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The child‐health nurse screening programme for the presence of congenital dislocation of the hip in Western Australia was evaluated by the use of data that were collected as a routine from the Congenital Malformations Registry, the records of child‐health nurses and the Hospital Morbidity System. The programme performed well operationally, with a sensitivity of 68.5%, a specificity of 98.8%, and a positive predictive value of 15.1%. Of the children who were born in the study period (1981‐1983), 450 children were diagnosed with congenital dislocation of the hip, and 46 (10.2%) of these children required 104 hospital admissions for the treatment of their dislocation. Of the children whose hips were found to be dislocated on screening (those who were screened as “true‐positive” cases), 10.1% were hospitalized, whereas 16.1% of those children who were missed by screening (who screened as “false negative”) required hospitalization (95% confidence interval [CI] for the difference in proportions, ‐7.8% to 19.9%). A comparison of the measures of hospitalization was made between the children who were found to be “true positive” on screening and the children whose dislocation was diagnosed in the hospitals of their birth — a group that generally is considered to show the best outcome. A somewhat‐higher proportion of children who were screened as “true positive” required an open surgical procedure (5.0% of children compared with 2.8% of children; 95% CI for the difference, ‐2.1% to 6.5%). The differences in the other measures of hospitalization between the two groups were small.

ABSTRACT The Western Australian Familial Polyposis Registry was established in 1985 by the Cancer Foundation of Western Australia in conjunction with the Health Department of Western Australia. The primary aim of the Registry is the prevention of colorectal cancer in a group of persons at high risk of the disease. Families are notified to the Registry by medical practitioners throughout the State and, at the request of family members and medical practitioners, the project officer for the Registry will arrange for the collection of data, counselling and reminders for follow‐up examinations for both cases and family members who are at risk of developing the disease. A total of 44 families with familial adenomatous polyposis is known to the Registry. The crude incidence of polyposis in Western Australia was 0.24 cases per 100 000 persons over the three‐year period 1986–1988. The prevalence of the condition in 1988 was 2.59 cases per 100 000 persons. There are 188 family members in Western Australia who are at a 50% risk of developing familial adenomatous polyposis. Registry data indicate that regular follow‐up and early treatment reduce the risk of the development of bowel cancer.

ABSTRACT A comparison of congenital malformations in Aboriginal and non‐Aboriginal children in Western Australia has been made by means of data from the WA Congenital Malformations Registry, for births from 1980 to 1987 inclusive. Although the birth prevalence of all malformations was 3.5% for both Aboriginal and non‐Aboriginal infants, significant differences were found in the birth prevalence of some individual malformations. Nervous‐system and cardiovascular defects, and cleft lip and palate were significantly more prevalent in Aborigines, and pyloric stenosis and urogenital defects were significantly less prevalent. While some of the observed differences may have been a result of biases in ascertainment, others are likely to represent true differences. A discussion of Aboriginal beliefs concerning conception and birth is included, in order that the quantitative findings from the study might be considered in an appropriate cultural setting.

ABSTRACT A population‐based case‐control study was conducted to test the hypothesis that the risk of the occurrence of neural‐tube defects in infants with no other birth defects (isolated neural‐tube defects) is associated inversely with the maternal dietary intake of free and/or total folate in early pregnancy. Information was collected from the mothers of 77 case subjects with isolated neural‐tube defects who were born in Western Australia from 1982 to 1984, from the mothers of 77 control subjects with birth defects other than neural‐tube defects (control group 1) and from the mothers of 154 control subjects with no birth defects (control group 2). The case and control subjects were matched individually by the date of the mother's last menstrual period. Odds ratios were adjusted for a number of potentially‐confounding variables, such as the country of birth of the parents, paternal social class, previous pregnancy outcome, interval between index and previous pregnancy and pregnancy order. Crude and adjusted odds ratios showed a protective effect of an increasing intake of free folate in the first six weeks of pregnancy. Adjusted odds ratios, with reference to the lowest quartile of intake, (and their 95% confidence intervals) were 0.72 (0.25‐2.08), 0.37 (0.11‐1.23) and 0.31 (0.10‐0.97) for quartiles 2‐4 when control group 1 was used, and 0.44 (0.17‐1.13), 0.34 (0.13‐0.90) and 0.16 (0.06‐0.49) when control group 2 was used. Similar, but weaker, trends were seen when total folate intake was the exposure variable. These findings support the hypothesis that the dietary intake of folate in early pregnancy protects against the occurrence of isolated neural‐tube defects in infants. Measures of postpartum dietary folate and of postpartum serum and red‐cell folate levels showed no association with the occurrence of neural‐tube defects in infants. (Med J Aust 1989; 150: 613‐619)