The Experts below are selected from a list of 204 Experts worldwide ranked by ideXlab platform
Eve A Roberts - One of the best experts on this subject based on the ideXlab platform.
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Neonatal Hepatitis syndrome
Seminars in Neonatology, 2003Co-Authors: Eve A RobertsAbstract:Abstract Conjugated hyperbilirubinaemia in an infant indicates Neonatal liver disease. This Neonatal Hepatitis syndrome has numerous possible causes, classified as infective, anatomic/structural, metabolic, genetic, neoplastic, vascular, toxic, immune and idiopathic. Any infant who is jaundiced at 2–4 weeks old needs to have the serum conjugated bilirubin measured, even if he/she looks otherwise well. If conjugated hyperbilirubinaemia is present, a methodical and comprehensive diagnostic investigation should be performed. Early diagnosis is critical for the best outcome. In particular, palliative surgery for extrahepatic biliary atresia has the best chance of success if performed before the infant is 8 weeks old. Definitive treatments available for many causes of Neonatal Hepatitis syndrome should be started as soon as possible. Alternatively, liver transplantation may be life saving. Supportive care, especially with attention to nutritional needs, is important for all infants with Neonatal Hepatitis syndrome.
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outcome of hepatobiliary scanning in Neonatal Hepatitis syndrome
The Journal of Nuclear Medicine, 1997Co-Authors: Susan M Gilmour, M Hershkop, Ram Reifen, David L Gilday, Eve A RobertsAbstract:UNLABELLED: To evaluate the diagnostic information gained from hepatobiliary scanning in infants, we reviewed 86 consecutive infants who were < or = 4 mo old and were treated for conjugated hyperbilirubinemia at the Hospital for Sick Children in Toronto between 1985 and 1993 and who had technetium iminodiacetic hepatobiliary scanning and a percutaneous liver biopsy performed in close temporal proximity. METHODS: Retrospective reviews of hospital charts and blinded reviews of hepatobiliary scans were performed. RESULTS: There were 58 male and 28 female infants (age range, 2-124 days; mean = 65 days). Hepatobiliary scanning failed to show biliary excretion into the gastrointestinal tract in 53 of 86 patients. Forty of these 53 had extrahepatic biliary atresia. The remaining 33 patients demonstrated biliary excretion into the gastrointestinal tract; 24 of 33 had Neonatal Hepatitis. Among 13 of the 53 patients who had no evidence of biliary excretion and who also did not have extrahepatic biliary atresia, 8 had idiopathic Neonatal Hepatitis, 4 had interlobular bile duct paucity and 1 had total parenteral nutrition-associated cholestasis. In this large series, no patient with extrahepatic biliary atresia showed bile drainage on hepatobiliary scanning. Fifty percent of patients with interlobular bile duct paucity but no extrahepatic obstruction failed to show biliary excretion of radionuclide. Twenty-five percent of patients (8 of 32) with idiopathic Neonatal Hepatitis demonstrated no biliary excretion. Hepatocellular extraction was examined by semiquantitative analysis in the nondraining, nonbiliary atresia patients (12 of 53). Four of these 12 patients demonstrated poor liver extraction. Three patients had idiopathic Neonatal Hepatitis, and one had bile duct paucity. Therefore, four of eight Neonatal Hepatitis patients had normal extraction, suggesting that poor versus good liver hepatocyte clearance cannot accurately identify Neonatal Hepatitis. CONCLUSION: Hepatobiliary scanning requires cautious interpretation. Nondraining scans may indicate severe Neonatal Hepatitis or the presence of interlobular bile duct paucity.
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Neonatal Hepatitis syndrome with paucity of interlobular bile ducts in cystic fibrosis.
Journal of Pediatric Gastroenterology and Nutrition, 1991Co-Authors: Katryn N. Furuya, Eve A Roberts, G. J. Canny, M. J. PhillipsAbstract:Summary A male infant presenting with Neonatal Hepatitis syndrome, characterized by conjugated hyperbilirubinemia and very mild liver function test abnormalities, at 2 weeks of age was found to have no excretion of radioisotope into the intestinal tract on hepatobiliary scan. Liver biopsy revealed severe interlobular bile duct paucity. Other features of Alagille's syndrome were not present; other conditions frequently associated with interlobular bile duct paucity were also excluded. Subsequently, the infant was found to have cystic fibrosis. Cystic fibrosis is thus another disease that may be associated with paucity of interlobular bile ducts presenting as Neonatal Hepatitis syndrome, and this represents a different pathogenesis of cholestatic jaundice in neonates with cystic fibrosis besides those previously recognized.
Magd A Kotb - One of the best experts on this subject based on the ideXlab platform.
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ursodeoxycholic acid in Neonatal Hepatitis and infantile paucity of intrahepatic bile ducts review of a historical cohort
Digestive Diseases and Sciences, 2009Co-Authors: Magd A KotbAbstract:We retrospectively reviewed the role of ursodeoxycholic acid in infants having nonsurgical cholestasis attending the Hepatology Clinic, New Children Hospital, Cairo University, Egypt, from 1985 until 2005. Files of 496 infants with Neonatal Hepatitis and 97 with intrahepatic bile duct paucity were included; of them 241 (48.6%) and 52 (46.4%) received 20–40 mg/kg/day ursodeoxycholic acid for 319.2 ± 506.9 days and 480.3 ± 583.3 days, respectively. The outcome of infants with Neonatal Hepatitis with intake of ursodeoxycholic acid and those without was: 108 (44.8%) and 179 (70.2%) successful (P = 0.000), 11 (4.6%) and 13 (5.1%) improved (P = 0. 474), 112 (46.5%) and 61 (23.9%) suffered failed outcome (P = 0.000), and 10 (4.1%) and 2 (0.78%) died (P = 0.014), respectively. Likelihood of successful outcome with ursodeoxycholic acid intake was 0.345 (P = 0.000), and that of deterioration was 2.76 (P = 0.000). For those having intrahepatic bile duct paucity likelihood of successful outcome with ursodeoxycholic acid intake was 0.418 (P = 0.040) and that of deterioration was 2.64 (P = 0.028). Ursodeoxycholic acid failed in management of this cohort of infants with nonsurgical cholestasis.
Anil Dhawan - One of the best experts on this subject based on the ideXlab platform.
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significance of low or normal serum gamma glutamyl transferase level in infants with idiopathic Neonatal Hepatitis
European Journal of Pediatrics, 2006Co-Authors: Jian She Wang, Anil DhawanAbstract:Introduction We evaluated the significance of low/normal serum gamma glutamyl transferase (GGT) level in infants with idiopathic Neonatal Hepatitis (INH).
Jian She Wang - One of the best experts on this subject based on the ideXlab platform.
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significance of low or normal serum gamma glutamyl transferase level in infants with idiopathic Neonatal Hepatitis
European Journal of Pediatrics, 2006Co-Authors: Jian She Wang, Anil DhawanAbstract:Introduction We evaluated the significance of low/normal serum gamma glutamyl transferase (GGT) level in infants with idiopathic Neonatal Hepatitis (INH).
Mei-hwei Chang - One of the best experts on this subject based on the ideXlab platform.
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differential diagnosis of extrahepatic biliary atresia from Neonatal Hepatitis a prospective study
Journal of Pediatric Gastroenterology and Nutrition, 1994Co-Authors: Mei-hwei Chang, Chengtau SuAbstract:SummaryThe clinical presentations of cholestasis in infancy caused by Neonatal Hepatitis and biliary atresia are very similar. Diagnosis may be difficult on many occasions, but the surgical treatment of biliary atresia should be performed as early as possible. We established a 3-day workup protocol for the differential diagnosis of biliary atresia and Neonatal Hepatitis and compared the diagnostic accuracy, sensitivity, specificity, and predictive values of various methods. One hundred and twenty-six infants, including 84 with Neonatal Hepatitis (age, 65.1 ± 24.1 days) and 42 with biliary atresia (age, 60.3 ± 31.1 days), were studied prospectively from July 1982 to December 1990. The diagnostic accuracy of various methods was as follows: liver histology, 96.8%; color of duodenal juice, 91.6%; peak radioisotope count in duodenal juice, 84.2%; ultrasonographic examination of the hepatobiliary system, 80.2%; and persistence of clay-colored stool, 80.2%. After stepwise logistic regression, the diagnostic methods of significance were liver biopsy, color of duodenal juice, abdominal ultrasonography, and stool color. However, stool color and the onset of jaundice could not differentiate severe Neonatal Hepatitis from biliary atresia. The diagnostic methods of significance then were liver biopsy and duodenal juice color. With this 3-day protocol, no biliary atresia was missed although four cases of Neonatal Hepatitis were misdiagnosed, resulting in unnecessary laparotomy; we found an overall diagnostic accuracy of 96.8%. We conclude that this 3-day diagnostic protocol is very helpful in the differential diagnosis of Neonatal Hepatitis and biliary atresia. Liver histologic examination is the most reliable single test for the differential diagnosis.
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Cytomegalovirus Infection and Neonatal Hepatitis
Molecular Aspects of Human Cytomegalovirus Diseases, 1993Co-Authors: Mei-hwei ChangAbstract:Neonatal Hepatitis is an inflammatory disease of the liver found in early infancy presenting with intrahepatic cholestasis. The etiologies remain unknown in about one half to two thirds of patients. Cytomegalovirus is an important etiologic agent of Neonatal Hepatitis. Either congenital or perinatal infection by cytomegalovirus can cause Neonatal Hepatitis. However, the role of cytomegalovirus may be underestimated. An extensive search for evidence of cytomegalovirus in patients with Neonatal Hepatitis, including urine or saliva culture, serology, immunocytochemistry, and molecular biology studies, may show that the proportion of patients with idiopathic Neonatal Hepatitis is less than is currently believed.
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polymerase chain reaction to detect human cytomegalovirus in livers of infants with Neonatal Hepatitis
Gastroenterology, 1992Co-Authors: Mei-hwei Chang, Huihsien Huang, Engshang HuangAbstract:Abstract Neonatal Hepatitis is closely related to human cytomegalovirus infection in Taiwan, a conclusion based on serological and urine culture studies. To obtain more direct evidence further relating cytomegalovirus to the pathogenesis of Neonatal Hepatitis, the cytomegalovirus genome was studied in the liver tissues of 50 infants with Neonatal Hepatitis using the polymerase chain reaction (PCR). Liver tissues from 26 infants with biliary atresia and another 30 infants and children with diagnoses other than Neonatal Hepatitis, cholestasis, or Hepatitis were also studied for comparison. Sequences from the immediate early gene 1 and 2 regions were used as primers. The liver tissues from 23 (46%) of the 50 infants with Neonatal Hepatitis were positive for cytomegalovirus genome, whereas those of 2 of the 26 infants with biliary atresia and none of the liver tissues from 30 infants and children without Neonatal Hepatitis were positive for cytomegalovirus genome, by PCR. The results of PCR correlated well with that of serology and urine culture. This study provides further evidence of cytomegalovirus in the pathogenesis of Neonatal Hepatitis.
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Cytomegalovirus-associated Neonatal Hepatitis.
Acta paediatrica sinica, 1992Co-Authors: Mei-hwei ChangAbstract:Fifty-five with cytomegalovirus (CMV) -associated Neonatal Hepatitis (NH) were followed for 12 to 90 months. Six patients (10.9%) died from either a fulminant course or a chronic liver disease. Among the remaining 49 patients, whose liver function was completely recovered, there were eight with retardation of developmental or growth status, and two with hearing impairment. Overall, 20.4% of the survivors suffered from a long-term impact. The unfavorable outcome was related to several clinical and pathological parameters. These included persistence of clay-colored stool, presence of splenomegaly, ascites or anemia, high peak total and direct bilirubin, low nadir albumin levels, diffuse giant cell transformation and cirrhosis of the liver. The seropositivity of CMV infection did not significantly correlate with the outcome.
