85 episodes of pancreatitis in 25 patients (16 girls, 9 boys; mean age, 9.5 +/- 4.4 years; 3.4 +/- 1.9 episodes per person) were documented. The recurrence rate of pediatric pancreatitis was 27.2%. Recurrent pancreatitis was associated with pancreaticobiliary structural anomalies (n = 7), biliary stones or sludge (n = 4), hyperlipidemia (n = 3), pseudopapillary tumor of the pancreas (n = 2), trauma (n = 2), hypoxic encephalopathy with recurrent bacteremia and sepsis (n = 1), and idiopathic (n = 6). The age and gender distribution according to etiologies were not different (p = 0.301 for age, p = 0.137 for gender). Complications included cholangitis or cholestasis (16%), pancreatic necrosis (16%), pseudocyst formation (12%), shock (8%), hemorrhagic pancreatitis (4%), and diabetes mellitus (4%). No patient died of recurrent pancreatitis. Long-term morbidity after recurrent pancreatitis presented as gout, diabetes mellitus, non-alcoholic steatohepatitis, and chronic pancreatitis., Conclusion: For children who suffer from recurrent pancreatitis, pancreaticobiliary structural anomalies should be considered first.

Two major forms of biliary atresia, the embryonic and perinatal type, are considered to have different pathogeneses and distinct prognoses. Embryonic biliary atresia is associated with worse prognosis. We report a case of embryonic biliary atresia in a preterm male infant of 31 weeks of gestation and weighing 1375 g, with the initial manifestation of intermittent acholic stool 5 days after birth. Kasai portoenterostomy was per-formed at the age of 51 days when he weighed 2164 g. Nevertheless, poorly restored bile flow and progressive cholestasis led to early liver transplantation at the age of 8 months. Liver function had recovered to normal levels by the age of 12 months. Diagnosis of biliary atresia in preterm infants is difficult and requires a high index of suspicion and careful workup. This case illustrates the poor outcome of embryonic biliary atresia and that early liver transplantation may be necessary to improve the prognosis.

Background/purpose: Viral infections are the most common causes of acute infectious diarrhea in the pediatric population. To explore any possible microbial etiologies of acute gastroenteritis in children, we detected stool viral antigen including rotavirus, adenovirus, norovirus and astrovirus. We also studied the possible precipitating factors., Methods: During a period of 1 year (from October 2003 to September 2004), children from birth to 15 years old admitted to the pediatric ward were prospectively surveyed. Stool specimens were collected within 48 hours after admission and then frozen at -40 degrees C until analysis. Enzyme immunoassay was used to detect rotavirus, astrovirus, norovirus and adenovirus. Bacterial culture was performed at the same time., Results: During the study period, 82 stool samples were collected due to acute gastroenteritis and fit the definition of a diarrhea episode. Forty-two (51.2%) patients with viral infection, 11 (13.4%) with bacterial infection, and six (7.3%) with mixed viral and bacterial infection were detected. The most prevalent virus was rotavirus (35.4%), followed by norovirus (29.3%). The most prevalent cause of bacterial infection was Salmonella (19.5%). With regard to clinical severity, rotavirus resulted in longer hospital stay, higher rate of vomiting, stool occult blood, leukocytosis, lower rate in stool pus cell, and C-reactive protein elevation more than 5 mg/dL as compared with norovirus. Only the difference in hospital stay reached significant statistical difference., Conclusion: Norovirus is an important cause of acute gastroenteritis in children, although rotavirus is still the leading cause of pediatric acute gastroenteritis.

Background/purpose: Home parenteral nutrition (HPN) is being increasingly used to treat children with intestinal failure. This study evaluated the long-term growth, outcome and complications in Taiwanese pediatric patients with intestinal failure who were treated with HPN., Methods: This retrospective study included 27 consecutive pediatric patients with intestinal failure who received long-term HPN between 1987 and 2002. These patients were categorized into two groups according to whether they had short bowel syndrome or a bowel motility disorder. Growth, prognosis and complications, including cholestasis, hypoglycemia, hyperglycemia and infections were compared between the two groups., Results: The median age of starting HPN was significantly younger in patients with short bowel syndrome (5 months) than in patients with motility disorders (1.9 years). The median duration of HPN treatment in the overall group was 13.5 months (range, 2.1-113.1 months); weight and height increased 1.7 +/- 2.3 and 1.0 +/- 1.6 in z score, respectively. The most common complications were cholestatic liver disease (52%), hypoglycemia (15%) and hyperglycemia (33%). All patients maintained stable serum glucose levels at follow-up. Cholestatic liver disease developed after 2.3 +/- 2.0 months of total HPN in 13 patients, which subsided after 9.7 +/- 6.9 months in 11 patients, while two patients died. The mean incidence of central venous infection was 3.0 +/- 3.3 per 1000 HPN days. The most common pathogens were Staphylococcus spp. (50%) and Candida spp. (30.6%)., Conclusion: HPN treatment can successfully provide a bridge to enteral nutrition in pediatric patients with intestinal failure. The metabolic disturbances and cholestasis are usually transient, but infection control is important throughout the period of HPN treatment.

Background and Purpose: Liver transplantation could be a useful treatment for selected inborn errors of metabolism. This study evaluated the outcome and viral infections after liver transplantation in young children and infants with these diseases., Methods: The outcome of liver transplantation and clinical characteristics of the following 4 patients were assessed: 1 infant with ornithine transcarbamylase deficiency (OTCD) who received liver transplant aged 3 years and 4 months; 1 infant with carbamyl phosphate synthetase I deficiency (CPSID) who received liver transplant at 14 months of age; and 2 infants with methylmalonic acidemia (MMA) who received liver transplant at 8 months and 11 months of age, respectively. All donors, except the 8-month-old infant with MMA, showed serologic evidence of previous cytomegalovirus (CMV) infection before transplantation. All 4 of these donors showed serologic evidence of previous infection of Epstein-Barr virus (EBV). None of the recipients had previous CMV infection. Both the infant with OTCD and the 8-month-old infant with MMA had previous EBV infection, while the other 2 patients did not. Preoperative hemodialysis was performed in both infants with MMA. Postoperative follow-up included metabolic stability, disability degree, and viral infections., Results: None of the patients developed severe metabolic decompensation after transplantation and all increased protein intake postoperatively. Symptomatic viral infections, however, were present in all patients postoperatively, including CMV infection in the infant with OTCD and the 11-month-old infant with MMA, reactivation of EBV infection in the infant with OTCD and the 8-month-old infant with MMA, and primary EBV infection in the infant with CPSID., Conclusions: Liver transplantation was an effective treatment for all 4 of these patients with inborn errors of metabolism. The risk of symptomatic viral infections for these patients was high. This was likely associated with conditions including immunosuppression, young age, endemic nature of CMV and EBV infections, and lack of CMV prophylaxis.

Background and Purpose: This study investigated the characteristics of intussusception in Taiwanese children of different age groups, including the incidence, length of hospitalization and hospital costs., Methods: Children with a diagnosis of intussusception who were hospitalized from 1999 through 2001 were identified from a nationwide health insurance claims database. The incidence of intussusception was calculated by age, gender, and season. Length of hospitalization and hospital costs were also analyzed., Results: A total of 6988 cases of intussusception were identified in Taiwan from 1999 to 2001. Among them, 4859 cases occurred in children below 15 years of age. The average incidence among children below age 15 years was 34.5 per 100,000, with a peak incidence of 118.8 per 100,000 observed among children younger than 24 months old. The highest incidence of intussusception in Taiwanese children occurred between 12 and 24 months of age. According to the data for patients below 15 years of age hospitalized for intussusception in year 2000, males were more likely to be affected than females (61.3% vs 38.7%). Intussusception-related hospitalizations were rare in infants in the first few months of life, increased in those 6 to 12 months old, and peaked among children 1 to 3 years old. Among the 952 patients with intussusception admitted to hospitals in 2000, 297 (31.2%) received surgery, incurring higher median medical costs (New Taiwan Dollars [NT dollars] 42,265 or US dollars 1234) and longer median hospital stay (6.2 days) than the 655 patients who did not require surgery (NT dollars 6290 or US dollars 185 for hospitalization of 2.4 days)., Conclusions: The study found that the incidence of intussusception peaked in the second year of life in Taiwanese children. There was also a male predominance and lack of seasonal variation in incidence.

Background and Purpose: Although chronic inflammatory bowel disease (IBD) is rare in Asian children, increasing numbers of pediatric patients with chronic IBD worldwide have been noted in recent years. This study was conducted to delineate the trend in incidence and clinical patterns of childhood IBD in Taiwan., Methods: All children admitted to National Taiwan University Hospital (NTUH) between 1979 and 2000 who met the criteria for IBD, Crohn's disease (CD), ulcerative colitis (UC), probable CD (PCD), or indeterminate colitis were included. The clinical features and outcomes were analyzed retrospectively. Incidence was calculated using cases of chronic diarrhea during the same period of time as the risk population., Results: IBD was diagnosed in 17 children (9 females and 8 males, aged 2 months to 18 years) during the study period. Six (35%) of these children had UC, 9 (53%) had CD, and 2 (12%) had PCD. The cumulative incidence of CD during 1979-1995 was 0.85%, and increased to 2.6% during 1996-2000 (p < 0.001), while the incidence of UC did not change significantly between these periods (from 0.85% to 0.99%, p = 0.16). The median interval from onset to diagnosis was 7.7 months. Eighty percent of patients had moderate to severe disease activity at diagnosis. The follow-up duration ranged from less than 1 year to 20 years, with a mean of 4.3 years. Two patients were lost to follow-up. Eighty six percent of patients responded to treatment, and 80% of patients had inactive to mild disease activity when re-evaluated at the end of 2000., Conclusion: There has been a marked recent increase in the incidence of childhood CD in Taiwan but the rate of childhood UC has remained unchanged. Eighty percent of cases of childhood IBD responded well to treatment.

Background and Purpose: Mortality of fulminant hepatic failure (FHF) is high in children. It is crucial to identify patients with poor prognosis for timely referral to liver transplantation. This study aimed to assess the clinical and laboratory characteristics of children with FHF and evaluate their correlation with outcome., Methods: Retrospective review was conducted of a total of 31 pediatric patients aged 1 month to 14 years with a diagnosis of FHF from January 1984 to December 2001. Twenty seven clinical and laboratory parameters were analyzed using univariate and multivariate analysis., Results: The etiology of FHF was HBV infection in 10 patients, Wilson's disease in 3, enterovirus infection in 1, herpes simplex virus infection in 1, herpes simplex virus 6 infection with hemophagocytic syndrome in 1, leptospirosis infection in 1, and cryptogenic in 14. Overall mortality was 71% (22/31). The patients who eventually died tended to be older in age, were more likely to have fever during the disease course, had higher peak bilirubin, indirect bilirubin, and ammonia levels, and had more advanced stages of encephalopathy. Multivariate analysis revealed that peak ammonia level > or = 200 microM/L was the only independent factor significantly associated with mortality. The mortality rate in patients with ammonia levels > or = 200 microM/L was 93% compared to 43% in patients with ammonia levels < 200 microM/L (p = 0.013)., Conclusion: Childhood FHF had a high mortality rate. Ammonia level > or = 200 microM/L was associated with a higher mortality rate. Liver transplantation is recommended for these patients.

Congenital generalized lipodystrophy (CGL, Berardinelli-Seip syndrome) is a rare autosomal recessive disorder with a clinical presentation of paucity of adipose tissue, muscular hypertrophy, organomegaly, and insulin-resistant diabetes. A 4-month-old Taiwanese female infant had hepatosplenomegaly and low body weight gain despite a voracious appetite. Hypermetabolism, hyperhidrosis, loss of subcutaneous fat, muscular hypertrophy, acanthosis nigricans, hypertrichosis, and marked hypertriglyceridemia were also noted. Liver histology revealed fatty change and portal-to-portal bridging fibrosis. Clinical features, serum biochemistry, and liver histology were compatible with the diagnosis of CGL. She was given a special diet characterized by calorie restriction and partial substitution of long-chain triglycerides with medium-chain triglycerides. The serum triglyceride concentration subsequently decreased. This present case suggests that extensive fatty infiltration and subsequent cirrhosis of the liver may be the earliest complication of CGL.

Background and Purpose: Hepatitis A is a disease that is heavily affected by sanitation status. Hepatitis A is much less prevalent compared with decades ago in Taiwan, as in many rapidly developing regions. Hepatitis A vaccine is still self-paid under the National Health Insurance program and is still not widely utilized by the general public in Taiwan. This seroepidemiologic study evaluated the prevalence of antihepatitis A virus (anti-HAV) seropositivity in Taipei in 1999., Methods: A total of 1017 serum samples from healthy inhabitants in Taipei were examined for anti-HAV antibody by qualitative enzyme immunoassay., Results: The overall seroprevalence rate was 25.2% (255/1013) in the nonvaccinated population. The seropositivity rate for anti-HAV antibody among children younger than 12 months old was 23.3%. The rates dropped to between 1% and 4.8% among subjects between 1 and 20 years of age. A markedly higher rate of 40% was observed in subjects aged between 20 and 30 years. The seropositivity rate in subjects aged 31 to 50 was 80%. More than 90% of subjects older than 50 years were seropositive. The vaccination rate was low (0.5%)., Conclusion: Our findings indicate that Taipei is an area of intermediate endemicity for hepatitis A virus. To achieve better herd immunity, a more active approach to the adoption of hepatitis A vaccine is warranted.

A large pelvic accessory spleen in a teenage female is rare and may be misdiagnosed as an adnexal tumor. A 17-year-old girl, gravida 0, had suffered from intermittent and dull pain in the lower abdomen for 1 month. Laboratory evaluation showed a low platelet count (136,000/microL). Abdominal sonography showed a large solid mass situated at the right pelvis, containing abundant vascular supply with low resistant blood flow. At laparotomy, an accessory spleen fed by a tortuous vascular pedicle from the omentum was found and then excised. Histopathologic examination revealed congestive accessory splenomegaly. Follow-up platelet count returned to normal levels (374,000/microL). The importance of including accessory spleen in the differential diagnosis of a solid adnexal tumor with thrombocytopenia is emphasized.

Volvulus of the sigmoid colon is rare in children. An early, accurate diagnosis can avoid unnecessary surgery and reduce the risk of complications. This condition is mainly due to a redundant sigmoid colon with a narrow mesosigmoid attachment. We describe two cases of sigmoid volvulus, which showed different clinical severities and were treated with different methods. Patient 1, a 9-year-old boy, presented with acute abdominal pain and vomiting. Patient 2, an 11-year-old boy, presented with abdominal pain, abdominal distention, and bloody mucoid stool. Plain abdominal radiographs revealed a distended colonic loop extending upward from the pelvis in patient 1 and a typical "coffee bean" sign in patient 2. Barium enema examination was used to confirm the diagnosis in both cases. The volvulus was reduced by insertion of a rectal tube in patient 1 and surgically in patient 2. Sigmoid colon volvulus should be included in the differential diagnosis of childhood abdominal pain or distention. This report suggests that nonsurgical reduction should be attempted first for uncompromised sigmoid volvulus in children, unless bowel ischemia or perforation develops.

Background: Several metabolic disorders such as encephalopathy and hepatic dysfunction have been described as Reye's-like syndrome because they present with similar clinical manifestations that mimic Reye's syndrome. We performed a retrospective study to explore the underlying metabolic etiologies of Reye's-like syndrome in patients treated at National Taiwan University Hospital., Methods: From January 1991 to June 1998, 19 children with a syndrome fitting the Reye's-like syndrome description were identified for study. Urine organic acid analysis, plasma amino acid analysis, liver pathology, and skin fibroblast enzyme assays were studied during the acute stage of illness., Results: The etiologies of patients' syndromes included urea cycle disorders (n = 7), glycogen storage disease type Ia (4), primary carnitine deficiency (2), hereditary fructose intolerance (1), methylmalonic acidemia (2), and 3-hydroxy-3-methylglutaric acidemia (1). Fatty acid oxidation defects were suspected in the remaining two cases., Conclusions: A significant number of patients who present with Reye's-like syndrome have an underlying inherited metabolic disorder. In patients with Reye's-like syndrome, an accurate diagnosis is essential to ensure normal growth and development and to prevent recurrence of the condition.

Protamine, used clinically as an antidote for heparin, is a small protein with high arginine content and is potent in folding DNA. Protamine and DNA can form a compact structure, protecting DNA from digestion by intracellular enzymes. Protamine may, therefore, enhance the efficiency of gene transfer. In this study, we tested the ability of protamine to improve liposome-mediated gene transfer efficiency in a human hepatoma cell line. The results of a preliminary gel retardation assay indicated that 10 micrograms was the minimal amount of protamine sulfate needed to completely bind 5 micrograms of a plasmid containing a reporter gene, green fluorescent protein (GFP). For transfection assays, protamine (0, 10, 50, 100, and 500 micrograms) was added to a DNA-liposome mixture (5 micrograms DNA and 20 micrograms of a mixed formulation of 2,3-dioleyloxy-N-[2(sperminecarboxamido)ethyl]-N, N-dimethyl-1-propanaminium trifluoroacetate and dioleoylphosphatidylethanolamine) to transfect cultured Huh7 cells. Transfected cells (those expressing GFP) were counted by using flow cytometry. The expression index (EI) was calculated as the transfection efficiency (% of transfected cells) with protamine divided by the transfection efficiency with DNA and liposome only. Our results show that protamine sulfate (in a range of 10-100, 10 micrograms being most efficient) addition to the liposome-DNA mixture significantly increases the EI, and transfection efficiency of GFP in Huh7 cells.

Primary sclerosing cholangitis (PSC) is a rare disease in Taiwan and has not been described in Taiwanese children previously. We report a 4-year-old girl who presented with prolonged fever, eosinophilia (11%), hepatomegaly, and markedly elevated serum levels of alkaline phosphatase (3,318 IU/L) and gamma-glutamyl transpeptidase (475 IU/L). Subsequent investigations including endoscopic retrograde cholangiopancreatography and liver histology confirmed the diagnosis fo PSC. Treatment with a low dose of prednisolone for 2 months and ursodeoxycholic acid during 32 months of follow-up resulted in clinical remission and halted disease progression. A high index of suspicion is necessary for physicians to diagnose this disorder in children with chronic liver disease. Our experience in this case indicates that therapy with prednisolone and ursodeoxycholic acid may be helpful for the treatment of PSC in children, and suggests the need for more trials of combined therapy.

A prematurely born 5-year-old boy with chronic lung disease, hypoxic-ischemic encephalopathy, cerebral palsy, repeated aspiration pneumonia, and stroke underwent percutaneous endoscopic jejunostomy (PEJ) to alleviate repeated aspiration pneumonia. Studies, including 24-hour esophageal pH monitoring, 99mTc gastric emptying time, upper gastroesophageal barium radiography, and endoscopic examinations showed severe gastroesophageal reflux and prolonged gastric emptying. Percutaneous endoscopic gastrostomy (PEG) was performed first, followed by placement of a polyurethane J-tube (9 French) through the preexisting gastrostomy site. We passed the style-guided J-tube through the pyloric ring endoscopically and advanced it to the jejunum. The position of the J-tube was confirmed by radiologic study. Feeding with an elemental formula, 20 mL/hour, commenced immediately after the procedure, and the rate was gradually increased to 50 mL/hour. No further episodes of aspiration pneumonia have occurred since J-tube placement. Our initial experience with jejunal feeding through a PEJ is encouraging.

Distinguishing extrahepatic biliary atresia from other causes of cholestasis in neonates and infants is important because surgical intervention before 2 months of age allows for long-term survival. The purpose of this prospective study was to evaluate the usefulness of magnetic resonance (MR) cholangiography in differentiating biliary atresia from other causes of cholestatic jaundice in neonates and infants. Nine anicteric infants (control group) aged 10 to 224 days (mean +/- SD, 8 +/- 65 days) and 15 neonates and infants with cholestatic jaundice, aged 22 to 142 days (mean +/- SD, 71 +/- 37) underwent MR cholangiography. The final diagnosis of extrabiliary atresia (6 patients) was based on laparotomy findings (4 patients) or autopsy (2 patients), while neonatal hepatitis (9 patients) was diagnosed according to the liver biopsy findings and clinical recovery during follow-up. Percutaneous liver biopsies were performed in all 15 patients. Results showed that the gall bladder and common bile duct (CBD) could be visualized using MR cholangiography in all patients in the control group. Nonvisualization of the CBD (6/6 patients) and demonstration of a small gall bladder (6/6 patients) characterized MR cholangiography findings in patients with biliary atresia. MR cholangiography failed to depict the CBD in one infant with hepatitis. We conclude that demonstration of the CBD by MR cholangiography in neonates and infants with cholestasis can be used to exclude the diagnosis of biliary atresia. In patients with cholestatic jaundice considered for exploratory laparotomy, preoperative MR cholangiography is recommended to avoid unnecessary surgery.

Although most hepatitis B virus (HBV) related chronic liver diseases manifest themselves in adulthood, HBV infection usually begins in infancy or early childhood in hyperendemic areas. The following factors affect the natural course of HBV: 1) host factors: a) initial age of infection: the younger the patient at infection, the more chance the disease will run a chronic course. Immune tolerance to hepatitis B e antigen (HBeAg) and core antigen persists for a period of time in young children. b) the immune status of the host: the usage and tapering of immunosuppressants may induce a severe course with a fatal outcome; 2) maternal carrier status: children of HBeAg seropositive mothers are more immune tolerant to HBV, and thus have lower rates of HBeAg and hepatitis B surface antigen (HBsAg) seroconversion; and 3) viral factors: mutations of the HBV precore, core or core promoter genes are related to fulminant hepatitis B or severe chronic hepatitis B. However, this is a matter of contention. Since July 1984, a nationwide HBV vaccination program has been successfully conducted in Taiwan. In 1994, the HBsAg carrier rate in Taipei city was reduced to < 1% in children younger than 10 years of age. Continuous efforts in immunoprophylaxis will hopefully further reduce the incidence of hepatitis and hepatocellular carcinoma in the next generation.

A 3-month-old Chinese male infant with typical manifestations of Beckwith-Wiedemann Syndrome (BWS), such as macroglossia, hepatomegaly, umbilical hernia and hypoglycemia, presented with a large hepatic tumor. The tumor measured 7.6 x 8.0 x 7.5 cm. An open biopsy of the tumor revealed hepatoblastoma. The family refused chemotherapy, so only supportive care was given. The tumor grew very rapidly and the infant died 17 days after admission due to respiratory failure. To our knowledge, this is the first report of BWS associated with hepatoblastoma in a Chinese infant. This patient was a typical example of the association of BWS and hepatoblastoma, and the possible effect of growth factors on the rapid proliferation of the neoplasm in BWS.

From 1985 to 1994, home parenteral nutrition (HPN) was used as the method of feeding nine pediatric patients. Indications for HPN included congenital or acquired short bowel syndrome. Crohn's disease, chronic intractable diarrhea, chronic idiopathic intestinal pseudo-obstruction and Hirschsprung's disease. During the period, two patients died; one of sepsis and the other from hepatic failure. Three of the remaining patients have since moved on to oral feeding, but four patients continued on HPN. The majority of these patients have attained a normal weight and height for age while receiving HPN. All patients were fed via an implanted silicone catheter. Catheters were removed and replaced due to complications including nine episodes of infection, four episodes of occlusion, three episodes of breakage and two episodes of dislodgement. Catheter-related sepsis was the most common cause of morbidity and hospital readmission in patients receiving HPN, but was acceptably rare. Staphylococcus aureus was the most frequently cultured organism from either the catheter tip or the blood. HPN is a relatively safe feeding method for patients who would otherwise remain hospitalized for prolonged periods on parenteral nutrition for permanent or prolonged intestinal failure.

After a trial of 1 month of nasogastric tube feeding, three infants (aged 5-14 mo) underwent percutaneous endoscopic gastrostomy (PEG) to enable long-term enteral feeding. The Ponsky-pull technique was employed for PEG insertion. In two patients, the procedure was complicated by superficial wound infection at the cutaneous exists of the PEG tubes: one with Pseudomonas sp and the other staphylococcal colonization. Both responded well to antibiotic treatment. All patients obtained good nutritional support. The preliminary experience reported here suggests that, even in infants, PEG is a safe and easy nonsurgical method of enteral feeding.

Pancreatitis following the administration of L-asparaginase (L-asp) has been well documented. However, the progression of such pancreatitis to pseudocyst formation in some patients has been rarely reported. The few reported cases have been teenagers, with the exception of one adult. All pseudocysts required surgical management. This report documents a pancreatic pseudocyst in a seven-year-old girl with acute lymphoblastic leukemia whose treatment regimen included L-asp. The pseudocyst was managed medically with nasogastric decompression, intravenous hyperalimentation, and antibiotics. The pseudocyst resolved spontaneously in one month without complication.

To determine the false-positivity of antibody to hepatitis C virus (HCV) (anti-C100-3), the correlation between ELISA optical density (OD), anti-HCV titer and recombinant immunoblot assay (RIBA) in 87 anti-HCV-positive sera was analyzed. The results showed that > 90% of the serum samples with an anti-HCV OD > 2.0 or with titers > 10 x were RIBA reactive. These findings are applicable in daily clinical practice.

Congenital short bowel is a rare anomaly. Among the 21 cases reviewed in the literature, only four (19%) survived beyond infancy. We present a female infant with short small bowel, malrotation and floating colon, who was successfully treated with home central parenteral nutrition. Improved long-term survival in this group of patients is expected with prolonged parenteral nutritional support.

Fasting serum gastrin values were measured by radioimmunoassay in 53 children with peptic ulcer disease. The mean fasting serum gastrin levels for children with chronic duodenal ulcer, acute gastric ulcer, acute duodenal ulcer who were younger than two years old and acute duodenal ulcer who were older than two years old were: 46.2 +/- 25.7 pg/mL, 46.9 +/- 43.5 pg/mL, 47.9 +/- 12.0 pg/mL and 37.5 +/- 17.8 pg/mL, respectively. Children with peptic ulcer disease did not have elevated fasting serum gastrin levels when compared with age-matched controls. We conclude that a fasting serum gastrin value cannot be used as a screening test for peptic ulcer disease in children, except for Zollinger-Ellison syndrome.

A male infant, born uneventfully from a consanguinous marriage, presented with intractable watery diarrhea from his third day of life, with subsequent malnutrition and failure to thrive. He received central parenteral nutrition beginning at three months of age after a poor response to a semielemental diet and peripheral parenteral nutrition. He was totally dependent on central parenteral nutrition thereafter. Although diarrhea disappeared with strict bowel rest, intolerance to minimal enteral feedings persisted throughout his 2 years 4 months of life. Investigations including stool examinations and repeated cultures, immune function studies, radiologic studies of the small bowel and screening for galactosemia and cystic fibrosis could not demonstrate a specific cause for the diarrhea. Repeated small intestinal biopsies at 1 month, 4 months and 1 year 5 months of age showed persistent villous atrophy with crypt hypoplasia and a low crypt mitotic index. Electron microscopic examination revealed normal-appearing microvilli. This child may have had a congenital enteropathy due to an inborn crypt regeneration defect causing lifelong intolerance to enteral feedings.

Non-sulfated bile acid levels including cholic acid (CA), chenodeoxycholic acid (CDCA), deoxycholic acid (DCA), lithocholic acid (LCA), ursodeoxycholic acid (UDCA), five taurine conjugates, and five glycine conjugates in duodenal juice were measured in 50 Chinese infants with cholestasis to test their diagnostic value. All 17 with biliary atresia (BA) cases, 11 out of 26 neonatal hepatitis (NH) cases and one case with paucity of the interlobular bile duct were without detectable bile acids. In those NH patients with detectable bile acids, the major components were conjugated forms of CA and CDCA, which was similar to all 6 cases of the comparison group with other diseases. The minor bile acid components identified in them were glycine conjugated UDCA, free CDCA, free CA, and free and conjugated DCA. Only one patient with NH had taurine conjugated LCA. The mean total duodenal bile acid level in 15 patients with NH was significantly lower than that in the 6 patients of the comparison group. Most patients with NH had a CDCA/CA ratio of less than one, indicating that cholic acid is the predominant form in their bile. Glycine conjugated bile acids were the predominant bile acids present in 11 out of 15 patients with NH and 4 out of 6 of the comparison group patients. The results suggest that the detection of duodenal bile acids by a sensitive HPLC method is of limited value in making a differential diagnosis between BA and NH.(ABSTRACT TRUNCATED AT 250 WORDS)

To evaluate the current status of hepatitis A virus (HAV) infection, a seroepidemiologic study to detect the prevalence of antibodies against HAV (anti-HAV) has conducted in Taipei City, from March to July, 1989. Serum specimens were collected from 602 male and 503 female apparently healthy children age 12 and under. The prevalence rate of anti-HAV was 27.5% in infants, and decreased to nearly 0% in preschool and early school children up to 10 years old. Compared to a previous study in the same area of Taipei in 1984, our study indicated a further and significant reduction in the prevalence of anti-HAV in almost every age group from 2 to 14 years of age. There was an average of a 5-year prevalence shift. These data suggest that there has been no significant endemic of HAV infection from 1984 to 1989. The antibody prevalence change in the different age groups represents an ongoing trend of HAV seroepidemiology in Taiwan where HAV infection is becoming rare. The age of primary infection in children has been extended to an older age group. The rarity of HAV infection in Taiwan has created a large and growing population of young adults susceptible to hepatitis A virus infection. The effective prevention of hepatitis A virus infection will become a major public health concern.

Diphenylhydantoin-induced hepatitis and mononucleosis are uncommon in children. The occurrence of these two diseases in the same individual, with progression to hepatic failure is rare and has not been reported in infants. This report represents a 6-month-old male infant who developed an infectious mononucleosis-like syndrome and hepatic failure 16 days after diphenylhydantoin administration. He took this anticonvulsant for controlling seizures after a head injury. Fever, skin rash, hepatosplenomegaly, lymphadenopathy, and atypical lymphocytosis led to the initial diagnosis of infectious mononucleosis. However, negative heterophil antibody did not support the diagnosis. Jaundice ensued in the following course and became more and more profound. Meanwhile, physical examination showed shrinking in liver size. Negative virology studies, including Epstein-Barr virus, cytomegalovirus, and hepatitis B virus, excluded them as causative agents. The patient lapsed into a stage I hepatic coma, but gradually recovered clinically and biochemically after eight successive exchange transfusions and supportive care. Two liver biopsies were performed 20 and 50 days after the onset of disease, respectively. Remarkable hepatic parenchymal loss, cholestasis, and fatty change were found on histologic examination of the first biopsy specimen, and portal fibrosis was noted on the second.

A three-and-a-half-year-old boy was transferred to our hospital under the impression of Reye's syndrome. The laboratory data showed hypoglycemia, hyperammonemia and elevated serum transaminases. A remarkable high serum amylase level of 2,223 IU/L and CAm/CCr of 36% was noted. A blood urea nitrogen level of 143 mg/dl was found on the third day and a creatinine level of 8.7 mg/dl on the fourth day. Disseminated intravascular coagulopathy (DIC) and systemic candidal infection complicated his final course. He died after intensive treatment for eleven days. Hemorrhagic pancreatitis and fatty change of the liver were noted at autopsy. Disseminated candidal invasion was noted within the kidneys, cerebrum, and lungs. Tonsillar herniation, systemic candidiasis and bronchopneumonia were believed to be the causes of his death. It is extremely rare for all three complications, acute pancreatitis, acute renal failure and DIC, to occur in Reye's syndrome at the e time.

With the continuing advancement in the treatment of childhood leukemia and the lengthened survival of these patients, an increased incidence of abdominal complications has been observed. A retrospective analysis of 364 patients with leukemia treated at the National Taiwan University Hospital from January 1977 through April 1988 was undertaken. Eleven patients (3.0%) developed abdominal complications during their course of disease, including acute appendicitis, intussusception, intestinal perforation, ovarian cyst rupture, etc. All of these patients had abdominal complications during the initial presentation or relapse of leukemia, and 9 (82%) of them had just received chemotherapy. Ten patients (91%) had thrombocytopenia and 7 (64%) had leukopenia. Blood cultures were positive in 5 patients (45%), and gram-negative enteric bacilli were isolated in 4 of them. All 5 septicemic patients had leukopenia or neutropenia. The clinical manifestations were nonspecific and were often masked. Most of the complications occurred in the right lower abdominal structures. Of the 7 children treated surgically, 3 had long term survival. Among the 4 patients who did not receive an operation, only 1 survived for more than 4 weeks. The mean length of survival tended to be longer in patients with additional surgical treatment. Prompt diagnosis and early aggressive treatment, under modern supportive facilities, appear to offer a more favorable outcome.