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 primary biliary cirrhosis (PBC) death rate registration became available in The Netherlands (15 million inhabitants). The objective of this study was to investigate death rates from 1979-1992 and analyse the impact of LT. PATIENTS AND METHODS: PBC was either a primary or secondary cause 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 calculation of regional differences.
RESULTS: In the 14 year period between 1979-1992, 417 persons died from and 179 persons died with PBC, totaling 596 PBC patients (6.3 per million inhabitants > or = 35 years). No person younger than 35 died. Eighty-two percent were female, with a corresponding female/male ratio of 4.2 per million females/males inhabitants. In region South there were significantly fewer 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 (males and females alike). At age 35-59, death from PBC in 1992 per million was 1.2, and for > or = 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, with two deaths only, LT appeared to have nearly eliminated death from PBC in the age category 35-59 years.
CONCLUSION: Death from PBC mainly occurs in the old and very old, who may never seek a specialized center. This indicates a more specific management and therapy for this particular group is needed.