1/15. alpha1-Antitrypsin deficiency and liver disease in children.This report describes the clinical, biochemical, and hepatic morphologic findings in ten children with severe serum alpha1-antitrypsin deficiency. Genetic protease inhibitor (Pi) phenotyping, using acid-starch gel and crossed antigen-antibody electrophoresis, demonstrated Pi phenotype ZZ in all our cases. In eight patients, manifestations of liver disease appeared during the first year of life. The case reports show that alpha1-antitrypsin deficiency should be suspected in any child with neonatal hepatitis, unexplained hepatomegaly or splenomegaly, or cirrhosis. In our report, one infant is normal at age 6 months, and one infant had progressive hepatic damage that culminated in liver failure and death at age 6 months. The variable clinical course and prognosis for infants with severe alpha1-antitrypsin deficiency is well illustrated by these two infants.- - - - - - - - - - ranking = 1keywords = hepatitis (Clic here for more details about this article) |
2/15. Neonatal cholestatic hepatitis from carbamazepine exposure during pregnancy and breast feeding.OBJECTIVE: To report a case of transient cholestatic hepatitis occurring in an infant between the third and seventh weeks of life, most likely due to carbamazepine exposure during pregnancy and breast feeding. CASE SUMMARY: A boy, born to an epileptic mother who had been treated with carbamazepine monotherapy throughout pregnancy and breast feeding, experienced asphyxia at birth with transient hepatic dysfunction in the first week of life. After full recovery from asphyxia, he experienced a second period of liver dysfunction, presenting as cholestatic hepatitis that lasted approximately 5 weeks. Infectious and metabolic etiologies as well as extrahepatic biliary atresia were excluded. DISCUSSION: carbamazepine is known to induce hepatic damage in children and adults. As the drug crosses the placenta and is excreted into breast milk, infants of mothers taking carbamazepine might also develop liver dysfunction. In addition to the present case, there are 2 well-documented case reports of cholestasis in association with transplacental and transmammary carbamazepine exposure. CONCLUSIONS: carbamazepine-induced hepatitis may occur in association with prenatal exposure and breast feeding. This may expose infants to unnecessary diagnostic procedures, and should therefore be mentioned in the company's product information.- - - - - - - - - - ranking = 7keywords = hepatitis (Clic here for more details about this article) |
3/15. Familial occurrence of giant cell hepatitis in infancy.The repeated occurrence within one family or giant cell hepatitis terminating in death is reported. The familial form of giant cell hepatitis has a poorer prognosis than that occuring sporadically. In the first case, reported earlier, primary liver cancer developed within a short time. In the second case, reported here, the progression of the course of the disease which terminated in cirrhosis was followed in a series of histological examinatons made at five different points of time. Electronmicroscopic examination were also conducted.- - - - - - - - - - ranking = 6keywords = hepatitis (Clic here for more details about this article) |
4/15. Fate of infants with neonatal hepatitis: pediatric surgeons' dilemma.Thirty-five cases of neonatal hepatitis (20 males and 15 females) were reviewed, 3 of whom were lost during the follow-up, leaving 32 patients for review. There were 10 late deaths and 22 patients survived, 18 of whom with a normal bilirubin level and 4 with a bilirubin level of greater than 1.0 mg/dL. In the 18, jaundice disappeared between the ages of 4 and 7 months. The current lifestyles of the patients include 4 adults aged 19 to 21 who are either working or at university, while the other 18 children are all making good progress at school. Except for moderate growth retardation in 3 children, all are growing well. In all 10 patients who died, liver failure persisted until the time of death. Three died of other causes and 7 died of neonatal hepatitis itself between 4 months and 7 years of age. Four patients ran a fulminating course resulting in death between the ages of 4 and 12 months. All 7 had growth and developmental retardation. A histological examination showed that in those who died, there was significantly more periportal fibrosis, inflammation in the periportal area, and diffuse giant cell transformation. These results indicate that some infants with neonatal hepatitis have a poor prognosis and, therefore, the identification of such a condition requires a careful, long-term follow-up.- - - - - - - - - - ranking = 7keywords = hepatitis (Clic here for more details about this article) |
5/15. Bacille Calmette-Guerin--associated neonatal hepatitis.We describe a full-term immunocompetent neonate who developed jaundice at 3 weeks of age. physical examination disclosed no abnormalities. Laboratory investigations showed direct reacting hyperbilirubinaemia and elevated liver enzymes. Liver biopsy revealed a non-caseating granulomatous hepatitis. The patient made an uneventful recovery within 4 weeks without therapy. Bacille Calmette-Guerin hypersensitivity reaction is suggested as the reason for this neonatal hepatitis.- - - - - - - - - - ranking = 6keywords = hepatitis (Clic here for more details about this article) |
6/15. Transient cholestatic hepatitis in a neonate associated with carbamazepine exposure during pregnancy and breast-feeding.We report a 3-week-old boy with cholestatic hepatitis, most likely due to carbamazepine exposure during pregnancy and breastfeeding. cholestasis resolved after cessation of nursing. Liver function test results and histological findings were compatible with a drug-induced hepatitis. Other causes were excluded. While carbamazepine-induced hepatitis is well known in children and adults, it has never been described in association with prenatal exposure and/or breast-feeding.- - - - - - - - - - ranking = 7keywords = hepatitis (Clic here for more details about this article) |
7/15. Serial ultrasonic examination to differentiate biliary atresia from neonatal hepatitis--special reference to changes in size of the gallbladder.We performed serial ultrasonic examinations to differentiate biliary atresia from neonatal hepatitis. The subjects studied were 144 children (100 normal neonates and infants, 31 patients with neonatal hepatitis and 13 patients with biliary atresia). They were examined by ultrasound before, during and after feeding. In 97 out of 100 normal children and all patients with neonatal hepatitis, the gallbladder was identified, and the change in size following oral feeding was observed. In four children with severe neonatal hepatitis which could not be differentiated from biliary atresia by clinical and laboratory data, we readily identified the gallbladder and observed the change in the size following oral feeding. In 8 of 13 patients with biliary atresia, we identified a small gallbladder whose size was not affected by oral feeding. In the other patients the gallbladder was not identified before, during or after oral feeding. On the basis of these results, we consider that serial ultrasonic examination with oral feeding aids in a differential diagnosis of biliary atresia and neonatal hepatitis.- - - - - - - - - - ranking = 9keywords = hepatitis (Clic here for more details about this article) |
8/15. Conjugating ability of bile acids in hepatic failure.A 5-month-old boy with giant cell hepatitis died of hepatic failure. Analysis of serum bile acids by high pressure liquid chromatography revealed a marked increase in total bile acid concentration and a decrease in the cholate to chenodeoxycholate ratio. However, contrary to expectation, the conjugating ability of bile acids with taurine or glycine was well preserved. This suggests that the amino acid conjugation can occur despite the presence of severe hepatocellular damage.- - - - - - - - - - ranking = 1keywords = hepatitis (Clic here for more details about this article) |
9/15. Neonatal hepatitis with obstructive jaundice in an SZ heterozygous alpha 1-antitrypsin-deficient boy and destructive lung disease in his SZ mother. A review of the literature.SZ-alpha 1-antitrypsin deficiency, leading to severe transient neonatal cholestasis with mild hepatitis is rare. In our patient, intrahepatic bile duct hypoplasia was suspected. Since cholecystography and hepatic scintigraphy failed to reveal intrahepatic bile ducts, a diagnostic surgical liver biopsy was performed. Typical intrahepatocytic PAS-positive granules and a low serum alpha 1-antitrypsin concentration allowed a correct diagnosis of the SZ phenotype. The administration of an elementary diet quickly improved the clinical condition of our patient. follow-up studies of this case and others will allow a better establishment of prognostic criteria and help in genetic counselling.- - - - - - - - - - ranking = 5keywords = hepatitis (Clic here for more details about this article) |
10/15. Kayser-Fleischer-like rings in patients without Wilson's disease.Three patients, one with cryptogenic cirrhosis, one with active chronic hepatitis and one with neonatal hepatitis, were found to have corneal pigmentation rings indistinguishable from early Kayser-Fleischer rings on slit lamp examination. They did not have the clinical features of Wilson's disease and their serum copper and ceruloplasmin concentrations were normal. Urinary copper excretion rates and hepatic concentrations were only slightly raised but were below the range found in symptomatic Wilson's disease. It is concluded that the Kayser-Fleischer ring would no longer be considered as pathognomonic of Wilson's disease.- - - - - - - - - - ranking = 2keywords = hepatitis (Clic here for more details about this article) |
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