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OBJECTIVES: Our goal was to investigate the physical and mental health of mothers who care for a child with Rett syndrome. METHODS: We assessed maternal physical and mental health by using the SF-12 version 1 physical component summary and mental component summary scores as the outcome measures of interest. Mothers (n = 135) of children with Rett syndrome completed the SF-12 measure as part of the Australian Rett Syndrome Study in 2002. The analysis investigated linear relationships between physical and mental health scores and maternal, family, and child characteristics. RESULTS: Mothers ranged in age from 21 to 60 years and their children from 3 to 27 years. Nearly half of these mothers (47.4%) indicated that they worked full-time or part-time outside the home, and 41% had a combined family (gross) income of <40,000 Australian dollars. The resultant model for physical health demonstrated that the following factors were positively associated with better maternal physical health: the mother working full-time or part-time outside the home, having some high school education, having private health insurance, the child not having breathing problems in the last 2 years, the child not having home-based structured therapy, and high scores on the Family Resource Scale (indicating adequacy of time resources for basic and family needs). The resultant model for mental health demonstrated that the following factors were positively associated with better maternal mental health: the mother working full-time or part-time outside the home, the child not having a fracture in the last 2 years, lesser reporting of facial stereotypes and involuntary facial movements, being in a well-adjusted marriage, and having low stress scores. CONCLUSIONS: Our study suggests that the most important predictors of maternal physical and emotional health are child behavior, caregiver demands, and family function. [ABSTRACT FROM AUTHOR]

The etiology of childhood cancers is largely unknown, although the early age at diagnosis has led to particular interest in in utero and perinatal factors. Birth weight is the most frequently studied perinatal factor in relation to risk of childhood cancers, and results have been inconsistent. We investigated whether the risk of CNS tumors and lymphomas in children was associated with three measures of the appropriateness of intra-uterine growth: proportion of optimal birth weight (POBW), birth length (POBL) and weight for length (POWFL). A cohort of 576,633 infants born in Western Australia in 1980-2004 were followed from birth to diagnosis of a CNS tumor (n = 183) or lymphoma (n = 84) before age 15, death, or December 31, 2005, and analyzed with Cox regression. Overall, there was little evidence of any association between fetal growth and risk of CNS tumors, although risk of ependymoma/choroid plexus tumors was positively associated with POBL and negatively associated with POWFL. The risk of Hodgkin and Burkitt lymphoma increased with increasing fetal growth among boys only, whereas the increased risk observed with non-Hodgkin lymphoma was only in girls. These associations between fetal growth and disease risk were also observed among children not classified as high birth weight, suggesting that accelerated growth is more important than birth weight per se. Results were similar when cases were compared with their unaffected siblings, suggesting that the increased growth associated with cancer risk was not general to the family. The associations we observed are consistent with causal pathways involving fetal growth factors., ((c) 2008 Wiley-Liss, Inc.)

Using population-based linked health data, we investigated whether the risk of certain childhood non-CNS solid tumours (n=186) was associated with intra-uterine growth. The risk of retinoblastoma and rhabdomyosarcoma, but not other tumour types, was positively associated with increased growth, suggesting a possible role of fetal growth factors. Larger studies are needed.

Increases in the incidence of childhood acute lymphoblastic leukemia (ALL) have been reported in some countries, while other reports from similar geographical regions have indicated stable rates. The reasons for the discrepancies have been debated in the literature, with the focus on whether the observed increases are "real" or an artifact resulting from improvements in diagnosis, case ascertainment and population coverage over time. We used population-based data from Western Australia to investigate trends in the incidence of childhood ALL between 1960 and 2006. Age-standardized incidence rates (ASRs) and rate ratios (indicating annual percent change) were estimated using Poisson regression. Between 1960 and 2006, the ASR was 3.7 per 100,000 person-years, with an annual percent increase of 0.40% (95% CI: -0.20, 1.00). Between 1982 and 2006, the ASR was 3.8, with an annual percent increase of 0.80% (95% CI = -0.70 to 2.30). This increased to 1.42% (95% CI: -0.30, 3.0) when a sensitivity analysis was undertaken to assess the effect of excluding the final 2 years of data. Annual increases of 3.7% (95% CI: -0.50, 8.00) among children aged 5-14 years, and of 3.10% (95% CI: 0.50, 5.70) in girls, were observed for this latter period. These results were supported by national Australian incidence data available for 1982-2003. There may have been a small increase in the incidence of ALL since 1982 among girls and older children, but an overall increase appears unlikely. No impact of folate supplementation or fortification is apparent., ((c) 2007 Wiley-Liss, Inc.)

This paper describes the development of a video-based evaluation tool for use in Rett syndrome (RTT). Components include a parent-report checklist, and video filming and coding protocols that contain items on eating, drinking, communication, hand function and movements, personal care and mobility. Ninety-seven of the 169 families who initially agreed to participate returned a videotape within 8 months of the first request. Subjects whose videos were returned had a similar age profile to those who did not provide a video but were more likely to have classical than atypical RTT. Evidence of the content and social validity and inter-rater reliability on 11 videos is provided. Video may provide detailed, objective assessment of function and behaviour in RTT.

The authors examined the relation between birth weight, intrauterine growth, and risk of childhood leukemia using population-based linked health data from Western Australia. A cohort of 576,593 infants born in 1980-2004 were followed from birth to diagnosis of acute lymphoblastic leukemia (ALL) (n = 243) or acute myeloid leukemia (AML) (n = 36) before their 15th birthday, death, or the end of follow-up (December 31, 2005). Data were analyzed using Cox regression. Risk of ALL was positively associated with the proportion of optimal birth weight--a measure of the appropriateness of fetal growth--particularly among children younger than 5 years; the hazard ratio for a 1-standard-deviation increase in proportion of optimal birth weight was 1.25 (95% confidence interval: 1.07, 1.47). Among children younger than 5 years not classified as having high birth weight (defined as >3,500 g, >3,800 g, and >4,000 g), a 1-unit increase in proportion of optimal birth weight was associated with an approximately 40% increase in ALL risk. This suggests that accelerated growth, rather than high birth weight per se, is involved in the etiology of ALL. These findings are consistent with a role for insulin-like growth factor I in the causal pathway. Findings for AML were inconclusive, probably because of small numbers.

Objective: To examine the prevalence, cumulative incidence, and survival in an Australian cohort with Rett syndrome (RTT)., Study Design: The Australian Rett Syndrome Database is a longitudinal data collection that included 276 verified female cases at the end of 2004. Survival was calculated using the Kaplan-Meier product limit method, and cumulative incidence was determined using the complement of the Kaplan-Meier method., Results: Most cases (88.4%) have had MECP2 mutation testing, with positive results in 73%. The prevalence of RTT was .88 per 10,000 females in 5- to 18-year-olds, and the cumulative incidence was 1.09 per 10,000 females by 12 years of age. The cumulative incidence by the age of 5 years increased from .39 per 10,000 in the 1980 to 1984 cohort to .76 per 10,000 in birth cohorts beyond 1984. Survival was 77.8% at 25 years, compared with 99.96% survival in the Australian female population. Pneumonia (10/25) was the most common cause of death., Conclusions: The availability of genetic testing has contributed to the changing pattern and timing of RTT diagnosis in Australia. Girls with RTT have worse survival compared with the general female population. When more data are available, it will be possible to evaluate the relationship between survival and specific MECP2 mutations.

Background: Previous studies reported an increase in the rates of operation following the publication of major trials that demonstrated the benefit of carotid endarterectomy in reducing stroke. The aim of this study was to determine whether carotid endarterectomy rates have continued to rise despite the reducing trend in most manifestations of atherosclerotic cardiovascular disease., Methods: Record linkage was used to select patients who had a carotid endarterectomy during the interval from 1988 to 2001. Incidence rates were age-standardized and trends were examined with Poisson regression., Results: The rate increased by 13.8 per cent per year between 1988 and 1998; however, from 1999 onwards the rate of carotid surgery fell by 15.8 per cent per year. In octogenarians, the rate increased steadily from 0.9 to 5.1 per 100,000 person-years between 1992 and 2000. The proportion of octogenarians also increased significantly from 0.9 per cent in 1988-1990 to 19.5 per cent in 2000-2001 (chi2=60.11, 4 d.f., P<0.001)., Conclusion: For the first time a recent decline has been observed in the rate of carotid endarterectomy, most likely owing to a combination of the deceasing incidence of atherosclerosis and more widespread use of effective drugs in the treatment of cardiovascular disease. The rate and proportion of operations in patients aged 80 years or older has increased steadily., (Copyright 2003 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd.)

Objectives: To investigate the trends in incidence and mortality and estimate survival for women diagnosed with ovarian cancer in Western Australia. CASE SELECTION AND METHODS: There were 1,336 women diagnosed with ovarian cancer in 1982-98. Age-standardised rates were calculated by the direct method. Age-period and age-cohort models were analysed by Poisson regression. The Kaplan-Meier method was used to estimate survival and Cox proportional hazards regression evaluated the relative risk of dying., Results: Trends in age-adjusted incidence and mortality rates showed little changed over the three time periods of diagnosis. A significant birth cohort effect showed a peak in the risk in the 1924 (mid-year) cohort followed by a general decrease in both incidence and mortality risk. Survival at five years was 34% (95% CI 31.3-36.5) overall, but was only 27% (95% CI 17.4-36.7) among women with stage III and IV disease. Aboriginal women showed a risk of dying twice that of non-Aboriginal women., Conclusions: The birth cohort analysis of ovarian cancer proved better at explaining disease trends than was time period of diagnosis. Survival continues to be poor, but Aboriginal women and those with serous and unspecified adenocarcinoma tumours fair much worse., Implications: As the leading cause of death from a gynaecological malignancy, ovarian cancer is of public health importance. Historical trends in birth rates and the use of oral contraceptives help to explain at least some of the observed birth cohort trends in this study. In the long term, an effective diagnostic technique needs to be developed or this disease will continue to be diagnosed at an advanced stage when treatment options for cure are limited.

Background and Purpose: Octogenarians were not included in the major trials of carotid endarterectomy. Concern has been expressed about the role of carotid endarterectomy in this age group. This concern is based in part on uncertainty about the long-term survival of elderly patients after carotid endarterectomy. The aim of the present study was to assess relative survival in those patients >or=80 years of age undergoing carotid endarterectomy., Methods: A population-based record linkage study of all patients who underwent carotid endarterectomy from 1988 to 1998 in Western Australia was undertaken. Long-term relative survival after carotid endarterectomy was assessed against age- and sex-matched controls., Results: During the period 1988 to 1998, 1796 (1306 male, 490 female) cases were identified. There were 151 patients >or=80 years of age. The cumulative survival at 5 years was 64.9% for those >or=80 years of age compared with 80.1% for those <80 years of age. Relative survival at 5 years was 118% (95% CI, 102 to 134) for those >or=80 years of age compared with 94.7% (95% CI, 92 to 97) for those <80 years of age., Conclusions: Long-term relative survival after carotid endarterectomy in patients >or=80 years of age was better than that of an age-matched population. The likelihood of living long enough to gain benefit from a carotid endarterectomy is not jeopardized by being too old.

Objectives: We aimed to review the utilisation and trends in surgical procedures for the primary management of ovarian cancer and the survival outcomes of patients surgically treated in Western Australia., Methods: The population-based Western Australia Data Linkage System was used to link hospital morbidity and mortality data for all women diagnosed with malignant primary ovarian cancer in the State Cancer Registry in the period 1982-1998. Poisson regression was used to analyse trends in surgical procedure rates. Logistic regression examined the likelihood of having a surgical procedure in the periods 1988-1993 and 1994-1998 compared with 1982-1987. Relative survival was used to adjust survival estimates for other causes of death occurring in the general female population., Results: There were 1,126 women who underwent a primary surgical procedure for ovarian cancer in Western Australia in the period 1982-1998. Women were more likely to undergo surgery in 1994-1998 (87.8%) compared with 1988-1993 (76.8%), but there was no difference when compared to 1982-1987 (89.2%) (P = 0.62). The likelihood of using specific surgical procedures to treat ovarian cancer increased for all but total abdominal hysterectomy. Bilateral salpingo-oophorectomy was 3.7 times more likely to be performed and omentectomy 5 times more likely to be performed in 1994-1998 compared with 1982-1987. The median length of hospital stay decreased from 15 to 12 days and emergency admissions decreased from 26.5 to 15.4% over the three time periods. Thirty-two percent of women were readmitted within 30 days of separation from their primary surgery, 23% of which were for the same-day treatment with either chemotherapy or radiotherapy. A 15% increase in relative survival was observed between the periods 1982-1997 (38.8%) and 1994-1998 (53.5%).Conclusion., Conclusion: Surgery remains a cornerstone in the primary management of ovarian cancer. There have been dramatic shifts in surgical practice in Western Australia, with more women undergoing certain surgical procedures today than they were 20 years ago. Coupling the increasing surgical trends are improved outcomes. Fewer women are presenting as an emergency, the length of hospital stay has been reduced, and survival outcomes have shown a significant improvement.