In a population based study of the prevalence of Wilson's disease in the Republic of Ireland from 1970-89, 26 definite and probable cases were ascertained. The adjusted birth incidence rate was 17-0 per million live births (95% (confidence interval 9*9 to 27.2)) for the 20 year period 1950-69. The gene frequency was 0-41% (95% (confidence interval 0-31% to 0-52%) indicating that one in 122 of the population was a gene carrier. Allowing for a maximal degree of consanguinity, the gene frequency is reduced to 0-36% and the proportion of heterozygotes to one in 139 of the population. The consistency of the adjusted birth incidence rates over the decades 1950-59 and 1960-69 suggests a high degree of ascertainment of diagnosed cases of Wilson's disease.
(-4%) for inshore fisherman, and an increase (+2%) for both combined. For deep sea fishermen, there were synchronous increases in mortality from vessel losses (+32%) and from personal accidents (+ 49%, p < 0-05) whereas the overall rate for inshore fishermen reflected a decrease (-33%, p < 0 01) in deaths from personal accidents but an increase (+ 52%) in deaths from vessel losses. Although pre-CITS mortality rates exhibited no statistically significant difference between deep sea and inshore fishermen, the post-CITS accident mortality rate for deep sea fishermen was significantly greater (p < 0-02). Compared with coal miners, fishermen were, on average, four times more likely to die from accidents at work. If CITS had reasonable cause for concern in 1969, the grounds for similar concern did not diminish because the evidence suggests that deep sea fishermen were at no lesser risk of death from accidents after CITS reported than before. Overall, both groups of fishermen experienced an accident mortality rate between 1961 and 1980 that showed no indication of improvement.The mortality from occupational accidents to United Kingdom fishermen was suspected by Tunstall of being higher than that of other occupations more than 20 years ago,' although it was only in 1965, when Schilling described trawler fishing as an "extreme occupation,"2 a description also used by Tunstall, that the nature of specific hazards appeared in partial focus. It was not until 1969, however, after publication of the final report of the Committee of Inquiry into Trawler Safety (CITS),3 that the extent of the excess mortality experience of deep sea fishermen was finally confirmed.4Schilling's contention that trawler fishing was an extreme occupation stemmed almost inadvertently from his personal exposure to actual working condi-
The trace element content of kidneys has been documented in very few international centres. Significant differences can be demonstrated between the cortical and medullary levels of calcium, zinc and cadmium. For the West of Scotland the values for the latter are similar to those found in Scandinavia. There are significantly higher cadmium contents in subjects who smoke and who have evidence of ischaemic heart disease.
Systematic studies of 618 fishing vessel losses between January 1961 and December 1982 have shown a significantly increased incidence of total losses from 1972 onwards. Comparison of mean pre-and post-1972 rates indicated an increase of 120% from 2 54 to 5 58 losses per 1000 vessels at risk. Parallel examination of the mortality of fishermen from all occupational accidents showed no improvement. For deaths arising from vessel losses alone, the mean crude death rate, for the same periods, increased by 13%. Despite these findings, formal investigations (courts of inquiry) into fishing losses, ordered by the Department of Transport according to specified criteria, decreased in number from about 1975 onwards. The mean number fell by 61% from 3 1 (1961-71) to 1 2 a year . Furthermore, the reduction in the rate of formal investigations into fishing vessel losses (-84%; p < 0-0001) was greater than the corresponding reduction (-61%; p < 0-001) in United Kingdom merchant vessel investigations and this difference was statistically significant (p < 0-01). During 1972-82 there were 61 fewer fishing investigations than predicted from the rate at which these were ordered during 1961-71. The last took place in 1979. None of the 141 fishing vessel losses between January 1980 and December 1982 proceeded to formal investigation. The possibility that the criteria for ordering formal investigations have not been fulfilled by the circumstances of fishing vessel losses from 1975 onwards is assessed as one explanation for this paradoxical finding of an inverse relation between losses and investigations. The accident investigation and public reporting procedures for aviation accidents should be adopted for marine accidents in general and fishing vessel losses in particular.
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