-Antitrypsin Deficiency and Neonatal Hepatitis

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PAPERS AND ORIGINALS ali - Antitrypsin Deficiency and Neonatal Hepatitis

Five out of 28 infants investigated in a regional survey of neonatal hepatitis were found to have genetically-determined deficiency of ai-antitrypsin (ZZ phenotype). The clinical course and pathological changes varied considerably. All five infants had an acute hepatitis-like illness, and although this subsided cirrhosis later developed in three cases. The remaining two infants had minimal abno...

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Neonatal hepatitis syndrome and alpha-1-antitrypsin deficiency: an epidemiological study in south-east England.

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 ...

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Alpha 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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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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-Antitrypsin Deficiency

Department of Biostatistics and Epidemiology, Cleveland Clinic Foundation, Cleveland, Ohio; Department of Pulmonary and Critical Care Medicine, Cleveland Clinic Foundation, Cleveland, Ohio; Department of Pulmonary and Critical Care Medicine, Oregon Health Sciences University, Portland, Oregon; Division of Pulmonary and Critical Care Medicine, The New York Hospital/Cornell University, New York, ...

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ژورنال

عنوان ژورنال: BMJ

سال: 1972

ISSN: 0959-8138,1468-5833

DOI: 10.1136/bmj.3.5824.435