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T helper (Th) cell cytokines modulate inflammation and play a role in biliary atresia (BA). The aim of the study is a cross-sectional assessment of the levels of Th cytokines in the jaundice-free post Kasai procedure patients.There were 40 jaundice-free patients with BA and 28 normal controls enrolled. Patients were divided into 3 groups, including normal liver function, impaired liver function, and portal hypertension. Plasma concentration of Th1 [interferon-γ (INF-γ), interleukin (IL)-2], Th2 (IL-4, IL-10), Th3 [transforming growth factor-β1 (TGF-β1)], Th17 (IL-17) cytokines, and stromal cell-derived factor-1α (SDF-1α) were investigated.The IFN-γ level was significantly higher in the BA patients with impaired liver function and portal hypertension than controls (P0.0001 and P0.0001, respectively). There was a significantly increase of TGF-ß1 in all BA groups compared with controls (P=0.003). The reduction of SDF-1α expression was found in BA groups (P0.0001). IL-10 levels significantly correlated with aspartate aminotransferase to platelet ratio index (r=0.496, P=0.001). For the cytokine correlations, there were no correlations of Th1, Th2 and Th17 cytokine with the other measured cytokines, but TGF-ß1 was negatively correlated with SDF-1α levels (r=-0.327, P=0.039).IFN-γ and IL-10 are likely to be involved in the disease progression in BA. Besides, TGF-β1 is found to be a suppression marker associated with SDF-1α levels and reduced production of TGF-β1 may be associated with the disease progression.

Background: The Identification and Management of Feeding Difficulties (IMFeD™) tool was introduced in 2011 for management of children with feeding difficulties and has been used in various countries around the world. This study aimed to assess its acceptance and effectiveness in clinical practice in Taiwan. Methods: This was a prospective, observational, multicenter study where subjects were followed over 3 months. Outpatients identified as picky eaters, having poor appetite, poor weight gain, or weight loss were enrolled. The acceptance and effectiveness of IMFeD tool were evaluated by caregivers and pediatricians. Results: Among 423 children who completed the study (235 boys, 188 girls; 78.8% aged

Background Cholangitis may affect liver failure of biliary atresia (BA) patients after Kasai portoenterostomy (KP). We examined whether the number of cholangitis episodes could be a prognostic marker for liver transplant (LT) in children with BA after Kasai portoenterostomy (KP). Methods Data for BA patients born after 1998 and undergoing KP were obtained from National Health Insurance Research Database (NHIRD), Taiwan. Patients were followed up until the end of 2011. Incidence and the number of cholangitis episodes were recorded and compared between patients based on LT status. Results Ninety-six (26.8%) of the 366 BA patients underwent LT. More patients who underwent KP at

OBJECTIVES: The pathogenesis of biliary atresia (BA) is unclear, but epidemiological studies may help to elucidate possible causes. The goals of this study were to identify BA incidence changes in Taiwan in 2004–2009 and to survey the factors that might influence incidence changes to elucidate the possible causes of BA. METHODS: A Taiwan national registry system for BA has been established since 2004. By using data from the national registry system for BA, we identified BA incidence changes in 2004–2009. We also evaluated the correlations between BA incidences and estimated rotavirus vaccine coverage rates and between BA incidences and the gross domestic product. RESULTS: A total of 185 patients with BA were identified in 2004–2009 in Taiwan, whereas the number of live births was 1 221 189. Compared with the incidence of BA in 2004–2006 (1.79 cases per 10 000 live births), the incidence of BA in 2007–2009 (1.23 cases per 10 000 live births) was decreased significantly (P = .01). BA incidences were negatively correlated with the gross domestic product (P = .02) and marginally negatively correlated with rotavirus vaccine coverage rates (P = .07). CONCLUSIONS: A significant decrease in BA incidence in Taiwan since 2007 has been noted and may be related to improvements in the general socioeconomic status and the popularity of rotavirus vaccination. Although more evidence is needed to establish a direct correlation, this phenomenon may shed light on possible causes of and preventive interventions for BA.

In Taiwan, a screening system using an infant stool color card to promote the early diagnosis of biliary atresia (BA) was established in 2002. This study aimed to investigate the 5-year outcome of BA before and after using the screening program. BA patients were divided into three cohorts according to their birth dates. The patients in cohort A (n = 89) were born before the stool card screening program (1990-2000); those in cohort B (n = 28) were screened by the stool card regional screening program (2002-2003); and those in cohort C (n = 74) were screened by the stool card universal screening program (2004-2005). The relative odds ratios were computed using logistic regression to compare the different factors affecting survival time. The rate of age at Kasai operation

Background Wilson's disease (WD) is an autosomal recessive defect of cellular copper export. Early diagnosis in children is difficult due to its obscure clinical presentations. The efficacy of zinc salts is well documented, although there are limited data concerning zinc use in pediatric patients with WD. Methods We performed a retrospective analysis of clinical features, laboratory results and treatment responses in children with WD diagnosed at Taichung Veterans General Hospital between 1996 and 2008. Diagnosis was established by low serum ceruloplasmin, high 24-hour urinary copper excretion, presence of Kayser-Fleischer rings, and mutation analysis. Results Eleven children were included in this study. The main initial presentations were impaired liver function tests (6/11) and hemolytic anemia (2/11). Gene studies in seven children showed six different mutations (G934D, R778Q, C490X, 304insC, IVS4-1 G > C, P992I) and one possible novel mutation (L1181P). All patients had improved liver function tests and hemoglobin levels after treatment with D-penicillamine, trientine and zinc supplement therapy. During a mean period of 3.4 ± 2.1 years with zinc therapy, six patients had serum zinc levels above the normal limit, and seven patients had serum copper levels below the normal range. Conclusion Serum ceruloplasmin and 24-hour urinary copper examinations could be used to rule out WD in children with chronic hepatitis and hemolytic anemia. Gene analysis is helpful for prompt diagnosis of asymptomatic siblings and patients with atypical features. Zinc treatment is generally safe in pediatric patients with WD. However, its adverse effects should be monitored.

Biliary atresia is the most common cause of death from liver disease in children. Although the Kasai operation before 60 days of age can significantly improve prognosis, delay in referral and surgery remains a formidable problem worldwide because of difficulties in differentiating it from benign prolonged neonatal jaundice. We established a universal screening system using an infant stool color card to promote the early diagnosis and treatment of biliary atresia. After a pilot regional study in 2002–2003, a national stool color screening system was established by integrating the infant stool color card into the child health booklet given to every neonate in Taiwan since 2004. Within 24 hours of the discovery of an abnormal stool color, this event is reported to the registry center. The annual incidence of biliary atresia per 10,000 live births in 2004 and 2005 was 1.85 (40/216,419) and 1.70 (35/205,854), respectively. The sensitivity of detecting biliary atresia using stool cards before 60 days of age was 72.5% in 2004, which improved to 97.1% in 2005. The national rate of the Kasai operation before 60 days of age increased from 60% in 2004 to 74.3% in 2005. The jaundice-free rate (

Subacute nonsuppurative cholangitis (cholangitis lenta) is an uncommon yet important histological finding in liver biopsies from pediatric liver transplant recipients. The histopathological features include proliferation of bile ductules at the edges of portal tracts, inspissated bile within dilated bile ductules, absence of acute inflammation within ducts or ductules, and normal structure of interlobular bile ducts.To describe the histopathology and clinical outcomes of pediatric liver transplant recipients with subacute nonsuppurative cholangitis.This is a retrospective analysis involving review of medical records and analysis of liver-biopsy specimens by a pathologist blinded to clinical diagnosis. We identified 9 pediatric patients meeting the criteria for nonsuppurative cholangitis. These patients were compared with a control group of patients with biliary obstruction.Liver histopathology clearly distinguishes subacute nonsuppurative cholangitis from biliary obstruction and other causes of posttransplant cholestasis. Clinical biochemistry cannot reliably distinguish between the 2, although statistically significant differences existed in levels of serum total and direct bilirubin, alanine aminotransferase, and alkaline phosphatase. The rate of proven bacterial or fungal infection in the study group was 100% in contrast to a rate of 54.5% in the control group (P.05). Graft and patient survival were similarly poor.: Finding subacute nonsuppurative cholangitis suggests the presence of severe local or systemic infection in liver-transplant recipients, and its recognition is important for clinical management.

Background Prior to the introduction of rotavirus vaccines, rotavirus was the leading cause of severe gastroenteritis in infants and young children, and it continues to be the leading cause in countries without vaccination programs. Rotavirus gastroenteritis results in substantial economic burden and has a pronounced effect on the family of those who are ill. Both in Taiwan and in Vietnam, rotavirus illness is viewed as a priority disease. This study assessed, in Taiwan and Vietnam, the impact of rotavirus gastroenteritis on the family among a group of parents whose children had recently been hospitalized for this illness. Methods In the first half of 2013, parents of children who had been hospitalized due to rotavirus infection were recruited from hospitals in Taiwan (n = 12) and Vietnam (n = 22), and participated in focus group sessions or in-depth ethnographic interviews. Results In both countries, the results point to a substantial burden on the parents concerning emotions and logistics of daily tasks, and to considerable disruptions of the family routine. Taiwanese parents reported satisfaction with the health care system, a great deal of effort to suppress emotions, a fair amount of knowledge about rotavirus, and little extra costs related to the illness. On the other hand, parents in Vietnam expressed concern about the emotional well-being of and the health care treatments for their children, were less knowledgeable regarding rotavirus infection, and experienced a substantial financial burden due to indirect costs that were related to accessing treatment. Conclusions Families in Taiwan and Vietnam suffer from a considerable economic and emotional burden related to rotavirus gastroenteritis. One way to substantially reduce this burden is to provide universal and affordable rotavirus vaccination to susceptible children, especially since cost-effectiveness studies have demonstrated that universal vaccination would be safe and efficacious against severe rotavirus gastroenteritis in these countries.

OBJECTIVE. We aimed to detect biliary atresia (BA) in early infancy to prevent additional liver damage because of the delay of referral and surgical treatment and to investigate the incidence rate of BA in Taiwan. METHODS. A pilot study to screen the stool color in infants for the early diagnosis of BA was undertaken from March 2002 to December 2003. We had designed an “infant stool color card” with 7 numbers of different color pictures and attached it to the child health booklet. Parents were then asked to observe their infant's stool color by using this card. The medical staff would check the number that the parents chose according to their infant's stool color at 1 month of age during the health checkup and then send the card back to the stool color card registry center. RESULTS. The average return rate was ∼65.2% (78184 infants). A total of 29 infants were diagnosed as having BA, and 26 were screened out by stool color card before 60 days of age. The sensitivity, specificity, and positive predictive value were 89.7%, 99.9%, and 28.6%, respectively. Seventeen (58.6%) infants with BA received a Kasai operation within 60-day age period. The estimated incidence of BA in screened newborns was 3.7 of 10000. CONCLUSIONS. The stool color card was a simple, efficient, and applicable mass screening method for early diagnosis and management of BA. The program can also help in estimating the incidence and creating a registry of these patients.

Epidemics of enterovirus 71 infections caused the rapid death of many children in Malaysia in 1997 and in Taiwan in 1998. Pulmonary edema occurred in most of the fatal cases and was considered to be neurogenic. The role of the heart was rarely investigated before. Between January 1998–January 2001, 34 consecutive patients who were admitted to the intensive care unit due to enterovirus infection were studied prospectively. Patients were divided into two groups: group I with pulmonary edema, and group II without pulmonary edema. Comparisons were made between the two groups based upon demographic, neurological, and cardiovascular manifestations. Group I consisted of 11 patients (5 boys, 6 girls; mean age, 22.8 months), and group II of 23 patients (12 boys, 11 girls; mean age, 28.8 months). There were no significant differences between the two groups in comparing sex, age, body weight, neurological severity, intracranial pressure, cell count, protein and glucose levels in cerebral spinal fluid, and blood pressure. All group I patients had left ventricular dysfunction, and their ejection fractions were significantly lower than those of patients in group II (37 ± 11% vs. 75 ± 6%, P

Budd-Chiari syndrome is a rare, heterogenous and potentially lethal condition due to hepatic venous outflow obstruction. Classic triads include hepatomegaly, ascites and abdominal pain. Most cases are caused by abnormal coagulopathy, infection, inflammation or tumor invasion. Status post liver transplantation was rarely mentioned in the pediatric group. Here we report a case of 4-year-old girl with biliary atresia status post living donor liver transplantation. Marked ascites developed 2 months later and Budd-Chiari syndrome was diagnosed. Cardiac catheterization showed significant stenoses of the hepatic vein and inferior vena cava. Initial balloon angioplasty of inferior vena cava only resulted in mild improvement. After successful balloon angioplasty of the hepatic vein stenosis, the stenosis of the inferior vena cava improved significantly. The ascites resolved soon, and no more happened up to 8 months' follow-up.

To investigate the role of hepatitis B virus (HBV) infection in pediatric fulminant hepatic failure (FHF) after the launch of universal HBV vaccination, the authors analyzed the data from patients with FHF collected from a nationwide collaborative study group. Children aged 1 month to 15 years who were diagnosed with FHF (62 males and 33 females) between 1985-1999 were included. HBV infection (hepatitis B surface antigen [HBsAg] and/or immunoglobulin M hepatitis B core antibody [IgM anti-HBc] seropositive) accounted for 46% (43 of 95 cases) of all the cases of FHF. The average annual incidence of FHF in the time period 1985-1999 was 0.053/100,000 in the group of patients ages 1-15 years and 1.29/100,000 in those patients age1 year. Approximately 61% (58 of 95 cases) of all FHF cases were infants. The percentage of HBV infection was found to be higher in infants (57%) compared with children ages 1-15 years (27%) (P = 0.004). The incidence rate ratio of those patients age1 year to those ages 1-15 years was 54.2 for HBV-positive FHF and 15.2 for HBV-negative FHF. Maternal HBsAg was found to be positive in 97% of the infants with HBV-positive FHF, and hepatitis B e antigen (HBeAg) was found to be negative in 84% of these infants. Approximately 74% of all HBV-positive FHF patients and 81% of the infantile HBV-positive patients had been vaccinated. In conclusion, within the first 15 years of universal vaccination, HBV was found to rarely cause FHF in children age1 year but remained a significant cause of FHF in infants. HBV-positive FHF was prone to develop in infants born to HBeAg-negative, HBsAg-carrier mothers; these infants had not received hepatitis B immunoglobulin according to the vaccination program in place.