93

RISK FOR CIRRHOSIS AND PRIMARY LIVER CANCER IN ALPHAI-ANTITRYPSIN DEFICIENCY: A CASE-CONTROL AUTOPSY STUDY

Sten Eriksson, Joyce Carlson and Ramon Velez Department of Medicine, Malm~ General Hospital, University of Lund, S-214 01MalmU, Sweden

Several case-series have suggested an assocation between homozygous alphal-antitrypsin- deficiency and cirrhosis and malignant hepatoma. To assess the risks of thes~ complications we conducted a retrospective case-control study based on al l cases of AAT-deficiency ascer- tained during the period 1963-82 in the ci ty of Malm~, Sweden. During the study period 38,520 or 68.2% of al l deceased patients had been autopsied. From the homozygote frequency in the population, 21 of these were expected to have AAT-deficiency. 20 of the expected 21 had been diagnosed and 17 (1 child and 16 adults) had been autopsied. Each case was matched to four controls selected from the same autopsy register and the Mantel-Haenszel odds ratio (O.R.) was calculated. The results indicated a strong relationship between AAT-deficiency and cirrhosis (O.R. = 7.8, 95% C.L. 2.4-24.7) and malignant hepatoma (O.R. = 20, 95% C.L. 3.5-114.4). When st rat i f ied by sex these associations were significant only for males.

We conclude that most cases of AAT-deficiency come under medical surveillance and that adult males are at high risk for cirrhosis and malignant hepatoma. The male predominance suggests the additive effects of exogenous factors.

94

THE N-TERMINAL PROPEPTIDE OF COLLAGEN TYPE I I I IN SERUM AS A PROGNOSTIC INDICATOR IN PRIMARY BILIARY CIRRHOSIS

Sten Eriksson and Olle Zett~rvall Department of Medicine, MalmU General Hospital, University of Lund, S-214 01MalmU, Sweden

The serum level of N-terminal propeptide of collagen I I I (Col 1-3) has received in- creasing attention as a possible marker of l i ver f ibrosis. Elevated levels have been reported in patients with primary b i l ia ry cirrhosis (PBC). We measured Col 1.3 levels in 24 patients with PBC (mean age 56 + 8 years) and compared i ts value as a prognostic marker with serum bi l i rubin and IgM leTels, the aminopyrine demethylating capacity (ABT) and presence of cl inical sjnnptoms. Mean observation time was 5.1 + 2.7 years. When these parameters and age were evaluated as predictive factors for survival only bi l i rubin, Col I-3 levels and symptom status variables were found to be significant. When tested as explanatory variables for survival in a stepwise linear logist ic regression model Col 1-3 was identif ied as the strongest significant (p < 0.001) explanatory variable followed by bil irubin (p < 0.01) whereas the symptom status turned out as a non-significant variable. The results suggest that the serum level of Col 1-3 may be a useful prognostic indicator in PBC, which is independent of the bi l i rubin level.

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