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A group of 46 bipolar probands and their first degree relatives were studied. A high rate of affective disorder (19.6 per cent) was found, including both unipolar (13.2 percent) and bipolar (6.4 per cent) types, with females predominating (3 : 1). The presence of four fatherson pairs suffering from affective disorder made the hypothesis of X-linked dominance untenable. Results compatible with polygenic inheritance were found, using both Slater's and Falconer's methods. There was no evidence for assortative mating or for increased total number of females (both well and ill) among first degree relatives. The probands and affectively ill first degree relatives who have died show an alarmingly high rate of suicide (46 per cent). Other forms of mental disorder, including alcoholism, were no more common than in the rest of the community. [ABSTRACT FROM AUTHOR]

This systematic review assessed the current evidence base of substance use prevention programs for Indigenous adolescents in the USA, Canada, Australia and New Zealand. The authors investigated (a) the outcomes, type, setting and context of prevention programs; (b) the common components of beneficial prevention programs; and (c) the methodological quality of evaluations of included prevention programs. The authors searched eight peer-reviewed and 20 grey literature databases for studies published between 1 January 1990 and 31 August 2017. Data extracted included type of program (culturally adapted, culture-based or unadapted), the setting (school, community, family or multi-setting), delivery (computerised or traditional), context (Indigenous-specific or multi-cultural environment) and common components of the programs. Program evaluation methodologies were critically appraised against standardised criteria. This review identified 26 eligible studies. Substance use prevention programs for Indigenous youth led to reductions in substance use frequency and intention to use; improvements in substance-related knowledge, attitudes and resistance strategies; and delay in substance use initiation. Key elements of beneficial programs included substance use education, skills development, cultural knowledge enhancement and community involvement in program development. Five programs were rated as methodologically strong, seven were moderate and fourteen were weak. Prevention programs have the potential to reduce substance use among Indigenous adolescents, especially when they are developed in partnership with Indigenous people. However, more rigorously conducted evaluation trials are required to strengthen the evidence base.

The role of major gene and multifactorial inheritance in the aetiology of club foot in the New Zealand Polynesian population was studied using 287 New Zealand Maori and Pacific club foot families. The club foot family data were analysed by complex segregation analysis under the mixed model using the computer program POINTER. This analysis shows that the best genetic model for club foot in this population is a single dominant gene with a penetrance of 33% and a predicted gene frequency of 0.9%. These data provide a scientific foundation for molecular studies in the Maori and Polynesian population to identify putative club foot genes.

Aim: To develop a DNA-based diagnostic test for adrenoleukodystrophy (ALD) in a large New Zealand family., Methods: Mutation screening of the X chromosome-linked ALD gene was undertaken by direct sequencing of PCR amplified products encompassing defined exons of the ALD gene. The identification of a mutation led to the development of a simple restriction enzyme digestion protocol of a PCR amplified product to identify those individuals with the mutation., Results: A nonsense mutation, resulting in deduced premature termination of translation of the ALD gene product, was detected in exon 4 of the ALD gene in an affected male. This mutation was found in three obligate gene carriers in the same ALD family. A DNA-based test was established to identify this mutation by Bgl II digestion of a PCR amplified product encompassing exons 3 and 4 of the ALD gene. The DNA-based test was applied to a chorionic villus sampling for prenatal diagnosis., Conclusions: A simple DNA-based test has been developed for ALD in a large New Zealand family. This test provides a rapid means of determining carrier status and for undertaking prenatal diagnosis for ALD in this family.

The incidence of facial clefts among live births in the Auckland urban area has been studied for the years 1960-1976. After correction for ascertainment, the live-born incidence of non-syndromal cleft palate among Maoris was 1.867/1000, and among Europeans it was 0.643/1000. The incidences for non-syndromal cleft lip, with or without cleft palate, were 0.397/1000 for Maoris, and 1.195/1000 for Europeans. The sex ratio for cleft palate showed the usual female preponderance, although there was evidence of heterogeneity between the races. Cleft lip occurred more commonly among males. Birth rank, maternal age, and paternal age had no apparent effect on the incidences, and there were no significant secular or seasonal changes in incidence.

The inheritance of A-V conduction time in Polynesians has been examined, using ECGs taken from the population of the Tokelau Islands. As has been demonstrated in other racial groups, A-V conduction time was influenced by sex, age, and heart rate. Complex segregation analysis provides evidence for a significant polygenic influence on A-V conduction, but no evidence for a major gene. Familial correlations and commingling analysis are in agreement with this conclusion. The heritability of A-V conduction time was 0.46. The hypothesis of Møller & Heiberg (1980a) that a major gene could be influencing A-V conduction, is not supported by these data.

Although the aetiology of anencephalus is unknown, changes in prevalence over time are thought to provide strong evidence of the effect of environmental factors in causation. In a number of countries the rate of this neural tube defect is declining. The rate in New Zealand has also fallen significantly (p less than 0.001) from 1.17/1000 total births in 1968 to 0.39/1000 in 1983, with the greatest decrease occurring in the female rate (approximately 4.16% per year). The aetiological implications of these findings and their consequences for programmes of prenatal diagnostic testing and maternal screening are discussed.

Data validity is a fundamental problem in epidemiology. An objective of birth defect registers is the collection of high quality data, often from a number of sources, for use in epidemiological research. This paper examines the use of data from a national register in New Zealand in the study of congenital heart defects. The prevalence rates of congenital heart defects are shown to depend on the definition of the terms employed, the methods and completeness of the case ascertainment, the correctness of the diagnoses, the nature and size of the population under study, and the duration of followup. Recommendations are made for increasing the utility of this register in the study of this major group of malformations.

Results from a dynamic computer model of rubella vaccination programmes indicate that consideration should be given to vaccinating all one-year-old girls and boys and revaccinating all girls at about 11 years of age, as well as continuing with the programme for susceptible women in the childbearing age group. With vaccine-induced immunity decaying at about 1% annually, the vaccination of 80 to 95% of all one-year-olds, 95% of 11 year old girls, and 5% of women aged 15 to 33 annually is expected to reduce congenital rubella syndrome deformities to less than 5% of the 1980 incidence by 1994, and to negligible levels thereafter. In comparison, continuation of the present scheme may reduce deformities to only 69% of 1980 levels by 1994 with a slow decline to 25% in 2010. (The 1980 levels used were computer generated to eliminate short-term fluctuations, and do not apply to actual figures from that year.) For convenience and better compliance, measles vaccine and the initial rubella vaccine may be given in combined form at 15 months without altering the effect of either. The rate of decay of immunity after vaccination is critically important in congenital rubella syndrome prediction, so that further accurate monitoring of immune status and congenital rubella incidence is essential.

194 cord blood samples were studied in both a fluorimetric assay and an electrophoresis method for Gal-1-PUT. From this the expected live birth incidence was 1:37000. The New Zealand neonatal screening programme has detected 5 cases in 223326 live births--an apparent incidence of 1:44600. The Beutler testing of dried blood spots collected on filter paper cards is a satisfactory method of detecting galactosaemia in the neonate.

A survey was conducted of professional New Zealand 2, 4, 5-T sprayers and a comparison group of agricultural contractors with a total of 989 respondents. The numbers of births, congenital defects, and miscarriages were identified from 1969 to 1980 by a postal questionnaire. Each pregnancy outcome was classified according to whether or not the father sprayed 2,4,5-T during the year of the pregnancy outcome, or the previous year. The relative risk estimates of 1.19 for congenital defects, and 0.89 for miscarriages, were not statistically significant. These results are reassuring as far as male professional 2,4,5-T sprayers are concerned. In addition, the extent of exposure of their wives from helping with spray activities, and from washing contaminated clothes, has not had a detectable reproductive effect.