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Four female members of the same family suffering from a rare combination of oesophageal leiomyomatosis and an Alport-like nephropathy are described. The disease is characterized by marked thickening of the oesophageal wall, usually also involving the proximal stomach, with or without discernible leiomyomatous nodule formation. All cases were treated surgically by oesophagectomy with symptomatic relief, and there was no evidence of recurrence on follow-up (2-37 years). The syndrome appears to be dominantly inherited, affects children and young adults, and may also be associated with leiomyomatosis of other viscera. Previously reported cases and possible aetiologies are reviewed, and evidence that this association represents a new variant of Alport's syndrome is discussed.

Background: Although most patients with hereditary haemochromatosis have HFE C282Y mutations, the lifetime risk to HFE C282Y homozygotes of developing fatal diseases such as hepatocellular carcinoma is uncertain. We have carried out a cross-sectional study to determine the proportion of diagnosed hepatocellular carcinoma patients who are homozygous for the HFE C282Y mutation; and to estimate the penetrance of this genotype with respect to hepatocellular carcinoma in East Anglia.

Table 1 Number of cases of cervical cancer in East AngliaCervical screening started in the East Anglian Region in the 1960s, but the programme lacked any central organisation and uptake was extremely variable. Since 1988, when the national programme was reorganised, the family health service authorities have been responsible for call and recall. This change led to a dramatic improvement in coverage, and now 80% of the eligible population participate in the programme. The East Anglian Region contains eight laboratories, and women aged 20-65 are screened at three or five yearly intervals. In East Anglia in 1994 there were nearly 620 000 women in the screening age range. MethodsIncidence data from 1971-94 were obtained from the East Anglian Cancer Registry for cases of invasive squamous cell carcinoma and adenocarcinoma of the uterine cervix. Cases of microinvasive squamous carcinoma (FIGO stages Ia 1 and Ia 2 ) were included. There are no established diagnostic criteria for microinvasive adenocarcinoma and so all stages of invasion were included. Cases of adenosquamous carcinoma were excluded.Similar incidence data from 1971-89 were obtained for England and Wales. The data were taken from the EUROCIM database of the European Network of Cancer Registries," who had obtained the information from the Office of Population Censuses and Surveys.Incidence rates, directly age standardised using the European standard population, were calculated for age bands 0-29, 30-39, 40-59, and 60+ years, over the whole study time period. Age specific incidence rates were calculated to compare patients diagnosed in 1971-76, 1977-82, 1983-88, and 1989-94. The results for the East Anglian Region were compared with those for England and Wales.The main purpose of this study was to determine whether there was an increased incidence of cervical adenocarcinoma in East Anglia. We did not consider mortality data or tumour stage at presentation.Trends in incidence were tested using Poisson (log-linear) regression, and the level of significance recorded. AbstractObjective-To determine trends in incidence of invasive adenocarcinoma of the uterine cervix in East Anglia. Methods--eervical cancer incidence data for both squamous cell carcinomas and adenocarcinomas were obtained from the East Anglian Cancer Registry for the period 1971-94. Similar data were obtained for England and Wales. European age standardised rates (ASRs) were used for comparisons. Results-The mean incidence (ASR) of cervical adenocarcinoma was 0.85 per lOS in 1971-76, rising to 2.54 per lOSin 1989-94. There has been a marked age shift, with the main increase in incidence occurring in younger women aged 30-39. The mean incidence (ASR) of squamous cell carcinoma of the cervix has decreased from 9.78 to 8.74 per lOS over the periods 1971-76 and 1989-94. Again there has been an age shift, moving from a single incidence peak in the 45-59 age band in earlier years to incidence peaks in both the 30-39 and 55-69 age bands in more recent years. Similar trends were noted when data for Eng...