PEDIATRICS Vol. 46 No. 6 December 1970, pp. 921-925
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Deficiency of agr1-Antitrypsin in Childhood Liver Disease

A. Myron Johnson M.D.1 and Chester A. Alper M.D.1

1 Blood Grouping Laboratory, Department of Medicine, Children's Hospital Medical Center Department of Pediatrics Harvard Medical School, 332 Longwood Avenue Boston, Massachusetts 02115

Visual examination of serum electrophoretic patterns and determination of total antitryptic activity using BAPNA as substrate are efficient screening techniques for deficiency of agr1-antitrypsin in patients with neonatal hepatitis, childhood cirrhosis, or pulmonary emphysema. However, the finding of a few infants and children with deficient levels but normal allotypes suggests that definitive diagnosis of the genetic disorder associated with qualitatively and quantitatively abnormal agr1-antitrypsin requries genetic typing and, when possible, confirmation by evaluation of the parents. The rather ominous outcome in those children so far diagnosed as having liver disease associated with this deficiency makes unequivocal diagnosis imperative for both prognostic and genetic counselling.




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