ASSOCIATION OF ALPHA-1-ANTITRYPSlN PHAENOTYPE MZ WITH NEONATAL LIVER DISEASE

J.Deutsch, M.G.Schimek, E.Paschke, H.Putz Department of Pediatrics, University of Craz, Austria.

An association of a lpha- l -ant i t ryps in phaenotype MZ with chronic cryptogenic l iver disease and malignant l iver tumors has already been demonstrated in adults; an association with neonatal l iver disease is stil l controversial (Moroz et al 1976, KCinzer 1977). Methods: A prospective study has been performed to test the hypothesis of an association of phaenotype MZ with neonatal l iver disease. During a period of 3 1/2 years all infants presenting with neonatal l iver disease have been investigated; the control group consisted of randomly selected sero of children without l iver disease or obstructive lung disease. Neonatal l iver disease was defined by hepatomegaly, direct hyperbil irubinaemia and increased activities of transferases in serum at two consecutive occasions; l iver biopsies were performed only when therapeutical ly indicated phaenotypes of a lpha- l -ant i t ryps in were determined by isoelectric focusing (polyacrylamidgel, LKB-mult iphor, pH 4-5, LKB Vienna). The results were compared by chi-square test. Results: 6 of 48 infants with neonatal l iver disease (= 12.5 %) and 4 of 131 controls (= 3.05 %) showed the phaenotype MZ (~L2=5.94,Ok.2crit=3.84, 0 .01<p<0.025) . Conclusion: The results indicate an association between a lpha- l -ant i t ryps in phaenotype MZ and neonatal l iver disease.

DECREASED PROLIFERATIVE ACTIVITY ASSOCIATED WITH ACTIVATION MARKERS IN PATIENTS WITH ALCOHOLIC LIVER CIRRHOSIS.

J. Devi~re~ C. Denys t L. Schand~ne t F. Romasco r M. Adler~ E. Dupont. Erasme Hospital, Free University of Brussels, Brussels, Belgium.

Peripheral blood mononuclear cells from 15 patients with alcoholic cirrhosis (AC) and 15 matched healthy controls were tested for their proliferative response to mitogens such as PHA and con A, tumour promotor PMA and OKT3 monoclonal antibody, for their capa- city to produce IL-2 and to respond to recombinant IL-2 (rlL-2). The expression of IL-2 receptor (Tac) together with two other activation markers, the receptor for trans- ferrin (T9) and la antigen have also been assessed. A profound decrease of prolifera- tive response was observed after stimulation by lectins and PMA. IL-2 production was significantly decreased (0.39~0.07 versus 0.82~0.009 units; P<0.001) in alcoholic cirrhosis. Rested PBMC of these patients disclosed spontaneous responsiveness to rlL-2 without requiring prestimulation with PHA as observed in healthy subjects. This abnormal response was associated with significantly increased percentages of Tac (19.4~3.1 versus 5.1~I.i %; P<0.001), T9 (13.6~3.3 versus 6.6~i.i %, P=0.04) and la positive cells' (21.4+5.4 versus ii.5~1.4, P<0.05) in alcoholic cirrhosis. Defective proliferative activTty and impaired IL-2 responsiveness could represent in vitro correlates for mechanisms leading to immunoregulatory disturbances in cirrhotic patients.

S121