Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Serum alpha-fetoprotein levels were measured using a sensitive radioimmunoassay in 77 infants presenting with persistent conjugated hyperbilirubinaemia. A breed range of alpha-fetoprotein concentrations occurred in both the 23 infants with extrahepatic biliary atresia and the 35 with idiopathic neonatal hepatitis but the 13 with alpha-1-antitrypsin deficiency had uniformly low levels. High alpha-fetoprotein concentrations (above 10 000 mug/1) favoured the diagnosis of neonatal hepatitis especially in the first ten weeks of life, but the overlap between neonatal hepatitis and extrahepatic biliary atresia was large and alpha-fetoprotein determination cannot be recommended as a reliable method for distinguishing the two conditions. Serial alpha-fetoprotein values showed no consistent relationship with standard liver function tests and gave no guide to prognosis. There was an association between alpha-fetoprotein production and needle biopsy evidence of hepatic giant cell transformation. The uniformly low alpha-fetoprotein levels in alpha-1-antitrypsin deficient infants with neonatal hepatitis is a new observation and possible mechanisms for disordered glycoprotein release are discussed.
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PMID:Serum alpha-fetoprotein levels in extrahepatic biliary atresia, idiopathic neonatal hepatitis and alpha-1-antitrypsin deficiency (PiZ). 6 Aug 72

A 35-year-old woman who had used Non-Ovlon for 3 1/2 years was treated for anicteric hepatitis and underwent vaginal extirpation of the uterus due to carcinoma. Point biopsies were taken of the liver at this time and 1 year later, and histological and electron microscopic studies were also performed. The examination of the hepatocytes revealed intracisternal, hyaloplasmic, and mitochondrial hyalin, i.e. protein deposits in the endoplasmic reticulum, the cytoplasmic ground substance, and the mitochondria ("Giant mitochondria"), respectively. Coagulation necrosis of the hepatocytes was also observed. These abnormal protein deposits could not be related to abnormal alpha-1-antitrypsin synthesis in the liver. No regression in the protein deposits was observed 5 months after Non-Ovlon use was discontinued. It was also ascertained that the histological discovery of globular hyaline bodies can indicate that any or all of the various hyalins or cell mecrosis can exist simultaneously.
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PMID:[Intracisternal hyalin and giant mitochondria in hepatocytes and oral contraceptives (author's transl)]. 9 11

Five patients with alpha-1-antitrypsin deficiency (PiZ) are reported. All these patients presented with the neonatal hepatitis syndrome and two fo them had developed cirrhosis at ages 5 and 8 years, respectively. Three patients, ages 1, 9 and 21 years, are asymptomatic. The oldest patient, 21 years of age, has only mild histologic changes in the liver. The prognosis for patients with alpha-1 antitrypsin deficiency (PiZ) presenting with neonatal hepatitis is not necessarily grave, a finding that differs from previous observations.
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PMID:Neonatal hepatitis and alpha-1-antitrypsin deficiency. The prognosis in five patients. 30 Apr 52

A case of a 70-year-old woman with a history of gastric ulcer and several pneumonias is presented. She was found to have pulmonary emphysema, severe alpha-1-antitrypsin (alpha1AT) deficiency and raised serum mitochondrial antibodies. Surgical liver biopsy showed portal liver cirrhosis, PAS-positive, diastaseresistant globules in the hepatocytes and changes interpreted as florid duct lesion of primary biliary cirrhosis. A brother has severe alpha1AT deficiency. Two daughters had raised mitochondrial antibodies. One of the latter had a granulomatous hepatitis, a common finding in primary biliary cirrhosis. The association of alpha1AT deficiency and primary biliary cirrhosis does not seem to have been described previously.
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PMID:Alpha-1-antitrypsin deficiency, mitochondrial antibodies and possible primary biliary cirrhosis. A case report and family study. 108 Sep 23

The authors report a case of neonatal hepatitis with alpha-1-antitrypsin occuring in a child of ZZ phenotype. The anatomopathological study carried out on two liver biopsies showed changes of common cholestatic hepatitis developing into cirrhosis, as well as intrahepatocytary globulins. Moreover, these globulins, P.A.S. positive after treatment by alphaamylase, fix an antialpha-1-antitrypsine antiserum. Ultrastructural analysis shows them to be masses of amorphous material, feebly osmiophilic, outlined by a unitary membrane the moniliform aspect of which recalls the ergastoplasmic membrane. These findings are identical to those already made in cases of cirrhogenous neonatal hepatitis by alpha-1-antitrypsine deficit reported in the literature. They point out the irreversibility of the affection which, after a stage of cholestatic hepatitis with or without inflammatory portal fibrosis, develops into cirrhosis. At this stage cholestasis has regressed or disappeared whereas portal sclerosis, often infiltrated with free elements, surrounds hepatic lobules and biliary neocanaliculi. But the globulins are still present and appear to be the specific feature of this deficit. By their ultrastructural and immuno-histochemical features, these globulins would represent a form of accumulation of alpha-1-antitrypsin in the hepatocytes which normally carry out the synthesis of this antienzyme. Accumulation in the hepatocytes proves excretory disturbance of hypothetical mechanism: structural anomaly, changes in the permeability of the membrane. Its role in the occurrence of hepatitis or cirrhosis lesions is still to be demonstrated but one may think that it consists in absence of inhibition of the enzymatic factors discharged during agressions.
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PMID:[Neonatal hepatitis with alpha-1-antitrypsin deficit. Apropos of a personal case]. 108 58

The authors evaluate the health status of children with alpha-1-antitrypsin deficiency, focused on liver disease in infant age. The children were selected by neonatal screening. Of 21 children one had severe neonatal hepatitis with progression to cirrhosis, 2 children had clinically apparent jaundice to the age of two months, 6 children had elevated total bilirubin and transaminase levels without clinical signs of the disease, 12 of the remaining children had no clinical and laboratory signs of liver disease. In the discussion the authors compare the results with data published abroad.
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PMID:[Liver diseases in children with alpha 1-antitrypsin deficiency in infancy]. 139 67

Low levels of alpha-1-antitrypsin can predispose deficient infants to the development of hepatitis and cirrhosis. Heterozygous PiMZ carriers can be affected by a subclinical liver involvement during their first half year of life. One pathogenic hypothesis of liver damage is that the process seems to be mediated by the activity of toxic oxygen waste products. In the present investigation it was found that the antioxidant vitamin E was able to significantly reduce the frequency of liver involvement in PiMZ carriers at two months of age but not at five months. These findings indicate that oxidative free radicals can promote liver damage in inadequately protected young infants, such as in alpha-1-antitrypsin deficiency. The protective role of vitamin E in relation to the developmental expression of other anti-oxidant scavengers is discussed.
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PMID:Oxidative radicals and liver involvement of infants with alpha-1-antitrypsin deficiency. 166 23

From 1985 to 1988 14,938 newborns were screened during the first days of life to determine their protease inhibitor phenotype (Pi) and 467 PiMZ and 456 PiMS were identified. Of these 101 PiMZ and 135 PiMS were followed-up and their clinical, biochemical and, in selected cases, histological data were recorded at two, five and twelve months of age. Nineteen out of 101 PiMZ infants showed hepatic dysfunction at two months, eight at five, and one at twelve months of age, respectively. In 20 of 135 PiMS infants, liver function tests were abnormal at two months, in ten at five and in none at twelve months. It appears that PiMZ and PiMS phenotypes can be associated with hepatic dysfunction during the first six months of life. The marked variability of serum levels of alpha-1-antitrypsin in heterozygotes, make Pi-typing in all cases of neonatal hepatitis advisable. This should also be done for screening purpose.
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PMID:Liver disease and heterozygous alpha-1-antitrypsin deficiency. 203 27

The hepatitis B virus X protein acts as a transcriptional transactivator in vitro. To elucidate possible biological effects of X protein on liver cells in vivo, we generated four lines of transgenic mice carrying the X gene open reading frame under the control of the human alpha-1-antitrypsin regulatory region. The plasmid construct used to introduce the transgene was shown to encode a 16-kDa X protein with transactivating capability. The expression of X protein was detectable in liver tissue of transgenic animals of three of the lines by immunoblot analysis; levels of expression were highest in the first month after birth of the animals. Over 80 animals from the expressing lines were examined histologically. Most transgenic mice, some of which were observed for up to 2 years, remained normal. However, a few transgenic animals developed mild focal hepatitis, nuclear pleomorphism, focal necrosis, and/or nodular hyperplasia in the liver. Increased mitotic activity of hepatocytes also was observed. We conclude that, at the level of expression achieved in these transgenic mice, the hepatitis B virus transcriptional transactivator X protein alone does not appear to mediate the development of serious liver damage or hepatocellular carcinomas.
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PMID:Hepatitis B virus transactivator X protein is not tumorigenic in transgenic mice. 224 80

About 15% of children with alpha-1-antitrypsin-deficiency with proteinase inhibitor type ZZ develop hepatopathy, uninfluenceable in its course. These children already show symptoms of severe cholestatic hepatitis in early infancy as became obvious from data of 13 children being patients in the authors care and suffering from hepatic cirrhosis with alpha-1-antitrypsin-deficiency. At present liver transplantation is the only causal possibility of therapy. Even without highly specialized laboratory the non-laboratory assistant will recognize at least the homozygous alpha-1-antitrypsin-deficiency (PI-ZZ). The therapeutic approach must be directed on treating the patients in such a way that liver transplantation will be possible at a favourable moment and under good conditions. Since PI-ZZ family members suffer similar course of hepatopathy, genetic counsel is of special significance.
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PMID:[Liver diseases in alpha 1 antitrypsin deficiency syndrome in children]. 233 11


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