Growth failure and metabolic bone disease in progressive familial intrahepatic cholestasis.
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Adenosine Triphosphatases↗
Biomedical subjects
Mei-Hwei Chang
Publications and source records attributed to Mei-Hwei Chang.
Stable seroprevalence of Helicobacter pylori infection in children during 1989-1999 in Taipei, Taiwan.
BACKGROUND: Helicobacter pylori (H. pylori) is thought to be acquired in childhood. The present study aimed to examine the changing seroepidemiology of H. pylori in children in the same area of Taipei city during 1989-1999, and to try to elucidate the possible transmission route of H. pylori in children by comparing hepatitis A virus (HAV) seroprevalence in Taipei during the same decade. METHODS: Sera were collected from the apparently healthy children (965 males and 835 females, aged 0-20 years) from the Chung Cheng District of Taipei city. H. pylori infection status was checked by enzyme-linked immunosorbent assay (ELISA). RESULTS: In 1999, the overall prevalence rate of H. pylori infection was 14.8% with no gender difference (P = 0.115). Children aged 1-3 years had the lowest prevalence rate (2.8%). The prevalence rate then increased steadily after 3 years of age. The annual infection rate was 1.25% after 3 years of age and the seroprevalence rate was 21.5% at 15-18 years. In comparison with the previous H. pylori seroepidemiology study performed at the same district of Taipei city one decade ago, there is no significant cohort effect in H. pylori prevalence in Taipei children during the past decade (Odds ratio = 1.13; 95% confidence interval: 0.74-1.72). The epidemiological pattern between H. pylori and HAV was found to be different. CONCLUSION: The seroprevalence of H. pylori in children did not change significantly in the past decade. This epidemiological feature is different from HAV. The authors propose that there are other transmission routes of H. pylori than fecal-oral.
Adolescent↗
Gastroesophageal reflux disease beyond infancy.
BACKGROUND: Children beyond infancy (>12 months of age) rarely have gastroesophageal reflux disease (GERD). Underlying diseases may contribute to the persistence of GERD from infancy to childhood. This study compares the clinical course of children with GERD with and without underlying diseases. METHODS: The authors studied the role of underlying diseases responsible for GERD in children beyond infancy by a retrospective analysis. From 1985 to 2000, GERD was confirmed in 34 children beyond infancy in the National Taiwan University (median age 2.5 years, range 1.1-9.7 years), according to the inclusion criteria of reflux symptoms and the fraction of pH < 4 above 5% in the 24-h esophageal pH study. The patients were divided into two groups: those without underlying diseases (n=10) and those with underlying diseases (n=24). The follow-up duration was 0.5-17.1 years (median 4.5 years). RESULTS: The underlying diseases responsible for GERD in 24 children included neurological impairment (n=14), repaired esophageal atresia (n=2), hiatal hernia (n=3), repaired congenital diaphragmatic hernia (n=2), and congenital heart disease (n=3). At the end of the study, 9 of 10 children with GERD beyond infancy and without underlying diseases were free of symptoms without any need for further medical treatment. In contrast, 10 of 14 children with neurological disorders had persisting reflux symptoms (Kaplan-Meier analysis, P=0.02, log-rank test). CONCLUSIONS: Neurological impairment and esophageal or diaphragmatic anatomic abnormalities were frequently associated with GERD beyond infancy. Children with underlying diseases, especially with neurological impairment, ran a refractory course, while those without underlying diseases enjoyed a longer symptom-free life.
Child↗
Outcome predictors of fulminant hepatic failure in children.
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.
Adolescent↗
Characteristics and trends in incidence of inflammatory bowel disease in Taiwanese children.
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.
Adolescent↗
A trend of improved survival of childhood hepatoblastoma treated with cisplatin and doxorubicin in Taiwanese children.
Hepatoblastoma is the second most common childhood malignant hepatic tumor in Taiwan. Its prognosis used to be poor. We reviewed our cases in this decade to see if there has been any improvement of survival in our patients with hepatoblastoma. From 1988 to 2000, 19 patients with hepatoblastoma in this institution were included in the study. These patients' clinical manifestations, laboratory and image studies, histological findings, treatment modalities and prognostic significance were analyzed. The mean age at diagnosis was 13.5 months, ranging from 0 to 4 years old (male:female =11:8). Abdominal distension was the most common symptom, and hepatomegaly was the most common physical finding. Laboratory abnormalities included elevated alpha-fetoprotein, thrombocytosis and abnormal liver function profiles. Treatment modalities included primary surgery with postoperative chemotherapy in three, chemotherapy only in four and preoperative chemotherapy plus surgery with or without postoperative chemotherapy in nine patients. The overall 2-year survival rate is 38.6%. The significant prognostic factors include patients' compliance, resectability and chemotherapy protocol. The introduction of a new chemotherapy protocol designed by the International Society of Pediatric Oncology Study (SIOPEL) in 1994 improved the 2-year survival rate from 12.5 to 58.4% ( P=0.01). In conclusion, the improved chemotherapy protocol enhances the survival rate of hepatoblastoma in Taiwanese children.
Antineoplastic Combined Chemotherapy Protocols↗
Tuberculous peritonitis mimicking peritonitis carcinomatosis: a case report.
UNLABELLED: A 13-year-old girl presented with non-specific symptoms of peritonitis, low grade fever and missed her normal menses. Computed tomography of her abdomen and pelvic cavity showed omental cake and a generalised thickened peritoneum. An elevated serum cancer antigen-125 (CA-125) level of 1248.5 U/ml was also noted and a diagnosis of peritonitis carcinomatosis was first suspected. Diagnostic laparoscopy revealed multiple tubercles over the whole peritoneum and the pathology report described granulomatous nodules with giant cells and epithelioid cells. Culture of the ascitic fluid revealed Mycobacterium tuberculosiswhich resulted in a final diagnosis of tuberculous peritonitis. The patient's fever and abdominal distension gradually subsided after anti-tuberculosis treatment. The serum CA-125 level also decreased significantly to 10.2 U/ml after treatment. CONCLUSION: cancer antigen-125 levels may serve as a potential follow-up marker of disease activity and treatment response in tuberculous peritonitis.
Adolescent↗
Percutaneous endoscopic gastrostomy to set up a long-term enteral feeding route in children: an encouraging result.
Percutaneous endoscopic gastrostomy (PEG) is now accepted as the preferred technique to establish long-term enteral feeding. A total of 30 pediatric patients (21 males and 9 females, aged from 5 months to 14 years) underwent PEG in the National Taiwan University Hospital from December 1994 to February 2001. The underlying diseases of the patients receiving PEG were neurological dysfunction ( n=26), gastrointestinal disease ( n=2) and metabolic disorders ( n=2). All patients received intravenous sedation and PEG was performed successfully. Prophylactic antibiotic treatment was given for 1 day. Tube feeding began 48 h after the placement. The Z-score of weight before and 6 months after PEG was -1.54+/-1.74 and -0.98+/-1.46, respectively, which is significant (paired t-test, p<0.0001). Accelerated weight gain was found after PEG. Complications of PEG in our patients included local wound infection ( n=15), gastrocolic fistula ( n=1), pneumoperitoneum ( n=1), dislodgement of tube ( n=6) and tube occlusion ( n=2). Of these patients 12 received button replacement after PEG and the gastrostomy tube was discontinued in 2 patients when they resumed adequate oral intake. Removal of PEG was performed in 2 patients due to complications of gastrocolic fistula and severe dislodgement. Our results encourage the use of PEG as a long-term route for nutritional supply.
Adolescent↗
TT virus infection in healthy children, children after blood transfusion, and children with non-A to E hepatitis or other liver diseases in Taiwan.
Serum samples from healthy and diseased children were studied for the presence of TTV DNA by nested PCR using primer sets generated from N-22 region and from the untranslated region (UTR) of the viral genome. N-22 positive TTV DNA was detectable in 33 (27%) of 122 healthy children, 47 (73.4%) of 64 polytransfused thalassemic children, 37 (46.3%) of 80 children who received transfusion during cardiac surgery, 8 (42.1%) of 19 non-A to E hepatitis, 10 (33.3%) of 30 HBV carrier children, and 5 (15.6%) of 32 infants with biliary atresia. A much higher prevalence of TTV DNA with rates varying from 78-100% in the above study groups was observed using the UTR primers. For children with N-22 positive TTV DNA, biochemical assessment of isolated TTV viremia in thalassemic children or children transfused during surgery showed no convincing association between raised ALT levels and TTV viremia. Coinfection with TTV in chronic HCV-infected or HBV-infected children did not result in higher peak ALT levels during follow-up, suggesting that TTV has no synergistic pathogenic effect. The phylogenetic analysis of the N-22 positive TTV DNA isolates revealed that most isolates from healthy children, children transfused during surgery, and non-A to E fulminant hepatitis children were type 1 TTV. These results indicate that TTV infection in children was significantly associated with transfusion. TTV infection is highly prevalent in early childhood in Taiwan but plays a minimal role in the induction of hepatitis in children.
Adolescent↗
The diagnostic value of sensory evoked potentials in pediatric Wilson disease.
We studied the sensory evoked potentials in pediatric Wilson disease to verify their subclinical neurologic involvement and to elucidate the role of cirrhosis in abnormal evoked potentials in non-neurologic Wilson disease. Thirty children (17 male, 13 female), diagnosed with Wilson disease before 18 years, were enrolled. The mean age during studies was 15.8 +/- 6.3 years, and disease duration since diagnosis was 3.0 +/- 3.3 years. In 12 neurologic Wilson disease cases, there were prolonged interpeak latencies of brainstem auditory evoked potentials III-V, I-V, somatosensory evoked potentials N13-N20 (P < 0.01 vs controls and non-neurologic cases), and P100 latency (P < 0.01 vs controls). All 12 patients had at least one abnormal evoked potential, including 91.7% brainstem auditory, 58.3% somatosensory, and 25% visual evoked potentials. In 18 non-neurologic Wilson disease cases, there were still prolonged interpeak latencies for brainstem auditory evoked potentials I-V and somatosensory evoked potentials N13-N20 (P < 0.05 vs controls), with 27.8% of them having at least one abnormal evoked potential, including 16.6% brainstem auditory, 5.6% somatosensory, and 11.1% visual evoked potentials. In those with non-neurologic Wilson disease, there were no significant differences in all the evoked potential parameters between the cirrhotic and non-cirrhotic patients.
Adolescent↗
Decreasing incidence of hepatocellular carcinoma among children following universal hepatitis B immunization.
Hepatocellular carcinoma (HCC) is one of the 10 most common malignant tumors worldwide. Chronic infection with hepatitis B or C virus is closely related to hepatocarcinogenesis. The outcome of current therapies for HCC is not satisfactory. Prevention is the best way to control HCC. Among the various strategies of HCC prevention, immunization against hepatitis B virus infection is the most effective. Universal hepatitis B immunization has proved to be effective in reducing the incidence of HCC to 1/4-1/3 of that in children born before the hepatitis B vaccination era in Taiwan. The problems we face in achieving global control of hepatitis-related HCC include: (1) no effective vaccine for the prevention of hepatitis C and its related HCC; (2) no immunization program for hepatitis B in areas with inadequate resources; (3) poor compliance to the immunization program as a result of ignorance, anxiety, or poverty; and (4) vaccine failure. Integration of the hepatitis B vaccination program into the expanded program of immunization for all infants throughout the world will be most urgent and important for HCC control. The reduction of the incidence of HCC will be seen in adults 30-40 years of age after the launch of the universal hepatitis B vaccination program. This concept of cancer vaccine can be applied to other infectious agents and their related cancers.
Carcinoma, Hepatocellular↗
Prophylactic oral antibiotics in prevention of recurrent cholangitis after the Kasai portoenterostomy.
PURPOSE: The aim of this study was to evaluate the efficacy of trimethoprim-sulfamethoxazole (TMP/SMZ) and neomycin as the prophylactic agents against the recurrence of cholangitis in children with biliary atresia (BA) after a Kasai portoenterostomy. METHODS: Nineteen BA patients aged 0 to 2 years, who had one episode of cholangitis after a Kasai portoenterostomy, were recruited in this study. Patients were assigned randomly into 2 groups: one (9 cases) with TMP/SMZ (TMP 4 mg/kg/d and SMZ 20 mg/kg/d, divided in 2 doses) and the other (10 cases) with neomycin (25 mg/kg/d, qid, 4 days a week). Another 18 BA patients aged 0 to 2 years, with cholangitis but not put on long-term prophylaxis, served as the historical control group. RESULTS: The mean prophylactic periods were 14.6 months and 14.7 months in the TMP/SMZ and neomycin groups. Patients who received prophylaxis with either TMP/SMZ or neomycin had lower recurrence rates of cholangitis than those in the control group (P =.042 and.011). There was no difference in the recurrence rates of cholangitis between the TMP/SMZ and neomycin groups (P =.641). The survival rates were higher in the TMP/SMZ and neomycin groups than in the control group (P =.09 and.018). CONCLUSIONS: Use of TMP/SMZ or neomycin is effective as a prophylactic agent against the recurrence of cholangitis after the Kasai portoenterostomy, but there is no difference in efficacy between these 2 regimens.
Antibiotic Prophylaxis↗
Role of hepatitis B immunoglobulin in infants born to hepatitis B e antigen-negative carrier mothers in Taiwan.
BACKGROUND: The efficacy of hepatitis B immunoglobulin (HBIG) in infants of hepatitis B e antigen (HBeAg)-negative hepatitis B surface antigen (HBsAg) carrier mothers in Taiwan is not clear. OBJECTIVE: To describe the responses of infants born to HBeAg-negative carrier mothers receiving HBIG combined with hepatitis B vaccine. METHODS: Term babies born to HBeAg-negative carrier mothers were assigned based on chart number to 1 of the 2 treatment groups. Group A infants (n = 94) received 0.5 ml (145 IU) of HBIG within 24 h of birth and 3 subsequent doses of recombinant hepatitis B virus (HBV) vaccine at 3 to 5 days, 1 month and 6 months of age. Group B infants (n = 122) received 3 doses of vaccines only. Infants (n = 19) born to HBeAg-positive carrier mothers were treated like those in Group A and are referred to as Group C. Sera obtained from infants at 2 and 7 months of age were tested for hepatitis B virus (HBV) markers. RESULTS: There were 2 (1%; one in Group A and one in Group B) subclinical breakthrough hepatitis B infections among studied infants. One (5%) child of Group C had asymptomatic HBV infection at the age of 7 months and became a chronic carrier. The rate of protective anti-hepatitis B surface antibody (anti-HBs) titers achieved (>10 mIU/ml) by 2 months of age was significantly higher in Group A than that in Group B (98% vs. 57%, P < 0.001). However, it was not different by 7 months of age. Infants (Group A) immunized with HBIG and vaccine had a significantly higher geometric mean titer (GMT, milli-International Units/ml) of anti-HBs than those (Group B) with vaccines only at 2 months of age (P < 0.001). Conversely at 7 months of age, the GMT of anti-HBs was significantly higher in infants who received vaccine only (P = 0.001). CONCLUSIONS: A protective level of antibodies was achieved earlier in those infants receiving both passive and active immunizations. However, infants receiving active immunizations alone achieved a higher GMT at 7 months of age. There was no clear benefit of passive-active vs.active immunization alone for chronic HBV infection in infants of HBsAg-positive, HBeAg-negative mothers.
Carrier State↗
Long-term immunogenicity and efficacy of universal hepatitis B virus vaccination in Taiwan.
The long-term immunogenicity of universal hepatitis B virus (HBV) vaccine is seldom studied in large-scale prospective community-based populations, especially in adolescents. This study enrolled 1200 children aged 7 years with complete HBV immunization in infancy and determined HBV surface antigen (HBsAg), its antibody (anti-HBs), and HBV core antibody (anti-HBc) annually until the children were aged 14 years. Eleven children had new HBV infections with anti-HBc positivity as the only marker. None became positive for HBsAg or had detectable HBV DNA by polymerase chain reaction. The percentage of protective anti-HBs in 951 children without booster vaccination gradually decreased from 71.1% at age 7 years to 37.4% at age 12 years. Only 1 of the 200 children in the booster group and 2 of the 258 children in the nonbooster group developed new anti-HBc positivity. The results suggest that routine booster vaccination may not be required to provide protection against chronic HBV infection before age 15 years.
Adolescent↗
Characterization of integration patterns and flanking cellular sequences of hepatitis B virus in childhood hepatocellular carcinomas.
Hepatitis B virus (HBV) DNA integration into host chromosomes is detected in more than 80% of HBV-related hepatocellular carcinomas (HCC), yet its significance in tumor development remains obscure. In this study, we re-examined the integration pattern of HBV in childhood HCC tissues, which has less environmental confounding factors than adult HCC. The HBV junctions and flanking cellular sequences were amplified from five childhood HCC patients by the inverse polymerase chain reaction (IPCR) method using primers located near HBV direct repeats (DR) 1 and 2. The viral junctions in nine of the ten obtained IPCR clones were demonstrated to be located near HBV DR1, and their patterns were classified to type I integrants. Southern blot analyses demonstrate that the cellular junctions derived from two of the five HCC tissues were male specific and contained sequences homologous to human long interspersed DNA elements (LINE-1). HBV integrant of one HCC tissue (1217T) was integrated into a RNA binding motif Y chromosome (RBMY) gene. The expression of RBMY, which is normally found only in male germ cells, was detected in HCC tissue 1217T by RT-PCR but not in the corresponding non-tumor liver tissue. The prevalence of RBMY expression in liver tissues from the tumor and non-tumor parts of ten other HCC children and seven biliary atresia (BA) children was studied by RT-PCR. No RBMY transcripts were detected in the non-tumor parts of HCC patients or the cirrhotic livers of BA children, whereas 30% (three of ten) of HCC tissues specifically expressed RBMY. The results indicate that HBV integration and activation of RBMY gene expression in liver cells may be associated with the development of childhood HCC.
Base Sequence↗
Multiple intrahepatic biliary cysts in children with biliary atresia.
PURPOSE: The aim of this study was to investigate the incidence, clinical course, and outcome of multiple intrahepatic biliary cysts in patients with biliary atresia. METHODS: From 1992 to 2000, 154 patients with biliary atresia were examined for intrahepatic cysts. They were followed and examined routinely using abdominal ultrasonography. RESULTS: Twenty-three patients had single intrahepatic cysts, and 16 patients had multiple cysts. The incidence of intrahepatic cysts in these patients were 25.3% (39 of 154) for all kinds of cysts and 10.4% (16 of 154) for multiple intrahepatic cysts. Of the 16 patients with multiple cysts, 13 (81.3%) had jaundice, and 15 (93.8%) had a history of cholangitis before cysts were detected. Image studies showed multiple discrete ovoid or round intrahepatic biliary cysts with various sizes along the biliary trees. The cysts decreased in size or number in 7 patients after antibiotic treatment and disappeared in only one patient. The mortality rate was higher in patients with multiple cysts than in those with single cysts (P =.037). CONCLUSIONS: Multiple intrahepatic biliary cysts are a common complication in patients with biliary atresia and suggests a poor prognosis. Prolonged parenteral antibiotics treatment should be administered when signs of biliary infection appear.
Adolescent↗
FIC1 and BSEP defects in Taiwanese patients with chronic intrahepatic cholestasis with low gamma-glutamyltranspeptidase levels.
To elucidate the frequency of FIC1 (ATP8B1) and BSEP (ABCB11) mutations in Taiwanese children with chronic intrahepatic cholestasis with low gamma-glutamyltranspeptidase (GGT) levels, we assessed 13 unrelated patients with infantile onset chronic intrahepatic cholestasis. Liver complementary DNA sequencing was performed in 7 infants for mutation analyses of FIC1 and BSEP genes. Two distinct liver histologic features were found. Group 1 (n = 5) was characterized by bland cholestasis and group 2 (n = 8) by giant cell transformation. Group 2 patients were associated with higher transaminase levels, alpha-fetoprotein levels, and early mortality. Novel FIC1 mutations were found in all 4 patients tested in group 1, including a 74-bp deletion, a 98-bp deletion, a nonsense, and 2 missense mutations. BSEP mutations were found in 2 of the 3 patients in group 2, including 2 missense mutations and a 1-bp deletion. Phenotypic characterization is useful to differentiate FIC1- from BSEP-related disease.
ATP Binding Cassette Transporter, Subfamily B, Mem↗
Acute and chronic hepatitis: Working Group Report of the First World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition.
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Acute Disease↗