Muckle-Wells syndrome: a treatable cause of congenital sensorineural hearing loss.

Muckle-Wells syndrome (MWS) is a rare autosomal dominant condition with variable expression. It is a subset of auto-inflammatory diseases characterised by recurrent inflammatory crises and is associated with chronic recurrent urticaria, sensorineural deafness, periodic arthritis and secondary amyloidosis. The diagnosis of MWS is a clinical one with sufferers classically presenting in childhood with a moderate fever and non-pruiginous urticaria. We describe a case of a six-year-old girl who was successfully diagnosed and treated with Anakinra. Muckle and Wells originally described this syndrome in 1962; however, only recently was it discovered to be genetically linked to chromosome 1q44 and subsequently to missense mutations in the CIAS1/NALP3/PYPAF1 gene. Since then, treatment has evolved and it remains one of few treatable causes of congenital profound sensorineural hearing loss.

Protective efficacy of ellagic acid on glycoproteins, hematological parameters, biochemical changes, and electrolytes in myocardial infarcted rats.

The cardioprotective property of ellagic acid in rats has been reported previously. The present study reveals the protective role of ellagic acid in biochemical parameters including serum iron, plasma iron binding capacity, uric acid, glycoprotein, and electrolytes along with hematological parameters. Rats were subcutaneously injected with isoproterenol (ISO) (100 mg/kg) for 2 days to induce myocardial infarction. ISO-induced rats showed a significant increase in their levels of serum iron, serum uric acid, and blood glucose, and a significant decrease in their levels of plasma iron binding capacity, serum total protein, albumin/globulin ratio, and heart glycogen, when compared with normal control rats. The altered hematological parameters were also observed in ISO-induced rats when compared with normal control rats. Pretreatment with ellagic acid at doses of 7.5 and 15 mg/kg produced significant beneficial effect by returning all the above-mentioned biochemical and hematological parameters to near normal levels.

Transfer from residential aged care to emergency departments: an analysis of patient outcomes.

BACKGROUND: In order to design optimal systems to meet the acute healthcare needs of the frail elderly living in residential care, good clinical information is essential. The aims of this study were to analyse the casemix and outcomes of patients transferred from residential aged care facilities to public hospital emergency departments in New South Wales. METHODS: Individual patient data from six hospital emergency departments and inpatient wards were obtained from merged databases and analysed using descriptive and comparative statistics. RESULTS: Outcomes in 4680 patient transfers over a 12-month period in 2006-2007 were analysed. Transfers occur mostly in high-acuity patients, with approximately three of every four transfers admitted; one in every 12 dying; and admitted patients undergoing an average of 2.4 interventions or procedures during each hospital stay. Several variables are associated with prolonged length of emergency department stay including triage urgency, type of hospital and transfers occurring in winter or out of hours. CONCLUSIONS: Patients transferred from aged care facilities to emergency departments are predominantly high-acuity patients with a substantial likelihood of hospitalisation, intervention and death. Nevertheless, scope exists for some episodes of acute care, in both discharged and admitted patients, to be provided outside a hospital setting.

A comparison of doctors', parents' and children's reports of health states and health-related quality of life in children with chronic conditions.

BACKGROUND: Health-related quality of life is an important outcome. Self-report is the gold standard, but in the paediatric setting we often rely on proxy reporting. Our understanding of the differences between self- and proxy reports and the factors that influence them is limited. These differences can impact on treatment choices and the patient-doctor relationship. OBJECTIVE: To evaluate differences between children's, parents' and doctors' perceptions of health states and health-related quality of life in children with chronic illness and explore factors which explain these differences. METHODS: Consecutive families attending eligible clinics at a tertiary paediatric centre were invited to complete the Health Utilities Index (HUI) 23 questionnaire. Percentage agreement and kappas were calculated as a measure of the agreement between pairs. Chi-squared tests or Fisher's exact test, if appropriate, were performed to determine if there was an association between level of agreement and participant variables. RESULTS: Data were collected for 130 parent-doctor pairs, 59 child-parent pairs and 59 child-doctor pairs. Overall health-related quality of life scores did not differ between responders, but there was poorer agreement for subjective domains. Doctor-child agreement was lower than parent-child agreement. Children with a diagnosis of cerebral palsy or chronic neurological condition were more likely to have lower inter-rater agreement for both subjective and objective domains. On the HUI2, agreement was lower for parent-child pairs when the father was the respondent. For child-doctor pairs, an increased frequency of patient-doctor visits and doctors' seniority were predictors of poorer agreement on the HUI3 and HUI2 respectively. CONCLUSIONS: We identified factors associated with level of agreement for self- and proxy reporting on the HUI23. Parent-child agreement was higher than doctor-child agreement. Patients with significant pain or emotional distress and patients with a diagnosis of severe cerebral palsy or chronic neurological conditions were more susceptible to under-reporting of subjective aspects of well-being by doctors and parents and may benefit from formal assessment of health-related quality of life in the clinical setting.

Increasing adherence with the use of hip protectors for older people living in the community.

UNLABELLED: For people at high risk of hip fracture living in community settings, providing hip protectors at no cost increased adherence, but the additional effect of an educational programme was limited. Overall, the level of adherence was modest. INTRODUCTION: The objective of the study was to increase adherence with hip protector use by older people at high risk of hip fracture. The study included two randomised controlled trials with 308 older people recruited from three hospital rehabilitation wards and 171 older people recruited from the community. METHODS: Participants were randomised into three groups. The control group received a brochure about hip protectors. The no cost group were fitted with free hip protectors and asked to use them. The combined group received free hip protectors and education sessions about their use. Adherence with the use of hip protectors at 3 and 6 months after recruitment was measured. Secondary outcomes were falls, fractures and hospitalisations. RESULTS: Very few participants in the two control groups bought a hip protector. Overall adherence in the four intervention groups was modest, but higher in the community recruitment setting (49%) than in the hospital recruitment setting (36%) at 6 months. In the community recruitment group, at 3 months of follow-up, a significantly higher number of participants in the combined group (62%) were wearing hip protectors compared to the no cost group (43%, p=0.04). Five hip fractures occurred during the study, with four sustained whilst not wearing the hip protectors. CONCLUSION: Providing hip protectors at no cost to community living older people at high risk of hip fractures modestly increases initial acceptance and adherence with hip protector use. Additional education may further increase hip protector use in people living in the community in the short term.

The benefits of using a mixed methods approach--quantitative with qualitative--to identify client satisfaction and unmet needs in an HIV healthcare centre.

Research into patient satisfaction with HIV ambulatory healthcare facilities is limited due to confidentiality restraints, the short history of the condition and the stigma attached. Furthermore, most satisfaction studies of this client group have not used mixed methods (quantitative with qualitative) despite its increasing use since the 1990s in healthcare research. This cross-sectional study demonstrates how a mixed methods approach is beneficial in assessing HIV client satisfaction and in identifying unmet needs in HIV healthcare. Conducted at the largest ambulatory HIV healthcare centre in Australia, this study consisted of two phases conducted sequentially: Phase One, a quantitative self-administered questionnaire survey, provided an overall statistical picture of satisfaction levels. This was followed by Phase Two, qualitative semi-structured face-to-face interviews, which enabled in-depth investigation of the reasons for satisfaction/dissatisfaction. Phase One had 166 respondents, giving a high response rate of 71%, while Phase Two had 22 participants. The study demonstrates the three advantages of using a mixed methods approach. Firstly, it increased the comprehensiveness of overall findings, by showing how qualitative data (Phase Two) provided explanations for statistical data (Phase One). Secondly, it expanded the dimensions of the research topic, as Phase Two enabled investigation of the determinants of satisfaction/dissatisfaction more broadly after assessing the client satisfaction levels in Phase One. Thirdly, it increased the methodological rigour as findings in both phases could be checked for consistency. Thus using a mixed methods approach can greatly enhance our understanding of client satisfaction in HIV/AIDS research.

Preventive effect of S-allyl cysteine sulphoxide (Alliin) on mitochondrial dysfunction in normal and isoproterenol induced cardiotoxicity in male Wistar rats: a histopathological study.

This study was undertaken to evaluate the preventive role of S-allyl cysteine sulphoxide (SACS) in isoproterenol (ISO)-induced cardiotoxicity in male Wistar rats. Myocardial infarction was induced by subcutaneous injection of ISO (150 mg/kg) once a day for 2 days. SACS (40 and 80 mg/kg) was given as pretreatment orally daily for a period of 35 days using an intragastric tube. SACS pretreatment significantly lowered thiobarbituric acid reactive substances (TBARS) and increased the activities of mitochondrial superoxide dismutase (SOD), catalase, glutathione peroxidase (GPx), glutathione S-transferase (GST), and the concentration of reduced glutathione (GSH) in myocardial infarcted rats. SACS pretreatment also increased significantly the levels of mitochondrial phospholipids and decreased the levels of mitochondrial cholesterol, free fatty acids (FFAs), triglycerides (TGs) and calcium, and the activity of xanthine oxidase (XOD) in heart. Further, the activities of isocitrate dehydrogenase (ICDH), succinate dehydrogenase (SDH), alpha-ketoglutarate dehydrogenase (alpha-KGDH), NADH-dehydrogenase, and cytochrome C-oxidase were significantly elevated in the mitochondrial fraction of the heart in the SACS-pretreated ISO-induced rats. Oral administration of SACS for a period of 35 days to the normal control rats did not show any significant effect. Histopathological studies of the myocardial tissue showed a protective role of SACS in the myocardial-infarcted rats. The effect at a dose of SACS 80 mg/kg was more effective than the dose 40 mg/kg. The results of the study conclude that SACS protect the mitochondria of the ISO-induced myocardial-infarcted rats.

Hopelessness, depression and oral health concerns reported by community dwelling older Australians.

OBJECTIVES: The purpose of this analysis was to explore whether, and if so to what extent, there is an association between self-reported oral and mental health problems, and if this association exists after controlling for self-rated physical health and age. RESEARCH DESIGN: A large cross-sectional population-based telephone health survey with participants selected using random stratified sampling. The response rate was 71%. Survey weights were used for estimating proportions and for all statistical inferences. SETTING AND PARTICIPANTS: Participants (n=8,881) were community-dwelling older people (65+ years) living independently in New South Wales, Australia. MEASURES AND ANALYSIS: The following measures were used in the analysis: physical health (1 item); oral health (5 items), mental health (2 items). Trends in prevalences across groups were analysed using the Cochrane-Armitage trend test. Logistic regression modelling was conducted to account for the main confounders of age and self-rated physical health, and attributable fractions calculated. RESULTS: Both measures of mental health, feeling hopeless and feeling depressed, were significantly and positively associated with increased reporting of oral health concerns in both males and females. Logistic regression modelling showed that most of the oral health items remained strongly associated with mental health after controlling for self-rated physical health and age. The attributable fractions demonstrated that a proportion of the mental health problems identified in older people would be reduced if oral health concerns were adequately addressed. CONCLUSIONS: The findings highlight the association between oral and mental health, and provide evidence to support the expansion of publicly funded dental health services.

Preventive effect of S-allyl cysteine sulfoxide (alliin) on cardiac marker enzymes and lipids in isoproterenol-induced myocardial injury.

The present study was designed to evaluate the preventive effect of S-allyl cysteine sulfoxide (SACS) in isoproterenol (ISO)-induced myocardial ischaemia in male Wistar rats. Rats were pretreated with SACS (40 and 80 mg kg(-1) body-weight) for 5 weeks. After the treatment period, ISO (150 mg kg(-1) body-weight) was administered subcutaneously to rats at intervals of 24 h for 2 days. The activities of creatine kinase, creatine kinase-MB, lactate dehydrogenase, aspartate transaminase and alanine transferase were significantly increased in serum and significantly decreased in the hearts of ISO-treated rats. Pretreatment with SACS decreased the activities of these enzymes significantly in serum and significantly increased the activities in heart in ISO-treated rats. The levels of cholesterol, triglycerides and free fatty acids increased in serum and heart, while the levels of phospholipids increased in serum and decreased in heart in ISO-treated rats. SACS pretreatment showed a significant effect on the lipids studied. The activity of 3-hydroxy 3-methyl glutaryl coenzyme A (HMG CoA) reductase was significantly increased and the activity of lecithin cholesterol acyl transferase (LCAT) was significantly reduced in ISO-induced rats. Oral pretreatment with SACS significantly decreased the activity of HMG CoA reductase and significantly increased the activity of LCAT in ISO-induced rats. The levels of plasma thiobarbituric acid reactive substances and hydroperoxides were increased in ISO-treated rats. Pretreatment with SACS significantly decreased the levels of lipidperoxides in ISO-treated rats. The effect at a dose of 80 mg kg(-1) body-weight was more effective than at a dose of 40 mg kg(-1) body-weight and brought back all the biochemical parameters to near normal levels. Thus our study shows that SACS has a lipid-lowering effect in ISO-induced rats. Our study may have clinical relevance.

Cost effectiveness of accelerated rehabilitation after proximal femoral fracture.

A randomised controlled trial comparing an accelerated rehabilitation program after proximal femoral fracture with conventional care and rehabilitation was conducted with 252 elderly patients treated at an Australian general hospital in 1989/1990. This paper presents a cost-effectiveness analysis of the accelerated rehabilitation program. The measure of cost was all direct costs of treatment and subsequent care (medical and non-medical) incurred during the 4 months after fracture. Effectiveness was defined as whether the patient returned to semi-independent living; or if moderately or severely disabled prior to the fracture to the premorbid level of physical independence. The cost for treatment up to 4 months after fracture was estimated at A$ 10,600 per accelerated rehabilitation patient and A$ 12,800 per conventional care patient (1990 Australian dollars, A$). Thus, accelerated rehabilitation releases resources equivalent to approximately 17% of costs for treatment per patient. When cost effectiveness is considered, the potential cost savings rise to 38% per recovered patient.

Problems in comparing findings on social class cross-culturally--applied to infant mortality (Australia and Britain).

This article addresses methodological problems in conducting cross-cultural comparisons of health findings analysed by social class. It then illustrates these problems by describing the difficulties faced and the methods adopted to conduct a cross-cultural comparison between Australia and Britain for infant mortality data analysed by social class. The specific findings on social class and infant mortality are reported, and the wider implications for cross-cultural research discussed.

What constitutes success in preventive health care? A case study in assessing the benefits of hip protectors.

The economic success of preventive health programs is typically assessed by the net health-related utility gain or loss to society relative to the cost. Issues relating to the positive or negative utility associated with participating in a preventive health program are often ignored. However, it is likely that calls for informed consumer choice and respect for patient autonomy will provide an impetus to examine utility associated with the process and outcomes of preventive health programs. In this paper, we outline the nature of the ex ante and ex post perspective in evaluating benefits and the presence of process utility and the utility of gambling in individual's utility function for preventive health care. The implications of including process attributes and psychological states when assessing benefits to society are discussed in relation to an empirical study on the value of external hip protectors for the prevention of hip fractures. We demonstrate that wearing hip protectors and the psychological outcomes of being a participant in the program can have a significant impact on individual's assessment of the benefits. Furthermore, point of reference plays a crucial role in their valuation. Individuals who did not consent to participate in a trial of hip protectors valued all states significantly lower than those who did participate in the trial. We argue that the utility associated with adherence to the intervention is an important issue for preventive health policy. From the viewpoint of applied welfare economics, evaluation of preventive health programs should allow for both process and outcome utility when assessing benefits. In this context, success might be viewed as maximising the opportunity for individuals to make an informed choice.

Suicide and unemployment in Australia 1907-1990.

Ever since Durkheim postulated a relationship between economic change and suicide there has been evidence of a general association between aggregate data on unemployment and the frequency of suicide. Quantitatively, however, the association has been variable and it is clear that due to differing cultural, social and individual determinants of suicide, the relationship is complex. Methodological difficulties abound with interpretation of aggregate data. Australian records for most of the present century are suitable for examining secular trends in suicide and unemployment by age group and gender to gain an indication of the extent to which both parameters may be causally related. An aggregate/ecological study was designed to incorporate quantitative and qualitative strategies. Annual age-adjusted male and female suicide rates and annual unemployment rates were derived for the period 1907-1990. Female suicide rates were generally stable throughout the period, whereas those for males demonstrated sharp fluctuations with the peaks coinciding with times of high unemployment. The association between suicide and unemployment for 15-24 year old males was comparatively high for the recent period, 1966-1990. The increasingly youthful contribution to male suicide was demonstrated by a rise in the loss of life years during 1973-1984. Despite the inability of any investigation based on aggregate data to establish an unequivocable causal relationship, no evidence was detected to suggest that relatively high population levels of unemployment were not related to the occurrence of suicide.

A cohort study of unemployment as a cause of psychological disturbance in Australian youth.

Data from the Australian Longitudinal Survey, conducted by the Commonwealth Department of Employment, Education and Training, were analysed to estimate relative risk of psychological disturbance accompanying unemployment in young people aged 15-24 years. Two cohorts were surveyed annually over 4 years during the mid-to-late-1980s; one from the general 15-24 year-old population (N = 8995), and the other selected from Commonwealth Employment Service records (N = 2403). Such large respondent numbers allowed control of confounding by exclusion to isolate employment transition groups suitable for hypothesis testing and quantification of causal relationships. Psychological morbidity was measured using binary outcomes of the 12 item psychological component of the General Health Questionnaire. Excluded from the analysis were those who: suffered from pre-existing physical health problems; were dissatisfied in their job; were self-employed; underwent marriage breakdown during the inter-survey period; had become widowed during the inter-survey period. A Bayesian probabilistic approach was used to calculate probabilities of psychologically normal respondents becoming psychologically morbid, given prior transition from employment to unemployment. Mantel-Haenszel analysis was utilised to estimate relative risks in comparison to a control group of those remaining employed, after controlling for age and gender. An overall relative risk of becoming psychologically disturbed as a consequence of becoming unemployed was estimated to be 1.51 (95% CI: 1.15-1.99). The overall relative risk of recovery from psychological disturbance upon re-employment in those with psychological disturbance was estimated to be 1.63 (95% CI: 1.08-2.48). Residual psychological effects of past unemployment experience and the effects of long-term unemployment were investigated, but found to be non-significant in this study. There was some evidence of psychological adaptation to unemployment, but this was statistically insignificant. Unemployment was a significant cause of psychological disturbance in young people who were initially employed, not suffering physical ill-health, and psychologically normal; conversely, re-employment reversed the effect.

A case-control study of employment status and mortality in a cohort of Australian youth.

Recent studies have demonstrated a link in young populations between unemployment and ill health. The purpose of this study is to correlate mortality with employment status in two cohorts of young Australian males, aged 17-25 years, from 1984 to 1988. Two youth cohorts consisting of an initially unemployed sample (n = 1424 males) and a population sample (n = 4573 males), were surveyed annually throughout the study period. Those lost to follow-up during the survey period were matched with death registries across Australia. Employment status was determined from weekly diaries and death certificates and was designated as: employed or student; unemployed; not in the work force (excluding students). Conditional logistic regression, using age- and cohort- matched cases (deaths) and controls (alive), was used to estimate the odds ratio (OR) of dying with regard to employment status, taking into account potential confounders such as ethnicity, aboriginality, educational attainment, pre-existing health problems, socio-economic status of parents, and other factors. Twenty three male survey respondents were positively matched to death registry records. Compared to those employed or students (referent group), significantly elevated ORs were found to be associated with neither being in the workforce nor a student for all cause, external cause, and external cause mortality other than suicide. Odds ratios were adjusted for age, survey cohort, ethnicity, pre-existing physical and mental health status, education level, and socio-economic status of parent(s). A statistically significant increasing linear trend in odds ratios of male mortality for most cause groups was found across the employment categories, from those employed or student (lowest ORs), through those unemployed, to those not in the workforce (highest ORs). Suicide was higher, but not statistically significantly, in those unemployed or not in the workforce. Suicide also was associated, though not significantly, with the respondent not living with their parents when they were 14 years of age. No association was found between mortality and past unemployment experience, as measured by length of time spent unemployed, or the number of spells of unemployment experienced during the survey. The results of this study underscore the elevated risk to survival in young males as a consequence of being neither employed nor a student.

Difficulties experienced by carers from non-English-speaking backgrounds in using health and other support services.

The proportion of the Australian population from non-English-speaking backgrounds is increasing (particularly among the elderly, who are more likely to be disabled), but their use of health care services and institutions is proportionately lower than that for other Australians, and many care for a family member with a disability or illness at home. The study objective was to document the experiences of such carers in using health and other support services in order to understand the reasons for underutilisation. This study differs from most other carer studies in concentrating on carers with little or no English, and by using a semistructured interview that enabled carers to describe their experiences and attitudes more openly. Forty women carers were interviewed, with bilingual interviewers or interpreters being used where necessary. Interviews were tape-recorded and content-analysed. The services used, sources of information and referral, and the reasons for not using a service were identified. Some of the difficulties experienced in using services were common to many carers, irrespective of background, whereas others related specifically to English language deficits and cultural differences. Carers were reluctant to institutionalise a family member, despite their own health problems, emphasising the need for greater support from existing and expanded services to enable the relative to be cared for at home.

Burn injuries and characteristics of burn patients in New South Wales, Australia.

This study describes the burn injuries and characteristics of patients admitted to the major adult burn unit in New South Wales, Australia. Data were collected from the medical records of all patients admitted in the 30-month study period (184 persons, 143 (78 per cent) males, 41 (22 per cent) females). The findings identified that patients were most likely to be males, aged between 25 and 34 years, who experienced a 11-20 per cent total surface burn area flame injury whilst using an accelerant in a domestic environment. Patients were more likely to smoke tobacco, drink alcohol to hazardous levels and take prescribed psychotropic medications than the general population. Preburn morbid conditions were prevalent, especially in the females. The actions of patients who had sustained a non-intentional burn commonly indicated a lack of understanding of the danger of certain equipment or the combustibility and volatility of some materials and flame. Prevention appears to be the most effective way to reduce burn injury through education of at-risk groups. However, because of the multicausality identified in the study, other preventive measures, such as improvement in product safety design, are suggested. Some similarities and differences with studies reported from Australia and overseas are noted.

External hip protectors: likely non-compliance among high risk elderly people living in the community.

The factors that may influence compliance with wearing of external hip protectors by potential users living in the community were investigated. Elderly women (median age 83 years) who were hospitalised after fracture, joint replacement or falls and were expected to return to community living participated. Five focus groups were conducted. Most participants said they would not use the hip protector demonstrated. The main objections were a perceived lack of comfort in wearing the appliance, particularly in bed, coupled with the belief that they were not at high risk. Other lesser issues were the extra effort needed to wear the device, appearance, accuracy of fit, cost and unfamiliarity with the protectors. These findings suggest that, in general, high risk elderly women living in the community will be unlikely to use external hip protectors unless there is considerable encouragement from family members and/or health professionals. Educational programmes could reduce some misconceptions about hip fracture, and reinforce the benefits of wearing a protective appliance. They may also increase awareness of personal risk. An introductory period of supervised wearing of the hip protectors, while in hospital or respite care, may enhance compliance.

Effect of submucosal diathermy to the inferior turbinates on unilateral and total nasal airflow in patients with rhinitis.

The efficacy of the controversial treatment of submucosal diathermy to the inferior turbinates (SMDIT) was evaluated objectively. Twenty-seven patients with chronic rhinitis were investigated by hourly posterior rhinomanometry to assess changes in total and minimum (Fmin) and maximum (Fmax) unilateral nasal airflow over 5 h, before and 2-3 months after standardized SMDIT treatment. Nasal airflow was recorded at a sample pressure of 75 Pa and the results are reported as medians with interquartile range. Whilst SMDIT caused a significant 51% increase (p < 0.0001) in total nasal airflow from 246 cm3/s (131) to 371 cm3/s (133) the changes in unilateral airflow provided further evidence which strongly supported the benefits of this operation. Unilateral Fmin significantly increased by 136% (p < 0.0001) from 69 cm3/s (82) to 163 cm3/s (74) and Fmax significantly increased by 23% (p < 0.0001) from 171 cm3/s (74) to 211 cm3/s (59). The effect of surgery was to "splint" to the turbinate in a state of relative vasoconstriction. Our findings therefore provide functional evidence of submucosal fibrosis following SMDIT. The greater percentage change in unilateral Fmin suggests that this parameter is a more sensitive index of the effect of nasal surgery than total nasal airflow measurements. The importance of considering the nose as two separate airways in the evaluation of nasal treatments is emphasized.