Volume 190 Issue 6

Victims of previous disasters have helped us learn much that can help those suffering now The horror and tragedy of the recent Victorian bushfires have affected all Australians, evoking both compassionate response and practical support. Alongside other members of their communities, doctors, nurses and other health professionals have all been directly affected, experiencing horrendous threats to life, loss, grief, and dislocation from their homes and way of life. General practitioners, community nurses, social workers and others will be called upon to provide care and to deal with the extensive mental health issues that arise in the aftermath of such incidents. It is important that any response is informed by the most up-to-date research findings in shaping the care provided. Studies, mainly by Australian researchers, have shown that the most important early responses involve protecting and comforting those most directly affected, linking them to loved ones and sources of support, and ensuring assessment and follow-up. A crucial issue is the central role of the GP in the provision of post-disaster services, as shown in a study of all the registered victims of the 1983 Ash Wednesday bushfires in South Australia.1 Local communities have a preference for their GP’s services in the post-disaster period, but they are also likely to need access to community recovery services for practical assistance and resources. Where possible, such services provided after the fires in Victoria should be linked to local clinics to facilitate access to health care. For the GP, assessing patients in terms of the nature of their experience of the disaster will be important — for instance, whether they were directly exposed to the fire, and whether they have lost family members or others close to them, or their home, property or other physical resources. A brief physical health check is important, alongside assessing levels of distress,2 providing guidance about health and wellbeing strategies, and assuring contact and outreach. Formal counselling is most effective after the early weeks, particularly for those with ongoing levels of acute distress related to the horror and life-threatening nature of the experience. Skilled management of bereavement in the early stages requires allowing patients to talk of their loved ones, and assisting them through any disaster victim identification and other formal processes. Follow-up over the months ahead is important for both physical and mental health needs in the post-disaster period. It is important to remember that the affected communities already carry a level of existing morbidity, which needs to be encompassed in planning a response. The magnitude of this problem is reflected in the 2007 National Survey of Mental Health and Wellbeing, which showed that 20% of Australian adults had a psychiatric disorder in the previous 12 months. Post-traumatic stress disorder (PTSD) was the most common disorder, with a 12-month prevalence of 6.4%.3 These findings suggest that the prevalence of traumatic events is much greater than is generally recognised in our community. Those already suffering are particularly at risk, but a further significant proportion may develop problems such as complicated grief, depression and PTSD as a direct consequence of the bushfire disaster.4 A lesson from the Ash Wednesday fires is that victims often delay seeking care for at least 18 months, despite experiencing considerable suffering.5 When they do present to GPs, it is often with physical symptoms,6 and the significance of these is missed. A recent treatment study after the London terrorist bombings that provided help by directly screening the high-risk victims found that many had presented to and requested help from GPs, who had discouraged them from seeking care, underestimating their distress.7 In the aftermath of the Victorian bushfires, one approach that should be considered is the use of screening for depression, PTSD and alcohol misuse in all GP presentations in affected areas. Clinical guidelines demonstrate that this approach leads to better outcomes if the screening is linked to adequate clinical services.8 However, GPs tend to prematurely terminate treatment, with subsequent loss of the demonstrated treatment gains, highlighting a need in fire-affected regions for continuing education programs that address the issues of diagnosis and treatment.9 Members of the emergency services also deserve particular attention because of the prolonged intensity of their exposure, particularly in light of the high number of fatalities.10 The community owes them a special duty of care. Active screening programs linked to occupational health services that are expert in managing traumatic reactions and grief should be instituted, as currently occurs in the Australian Defence Force. The willingness of victims of previous Australian disasters to participate in disaster research has resulted in the capture of many lessons and should be acknowledged. This knowledge needs to be used in the coming months so that the lessons already learned do not have to be rediscovered, as is too often the case after disasters. Future studies should build on what we already know, rather than simply replicating what has been studied before. Research that makes demands on people who are suffering has no role if it is not innovative. Image courtesy: Inspector Ben Shepherd, Rural Fire Service, NSW.

Alexander C McFarlane MB BS(Hons), MD, FRANZCP · Beverley Raphael AM, MB BS, MD, FRANZCP

A stringent, preventive risk-management approach could ensure potable reuse is a safe option As a result of prolonged drought, Australians are increasingly relying on alternative water sources — including rainwater, greywater, and water recycled from stormwater or sewage — for many community and household uses. Health professionals therefore need to be aware of the likelihood, if any, of illness related to water usage. In particular, careful consideration should be given to the safety of water recycling, especially as, at face value, it seems to represent a backward step from John Snow’s mid 19th century discovery of the importance of keeping drinking water and sewage separate.1 The obvious question is: is water recycling safe? There are different types of recycling, and different end uses for recycled water. In Australia, recycled water is currently used for irrigation of parks, golf courses and certain crops, and by various industries. Increasingly, new housing developments are incorporating separate pipes to distribute two grades of water: drinking water, supplied to kitchens and bathrooms; and recycled water for outdoor purposes (garden watering, car washing) and limited indoor use (toilet flushing, sometimes in laundries). As these uses of recycled water should not involve intimate human exposure, they are generally considered to incur negligible health risks. However, plumbing errors or use of recycled water for unendorsed purposes could result in intentional or unintentional ingestion. Fortunately, no recognised disease outbreaks associated with these schemes have so far occurred, and the public seems to be tolerant of these forms of water recycling. The more controversial issue relates to the potential health consequences of using treated recycled water to augment drinking water supplies (also called “potable reuse”). Australian water and health agencies have recognised the need for detailed technical guidance on minimising health risks from reuse schemes, resulting in production of the Australian guidelines for water recycling (AGWR).2,3 Phases 1 and 2 of these guidelines have been endorsed by the Australian Health Ministers’ Conference, and Phase 2 by the National Health and Medical Research Council (NHMRC). The AGWR recognise that the health consequences of system failure in a water recycling scheme could be catastrophic, with the potential for large gastroenteritis outbreaks, and that the greatest threat to public heath is from poor maintenance or poor quality control of water treatment processes. Consequently, the guidelines recommend proactive identification and management of risks, and include a “framework for management of recycled water quality and use”.2,3 One of the framework’s 12 elements involves undertaking a comprehensive risk assessment of each reuse scheme to determine the appropriate type and number of treatment steps required to “clean” the water. Another fundamental aspect of the framework is continuous monitoring of operational characteristics such as disinfection (chlorine concentration) and filtration (turbidity) that indicate water treatment processes are functioning efficiently. In other words, assuring adequate water quality is best achieved by monitoring the performance reliability and integrity of water treatment systems, and not by testing for all possible microbial or chemical contaminants.2,3 This lack of reliance on water quality testing is likely to seem counterintuitive to clinicians; however, the degree of assurance provided by negative test results is constrained by detection limits and the representativeness of small water sample volumes. Water quality testing is thus better suited to providing verification of system performance rather than being used as a routine management tool. There are two main issues raised by opponents of recycling schemes. First, they question whether water treatment processes can be guaranteed to always function effectively. This is a key focus of the AGWR, and it is essential that operators of potable reuse schemes have adequate skills and resources to provide the high level of quality control required to ensure safety. The second issue relates to adequate removal of chemical contaminants, given the large number of substances present in sewage (eg, hormones, pharmaceuticals, personal care products). This is a complex issue, particularly as the health consequences of low-level exposure to many of these substances are currently ill defined by toxicological and health data. The AGWR take a conservative approach to this issue and are consistent with international practices. Moreover, any incremental risks associated with potable reuse are likely to be negligible compared with other sources of direct exposure to these chemicals and with exposure from conventional drinking water supplies. So what is the bottom line? Evidence shows that current public supplies of drinking water in Australian cities do not incur an increased risk of gastroenteritis.4,5 Potable reuse is not yet occurring in Australia, but such water recycling schemes have existed internationally for more than 30 years, with schemes in Europe, the United States and Singapore having no recognised adverse health outcomes.6 Gastroenteritis outbreaks are unlikely when water treatment processes are well managed, so clear guidelines supporting prospective implementation of a preventive risk-management framework are fundamental to ensuring the safety of potable reuse.2,3 Health surveillance, although a relatively insensitive marker, can provide a retrospective indication of health outcomes associated with such schemes. The AGWR acknowledge that safety is not predicated on achieving zero health risk, but that there is an upper limit of tolerable risk (10-6 disability-adjusted life-years per person per year).3 The Australian drinking water guidelines, although now under review, currently contain no health-based target for microbial risk.7 This means that more stringent requirements for water quality are currently applied to potable reuse schemes than to conventional drinking water. While not advocating for or against potable reuse, it is thus paradoxically possible that planned potable reuse could be among the safest approaches to the provision of drinking water.

Karin S Leder MB BS, FRACP, PhD · Joanne E O’Toole BAppSc, MBA · Martha I Sinclair BSc(Hons), PhD

Evidence shows that cost does affect alcohol consumption, and reducing consumption improves public health There is strong evidence that increasing the cost of alcohol reduces the overall amount that is consumed.1 In a range of countries, price increases have been consistently shown to reduce alcohol consumption and related harms in both the general population and at-risk populations such as young people and heavy drinkers. Conversely, price decreases have resulted in an increase in consumption and harm.1-3 In this context, the Australian Government’s April 2008 increase in excise tax (Bill introduced on 11 February 2009) on ready-to-drink (RTD) spirit-based products (RTDs; “alcopops”) is an evidence-based strategy to reduce excessive RTD consumption among young people. The alcoholic content of RTDs is now taxed at a similar rate to that of other spirits (tax increased from $39.36 to $66.67 per litre of pure alcohol). Critics have argued that the RTD tax increase has not reduced alcohol consumption by young people, and will not do so. One claim is that young people will merely switch to other beverages. These arguments have been made by some from the alcohol industry and some researchers. Doran and Shakeshaft, for example, argued that young people “ seem to be price inelastic about their alcohol demand”.4 Citing a national school survey, they claimed that “spirits are by far the beverage of choice for the 45% of 16–17-year-old Australians who drink, despite spirits being the most highly taxed beverage in Australia, and the most expensive per litre of alcohol”. This is not evidence for price inelasticity. They also argued that “overall rates of usual or binge consumption in Australia are unlikely to substantially fall, because spirits hold a smaller market share than beer, and young people will more than likely switch their preference”.4 The weight of scientific evidence suggests otherwise — that overall consumption is likely to decline because young people’s demand for alcohol is elastic.1-3 The survey series on which Doran and Shakeshaft rely shows that beverage preferences vary between boys and girls and over time. In 1999, before reductions in tax and in the retail price of RTDs in 2000, RTDs were the preferred beverage of about 23% of 12–17-year-old female drinkers. By 2005, after the tax decrease, 48% of young females drank RTDs, while the preference for higher-taxed spirits fell from 42% to 30%. For 12–17-year-old males, RTD consumption increased from 6% to 14%, a small share compared with spirits (39%) and beer (33%).5 Although new products and marketing strategies may have contributed to this substantial change, these data suggest that young Australians, like their counterparts in other countries,2 do alter their beverage choices in response to price changes. Definitive statements about the impact of the “alcopops tax” are premature in the absence of independent alcohol sales data. It is regrettable that there are no readily available, official monthly sales data for all alcoholic beverages, like those obtained by the detailed monitoring that we know is conducted by private industry.6 However, available evidence does indicate that the tax has reduced sales of RTDs and the reduction was far from wholly offset by a switch to other beverages. A market research company that regularly compiles reports on sales of alcohol products has estimated national monthly sales of packaged alcohol (sold for off-premises consumption by liquor licensees across the five mainland states of Australia) by beverage type for 2007 and 2008 ().7 These data show that in the 3 months after the April 2008 tax increase, 91 million fewer standard drinks were sold as RTDs than in the same months in the previous year. Standard drinks sold as spirits and beer increased but wine sales decreased. The increase in spirit and beer sales (48 million standard drinks) was only 53% of the 91 million fewer RTD drinks sold. A decline in RTD sales was also reported on the basis of Australian Tax Office data. These showed a 54% reduction in sales of RTDs and a 7% increase in spirit sales from April to June 2008.8 In presenting the Excise Tariff Amendment Bill to Parliament, the Minister for Health and Ageing confirmed that: “Tax office figures drawn from the first nine months of this measure show that alcopops sales have dropped by 35 per cent compared to the previous year”.9 Critics have been hasty in predicting that young people’s drinking would be unresponsive to the RTD tax increase. In keeping with a large body of research evidence, the early indications are that RTD sales declined in the first few months after the tax increase. Previous research suggests that this decline in alcohol sales (a reliable proxy for consumption10) will produce a public health benefit.1-3 Further investigation is needed to determine specifically in which population group(s) the benefit accrues; for example, whether this reduction in RTD purchases occurred primarily among young drinkers (the target of the tax increase), and what other factors may have contributed to the reduction. Informed policy debate requires independent evaluations of short-term and long-term effects of these tax changes on consumption and harm indicators (eg, injuries). Nevertheless, the evidence to date is that the “alcopops” tax is a step in the right direction. Number of standard drinks* consumed in May to July, 2007 and 2008, by beverage type Beverage type Million standard drinks consumed Difference in million standard drinks % Change 2007 2008 RTDs 348 257 − 91 − 26.1 Beer 886 899 13 1.5 Wine 797 776 − 21 − 2.6 Spirits 313 348 35 11.2 Total 2344 2280 − 64 − 2.7 Source: Nielsen Liquor Services Group (NLSG) 2008.7 RTDs = ready-to-drink spirit-based products. * One standard drink = 10 g pure alcohol. To accurately convert beverage volumes to pure alcohol, the NLSG applies alcohol conversion factors at the subsegment level for beer (eg, regular, mid-strength, low-strength beer) and RTDs. Average alcohol contents by beverage type: RTDs 5.0%; beer 4.8%; straight spirits 38.0%; and wine 13%.

Tanya N Chikritzhs BA(Hons), PostGradDip(Epi · Paul M Dietze BSc(Hons), PhD · Steven J Allsop BSc, PhD · Michael M Daube BA(Hons), HonDSci · Wayne D Hall BSc(ApplPsych), PhD · Kypros Kypri BA(Hons), PhD

Objective: To estimate the incidence and survival rates of acute myocardial infarction (AMI) for Northern Territory Indigenous and non-Indigenous populations.Design and participants: Retrospective cohort study for all new AMI cases recorded in hospital inpatient data or registered as an ischaemic heart disease (IHD) death between 1992 and 2004.Main outcome measures: Population-based incidence and survival rates by age, sex, Indigenous status, remoteness of residence and year of diagnosis.Results: Over the 13-year study period, the incidence of AMI increased 60% in the NT Indigenous population (incidence rate ratio [IRR], 1.04; 95% CI, 1.02–1.06), but decreased 20% in the non-Indigenous population (IRR, 0.98; 95% CI, 0.97–1.00). Over the same period, there was an improvement in all-cases survival (ie, survival with and without hospital admission) for the NT Indigenous population due to a reduction in deaths both pre-hospital and after hospital admission (death rates reduced by 56% and 50%, respectively). The non-Indigenous all-cases death rate was reduced by 29% as a consequence of improved survival after hospital admission; there was no significant change in pre-hospital survival in this population. Important factors that affected outcome in all people after AMI were sex (better survival for women), age (survival declined with increasing age), remoteness (worse outcomes for non-Indigenous residents of remote areas), year of diagnosis and Indigenous status (hazard ratio, 1.44; 95% CI, 1.21–1.70).Conclusions: Our results show that the increasing IHD mortality in the NT Indigenous population is a consequence of a rise in AMI incidence, while at the same time there has been some improvement in Indigenous AMI survival rates. The simultaneous decrease in IHD mortality in NT non-Indigenous people was a result of reduced AMI incidence and improved survival after AMI in those admitted to hospital. Our results inform population-specific strategies for a systemwide response to AMI management.

Jiqiong You MSc, MBA, MB · John R Condon MPH, PhD, FAFPHM · Yuejen Zhao MB BSc, MBiostats, PhD · Steven Guthridge MB BS, MTH, FAFPHM

Objective: To describe the natural history and risk of early chronic kidney disease (CKD) in Indigenous Australian populations.Design, setting and participants: A prospective cohort of 2266 Aboriginal and non-Aboriginal children enrolled from primary schools throughout New South Wales from February 2002 to June 2004 and followed for 4 years.Main outcome measures: Urinalysis, height, weight, blood pressure, birthweight and sociodemographic status at baseline and 2- and 4-year follow-up; CKD risk factors: haematuria, albuminuria, obesity, and systolic and diastolic hypertension.Results: 2266 children (55% Aboriginal; 51% male; mean age, 8.9 years [SD, 2.0 years]) were enrolled at baseline. 1432 children (63%) were retested at 2-year follow-up, and 1506 children (67%) at 4-year follow-up. Prevalence of baseline CKD risk factors was frequent (2%–7%), but most abnormalities were transient. Besides persistent obesity (5.0%), persistence of CKD risk factors at final follow-up was low: haematuria (1.9%), albuminuria (2.4%), systolic hypertension (1.5%) and diastolic hypertension (0.2%). There was no difference in prevalence of persistent CKD risk factors between Aboriginal and non-Aboriginal children.Conclusions: Over 4 years of follow-up, Indigenous Australian children had no increased risk for early evidence of CKD. More than 70% of baseline risk factors were transient, and persistent risk factors were uncommon. Our findings suggest the increased risk for end-stage kidney disease seen in Indigenous adults is not yet manifest in these schoolchildren, and may be potentially preventable.

Leigh Haysom MB BS, MClinEpi, FRACP · Rita Williams BA · Elisabeth M Hodson MB BS, FRACP · Pamela A Lopez-Vargas BN, BSc, BHSc(TCM) · Leslie P Roy MB BS, MD, FRACP · David M Lyle MB BS, PhD, FAFPHM · Jonathan C Craig MB BS, PhD, FRACP

Objective: To quantify Indigenous mortality in the Northern Territory by remoteness of residence.Design, setting and participants: Australian Bureau of Statistics mortality data were used to compare rates of death from chronic disease in the NT Indigenous population with rates in the general Australian population over the period 1998–2003. Rates were evaluated by categories of remoteness based on the Accessibility/Remoteness Index of Australia: outer regional areas (ORAs), remote areas (RAs) and very remote areas (VRAs).Main outcome measures: Mortality from cardiovascular disease, diabetes and renal disease; standardised mortality ratios (SMRs); percentage change in annual death rates; changes in mortality between 1998–2000 and 2001–2003.Results: In 1998–2000, SMRs for all-cause mortality were 285% in ORAs, 875% in RAs and 214% in VRAs. In 2001–2003, corresponding SMRs were 325%, 731% and 208%. For the period 1998–2003, percentage changes in annual all-cause mortality were 4.4% (95% CI, –2.2%, 11.5%) in ORAs, –5.3% (95% CI, –9.6%, –0.8%) in RAs, and 1.1% (95% CI, –7.2%, 11.3%) in VRAs. In 2001–2003, compared with 1998–2000, changes in the number of Indigenous deaths were +35 in ORAs, –37 in RAs and +32 in VRAs. Similar patterns were observed for cardiovascular mortality.Conclusions: Compared with mortality in the general Australian population, Indigenous mortality was up to nine times higher in RAs, three times higher in ORAs and two times higher in VRAs. The fact that rates were lowest in VRAs runs contrary to claims that increasing remoteness is associated with poorer health status. Despite the high death rate in RAs, there was a downward trend in mortality in RAs over the study period. This was partly attributable to a fall in the absolute number of deaths.

Karen Andreasyan DMD, MPH · Wendy E Hoy BSc, MB BS, FRACP

Objective: To determine whether the proportion of babies born large for gestational age (LGA) in New South Wales has increased, and to identify possible reasons for any increase.Design and setting: Population-based study using data obtained from the NSW Midwives Data Collection, a legislated surveillance system of all births in NSW.Participants: All 1 273 924 live-born singletons delivered at term (≥ 37 complete weeks’ gestation) in NSW from 1990 to 2005.Main outcome measures: LGA, defined as > 90th centile for sex and gestational age using 1991–1994 Australian centile charts; maternal factors associated with LGA were assessed using logistic regression.Results: The proportion of babies born LGA increased from 9.2% to 10.8% (18% increase) for male infants and from 9.1% to 11.0% (21% increase) for female infants. The mean birthweight increased by 23 g for boys and 25 g for girls over the study period. Increasing maternal age, higher rates of gestational diabetes and a decline in smoking contributed significantly to these increases, but did not fully explain them.Conclusions: There is an increasing trend in the proportion of babies born LGA, which is only partly attributable to decreasing maternal smoking, increasing maternal age and increasing gestational diabetes.

Ruth M Hadfield BSc, GCBiostat, DPhil(Oxon) · Samantha J Lain BComm, BHlthSci(Hons), MPH · Judy M Simpson PhD, CStat · Jane B Ford BA(Hons), PhD · Camille H Raynes-Greenow BA, MPH, PhD · Jonathan M Morris MM, FRANZCOG, PhD · Christine L Roberts MB BS, DipObs(RACOG), DrPH

This position statement was prepared by the Working Group of the Australian and New Zealand Bone and Mineral Society and Osteoporosis Australia. The final statement was endorsed by the Endocrine Society of Australia. Currently, the balance of evidence remains in favour of fracture prevention from combined calcium and vitamin D supplementation in elderly men and women. Adequate vitamin D status is essential for active calcium absorption in the gut and for bone development and remodelling. In adults with a baseline calcium intake of 500–900 mg/day, increasing or supplementing this intake by a further 500–1000 mg/day has a beneficial effect on bone mineral density. Calcium intake significantly above the recommended level is unlikely to achieve additional benefit for bone health.

Kerrie M Sanders GradDipDiet, MHumNutr, PhD · Caryl A Nowson DipNutrDiet, PhD · Mark A Kotowicz MB BS, FRACP · Kathryn Briffa BAppSc(Physio), PhD · Amanda Devine GradDipDiet, PhD · Ian R Reid MB BS, FRACP

Recently, two major epidemiological studies found that hormone replacement therapy (HRT) in postmenopausal women increased the risk of breast cancer. One of the studies also found that HRT increased the risk of cardiovascular disease and thrombosis. As a consequence, women were advised to cease this therapy. However, detailed analysis of these studies suggests that the conclusions may be erroneous. Other studies suggest that the timing of initiation of HRT for healthy women is critical to achieving a beneficial outcome. When begun within 5 years of menopause in healthy women, oestrogen-based HRT results in far greater benefits than adverse outcomes. There is substantial objective evidence that the benefits of HRT include: Reduced distressing symptoms of menopause. Reduced risk of osteoporotic fractures, dementia and colorectal cancer. Improved wellbeing, quality of life; improved vaginal epithelium, sexual enjoyment and bladder capacity. Improved cardiovascular system, with reduced myocardial ischaemia and cardiovascular-related death. Increased longevity. The adverse effects of HRT include: Oral HRT doubles the risk of thromboembolism. HRT promotes growth of pre-existing breast cancer.

Barry G Wren MD, FRANZCOG, FRCOG

New treatments that are potentially as effective as existing treatments are increasingly being developed, some of which may be preferred because of lower cost, fewer side effects, easier administration or less harm. Non-inferiority trials attempt to establish whether or not a new treatment — drug or non-drug — is no worse than an established treatment for which efficacy has been determined in placebo-controlled trials. Critical issues in the design and conduct of non-inferiority trials include: defining the acceptable margin of adverse events that, if exceeded, will render the new treatment inferior to the standard treatment (the non-inferiority margin); calculating the sample size needed to demonstrate non-inferiority; assessing the robustness of results in terms of absolute versus relative effects, intention-to-treat versus per-protocol analyses, one-sided versus two-sided statistical tests, and observed versus expected event rates for standard treatment; evaluating all relevant outcomes, including harm; and stating conclusions that are consistent with aims and results. Many non-inferiority trials fail to meet basic quality criteria, report biased and misleading conclusions, and are unduly influenced by commercial sponsors, with some commentators going so far as labelling them unethical. Clinicians and trial investigators need to exercise caution when interpreting results of non-inferiority trials which, because they lack a placebo group, can only provide an indirect assessment of the efficacy of a new treatment compared with an existing standard, and where the choice of non-inferiority margin can be highly subjective.

Ian A Scott FRACP, MHA, MEd

Clinical records Over the past 3 years, we have identified five cases of coronary syndromes attributable to a left subclavian stenosis proximal to a left internal mammary artery (LIMA) graft for coronary artery disease. All occurred in men aged between 56 and 73 years, presenting a median of 52 months (range, 26–138 months) after coronary artery bypass grafting. One patient presented with stable angina, three had unstable angina, and one had a non-ST elevation myocardial infarction. Arm claudication was present in one patient. Three of the five patients had an exercise or pharmacological stress test — all with anterior wall ischaemia. At cardiac catheterisation, the diagnosis was recognised by careful comparison of the pressure tracings in the subclavian artery and aorta. All patients had a significant pressure gradient across the subclavian stenosis (median, 35 mmHg; range, 20–85 mmHg), measured by a 5F or 6F diagnostic catheter, and a significant angiographic stenosis (median, 70%; range, 50%–80%). Non-invasive left arm blood pressure measured at the time of cardiac catheterisation was substantially lower than aortic pressure (> 20 mmHg difference in all patients). Retrograde flow up the LIMA graft during native left coronary angiography, a characteristic of subclavian steal, occurred in one of the five patients (Figure 1). All patients were treated with percutaneous stenting, using 9–10 mm balloon expandable stents, with successful abolition of the pressure gradient in the proximal subclavian artery (Figure 2). The difference in non-invasive blood pressures between the arms after stenting was less than 5 mmHg in all patients. All patients had relief of their angina symptoms over a median follow-up of 20 months (range, 5–29 months). 1 A: Subtracted angiogram showing the subclavian stenosis prior to the left internal mammary artery (LIMA) origin. B: Selective left coronary angiogram showing flow down the left anterior descending artery (LAD) (1) then retrograde up the LIMA (2 and 3). C: Diagram showing the relationship of the stenosis to the left vertebral and LIMA origins. 2: Angiograms of the left subclavian stenosis before (A) and after (B) stenting, showing the improvement in antegrade LIMA flow. The intra-arterial subclavian pressure tracings are shown below the angiograms. Before stenting (left) the pressure tracing is blunted, while after stenting (right), there is an improvement in blood pressure and normalisation of the arterial waveform. A potentially clinically significant stenosis in the subclavian or brachiocephalic arteries will produce a difference in systolic blood pressure between the right and left brachial arteries of 15–20 mmHg or more.1 Therefore, bilateral arm blood pressure measurements should be taken in symptomatic patients after coronary artery bypass using internal mammary artery grafts. Unfortunately, this simple non-invasive assessment is often overlooked, as it was in the patients reported here. A haphazard approach to catheterisation of the left internal mammary artery (LIMA) graft can also cause the diagnosis to be missed, as the catheter can often cross a significant subclavian stenosis. Meticulous comparison of the subclavian and aortic pressure tracings will identify the gradient and enable diagnosis. The difference in pressure between the arms may be reduced or absent in patients with significant bilateral subclavian and/or brachiocephalic disease, a scenario more likely in patients with extensive atherosclerotic peripheral vascular disease elsewhere.2 Coronary subclavian steal syndrome describes angina related to a subclavian stenosis with retrograde flow up the LIMA graft. This reverse LIMA flow results from lower vascular resistance and blood pressure in the arm compared with the myocardial territory supplied by the LIMA. However, steal with reverse LIMA flow is not an absolute requirement for angina. In our patients, none had angina precipitated by left arm activity that would uncover a latent steal syndrome. In many cases, subclavian stenosis behaves physiologically more like a very proximal LIMA stenosis. In this case, “LIMA-inflow syndrome” may be a more accurate term. Regardless of the semantics, this is a rare phenomenon that is reported in 0.1%–5.0% of patients after coronary artery bypass grafting.2-4 The differential diagnosis includes other diseases obstructing large arteries such as Takayasu’s arteritis (especially in young women), post-radiation arteritis, and giant cell arteritis (especially in older patients). The management of subclavian and brachiocephalic stenoses depends on the clinical presentation. All patients require medical therapy with intensive atherosclerosis risk factor reduction, as the 10-year total and cardiovascular mortality approaches 40%–50%.1 Asymptomatic individuals do not need intervention, so non-invasive imaging is unnecessary. In symptomatic patients, non-invasive imaging with contrast computed tomography or magnetic resonance or conventional angiography can confirm the diagnosis when an intervention is anticipated. Traditionally, symptomatic patients were treated with carotid-subclavian bypass. However, percutaneous angioplasty and stenting are increasingly used because of their lower morbidity and similar long-term results.2,5-8 The risk of stroke from stenting is low, and no higher than for surgical bypass.2,5-8 Post-procedural management includes treatment with aspirin for life and clopidogrel for at least 1 month. Clinical follow-up includes surveillance for recurrent symptoms with a difference in brachial blood pressures. Although the LIMA-inflow syndrome from subclavian stenosis is a rare cause of angina, it is easily identified non-invasively by comparing the brachial blood pressures in both arms. Advances in percutaneous stenting permit a relatively easy and durable treatment in a cardiac catheterisation laboratory. Lessons from practice Systolic blood pressure should be measured in both arms with a standard sphygmomanometer in all patients with past coronary artery bypass grafting and progressive angina or acute coronary syndromes. A difference in systolic blood pressure of greater than 15–20 mmHg between the right and left arms is strongly suggestive of subclavian stenosis. Asymptomatic subclavian stenosis does not require imaging or revascularisation, but does denote high cardiovascular risk warranting intensive risk factor reduction. Symptomatic subclavian stenosis can be successfully treated with percutaneous stenting.

Daniel Tsyvine MD · Maryanne Hartzell MD · Marc P Bonaca MD · Gerard Connors MB BS, FRACP · Scott Kinlay MB BS, PhD, FRACP

A 32-year-old woman with a 4-year history of multiple sclerosis presented with persistent clawing of the right hand. History revealed that she and five family members had lifelong symptoms of paradoxical myotonia (impaired relaxation of muscles following muscle contraction), exacerbated by cold. The family was diagnosed with paramyotonia congenita, based on neurophysiological and genetic studies. To our knowledge, this is the first report of an Australian family with paramyotonia congenita. Clinical record A 32-year-old woman of European ancestry was referred to a movement disorder clinic for evaluation of mild persistent clawing of the right hand (Figure, A), which had developed over the past year. She had no weakness of the hand or other neurological signs. The patient had been diagnosed with relapsing remitting multiple sclerosis 4 years earlier, based on clinical features (typical exacerbations), characteristic white matter changes on magnetic resonance imaging scans, and oligoclonal bands restricted to the cerebrospinal fluid. Her condition was managed in a multiple sclerosis clinic. Before her referral, she was in good health between exacerbations of multiple sclerosis. Since early childhood, the patient had experienced persistent cramping of her hands after their use, particularly in cold conditions. She had difficulty releasing tightly gripped objects, and cramping usually worsened, rather than improved, with ongoing exertion, such as when using clippers to groom a dog (ie, there was no “warm-up” phenomenon). She found it difficult to talk after ingesting cold food or drink, and difficult to open her eyes after jumping into a cold pool. In extremely cold conditions, she experienced widespread cramping of her muscles with clawing of her hands, and a tendency for her toes to curl inwards. These symptoms would typically improve over several hours as her body warmed. As the cramping improved, there was notable weakness of affected muscles. A diagnosis of paramyotonia congenita was suspected after referral to the clinic. On questioning, the patient reported that other members of her family had similar symptoms. Five were interviewed; their symptoms began in early childhood, and included muscle cramping induced by exposure to cold or exertion (both in most cases). Face and hand muscles were predominantly affected. These family members also described generalised cramping and weakness in response to severe cold, but none had a permanent deformity similar to the patient’s clawing of the right hand. Physical examination of the patient and five affected family members revealed that all had paradoxical myotonia of the orbicularis oculi muscles and the hands, and percussion myotonia over the thenar eminence. None had weakness or muscle hypertrophy. Owing to the unusual coincidence of white matter disease and suspected paramyotonia congenita in the patient, magnetic resonance imaging of the proband’s affected brother (Figure, B; V-4) was performed. A small area of gliosis in the left caudate nucleus was identified but no white matter abnormalities were present. A family pedigree was constructed (Figure, B). This revealed that the paradoxical myotonia was inherited in an autosomal dominant pattern, spanning at least six generations. Further investigations of the patient revealed a slightly elevated creatine kinase level, myotonic discharges on electromyography and evidence of cold paralysis on nerve conduction studies. DNA sequencing revealed that the patient had a sodium channel gene mutation (Box). The five affected family members were subsequently identified as being heterozygous for the same mutation, and an unaffected family member was shown to lack this mutation (Box). Paramyotonia congenita was diagnosed, and acetazolamide therapy was begun, which led to moderate amelioration of symptoms. Her multiple sclerosis remained well controlled on glatiramer acetate. A: Permanent deformity of the patient’s right hand. B: Pedigree of the patient’s family, demonstrating that the paramyotonia congenita is inherited in an autosomal dominant pattern (patient [proband] = V-1). * Individuals IV-3 and V-1 each appear twice in the pedigree, as indicated by the dashed lines. To our knowledge, this is the first report of an Australian family with paramyotonia congenita. Paramyotonia congenita is a rare autosomal dominant condition characterised by paradoxical myotonia — impaired relaxation of muscles following muscle contraction, which worsens with repetitive muscle activity.1 It is distinct from other forms of myotonia, which typically improve with repetitive activity (the warm-up phenomenon).4 Paramyotonia is typically exacerbated by exposure to cold, and can be associated with cold-induced paralysis.1 Mutations in the skeletal muscle voltage-gated sodium channel gene SCN4A cause paramyotonia congenita,5 and can also cause hyperkalaemic periodic paralysis, potassium-aggravated myotonia, and a small proportion of hypokalaemic periodic paralysis cases.6 Patients carrying an SCN4A mutation may have manifestations of more than one of these allelic disorders. Our patient’s neurophysiology findings and the clinical features of her affected family members are typical for paramyotonia congenita associated with cold paralysis, and similar to those described in the largest reported case series for this condition.7 In this case series, age of onset was typically during early childhood, and clinical myotonia was evident in 100% of the 56 patients studied. Cold was identified as a precipitant in 91% of patients, and exercise was identified as a precipitant in 46%. Electromyographic evidence for myotonia was seen in 100% of the patients, and 92% had a reduction in compound muscle action potential in response to cold (objective cold paralysis). Forty-nine of the patients (88%) had a mutation of the SCN4A gene. The electromyographic changes evident with cooling in our patient were typical for paramyotonia congenita — cooling initially resulted in abolition of the myotonic discharges, and electrical silence was recorded at lower temperatures.1 The T1313M mutation was identified in our patient and the five affected family members, but not an unaffected family member. This is one of the more common mutations that causes paramyotonia congenita,4,8,9 and has been reported to exclusively cause paramyotonia with the cold-paralysis phenotype.9-13 The hands and face are predominantly affected in patients who carry the T1313M mutation.9-13 A report of French families with paramyotonia congenita noted significant clinical variability in patients carrying the T1313M mutation — in both severity of myotonia and its permanence — and myotonia permanens was evident in six of eight of these patients.9 In our patient, the clawing of the right hand is also likely to reflect myotonia permanens, rather than an interaction between the paramyotonia and multiple sclerosis. The T1313M mutation has been predominantly described in families with French ancestry, and to a lesser extent in families of English and Japanese background. 5,9,11 One de-novo mutation has been identified in the literature, which occurred in a Japanese man.12 All individuals from the six generations of the family we studied were born in Australia and lived in Australia, but the ancestral origins of the family are unknown. Present knowledge of SCN4A channel physiology provides insight into the clinical manifestations seen in our patient and the affected members of her family. Voltage-gated sodium channels are heteromultimeric, integral membrane proteins; they are composed of a single large pore-forming α subunit, and 1 or 2 smaller β units.6 There are nine subtypes of α subunit, one of which is expressed in skeletal muscle — SCN4A.6 The α subunit is composed of four structurally homologous domains (D1–D4), with six membrane spanning segments (S1–S6) within each domain.6 The sodium channel is important for generating and propagating action potentials and switches through three functional states: activation (the open-channel state), inactivation, and recovery from inactivation.6 The consequences of the T1313M mutation have been studied by patch-clamp studies using various cell lines. 10,14-17 Overall, the mutation has been shown to slow the rate of channel inactivation, diminish the voltage dependence of inactivation, and increase the rate of recovery from inactivation. 10,14-17 These effects result in increased sodium conductance and prolongation of action potentials, which causes persistent activation of potassium channels, leading to relatively high levels of extracellular potassium.6,10,14-17 Elevated extracellular potassium levels increase the likelihood of afterdepolarisations, which may lead to action potentials on adjacent surface membranes.6 This can result in ongoing muscle contraction and slowed relaxation — the features of paramyotonia.6 Threonine-1313 is a highly conserved residue in the D3–D4 linker; it is important for channel inactivation, and thought to occlude the cytoplasmic portion of the channel.16 The change from the polar hydrophilic threonine to the larger non-polar methionine is thought to impair occlusion of the channel, and therefore impair inactivation.13,16 Although the effects of the mutation have been shown to be potentiated at lower temperatures, as might be expected, no study has shown an increased sensitivity to temperature compared with normal cells.15-17 We have described the clinical, neurophysiological and genetic features of a large Australian family with paramyotonia congenita. The diagnosis of myotonia may be difficult and cause confusion, and the sodium channel disorder described here should be considered in patients with the symptoms described — especially as the symptoms respond to treatments such as acetazolamide and mexiletine, and as there are implications for genetic counselling and prognosis. Clues to the diagnosis include paradoxical myotonia — myotonia that worsens with ongoing exertion — and significant worsening of symptoms in cold conditions. Investigations Biochemistry and neurophysiology The patient’s serum potassium level was 4.2 mmol/L (reference range [RR], 3.2–4.3 mmol/L) and her creatine kinase level was slightly elevated at 191 U/L (RR, < 150 U/L). Routine nerve conduction study results were normal. Electromyography of the left abductor pollicis brevis and flexor digitorum superficialis revealed electrical myotonia and dense fibrillations. Cooling to below 28°C abolished the myotonic discharges and cooling below 22°C resulted in abolition of all spontaneous activity. The cooling test described by Streib1 was also performed. Before cooling, the amplitude of the left median compound muscle action potential recording over the abductor pollicis brevis muscle was 16.7 mV. The arm was then cooled to below 20°C by immersion in an ice bath and then rewarmed to 32°C using heat packs. The compound muscle action potential after rewarming was 4.5 mV (a 73% decrease). Molecular genetics Venous blood from the patient was sent to a commercial laboratory (PathWest, Royal Perth Hospital, Perth) for sequencing of the SCN4A gene. A heterozygous C-to-T nucleotide substitution at position 3938 in exon 22 was identified, resulting in a threonine-to-methionine amino acid change at codon 1313 (T1313M). Venous blood was then obtained from five family members who had symptoms that were similar to those of the patient (Figure, B; IV-1, IV-3, V-3, V-4, VI-4), as well as an unaffected family member (the patient’s half-sister), and mutational analysis was carried out. DNA was extracted by a proteinase K digestion method2 and amplified using the polymerase chain reaction (PCR) (forward primer sequence, 5'-TGGAGGCAGGAAGGGGAACT-3'; reverse primer sequence, 5'-GGCAGCACACACAGGACAGG-3'). The cycling conditions were: 3 min at 94°C × 1; and 30 s at 94°C, 40 s at 57°C, 1 min at 72°C × 30. The PCR reaction mixture contained 100 ng DNA, 0.5 μM of each primer, 80 μM of each deoxynucleoside triphosphate, 20 μM Tris-HCl (pH, 8.5), 50 μM KCI, 1.5 mM MgCI and 0.5 U Taq Ti DNA polymerase (Fisher Biotech, Perth, Australia). Amplified products were separated on 3% agarose gels. The separated fragments were diluted to 10 ng/μL per 100 base pairs and then sequenced by the dideoxy termination method,3 using BigDye Terminator v3.1 chemistry (Applied Biosystems, Foster City, Calif, USA) and the 3100 Genetic Analyzer (Applied Biosystems). Sequencing results showed that the five affected family members were heterozygous for the T1313M mutation and that the unaffected family member did not carry the mutation.

Sharavanan Parasivam MB BS, FRACP · Malgorzata Krupa BSc(Hons) · Mark Slee MB BS, FRACP · Dominic E Thyagarajan MB BS, MD, FRACP

To the Editor: Macneil and colleagues1 present evidence of how inadequate resourcing of acute kidney stone care for public patients in New South Wales is compromising their surgical outcomes compared with private patients. In our experience, the same difference exists elsewhere in Australia. Macneil and colleagues show how poorly planned, under-resourced and badly coordinated acute surgical services lead directly to adverse surgical outcomes. All too often, a lack of appropriate modern lasertripsy equipment, staff trained to operate it, or provision of adequate emergency theatre time leaves public patients languishing with a double-J stent (inserted as a temporising measure) for an extended period. Not treating stones definitively at presentation (in cases where surgery is appropriate) adds unnecessary morbidity and necessitates readmission, further compounding the inefficiencies of an already overstretched public system. Given such important findings, it is disappointing that the Journal chose only to publish Macneil’s study as a letter. The conclusions of this study should provide an impetus for addressing proper coordination and resourcing of surgical services in the public system throughout Australia.

Robert J Davies · R Denby Steele · John Kourambas

To the Editor: Access to timely, definitive management of kidney stones after initial short-term management in the New South Wales public hospital system has long been a source of frustration for urological surgeons and their patients. These patients will, for the most part, ultimately receive treatment that is successful. Although this may be reflected in final outcome data, the financial and personal costs associated with unacceptable delays between staged treatment episodes is unlikely to be documented. The assessment of kidney stone management by the Greater Metropolitan Clinical Taskforce reported by Macneil and colleagues highlights the types of problems that are endemic in NSW,1 and is based on strong input from clinicians who are not under pressure to manipulate data to give the most favourable assessment of the state of health care delivery. It has broader implications regarding appropriate and timely management of staged treatment of other acute conditions that require surgery — in particular, the treatment of urinary retention due to benign prostatic obstruction. Until now, the saving grace for the NSW Department of Health with respect to these issues has been a lack of resources and will to capture this information. Such information creates embarrassment regarding an inadequately resourced and organised approach to the staged care of acute conditions requiring surgery. Inadequate basic access to kidney stone treatment in the public hospital system is just the “tip of the iceberg”, and is one of many examples of the substantial divide that exists between access to optimal health care in public versus private hospital systems. The real state of health in NSW has been spared from exposure by a lack of resources and will to define the true extent of problems. It seems unlikely that the problems that have been exposed in the public hospital system are isolated to NSW.

Henry H Woo · Michael P Wines

To the Editor: Critics of the proposal to legalise trade in kidneys have pointed out the low rates of renal transplantation in Australia compared with the United States,1 where the trade in organs is also illegal. However, it is unclear if the lower rate of renal transplantation in Australia is a result of a shortfall in transplants from living or deceased donors. First, I ranked renal transplantation rates in 2005 in US states and from Australia using the numbers of transplants from deceased and living donors from the United States Renal Data System (Beth Forrest, Coordinating Center, US Renal Data System, National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, Md, personal communication), the Australia and New Zealand Dialysis and Transplant Registry,2 and populations from the US Census Bureau.3 In 2005, there were 12.1 per million population renal transplants from living donors in Australia. All of the states of the US except Oklahoma had higher rates of renal transplantation from the living, and the rate in Minnesota was four times higher. In contrast, the rate of renal transplantation from deceased donors in Australia was 18.6 per million population, which was higher than 24 American states and lower that 26 states and the District of Columbia. Some US states had twice the Australian rate of kidney donation from the deceased. I then used multiple linear regression to examine associations between rates of renal transplants from the deceased and rates of homicide, suicide and motor vehicle accident deaths in US states.4 Rates of renal transplantation from living donors were included as a covariable to control for differing degrees of development in US state transplant services. Homicide rates were associated with rates of transplants from the deceased (R = 0.607, R2 = 0.386; Box), but deaths from suicide and motor vehicle accidents were not. Hence, the higher rate of renal transplantation from deceased donors in the US appears to be the result of greater availability of organs from homicide victims. The high mortality and morbidity associated with endstage renal failure, despite renal dialysis treatment, suggests a need for new approaches to increasing the availability of donor kidneys. Assumed consent for organ donation might increase donation rates from the deceased, but this has been resisted in both Australia and the US on the grounds that failure to opt out might only indicate lack of forethought and not consent. While Australia continues with an opt-in system for deceased donation, measures to encourage live donation might have more realistic prospects of success than attempts to increase the rate of renal transplantation from the deceased.

Matthew M Large

To the Editor: A 60-year-old Hispanic woman, who had lived in the United States for 10 years, presented with a 1-day history of altered mental status. Physical examination revealed ascites and enlarged right axillary lymph nodes. Magnetic resonance imaging (MRI) of the brain showed multiple intracranial lesions (Box, A). Computed tomography of the chest and abdomen showed massive adenopathy in the right axilla, multiple nodules in upper lung fields, ascites and retroperitoneal adenopathy. Her cancer antigen (CA) 125 level was 1469 U/mL (reference range, 0–35 U/mL); CA27.29 and CA19-9 levels were within the upper limit of the normal ranges. She was initially thought to have metastatic cancer of unknown primary site. However, a right axillary node biopsy revealed necrotising granulomas and no malignancy; an acid-fast bacteria (AFB) stain was negative. Ultrasound-guided retroperitoneal lymph node biopsy showed necrotising granulomas and no malignancy; an AFB stain was positive. We began investigations for disseminated tuberculosis (TB). A QuantiFERON-TB Gold test (Cellestis, Valencia, Calif, USA) and sputum and right axillary node cultures were positive for Mycobacterium tuberculosis; peritoneal and cerebrospinal fluid cultures were negative. Polymerase chain reaction (PCR) of samples of peritoneal fluid and from bronchoalveolar lavage was negative for M. tuberculosis DNA, but a sample from the retroperitoneal lymph node tested positive. The patient was started on four-drug therapy for TB and her condition progressively improved. Follow-up MRI of the brain 5 months later showed a decreased size of all intracranial lesions (Box, B), and her CA125 level was 84 U/mL. Peritoneal TB can mimic advanced ovarian cancer because of similarities in clinical signs and symptoms, such as ascites, abdominal pain and elevated CA125 levels.1 The association of peritoneal TB with high CA125 levels was first described in 1987.2 The positive predictive value of CA125 levels to detect malignancy is estimated at 60%, rising to 98% in postmenopausal women.3-4 In most reported cases of peritoneal TB, CA125 levels were below 500 U/mL; rarely, levels up to 1200 U/mL have been seen.1-4 Culture is of limited clinical usefulness, as results take up to 6 weeks. Although microscopy is rapid, cheap and highly specific, its sensitivity has been shown to be as low as 31% for extrapulmonary TB.5 PCR is of limited value in diagnosing peritoneal TB. Detecting M. tuberculosis DNA by PCR in ascitic fluid poses many challenges — differences in technique, contamination with other bacteria, and the variable number of acid-fast bacilli in samples have been shown to influence its reliability.4-5 Negative results from microscopy, culture and PCR should not distract from a diagnosis of TB. In the face of a growing international incidence of TB, it is important to consider this transmissible and treatable disease in the context of abdominal symptomatology, ascites and raised serum CA125 levels — especially in ethnic groups in which TB prevalence is high. Misdiagnosis or delayed diagnosis can lead to infertility, premature menopause and death. Magnetic resonance (MR) imaging of the brain before and after treatment A: B rain MR image showing multiple intracranial lesions involving the left cerebellum, left occipital lobe, left parietal lobe and corpus callosum. B: MR image of the brain 5 months post-treatment, showing a decrease in the size of all intracranial lesions.

Riad O El Fakih · Bassem M Chehab · Rami A Mortada · Maha Assi

To the Editor: Keogh and Clifton’s call for salt reduction in the food supply1 comes when the National Heart Foundation is telling doctors who treat patients with hypertension that they should “recommend low-salt and reduced-salt foods as part of a healthy eating pattern”.2 Humans evolved while eating foods that (with rare exceptions) are low in salt — fresh fruit, vegetables and nuts, supplemented sometimes with fresh meat, poultry or fish — and these foods are still abundant. Using cooking methods that conserve flavour and any of about 300 herbs and spices, they make delicious meals that are low in fat, saturated fat, sugar and salt. Keogh and Clifton’s point is that we need more processed foods that are low in salt. This could be brought about if all doctors prescribed low-salt diets for their patients with hypertension.2 The food industry could hardly fail to respond to an unprecedented demand from the 3.7 million hypertensive Australians who need low-salt foods. Low-salt foods (sodium ≤ 120 mg/100 g) are easy to prescribe, requiring neither a diet nor a dietitian. In Britain, “traffic light” labels identify them at a glance, with green lights for salt. Australian consumers wishing to identify them have to check the mandatory sodium figure in the nutrition information panel provided on all processed food packages for a value of 120 mg/100 g or less. Further information to help consumers identify low-salt foods is available on the SaltMatters website (http://www.saltmatters.org), and a comprehensive handbook for practitioners and motivated patients has also been published.3 A low-salt diet is also particularly important for patients with Ménière’s disorder, because sodium excretion < 50 mmol/day is “more effective and less troublesome than diuretics” for controlling their vertigo.4 This level of sodium excretion occurs when all meals are low in salt.5 The National Heart Foundation’s marginally easier limit of 65 mmol/day allows room for a small mistake or a reduced-salt food, but practitioners must remember that the “combination of diuretic treatment and low dietary salt intake may result in unacceptable volume depletion and hyponatraemia”.6 The National Heart Foundation recommends 24-hour urine sample collections for monitoring dietary compliance (see “Measuring Salt Intake” at the SaltMatters website).

Trevor C Beard

To the Editor: We read with great interest Drini and colleagues’ recent report of hepatosplenic T-cell lymphoma (HSTCL) associated with inflammatory bowel disease. The occurrence of this rare lymphoma is partly driving a move away from the use of combination thiopurine and anti-tumour necrosis factor alpha therapy. It is important to recognise that risk of HSTCL is not only associated with exposure to thiopurine drugs with or without infliximab. It is associated with immunocompromise in general, and also occurs de novo. Explaining relative risks to patients is never easy and needs to be balanced with the need for treatment in properly selected patients. Contrary to a comment by Drini and colleagues, HSTCL has been reported in association with exposure to adalimumab, another anti-tumour necrosis factor alpha agent.2 Two of these cases occurred in patients with prior exposure to thiopurines and infliximab, and the third occurred in a patient with rheumatoid arthritis who was not exposed to thiopurine or infliximab. There has been a recent spate of reports of HSTCL in patients with inflammatory bowel disease treated with immunomodulator therapy, but most HSTCL appears to occur in patients without exposure to immunosuppressants.3 Also, although cases predominantly occur in young men (under 22 years), HSTCL is not limited to the paediatric age group.3,4 The presentation also occurs in females, including during pregnancy. More generally, in patients with inflammatory bowel disease, treatment with thiopurines appears to increase the risk of lymphoma two- to fourfold.5 Assuming a fourfold increase in risk, this translates into one additional lymphoma per year for every 4357 patients treated with thiopurines at age 20–29 years.5 This should be compared with the lifetime attributable risk of death from any cancer due to a single abdominal computed tomography scan performed at the age of 20, which is in the order of 1 : 2000.6 Currently, it is difficult to completely disentangle the many different factors — including patient age; severity, duration and course of disease;7 type of immunosuppression; and exposure to radiation — when considering the risk of lymphoma in inflammatory bowel disease. However, in many patients with severe disease, this risk appears to be outweighed by the benefits of adequate medical control.

Daniel C Burger · Timothy H J Florin

To the Editor: The article by Outram and Nair on peer physical examination1 misses the point that consent by medical students for physical examination by their peers can never be freely given. It always contains elements of coercion. Ethics committees usually do not allow medical students to enter clinical trials run by staff, who at some point may be their assessors, because there may be subtle duress to participate. Even if consent for peer physical examination is sought by staff who will be neither assessors nor tutors of these students, non-consenting students are immediately apparent to their colleagues, creating peer pressure to participate. The emotionally vulnerable are the most likely to accede to this and the most likely to be distressed. It is unsafe to rely on all staff seeking consent and behaving appropriately at all times. In one Australian medical school, students were told that they all had to “bring their swimmers” to wear so that they could be examined by other students. The statement that peer physical examination “has high acceptability”1 is not supported by the cited literature, and it is not clear to whom it is highly acceptable. There are good arguments for learning physical examination skills on the healthy. If young bodies are needed for this, universities are full of students from other faculties, and medical schools are not so poor they cannot pay the small amounts of money valued by these students for their participation.

John E Marley

In reply: Marley states that consent for peer physical examination “always contains elements of coercion”. It could be argued that this applies equally to medical practice and research, as one can never be 100% sure that participants have not felt some coercion. The ethical imperative is to balance the issues, to gain the best outcome. The article by Nair and myself reviewed the literature, reported additional research in the main area where difficulties had been noted (culturally and linguistically diverse students) and, on the basis of that evidence, suggested best practice.1 Contrary to Marley’s assertion, the articles we reviewed do support high levels of acceptability: 98%,2 97%,3 and 94%,4 respectively. Marley’s statement that emotionally vulnerable students are most likely to accede to peer physical examination and then be distressed may sound correct, but there is no evidence for this. In our experience, medical students are assertive, including those from the minority groups surveyed. Although the cost of direct payment to non-medical university students acting as “models” may be relatively small, the process of recruitment, training and processing by overstretched academic staff has significant opportunity costs. Additionally, medical students acting as models have the opportunity to experience the patient’s perspective. We acknowledge some criticisms of the practice of peer physical examination. However, it is currently widely used and will continue to be. The intent of our article was to draw attention to unforeseen difficulties and to improve practice.

Suzanne Outram

In developing its first seven standards for implementation by all health service providers in Queensland, the Health Quality and Complaints Commission (HQCC)1 considered the questions raised by Brand and colleagues2 regarding health care standards. We believe that the HQCC standards fit the framework suggested by Brand et al in that they are regulated, the measures of processes and outcomes are quantitative, and the criteria used for their development are evidence-based. In seeking to minimise conceptual confusion, the HQCC has “regulated” existing clinical guidelines and health standards with the aim of improving the quality of health services by requiring providers to establish systems to monitor and report on key aspects of care. The standards address the following areas: Review of hospital-related deaths; Management of acute myocardial infarction on and following discharge; Surgical safety, including antibiotic prophylaxis, prevention of venous thromboembolism, and correct surgery; Hand hygiene; Credentialling and scope of clinical practice; Complaints management; and The duty of health providers to improve the quality of care. The HQCC is now establishing a responsive regulatory framework to monitor compliance with the requirement for all health service providers to implement and maintain quality improvement processes. Brand and colleagues are critical of initiatives that burden providers with data collection and potentially distract from efforts to improve quality.2 But we contend that, without collection, analysis and review of data, the capacity to improve quality is limited. Since July 2007, the HQCC has required all acute care facilities to regularly report against the standards. Our intention is not to make the HQCC into a data repository, but rather to ensure that providers have the ability and the motivation to measure and monitor their own performance. Nevertheless, as a consequence, a unique dataset has been created that reflects wide variation across the state. This approach aligns with the model of metaregulation (or enforced self-regulation) and triple-loop learning espoused by Healy and Braithwaite.3 Ultimately, the purpose of health care standards is to improve the quality of care and drive a culture of quality improvement. Although the HQCC designed its compliance framework to evaluate the impact of its standards on quality and culture of the health system over an extended period, there are already promising indications of a positive effect after only 12 months.

Teresa A Lynne

To the Editor: The article by Statham and colleagues raises important issues about the accuracy of diagnosis by primary eye care providers, with all professionals in the study recording a diagnostic accuracy rate of less than 50%.1 From the general practice perspective, the authors raise a number of important contributors — lack of equipment, ophthalmological expertise and time. Additional factors, such as undergraduate and postgraduate exposure, and targeted training in the diagnosis of sight-threatening acute eye conditions, are also crucial considerations. From a postgraduate point of view, the Royal Australian College of General Practitioners offers a comprehensive continuing professional development program to support broad-based GP training, including in eye disease.2 The Master of Medicine (GP) offered by the University of Queensland also includes a dedicated subject on primary eye care, with particular emphasis on sight-threatening presentations.3 The Division of General Practice in which one of the sites in the report by Statham and colleagues1 sits is currently working with that hospital’s Department of Ophthalmology to institute an education/continuing professional development reform program to better target hospital eye referrals from primary care (Shelley Kleinhans, Health Systems Improvement Program Manager, GPpartners [Brisbane North] Division of General Practice, personal communication). It is very important to describe accurately the dimension of missed diagnosis within primary care — the ensuing challenge is to address it by harnessing the significant momentum within the primary care community for quality improvement.

Claire L Jackson

A good death. An argument for voluntary euthanasia. Rodney Syme. Melbourne: Melbourne University Press, 2008 (xviii + 301 pp). ISBN 978 0 522 85503 6. In facing the management and termination of intolerable situations, a caring doctor involved in alleviating the final agonies of patients can berate the legal system’s impotence and governmental procrastination. Oncologists are frequently confronted by situations involving the end of a painful existence. Most are now assisted by specialists in palliative care, often disempowered by shortages of beds and frustrated by staff inadequately trained or less than sympathetic to the views of those who would like to see “physician-assisted by specialists in palliative care, often disempowered by shortages of beds and frustrated by staff inadequately trained or less than sympathetic to the views of those who would like to see “physician-assisted dying” as an alternative to only partially effective, cerebrally numbing analgesia. Cancer, of course, is not the only cause of a prolonged and tormented end to life. Some of the most distressing cases are associated with the neurological prisons that mean a fully conscious person is cut off from mobility, self-care, sensory input and communication. In a deeply personal, well researched and detailed book, Rodney Syme relates his own experiences which, over 30 years, have honed his ideas of how best to achieve the relief requested by the sufferer. Conditions that have to be met include a clear, unequivocal request to die by the patient. This is extremely difficult when communication has become impossible, and points to the need for a “living will”, assigning that responsibility to a nominated person. Nevertheless, euthanasia on demand is not his aim. Rationality is paramount, as is the physician’s responsibility in the decision process. Syme lays emphasis on the need to allow relatives and/or close associates the opportunity to say farewell and discusses the reasons for recommending the methods he does. He makes it clear why he has never adopted the execution-style injections of sedatives, analgesics and cardio-respiratory paralytics adopted in other countries. Although this is scarcely a textbook of suicidal methods or a scientific treatise, it is a valuable addition to the discussion of the subject. It may help medical practitioners to know that others have had to go through the same convolutions of conscience, and be of comfort to family members to follow the thoughts of a clearly caring doctor.

Thomas F Sandeman

Biting anorexia. A first-hand account of an internal war. Lucy Howard-Taylor. Sydney: Finch Publishing, 2008 (ix + 214 pp). ISBN 9781876 451929. I undertook reviewing this book with some trepidation. I presumed it would be yet another distant “tortured” account of anorexia. I found it quite the opposite. Lucy is an 18-year-old Australian university student. Her depiction of her mental state in anorexia is quite extraordinary. I know that anorexia affects the brain negatively, as a result of both malnutrition and the pervasive thinking disorder. It isn’t until the later chapters that you realise the extreme blunting of her intellect that has occurred. Lucy’s diary is an extraordinarily honest, most intelligent account detailing the process of her illness and pathway towards recovery. I was particularly struck by the difficulties she experienced at all times, and by the depth and sophistication of her thoughts and the enormity of the struggle she went through in attempting to overcome her illness. This book really is the most lucid document and one I have started to recommend to colleagues and to patients. It gave me an insight I had not expected to gain into the extent of the damage done by this illness and the extreme difficulties endured to overcome it.

Simon D Clarke

Paul Large was born in Beira, Mozambique, on 12 August 1920 and brought up in Durban, South Africa. He was educated at Michaelhouse, in rural Natal, and enrolled at Guy’s Hospital Medical School, London, in 1938. During the war, many Guy’s Hospital patients, staff and students were evacuated to Kent and Sussex because of the German bombing. Paul and other students were strained by decentralisation, by Home Guard and air raid precaution duties, and by a disjointed curriculum. After graduating in 1943, Paul joined the South African Air Force, but returned to Guy’s in 1946. He distinguished himself as a teacher and qualified for Fellowship of the Royal College of Surgeons (London) in 1948 and a Master of Surgery in 1952. Paul returned to Durban in 1953 to work in private surgical practice. He also did honorary work at McCord Hospital and Durban’s fledgling medical school. Both of these institutions catered for people of dark skin. Educating underprivileged South African students of Bantu and Indian origin was in keeping with his compassionate philosophy of life. In 1958, disillusioned with the political situation in South Africa at the time, Paul migrated to Melbourne, Australia, where he built up a solid private and hospital practice. A staff surgeon for many years at the Western General Hospital, he taught many medical students and junior doctors and always showed deep concern for his patients. In 1966, 1969 and 1972, Paul served (as leader) with civilian Australian surgical teams in battle areas in rural South Vietnam. With guerrilla war in progress, much of the surgery dealt with battle trauma. Although dedicated to his profession, Paul’s family and home life were all-important to him. He was erudite, civilised, inquisitive, charming, humorous, kind and selfless. He had a passion for history and a love of the English language. His electoral choices were shaped by the personal qualities of candidates, rather than political parties, and his disgust at exploitation of the weak. He retained a boyhood enthusiasm for dinghy sailing, and was involved in building, racing and administration. He also followed international rugby, retaining a keen interest even as he became more infirm. Paul’s final years were of gentle physical decline until death from an embolic blockage of a coronary artery on 24 October 2008. He is survived by his wife Susan and children Peter, Susanna, Richard, Catherine and Jonathan.

Anthony V Large

A 28-year-old man with no past ophthalmic history presented with sudden loss of left central vision after dancing vigorously at a night club. On examination, visual acuity in the left eye was 6/60. Fundoscopy revealed a well circumscribed, dense preretinal haemorrhage obscuring his left fovea (Figure A). Optical coherence tomography confirmed the haemorrhage to be located just under the internal limiting membrane (Figure B). Conservative management was applied, and the patient’s vision returned to 6/6 after 3 months. First described by Duane in 1972,1 Valsalva retinopathy is now a well recognised cause of retinal haemorrhage that typically occurs in association with strenuous activity and the Valsalva reflex.

Henri Sueke

Re: “National mental health reform: less talk, more action”, by Sebastian Rosenberg, Ian B Hickie and John Mendoza, in the 16 February 2009 issue of the Journal (Med J Aust 2009; 190: 193-195). On page 193, in the second column, it was stated that “the federal government has only managed to spend $87.3 million of the $1.9 billion allocated . . . or 6.6% of the total to be spent”. The percentage should have been 4.6%, not 6.6%. The error only appeared in the printed edition. The html and pdf versions of the article published in the eMJA were correct on publication.

Sebastian Rosenberg · Ian B Hickie · John Mendoza

Can sick tonsils stunt growth? Children who experience chronic sleep disordered breathing (SDB) because of adenotonsillar hypertrophy may present with retarded growth, say UK and US researchers. In a systematic review and meta-analysis of 20 studies, the authors analysed the outcomes pre- and post-adenotonsillectomy for standardised height and weight, and the serum biomarkers insulin-like growth factor 1 (IGF1) and IGF binding protein 3. The results showed that height, weight and both serum biomarkers increased significantly after adenotonsillectomy. The authors comment that there are some limitations to the study, including a paucity of literature, and no suitable trials of surgery versus no surgery (“watchful waiting”) were identified for inclusion in the analysis. They also discuss problems inherent in the use of serum biomarkers, for which it is difficult to find accepted reference ranges for healthy children. Despite these limitations, the researchers conclude that their findings support the association between SDB and growth failure, and that the condition should be considered by clinicians in their investigations for growth failure and short stature in children. Arch Dis Child 2009; 94: 83-91 Maternal obesity and birth defects Maternal obesity appears to be associated with some congenital abnormalities, according to the results of a systematic review and meta-analysis conducted by UK researchers. Observational studies estimating maternal weight or body mass index and birth anomalies were included in the analysis. Obese mothers were at increased odds of pregnancies affected by several congenital abnormalities including neural tube defects, cardiovascular anomalies, cleft palate and hydrocephaly. The authors comment that although larger, population-based studies would shed more light on the issue, the health implications of the study are considerable, particularly considering the rising prevalence of obesity around the world. JAMA 2009; 301: 636-650 Support for ankle sprains Severe ankle sprains are common, but there is surprisingly little consensus on the best treatment for these injuries, which can result in considerable morbidity. A randomised controlled trial conducted by UK researchers recruited over 500 participants with severe ankle sprain from hospital emergency departments. Patients were provided with mechanical support for the injury — Aircast brace, Bledsoe boot, or 10-day below-knee cast. Quality of ankle function at 3 months was the primary outcome. When compared with a compression bandage, the below-knee cast resulted in more rapid recovery, abatement of pain and other symptoms, and improved activity and quality of ankle function at 3 months. The Aircast brace improved ankle function at 3 months compared with the bandage, but did not make a significant difference to pain, symptoms or activity. At 9 months, there was no difference between treatments or in the incidence of adverse events. The authors recommend the use of a 10-day below-knee cast or Aircast brace for severe ankle sprains. Lancet 2009; 373: 575-581 HRT risks reduce over time The increased risk of breast cancer observed in postmenopausal women taking oestrogen and progestogen declines rapidly after discontinuation of therapy, say researchers analysing the findings from the US 2002 Women’s Health Initiative (WHI) trial. In the original trial, one group received a combination of conjugated oestrogen and medroxyprogesterone daily; a second group received a placebo. The trial was stopped after 5.6 years when the risks of combined therapy, which included a higher incidence of breast cancer, were found to outweigh the benefits. Breast cancer diagnoses, frequency of mammography, and risk factors for breast cancer were assessed in the trial participants after cessation of the trial. The researchers also analysed data from over 41 000 women involved in a separate WHI observational study with similar eligibility criteria to the clinical trial, including about 16 000 women taking combined hormonal therapy. Temporal trends in breast cancer diagnoses were examined in this group. In the clinical trial cohort, the elevated risk of breast cancer decreased rapidly after the participants stopped taking the combined therapy, despite a similar frequency of mammography. In the observational study, those taking hormone treatments had an incidence of breast cancer that was initially twice as high as in the non-treatment group, but this also decreased rapidly over 2 years; there was no difference in the frequency of mammography between these groups. The authors suggest that the effect of combined hormone therapy on breast cancer risk is time-limited and not explained by changes in frequency of mammography. N Engl J Med 2009; 360: 573-587

Tanya Grassi