PRIMARY BILIARY-CIRRHOSIS (PBC) IN AN EUROPEAN COUNTRY - A DESCRIPTION OF DEATH RATES IN THE NETHERLANDS (1979-1992)

Background/Aims: In 1979, separate liver transplantation (LT) and prim ary biliary cirrhosis (PBC) death rate registration became available i n The Netherlands (15 million. inhabitants). The objective of this stu dy was to investigate death rates from 1979-1992 and analyse the impac t of LT. Patients and Methods: PBC was either a primary or secondary c ause of death. Rates were expressed as absolute numbers or per million inhabitants in the corresponding age category. Age classes of 5 years were used. The Netherlands was divided in four regions, North, South, East and West. Standardized mortality ratios (SMR) were used for calc ulation of regional differences. Results: In the 14 year period betwee n 1979-1992, 417 persons died from and 179 persons died with PBC, tota ling 596 PBC patients (6.3 per million, inhabitants greater than or eq ual to 35 years). No person younger than 35 died. Eighty-two percent w ere female, with a corresponding female/male ratio of 4.2 per million females/males inhabitants. In region South there were significantly fe wer deaths (SMR 66%, p<0.001) and in region North significantly more ( SMR 141%, p<0.05). The median age class at death was 70-74 (mates and females alike). At age 35-59, death from PBC in 1992 per million was 1 .2, and for greater than or equal to 65 years 15.7. In age class 80-84 , the highest death rate from or with PBC was found with 28 deaths per million inhabitants and with a female/male ratio of 3.6. In 1992, wit h two deaths only, LT appeared to have nearly eliminated death from PB C in. the age category 35-59 years. Conclusion:Death from PBC mainly o ccurs in. the old and very old, who may never seek a specialized cente r. This indicates a more specific management and therapy for this part icular group is needed.