Neonatal hepatitis syndrome and alpha-1-antitrypsin deficiency: an epidemiological study in South-East England

Summary A prospective epidemiological study of the Neonatal Hepatitis Syndrome in S.E. England showed Alpha-1-Antitrypsin Deficiency (Pi ZZ) to be present in seven out of fifty-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 variability, but in general the hepatitis is more severe in patients with Alpha-1-A.T. deficiency than in those in whom no aetiological factor was found.