Modification of the alpha 1-antitrypsin phenotype in neonatal hepatitis.
نویسندگان
چکیده
منابع مشابه
Modification of the alpha 1-antitrypsin phenotype in neonatal hepatitis.
Two cases of neonatal hepatitis are described, one related to cytomegalovirus infection and the other idiopathic. In both infants a transient abnormality of the alpha 1-antitrypsin phenotype, inconsistent with the parent's phenotypes, reverted to normal during the convalescent phase of the illness.
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The finding is recorded of typical a1-antitrypsin globules, confirmed by immunofluorescence and immunoperoxidase methods, in the hepatocytes of a patient shown to have a normal serum antitrypsin level and normal phenotype (TiM) for a1-antitrypsin. The identification of such globules can no longer be regarded as conclusive evidence of an abnormal a1-antitrypsin phenotype.
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Typical liver cell inclusions of alpha-1-antitrypsin deficiency were found in a patient with the Pi S phenotype and acute alcoholic hepatitis. It is suggested that the inclusions resulted from the combined effects of the S phenotype and alcohol and, on the basis of the known chemistry of the variant antitrypsins, that the lowered serum concentration of antitrypsin associated with the S allele m...
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A prospective epidemiological study of the Neonatal Hepatitis Syndrome in S.E. England showed Alpha-1Antitrypsin Deficiency (Pi ZZ) to be present in seven out offifty-two patients. Data are considered from these seven patients, and from a further six cases from outside this area. The nature of acute illness, pathological changes on early liver biopsy, and short-term prognosis show considerable ...
متن کاملAlpha 1 Antitrypsin Deficiency in Infants with Neonatal Cholestasis
OBJECTIVE Alpha1-antitrypsin deficiency (A1ATD) is the most important indication for liver transplantation in children. The gene frequencies vary in different ethnic groups. In the present study, we attempt to determine the frequencies of the most common defective alleles, Z and S, in Iranian children suffering from idiopathic neonatal cholestasis. Eighty-seven infants were typed for Z and S al...
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ژورنال
عنوان ژورنال: Archives of Disease in Childhood
سال: 1985
ISSN: 0003-9888,1468-2044
DOI: 10.1136/adc.60.4.378