Child health

This state-wide Aboriginal community child health survey, the first of its kind in Australia, describes physical and mental health and their antecedents in Western Australian Aboriginal children and young people. Aboriginal young people had significantly more physical and mental health problems and were more likely to engage in lifestyle risk factors than non-Aboriginal young people. Aboriginal young people tend to be caught up in a cycle of disadvantage that includes family and community factors as well as recent history, facilitating their making less optimal life choices, thereby perpetuating the cycle. A coordinated approach will be required to break this cycle, in which appropriately and sympathetically provided medical attention is necessary but not sufficient.

Eve M Blair PhD · Stephen R Zubrick PhD · Adele H Cox DipAppSci

In Australia, where about 16% of young people are born overseas and 24% are from a non-English-speaking background, adolescent health care is a multicultural challenge. “Cultural competency” involves challenging one’s own cultural assumptions and beliefs, developing empathy for people from other cultures, and applying specific communication and interaction skills in clinical encounters. For health professionals, sensitivity to the cultural, ethnic, linguistic and social diversity among young people helps to avert problems and misunderstandings, improves satisfaction for all concerned and leads to better outcomes. Engaging the family and gaining the trust of parents is critical in treating young people from cultural backgrounds in which participation in health care is a family concern rather than an individual responsibility.

David L Bennett MB BS, FRACP, FSAM · Peter Chown BSc(Psych), MAPS(Hons) · Melissa S-L Kang MB BS, MCH

Dagmar M Haller,* Lena A Sanci,† George C Patton,‡ Susan M Sawyer§ * General Practitioner and PhD Candidate, ‡VicHealth Professor and Director of Adolescent Health Research, §Professor of Adolescent Health, and Director, Centre for Adolescent Health, Murdoch Children's Research Institute and The University of Melbourne, 2 Gatehouse Street, Parkville, VIC 3052. †Senior Lecturer, Department of General Practice, The University of Melbourne, Melbourne, VIC. dagmar.hallerATmcri.edu.au To the Editor: We recently advocated the inclusion of a mature-minor clause in the National Health and Medical Research Council ethics guidelines on teenagers’ participation in research.1 This would allow minors (teenagers younger than 18 years) who show sufficient maturity and understanding to consent to participation in minimal risk research (eg, observational studies and quality assurance studies) without requiring parental consent. This is important in primary care research, as teenagers presenting to general practitioners without an accompanying parent or carer frequently wish their visit to remain confidential. To exclude this group from research would deny them the benefit of potential improvements to their health care as a result of research. As no such data were available in Australia, we sought to document, as part of a larger primary care study of young people’s perspectives on their health problems and their expectations from a GP consultation, the proportion of teenagers aged 16 to 18 years who potentially would not be able to participate because they presented without a parent. We also documented whether, when present, parents would express concern about not being asked to provide consent for their teenaged child to participate. We recruited up to 20 consecutive patients aged 16 to 24 years in each of 26 randomly selected practices throughout Victoria and invited them to participate in an interview on their perspectives on the health problem for which they had come to see the doctor and their expectations from the consultation. Our institutional ethics committee approved inclusion of minors aged 16 to 18 years without parental consent. One of the authors, a GP (D H), obtained consent from participants and conducted the interviews. Results are reported with 95% confidence intervals adjusted for clustering within practices. Of the 501 young people approached, 101 were minors. Five minors (5%; 95% CI, 0.6%–9%) were excluded (too unwell, intellectually disabled or non-English speaking), and another five (5%; 95% CI, 1%–8%) declined participation. Of the 91 minors who consented to participation, 40 (44%; 95% CI, 35%–53%) had come without a parent. Although 37 of the parents of the 51 minors who were accompanied (73%; 95% CI, 59%–86%) went into the GP consulting room with their children, only one was concerned about her child consenting and participating in an interview on his own. Had parental consent been mandatory, nearly half the patients aged 16 to 18 years could not have been included in this primary care study. When present, the overwhelming majority of parents did not disapprove of their children consenting and participating on their own. These findings support the idea that, for low-risk studies in primary care, mature minors should be given the opportunity to consent to participation on their own.

Dagmar M Haller · Lena A Sanci · George C Patton · Susan M Sawyer

Graham D Hocking Child Psychiatrist, 25 High St South, Kew, VIC 3101. ghockinATiprimus.com.au To the Editor: Catto-Smith gives a very good account of the medical management of constipation and soiling in children,1 but fails to mention psychological, interpersonal and social factors in the main part of his article. He does mention “behavioural abnormalities” towards the end, in the section “When to refer”. I think it is generally accepted among paediatricians and child psychiatrists that the problem of constipation and soiling, or encopresis, often has multiple determinants and varied psychological effects on the child and the family. Certainly, the older the child is, the more likely these effects will be present.2 If this condition is to be managed in general practice over a period of “6–12 months”, general practitioners need to be aware of these factors so they can be addressed. Twelve months is a long time in the life of a 5 year old, and in that time pathological patterns can become well established and hard to shift. Most children over 5 years with soiling have developed secondary psychological problems as a result of the soiling.3 At this stage, assessment by a child psychiatrist will often reveal that the child has developed a pathological fantasy world around what they believe is happening inside them. The physical management of constipation and soiling is an essential part of the management no matter what the aetiology, but addressing the psychological interpersonal and social factors is equally important. If these factors are obvious to the GP and are not responding to intervention, the family should be referred to a child psychiatrist. With children over 5 years, the secondary effects have almost always become significant, and I believe that all these families should be referred for assessment. Catto-Smith quotes a 30%–50% relapse rate,1 and “there is evidence that they do not improve on reaching puberty”. This is not my experience with families that have the benefit of a multidisciplinary approach to the disorder.

Graham D Hocking

Anthony G Catto-Smith Director, Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Flemington Road, Parkville, VIC 3052. tony.cattosmithATrch.org.au In reply: Hocking emphasises secondary behavioural and emotional effects that occur in some children with longstanding faecal soiling. Fortunately, there is good evidence that these tend to resolve with effective multimodal treatment of the constipation.1 The relatively high long-term relapse rate of soiling among children who have been treated in tertiary centres has only recently been recognised,2 but the psychological features of this relapsing group are not well defined. My review was directed toward general practitioners and was as much as possible evidence-based. I am unaware of any good quality evidence to support Hocking’s assertion of the benefits of automatic referral to a child psychiatrist of all children over the age of 5 years with ongoing faecal soiling. Given the beliefs of both myself and Hocking, that constipation and soiling are likely to have multiple determinants and varying psychological effects, it would seem to be appropriate to triage “problem” patients through a general paediatrician, with referral for psychological assistance if deemed appropriate. This is best summed up in my article in the section that Hocking mentions, “When to refer”.3

Anthony G Catto-Smith

Objectives: To determine the incidence of childhood type 1 diabetes mellitus (T1DM) in New South Wales from 1997 to 2002; to compare with previously published rates (1990–1996); and to analyse trends in incidence from 1990 to 2002.Design, setting and participants: Prospective population-based incidence study. Primary ascertainment of incident cases aged < 15 years was from the Australasian Paediatric Endocrine Group NSW children’s diabetes register. Secondary ascertainment was from the National Diabetes Supply Scheme until 1999 and from the Australian Institute of Health and Welfare thereafter. Childhood population data were obtained from the Australian Bureau of Statistics.Main outcome measures: Age-standardised incidence; trends in incidence by calendar year, and sex and age at diagnosis.Results: There were 3260 incident cases (1629 boys, 1631 girls) in the 13 years. Case ascertainment was 99.7% complete using the capture–recapture method. Mean age-standardised incidence per 100 000 person-years was 20.9 (95% CI, 19.9 to 21.9) from 1997 to 2002 compared with 17.8 (95% CI, 17.0 to 18.7) from 1990 to 1996; there was a plateau in incidence between 1997 and 2002. Overall, the incidence increased on average by 2.8% per year (95% CI, 1.9% to 3.8%, P < 0.001) and increased with age, being 12.2 (95% CI, 11.3 to 13.1) in 0–4 year olds; 18.9 (95% CI, 17.8 to 20.0) in 5–9 year olds and 26.7 (95% CI, 25.4 to 28.1) in 10–14 year olds. The increase per year in 0–4 year olds (3.9%) was not significantly higher than in older children. The mean incidence of T1DM was 19.8 (95% CI, 18.8 to 20.7) in girls and 18.8 (95% CI, 17.9 to 19.7) in boys (P = 0.02).Conclusions: The incidence of childhood-onset T1DM has increased significantly in all age groups in NSW since 1990. Resource planning in the management of childhood diabetes in NSW should take these findings into account.

Craig E Taplin MB BS · Maria E Craig PhD, FRACP, MMed(ClinEpid) · Margaret Lloyd RN · Martin Silink AM, MD, FRACP · Neville J Howard FRACP, FRCP · Claire Taylor MB ChB · Patricia Crock FRACP

Andrew S Kemp Professor, Allergy Immunology and Infectious Diseases, The Children’s Hospital at Westmead, Locked Bag 4001,Westmead, NSW 2145. andrewk5ATchw.edu.au To the Editor: Publicity such as that on the recently televised “Sunday” show (Channel 9) entitled “When food can be fatal” (http://sunday.ninemsn.com.au/sunday/cover_stories/transcript_1770.asp), which contained statements that “30 in every 1000 [3%] children in Australia are at risk of a severe allergic reaction [anaphylaxis] to a food”, and a reference to a “tsunami of children” with serious allergies, provokes understandable concern and anxiety. Some perspective on this issue is required. To determine the risk, it is essential to study a population-based cohort. Allergies to peanuts or tree-nuts are the most common cause of severe childhood food anaphylaxis and death.1 What is the risk for Australian children of peanut-induced anaphylaxis that is likely to require adrenaline? Of a population-based cohort of 456 Tasmanian children aged 7–8 years, none reacted to a peanut skin-prick test.2 In the Australian Childhood Asthma Prevention Study (CAPS),3 a high-risk cohort, 4.9% of 3 year olds were prick-test positive to peanut (unpublished data) using a liberal cut-off of ≥ 2 mm (for clinical testing the usual cut-off is ≥ 3 mm). Perhaps the most helpful information comes from a population-based study of 13 971 preschool children in the United Kingdom who were followed from birth to 6 years of age. Forty-nine (0.35%) children had an allergic reaction to peanut, of whom only two (0.014%) had what was described as anaphylaxis.4 Thirty-six of the children underwent formal peanut challenge, 23 reacted and three had reactions for which adrenaline was given. Combining these three with the previous two gives a severe reaction rate requiring adrenaline of 0.036%. This suggests that, of the 49 children in the UK study who had an allergic reaction to peanut, only 10% were at risk of a severe reaction requiring adrenaline. Only a third to a half of children with a positive peanut skin test will react if exposed.5 Applying these considerations to Australian children indicates that the proportion at risk of a severe peanut reaction is only 0.25% (4.9% × 1/2 × 5/49) even in a high-risk cohort such as the CAPS. This would be substantially lower in a population-based cohort. For the cohort of 7–8-year-old Tasmanian children referred to above, the risk would be much less than 0.2%, considering none of 500 children was prick-test positive to peanut allergen. There has been a substantial increase in childhood food allergy in recent decades;5 however, sensationalist statements and inaccurate figures are unlikely to be helpful in developing appropriate responses. The Australasian Society of Clinical Immunology and Allergy recently published guidelines for the prevention of food anaphylactic reactions,6 and has other useful information for patients and medical practitioners on its website (http://www.allergy.org.au/).

Andrew S Kemp

Re: the letter “Reliability of parental reports of head lice in their children”, by Megan L Counahan, Ross M Andrews and Rick Speare, in the 7 February issue of the Journal (Med J Aust 2005; 182: 137-138). There was an error in Box 2, Sensitivity and specificity of parental report versus screening. The specificity given as 94.0% (969/1030) should have been 98.8% (1018/1030). The html and pdf versions of this article published online were corrected on 5 August 2005.

Megan L Counahan · Ross M Andrews · Rick Speare

There is growing recognition in Australia of the importance of early childhood to later health and wellbeing, with developments such as the National Agenda for Early Childhood and the National Public Health Action Plan for Children. To sustain a policy agenda for children and improve long-term outcomes, we need timely, comprehensive and accurate indicators and data on child health, development and wellbeing. Building this evidence requires a national monitoring and surveillance system that involves more than aggregating or linking existing data. Steps to building a national system are: to agree on key indicators of child health, development and wellbeing for regular reporting, to research a comprehensive set of indicators for each domain and ascertain data gaps, and to ensure development and coordination of data relevant to policy-making.

Sharon R Goldfeld FRACP, PhD · Frank Oberklaid FRACP, MD

Caroline F Finch Director, New South Wales Injury Risk Management Research Centre, University of New South Wales, Level 8, Applied Science Building, Sydney, NSW 2052. c.finchATunsw.edu.au To the Editor: I am prompted to write to you in response to a recent MJA editorial.1 It amazes me that the health sector in Australia, as I think the editorial did, continues to largely ignore the magnitude of the problem of injury in our children. This is despite clear evidence of the excess ill-health burden that injury places on our children, according to the Australian Institute of Health and Welfare (AIHW) report (the subject of the editorial)2 and other reports.3-5 Having said this, the editorial did highlight a very pleasing trend — there has been a steady decline in injury deaths in later childhood. Unfortunately, however, this was the only mention of injury in the editorial, and readers could be forgiven for thinking that this is the end of the story: the injury death rate is declining; therefore, we are doing all we can, and injuries are not a major issue. Nothing could be further from the truth. Our children continue to die from road and drowning accidents and will do so until injury prevention is recognised as paramount. The AIHW report clearly states that the single highest cause of death in children remains injury and poisoning.2 Accordingly, trauma is the single highest contributor to premature mortality and years of potential life lost of any health condition in Australia. If we don’t develop new approaches to reducing the incidence of drownings and road deaths, in particular, we will not see further declines in injury-related death rates, and injury will continue to rate highly as a killer of young people. Importantly, injuries do not only kill young people — they also hospitalise and maim them. The second most common reason for hospitalisation in Australian children is injury.2 Unlike injury deaths, there has been no trend in the rate of hospitalisation for injury. Across age groups, there appears to be a shift from fatalities to an increasing number of people with a high lifetime burden of significant disability, including brain and spinal cord damage. Imagine what this does to the quality of life and life expectancy of a child. How many of these children will be able to lead physically active lives? It is time for the health sector, particularly public health agencies, to properly recognise injury as a critical issue for the ongoing health of Australian children and to formally commit to appropriate preventive actions, commensurate with the priority ranking of childhood injuries.

Caroline F Finch

George C Patton,* Sharon R Goldfeld,† Indrani Pieris-Caldwell,‡ Meredith Bryant,§ Graham V Vimpani¶ * VicHealth Professor of Adolescent Health Research, Centre for Adolescent Health, Murdoch Childrens Research Institute, Flemington Road, Parkville, Melbourne, VIC 3052; † Paediatrician and Research Fellow, Royal Children’s Hospital, Melbourne; ‡ Senior Analyst, § Project Officer, Australian Institute of Health and Welfare, Canberra; ¶ Clinical Chair in Paediatrics, University of Newcastle, NSW. george.pattonATrch.org.au In reply: There is little to disagree with in this excellent summary of injury morbidity and mortality in Australian children. However, the principal point of our editorial1 was to highlight important problems where adequate data are currently unavailable. The Australian Institute of Health and Welfare report was able to give extensive coverage to injuries and accidents in children.2 Indeed, seven indicators specifically addressed aspects of childhood injury, with a range of others (eg, child abuse and neglect, neighbourhood safety) addressing relevant aspects of the family and social context. This emphasis reflected not only the importance of childhood injury, but the extent to which reasonably good data are available. We agree that, despite some favourable mortality trends, the burden of childhood injury remains high, as are associated health care costs. However, childhood injury is an area where advocacy has translated into action.3 One of the reasons for the success of that advocacy has been the availability of sound data, both to make the case and to ensure an appropriate focus in policy responses.4 While there is undeniably much more to do, we can learn much from injury prevention about how to tackle the newly emerging problems of childhood.

George C Patton · Sharon R Goldfeld · Indrani Pieris-Caldwell · Meredith Bryant · Graham V Vimpani

Although children in Australia generally have good health, some alarming indicators of poor health and wellbeing exist, which are related to major socioeconomic discrepancies. The pathways connecting socioeconomic disadvantage to child health outcomes are complex and poorly understood. Reducing social disadvantage requires strategies beyond the health arena, involving political, moral, cultural and economic initiatives. Developing “social capital” — cohesion in communities, a sense of belonging and involvement in community affairs — may be a key strategy in improving health indicators. Overseas studies of early intervention and home visiting programs in early childhood have shown improvements in child health and development outcomes. Similar programs have been introduced in Australia and face considerable challenges in their widespread roll-out and evaluation. Health professionals need to develop practical ways to interact with community programs and thus improve social capital.

Karen J Zwi FRACP, MRCP, MSc · Richard L Henry MD, FRACP

Peter W Holmes,* David Armstrong,† Nicholas Freezer‡ * Deputy Director, Adult Respiratory Medicine, † Director, Paediatric Cystic Fibrosis Unit, ‡ Director, Adult and Paediatric Respiratory Medicine, Department of Respiratory and Sleep Medicine, Monash Medical Centre, Locked Bag 29, Clayton, VIC 3168. peter.holmesATsouthernhealth.org.au To the Editor: We congratulate Lam et al1 for identifying the major problems in transferring adolescents from the Royal Children’s Hospital, Melbourne, to adult care. The article and the accompanying editorial2 address a difficult problem relating to the transfer of adolescent patients from a stand-alone paediatric hospital to adult services. Lam et al conclude that there needs to be a change of attitude among adult physicians, and recommend the provision of additional resources to enhance the smooth transition to adult care. As long as paediatric services remain geographically separated from their adult counterparts in stand-alone hospitals, these problems will continue, regardless of any increase in resources. In New South Wales, tertiary paediatric services have now been incorporated onto the same campus as tertiary adult hospitals in shared-site arrangements. This facilitates the transition process, as adult physicians are more closely linked to their paediatric colleagues via shared clinical and research infrastructures. Such close cooperation allows paediatric and adult physicians to share their care during transition and provides the adult physicians with full access to the patients’ medical records and radiology, microbiology, laboratory and pulmonary function data. At Monash Medical Centre, we have taken this further by totally incorporating our adult and paediatric services into one single Department of Respiratory and Sleep Medicine. This arrangement allows an integrated approach to childhood, adolescent and adult care. The combination of services generates trust between all members of staff (an issue raised in the editorial2) and gives adult physicians a greater understanding of the needs of adolescents with complex health problems. One solution to the difficult problem of transition to adult care is to phase out stand-alone paediatric services with their own costly management infrastructure. A shared campus arrangement allows greater integration of the full range of tertiary paediatric and adult services and offers many advantages in providing a seamless transition to adult care.

Peter W Holmes · David Armstrong · Nicholas Freezer

Canteens are not the main source of food for Australian school kids, but their symbolism is big There is widespread awareness of the obesity epidemic in Australian children,1 and the focus has now, quite appropriately, turned to action. In the United Kingdom, celebrity chef Jamie Oliver is trying to transform a 100-year-old school lunch service from “soggy and fried” to “crisp and fresh”. In Australia, the question is whether school canteens should be a high priority for action, because of their accessibility and visibility, or a low priority, on the grounds that canteen foods contribute little to children’s energy intake. Negative ripples from canteensOver the period of a year, children aged 5–15 years obtain only about 16% of their total energy intake from food eaten at school, and probably less than 3% comes from canteens.2 But while the energy contribution is small, the symbolism is big. Canteen users consume significantly greater amounts of foods likely to promote unhealthy weight gain, such as fast foods, confectionery and packaged snacks.2 The types of foods and beverages that predominate in school canteens not only undermine the health and nutrition curriculum, but also create the impression that foods and drinks that are high in fat, sugar and salt belong on the plate as “everyday foods”, rather than on the side as “occasional foods”. Other common practices in schools that undermine healthy eating messages include rewarding children with sweets, having soft-drink and confectionery vending machines, holding sporting events with fast-food vouchers as prizes, and using chocolate drives for fundraising. All these practices create negative ripple effects on Australian family eating practices and beliefs.3 Children are developing the food preferences that they will carry with them into adulthood, so strengthening family and school environments for enjoying healthier food choices is critical. In a 2004 survey of 18 Victorian primary schools (unpublished data), we found that, of the 17 with a food service, all sold meat pies, but only five sold fruit on a regular basis. As a rule, canteen managers provided foods that sold well and had a long shelf life. They usually had no mandate or support to do otherwise. A reliance on profits from canteens, vending machines and “junk food fundraising” also makes it hard for schools, particularly high schools, to model healthy eating. In common with a survey of 500 New Zealand schools,4 we found that schools readily recognise the rather poor job they do of providing a healthy food environment. Most schools do not see food provision as part of their core business and lack the inclination or resources to take on this “added” responsibility. Private enterprise fills this vacuum, with the result that the health of profits increasingly dominates the health of pupils. An extreme example is the “cola war” in the United States, in which the weapons of choice have been contracts with schools to sell minimum volumes of Coca Cola or Pepsi.5 Creating a new epidemic?Perhaps we should consider the task ahead of us as the creation of a new epidemic of healthy eating rather than reducing an obesity epidemic. Using the principles in Gladwell’s recent bestseller The tipping point,6 the school canteen and students themselves could be the catalyst for healthier eating among children and adolescents — turning negative ripples into positive waves. Can a “tipping point” be created from a handful of champion schools that decide to embrace the whole-of-school policies and strategies needed to get their canteens right (healthy, enjoyable, profitable and supported), hoping that others will follow their lead? This is almost certainly too much to expect to happen in 9000 schools across Australia, which tend to function semi-autonomously on these matters. Lessons from successful public health programs, such as sun protection and injury prevention, show that tipping the balance in targeted behaviours from unhealthy to healthy requires a backbone of strong central policy, ongoing social marketing, and supported and coordinated implementation of programs. Some Australian modelsVarious government-supported models influence how school canteens operate in Australia. Probably the least effective include the Victorian model of simply disseminating canteen guidelines7 and the Australian Government model from the pre-election spending spree, wherein each school could apply for $1500 to reinvent the “healthy canteen” wheel. Neither has policy, social marketing or implementation support. A third model, which has some merit, is exemplified by the Western Australian (StarCAP8) and Tasmanian (Cool CAP9) school canteen accreditation programs. Both have well developed criteria and processes for schools to work through to achieve program accreditation. StarCAP is backed by the WA government, but is managed on a shoestring budget, without policy and social marketing support, and thus has a low accreditation rate (7% of schools) and declining reach.10 Cool CAP is newer, with a higher accreditation rate (42% of schools accredited or working towards it), and so far has been successful in securing legislative and monetary support. Ultimately, however, the impact of these types of programs will probably be modest as long as the impetus to change remains with each individual school. Because they are well supported and centrally driven, the most promising models come from New South Wales and South Australia. The NSW Healthy School Canteen Strategy (“Fresh Tastes @ School”)11 grew out of the NSW Government Childhood Obesity Summit in 2002. It is now mandatory for state schools to provide food and beverage choices consistent with the Australian guide to healthy eating.12 NSW Health has also boosted support for the NSW Canteen Association so that it, in turn, can support schools to operate economically viable, nutrition-oriented school canteens. Early positive waves include support from parents, canteen managers, some food companies and, increasingly, local health and education services. A similar model released in 2004 in South Australia brings SA government backing to a set of healthy eating guidelines.13 The guidelines encourage links between the canteen, the community and teaching about nutrition food skills. Both the NSW and SA government models would now benefit from social marketing explaining the rationale, processes and support for the program. It would make sense, for example, to link these strategies with the national “Go for 2&5” (2 serves of fruit and 5 serves of vegetables) campaign.14 If we are serious about the childhood obesity epidemic, school canteens are a good place to start, because they carry a symbolism that ripples into the Australian diet far beyond their contribution to energy intake. At the moment, the ripples are a negative and undermining force. However, full implementation of the NSW or SA models for school canteens throughout the country could just tip the balance towards an outbreak of healthier eating.

A Colin Bell BSc(Hons), MSc, PhD · Boyd A Swinburn MB ChB, MD, FRACP

Brain development from late pregnancy to 3 years of age affects a child’s learning, behaviour and health throughout life. Behavioural difficulties in children are usually symptoms of underlying problems. Observing a child’s appearance and performance, and taking a detailed history (considering factors in the child, the home, the school and the wider environment) provide most of the information needed for diagnosing behavioural problems. It is important to know what is “normal” for all stages of a child’s development, but equally important not to confuse behavioural difficulties with normal variations and behaviours associated with developmental stages. Assessment and early intervention for behavioural and learning difficulties in children require a multidisciplinary team approach. As well as the recommended “multimodal” approach for managing attention deficit hyperactivity disorder, shared care with a general practitioner is available in some states, but medication is likely to be the trigger for a positive outcome.

Trevor S Parry FRACP, DCH, DPH, FRACMA

Fractures in children are common, but the plasticity of children’s bones means that they may be incomplete. If a child has deformity, swelling or bony point tenderness in a limb after a fall, it is likely to be fractured. A fractured limb that appears deformed will most probably need to be reduced. Effective splinting, using whatever means is readily available, and early, adequate analgesia, can ameliorate the severe pain associated with a fracture. In young children with open growth plates, Salter–Harris type I injuries of the distal fibula are more common than ligament injuries of the ankle. After an ankle ligament injury, functional treatment — brace or tapes, with active physiotherapy — results in a better outcome than immobilisation. A child with a head injury, who does not lose consciousness, has only one or no episodes of vomiting, and is stable, alert and interactive, and neurologically normal, is extremely unlikely to have sustained an intracranial injury.

Simon J Young MB BS, DipCrim, FACEM · Peter L J Barnett MB BS, FRACP, FACEM · Ed A Oakley MB BS, FACEM

On 14 January 2005, Western Australia lost one of its finest paediatricians. Paul Carman died suddenly of acute myocardial infarction while on holiday with his family in Capel. The loss to the medical and Catholic community is immense. In the 19 years that Paul was in Western Australia, he made significant contributions in many fields. He was Chairman of the Paediatric Clinical Care Unit at Princess Margaret Hospital for Children. He ran regular remote paediatric clinics in the Pilbara region, serving the local mining and Aboriginal communities with compassion and care — qualities that he exercised in all other aspects of his professional life. He was a foundation member of the (now disbanded) Advisory and Coordinating Committee on Child Abuse, an advisor to the Christian Brothers commission to support the needs of former child migrants who had been under their care, and an active member of St Thomas’ parish and school, which his seven children attended and where his funeral was held. However, his lasting legacy for paediatric hospitals in Perth will be his vision to establish a Child Protection Unit to provide specialised medical and forensic services to children. Paul was born in Melbourne on 17 September 1950. When he was 4 years old, his family moved to Cairns, in Queensland, and then, 8 years later, to Warrnambool, in Victoria. In Warrnambool, his father established a very successful dental practice. Paul matriculated from St Joseph’s Christian Brothers College in Warrnambool. However, his university entrance score was not sufficient to enter medicine, which was his passion (a passion sparked by the television serial “Dr Finlay’s Casebook”). The following year, Paul repeated his matriculation exams and gained entrance to the University of Melbourne Medical School in 1969. During the fifth year of his course, he met Margaret, whom he married in 1977. Paul worked for 2 years at Queen’s Medical Centre in Nottingham under the guidance of Sir David Hull. It was here that Paul developed his interest in child protection. He obtained his Fellowship of the Royal Australasian College of Physicians in 1985 and, in the following year, took up an appointment as a staff paediatrician at the Princess Margaret Hospital in Perth and as a rural paediatrician in conjunction with the legendary Dr Rex Henderson. Paul’s ability to listen, discuss and draw on his vast knowledge of Russian and European history, ethics, philosophy and common sense was a driving force in the outstanding contribution he made to children’s health in Western Australia. The love, trust and enduring friendship in his relationship with Margaret is reflected in the lives of their seven children.

Peter M Winterton BA, MB BS, FRACGP

Questions of cost-effectiveness, vaccine efficacy and feasibility are yet to be answered In the United States, routine immunisation of all healthy children aged 6–23 months against influenza has recently been introduced. The principal justification for this is the relatively high morbidity and mortality from this disease in very young children.1 The United States is also considering routine influenza immunisation of all children aged over 6 months, in view of the herd protection it would provide to the adult population. Currently, Australian guidelines recommend immunisation of children in groups considered at high risk of severe influenza.2 Should Australia introduce universal childhood immunisation? There is no doubt that children have an extremely high incidence of influenza. It is estimated that, on average, 20%–43% of children are infected during typical influenza seasons.3-5 The incidence is highest in young children less than 2 years old, who are often hospitalised.1,3-5 The mortality due to influenza in infancy is second only to that in the most elderly patients.1 In the severe 2003–04 influenza season, 143 children died from influenza in the United States, of whom 58 (41%) were less than 2 years old and 65 (45%) had no underlying condition.1 These data emphasise the importance of protecting children with annual influenza immunisation, if feasible. Another reason to consider universal childhood influenza immunisation is herd protection. In what turned out to be an illuminating natural experiment, 50%–85% of Japanese schoolchildren were immunised annually against influenza from 1962 to 1987, but there was no routine immunisation of the elderly. When mandatory immunisation of schoolchildren was relaxed in 1987 and repealed in 1994 (because of doubts about safety and effectiveness), influenza immunisation rates dropped to very low levels. A retrospective study comparing excess mortality from pneumonia and influenza in Japan and the United States concluded that the vaccination of Japanese schoolchildren prevented about 37 000 to 49 000 deaths per year, mostly of elderly people. (This represented about one death for every 420 children vaccinated.)6 In considering the feasibility of universal childhood vaccination, vaccine efficacy is one of the factors that needs to be taken into account. In healthy adults under 65 years of age, inactivated influenza vaccine is 70%–90% effective when the match between vaccine and circulating viruses is close.1 However, the same vaccine may be less immunogenic in children. Studies in children aged 6 months to 15 years show a vaccine efficacy of 31%–91% against influenza A and 45% against influenza B.7,8 However, very few of the studies have examined children aged 6–23 months,9,10 the age group currently recommended for routine influenza vaccination in the United States. An alternative form of vaccine administration is on the horizon — live attenuated influenza vaccines. A recent systematic review10 suggested that live vaccines may be more effective than inactivated vaccines in children over 2 years of age (79% versus 65%). Live attenuated influenza vaccines have been licensed in the United States, and might be more acceptable because they are given intranasally.11 However, live vaccines cost a lot more and are not licensed for use in children under 5 years in the United States (because of limited safety data). They are not yet licensed in Australia for use in any age group. The high morbidity of influenza in children and the likely benefits due to herd immunity do make annual childhood influenza immunisation appear economically attractive. However, against this must be weighed the need to immunise with a new influenza vaccine each year, because of antigenic drift in influenza strains, and the need to give two doses of vaccine to children under 9 years in the first year they are immunised.1,2 In addition, the severity of influenza seasons varies unpredictably from mild to severe, and it costs as much to immunise in a mild year as in a severe one. In the United States, indirect costs (mainly days of work lost by parents) dominate economic analyses supporting the use of influenza vaccines in children.12 In Australia, by contrast, the Pharmaceutical Benefits Advisory Committee considers only direct costs of illness, so it is unlikely that a universal, publicly funded childhood immunisation program could be justified using such cost-effectiveness criteria. There may also be practical problems with attempts to introduce routine childhood immunisation. In 2004–05, the uptake of influenza vaccine for children aged 6–23 months in the United States, when the vaccine was recommended universally, was estimated to be only 48%.13 In Ontario, Canada, where all residents aged over 6 months have been offered free annual influenza immunisation since 2000,14 the 2003 uptake in children was only 27%.15 Parents of unimmunised children were more likely to believe that immunisation resulted in a flu-like illness, caused adverse effects more severe than the disease, or weakened the immune system.15 Such immunisation myths are common, although studies have repeatedly shown inactivated influenza vaccine align="right" to be safe, with low rates of adverse events and the benefits clearly outweighing the risks.1,2,9 Another practical issue is the question of how to fit the vaccine into an already crowded childhood vaccination schedule. In general, annual immunisation against influenza is recommended in autumn at the start of the influenza season; there is no fixed age of administration of vaccine. Implementing universal influenza vaccination would place a substantial extra burden on primary care practices.16 Considering all the available information, I believe that there is currently insufficient reason for introducing universal childhood vaccination for this disease in Australia. There are too many unanswered questions about the cost-effectiveness, efficacy and feasibility of universal immunisation of healthy children, whether infants or school-aged. For the time being, we should maintain a watching brief. Future data emanating from Ontario and the United States may provide us with a clearer answer as to whether large-scale programs of routine childhood influenza immunisation are feasible and effective. Further, if the US experience with live vaccines shows consistent immunogenicity and improved ease and acceptability of administration, live vaccines may yet prove to be a cost-effective way to implement universal childhood influenza immunisation in Australia. One thing is clear: influenza vaccination is most cost-effective for children considered at high risk of severe influenza, such as those with chronic cardiopulmonary and other chronic illness. These children should clearly be vaccinated annually against influenza.1,2 Yet vaccine coverage of high-risk groups aged 2–17 years is only 35% in the United States,13 and probably lower in Australia, although we lack age-specific data. Australian immunisation providers should redouble their efforts to ensure that children at high risk are immunised annually.2 Also, it should be remembered that the Australian immunisation handbook2 does not preclude vaccinating others who are not at high risk. It states that “influenza vaccine should be administered to any person who wishes to reduce the likelihood of becoming ill”.

David Isaacs MD, FRACP, FRCPCH

Minor injuries in children (those that could reasonably be expected to heal with minimal medical intervention) are extremely common. The possibility of more serious injuries should be considered and excluded early. Successful examination requires gaining the child’s trust, relieving pain early, and using a flexible and creative examination technique. Bruising may suggest a more serious underlying injury, or the bruising pattern may indicate non-accidental injury or a bleeding disorder. Superficial abrasions and lacerations can be safely cleaned with good quality water, and all foreign material should be removed. Deeper wounds with suspected damage to nerves, tendons or circulation need formal exploration under a general anaesthetic. Good local anaesthesia can be produced by topical preparations, and many wounds can be closed with tissue adhesives with an excellent cosmetic result. Antibiotics should be prescribed for specific circumstances, such as wounds with extensive contamination or tissue damage, and all children with injuries should be checked for adequate tetanus cover for prophylaxis.

Simon J Young MB BS, DipCrim, FACEM · Peter L J Barnett MB BS, FRACP, FACEM · Ed A Oakley MB BS, FACEM

Elizabeth A Hender,* Corrine R Balit† * Scientific Officer, Hazardous Substances Section, Environmental Health Service, Department of Health, PO Box 6 Rundle Mall, Adelaide, SA 5000; † Research Pharmacist, New South Wales Poisons Information Centre, The Children’s Hospital, Westmead, NSW. elizabeth.henderAThealth.sa.gov.au To the Editor: Although there are few deaths due to poisoning in Australian children, from 1993 to 1997 there was an average of more than 2500 admissions to hospital per year for assessment of poisoning with medicines in children younger than 5 years.1 Child-resistant packaging has been effective in preventing accidental poisoning with prescription medicines and aspirin in young children in the United States.2,3 In the US, both reclosable and non-reclosable (blister or strip) packaging used for pharmaceuticals required to be in child-resistant packaging is tested to confirm its effectiveness in preventing access by children.4 In Australia, only reclosable packaging is required to be child-tested. Blister or strip packaging, which has not usually been child-tested, is accepted as an alternative to child-resistant reclosable packaging.5 We conducted a study at the New South Wales Poisons Information Centre (NSWPIC) over 9 weeks from 18 July to 17 September 2003. Our aims were to ascertain which medicines children younger than 5 years access directly from blister or strip packaging, and whether assessment at a hospital was recommended. The study was approved by the Ethics Committee of the Children’s Hospital, Westmead. Callers ringing about a suspected accidental ingestion of a solid dose medicine in a child younger than 5 years were asked whether the child accessed the medicine directly from a blister or strip pack. There were 318 accidental exposures to solid dose medicines in these children during the study period. In 186 exposures (58%), the caller said the medicine was normally in a blister or strip pack and the child obtained it directly from the pack. A wide range of medicines (40 different drugs or drug groups) were associated with the exposures; the most common were oral contraceptives (49 exposures) and paracetamol (27 exposures). Some of the exposures involved medicines that can cause severe toxicity when children ingest a small number of dose units, such as clonidine, olanzapine, narcotic analgesics, and tricyclic antidepressants. In 36 exposures where the child obtained the medicine directly from the pack, the caller was advised to take the child to hospital (Box). Many of the medicines associated with these exposures (eg, paracetamol, preparations containing narcotic analgesics, antidepressants, antihistamines, iron and clonidine) are required to be in child-resistant packaging.5 Our study shows that blister or strip packs currently in use did not prevent children accessing drugs. This finding calls into question whether blister or strip packaging that has not been child-tested presents an adequate safety barrier. No outcomes of drug ingestion are known in this study, which is a limitation. However, assessment of these children in hospital represents a financial burden to the health care system regardless of the outcome. Further studies would be required to quantify the harm associated with exposures to medications packaged in blister or strip packaging in young children and to assess the effectiveness of such packaging in the prevention of poisoning. Drugs accessed from blister or strip packs where child required referral to hospital Drug or drug group Number of exposures Paracetamol 8 Paracetamol/narcotic combination analgesics 3 Selective serotonin re-uptake inhibitors 3 Antidepressant: other/unknown 2 Antiemetics 2 Antihistamines 2 Cough/cold preparations, no paracetamol 2 Iron 2 Other (eg, clonidine, olanzapine) 12 Total 36

Elizabeth A Hender · Corrine R Balit

Graham R Wicks Medical Hypnotherapist, Department of Psychological Medicine, Women’s and Children’s Hospital, North Adelaide; 9 Collins Street, Collinswood, SA 5081. grwicksATbigpond.net.au To the Editor: I was interested to read the article about managing nocturnal enuresis in children,1 but was surprised and disappointed that there was no mention of the place of medical hypnotherapy. Hypnotherapy can be particularly valuable in the treatment of monosymptomatic nocturnal enuresis in children aged from 7 or 8 years upwards, and has the advantage of being completely non-invasive with no side effects. It focuses on empowering the children to take control of their own bodily functions.2 Hypnotherapy is also of value in the management of nocturnal enuresis associated with day-time symptoms, such as urgency with or without incontinence, and can also be used to enhance the efficacy of treatments like enuresis alarm systems. While there are few well documented comparative studies3 on the benefits of hypnosis versus other treatments for nocturnal enuresis, there are numerous anecdotal reports and studies involving a series of patients being successfully treated with hypnotherapy. Hypnosis should only be used by properly trained doctors or psychologists who have access to the full range of medical investigations. Hypnosis is of course not a panacea, but is an excellent first-choice treatment for monosymptomatic nocturnal enuresis, the commonest type seen by general practitioners. If, after three or four treatment sessions, hypnosis is not effective, other approaches can be employed. A quick search of the internet using the terms “enuresis and hypnotherapy” will reveal over 700 sites with information on the subject, and there are several highly respected professional journals that publish clinical and research papers and articles on the use of hypnosis in medicine and psychology. All of these are published by reputable professional societies whose membership is limited to registered health professionals. The Australian Society of Hypnosis (http:// www.ozhypnosis.com.au) conducts ongoing training courses in all states of Australia for graduates in medicine, psychology and dentistry. Hypnotherapy is now becoming more and more accepted worldwide as a valuable and legitimate tool that can be used, in conjunction with the more traditional approaches, in a wide variety of medical and psychological problems. It is a great pity that many clinicians are either not aware of its value or are still loathe to accept it because of negative connotations associated with its use for entertainment purposes and in the hands of non-professional therapists.

Graham R Wicks

Patrina H Y Caldwell,* Denise Edgar,† Elisabeth Hodson,‡ Jonathan C Craig§ * Staff Specialist and Lecturer, † Head, Department of Nephrology, § Associate Professor and Head of Clinical Research, NHMRC Centre of Clinical Research Excellence in Renal Medicine, The Children’s Hospital at Westmead, Locked Bag 4001, Westmead, Sydney, NSW 2145; ‡ Clinical Nurse Consultant, The Continence Foundation of Australia in NSW, Sydney, NSW. PatrinacATchw.edu.au In reply: Thank you for your interesting comments regarding hypnotherapy in the treatment of nocturnal enuresis in children. There are a number of therapies, such as hypnotherapy, electrotherapy and acupuncture, which show great promise for the management of nocturnal enuresis. We only included in our article1 treatments that were supported by evidence from well documented comparative studies. Using comprehensive search strategies, we have not found comparative studies for these complementary therapies. We would be very interested to be directed to studies that have formally evaluated other interventions. There is a huge need for randomised controlled trials comparing alternative treatment strategies with conventional therapy in this area.

Patrina H Y Caldwell · Denise Edgar · Elisabeth Hodson · Jonathan C Craig

Objective: To evaluate the role of zinc and vitamin A supplementation in the recovery of Indigenous children hospitalised for acute diarrhoea.Design: A randomised controlled 2 by 2 factorial trial of supplementation with zinc and vitamin A.Setting and participants: Aboriginal children (aged < 11 years) hospitalised for acute diarrhoea at Alice Springs Hospital, Northern Territory, April 2001–July 2002.Main outcome measures: Duration of diarrhoeal illness; re-admission for diarrhoeal illness within 120 days.Results: Our study involved 392 Aboriginal children with 436 episodes of diarrhoea. Supplementation with zinc, vitamin A, or combined zinc and vitamin A had no significant effect on duration of diarrhoea or rate of re-admission compared with placebo. Median diarrhoea duration after starting supplementation was 3.0 days for the vitamin A and zinc supplemented and placebo groups (P values 0.25 and 0.69, respectively). The number of re-admissions did not differ significantly between those receiving vitamin A or zinc and the relevant placebo groups (relative risk [95% CI], 1.2 [0.7–2.1] and 1.3 [0.8–2.1], respectively).Conclusion: Vitamin A and zinc supplementation may not be indicated for in-hospital management of acute diarrhoeal disease in Aboriginal children living in remote areas. This finding may not apply to children with malnutrition, for whom other studies suggest a benefit. Larger trials incorporating more comprehensive data on the vitamin A and zinc status as well as nutritional status of study populations might help to explain the different results in different populations.

Patricia C Valery MD, MPH, PhD · David M Purdie BSc(Hons), PhD · Paul J Torzillo MB BS, FRACP, FFICM · Peter A Stewart MB BS, FRCPA · Naomi C Boyce B Nursing · Anne B Chang MPHTM, FRACP, PhD · Andrew V White MB BS, FRACP · Gavin R Wheaton MB BS, FRACP · John Wakerman MB BS, MPH

Do we have a clear enough picture to guide rational health and social policy responses? Australia’s economic prosperity has long brought incremental health gains through better living conditions, sanitation, education, medical care and vaccination.1 The effects on child health and mortality have been striking. The latest report from the Australian Institute of Health and Welfare (AIHW), A picture of Australia’s children, documents this continuing trend. Infant and child mortality rates halved again in the past 20 years.2 The fall in deaths from sudden infant death syndrome (SIDS) to a third of 1991 rates is a tribute to outstanding Australian child health research, as well as the work of child and family health nurses and the SIDS Council of Australia.3 A steady decline in deaths from injury in later childhood has also contributed to lower childhood mortality. Judged by these indices, the present generation of Australian children is the healthiest ever. Key findings of A picture of Australia’s children The infant mortality rate in Australia halved over the past two decades, from 9.6 per 1000 livebirths in 1983 to 4.8 in 2003. The Indigenous infant mortality rate also declined by 3.3% per year, but was still 2.5 times that of other Australian infants. Rates of non-communicable health problems, such as obesity and mental disorders, appear to be rising, but lack of up-to-date national data makes it difficult to accurately assess the current rates. Rates of vaccination among children aged 1 and 2 years have increased over time, with the coverage in 2004 being over 90%. Between 1990 and 2000, children’s dental health improved, with a decrease in the mean number of decayed teeth in 6 year olds (from 2.1 to 1.7), and 12 year olds (from 1.4 to < 1). However, since 2000, tooth decay in Australian children seems to be on the increase again. The number of children on care and protection orders has risen almost 50% in the past 6 years, with the rates sixfold higher in Indigenous children. The proportion of children placed in out-of-home care also rose from 3 per 1000 children in 1997 to 5 per 1000 in 2004. Economic progress has also altered the lives of children through changing the social context of development. The transformation of Australian families has been striking. Fewer children, smaller households, older parents, working mothers, and parental separation and divorce, all affect the way in which families provide a nurturing and secure base.4 There are concerns that a greater investment in fewer children, tied with heightened parental anxieties, has produced a “bubble-wrap generation”. The effects of limiting independent exploration, risk taking and physical activity on children’s physical, cognitive and emotional development may be profound.5 Socioeconomic changes have also affected child health in other ways, such as altering material consumption and lifestyle. Industries, ranging from fashion to food and entertainment, now market to children, regarding them not only as the consumers of tomorrow but as major agents of influence on family spending.6 In this changing social context, the AIHW report attempted to capture a broad picture of the health and development of our children (Box). In preparing the report, emerging morbidities, such as childhood obesity, were to be an important focus. Obesity not only poses risks for later cardiovascular disease and diabetes, but also profoundly affects children’s quality of life and self-concept.7 However, the best available national data are 10 years old, from a time when around one in five children were overweight or obese. Moreover, national data are not available on patterns of physical activity or nutrition. Because of longer-term effects on adult health and prosperity, the socioeconomic circumstances of childhood are central in social policy considerations.8 Nowhere are these continuities between childhood circumstances and adult health clearer than in Aboriginal and Torres Strait Islanders. For this reason the report attempted to capture broader data on family functioning, local neighbourhoods, educational attainment, and the welfare of children in contact with health and social services. Some of the trends revealed by the study provide food for thought. The number of children on care and protection orders has increased almost 50% in 6 years, and rates in Indigenous children are over sixfold higher. The proportion of children in out-of-home care (ie, having to live away from their parents) has risen over 60% in the same period. Around one in ten families with children currently report that their neighbourhoods feel unsafe at least some of the time. This experience is three times commoner in poorer families. What effects these trends may be having on the mental health and emotional development of children is uncertain. Again our picture is incomplete, with the best available national data on child mental health now 7 years old.9 Data from this 7-year-old study suggested that, at any point in time, one in eight children had a diagnosable mental or behavioural disorder. These rates were twice as high in sole parent and blended families (ie, families formed by second marriages between parents with children). Thus, in attempting to paint a bigger picture of child health, development and wellbeing, the AIHW report has exposed huge gaps in the information needed for rational health and social policy responses. Perhaps the clearest gaps concern the emerging non-communicable illnesses of childhood. A need for up-to-date national data on the social and geographic distribution of childhood obesity and mental disorders stands out as a priority. What data we have suggest that these problems vary greatly according to geographic location and socioeconomic status and are worsening. If current social changes persist, the worsening trends in obesity and mental disorders seem likely to continue, and the children most affected will be those in disadvantaged and disrupted families. The federally funded Longitudinal Study of Australian Children will address some of the gaps by providing a better understanding of how current social and family contexts affect children.10 However, the study is of two cohorts separated by 4 years and will not be able to adequately capture the continuing and ongoing changes in the social context of childhood that we may expect to see in the coming years. Other gaps relate to our service systems for children and families. The aggregation of service system data to create ongoing national minimum datasets for areas such as juvenile justice, child protection and children’s services is an important first step in understanding how these systems are working. But much more is needed. The development of brief measures of development and social context in early and later childhood11,12 heralds the possibility of efficiently capturing ongoing shifts in the lifestyles, social development and health of our children. The new health problems of childhood are complex in their origins and likely to be complex in their solutions. A clearer picture of our children is needed to guide our responses — whether these be through priority research, informed government policy, better functioning of our service systems or, most importantly, the efforts of Australia’s parents, schools and local communities.

George C Patton MD, FRANZCP · Sharon R Goldfeld FRACP · Indrani Pieris-Caldwell PhD · Meredith Bryant MA · Graham V Vimpani FRACP

Objective: To report the outcome of gene therapy in an infant with X-linked severe combined immunodeficiency (SCID-X1), which typically causes a lack of T and natural killer (NK) cells.Design and setting: Ex-vivo culture and gene transfer procedures were performed at The Children’s Hospital at Westmead, Sydney, NSW, in March 2002. Follow-up to March 2005 (36 months) is available.Patient: A 9-month-old male infant with confirmed SCID-X1 (including complete absence of T cells) with an NK+ phenotype (a less common variant of SCID-X1), and no HLA-identical sibling donor available for conventional bone marrow transplantation.Procedure: CD34+ haemopoietic progenitor cells were isolated from harvested bone marrow and cultured with cytokines to stimulate cellular replication. Cells were then genetically modified by exposure to a retrovirus vector encoding human γc (the common γ chain of several interleukin receptors; mutations affecting the γc gene cause SCID-X1). Gene-modified cells (equivalent to 1.3 × 106 CD34+/γc+ cells/kg) were returned to the infant via a central line.Results: T cells were observed in peripheral blood 75 days after treatment, and levels increased rapidly to 0.46 × 109 CD3+ cells/L at 5 months. Within 2 weeks of the appearance of T cells, there was a distinct clinical improvement, with early weight gain and clearance of rotavirus from the gut. However, T-cell levels did not reach the reference range, and immune reconstitution remained incomplete. The infant failed to thrive and developed weakness, hypertonia and hyperreflexia in the legs, possibly the result of immune dysregulation. He went on to receive a bone marrow transplant from a matched unrelated donor 26 months after gene therapy.Conclusions: This is the first occasion that gene therapy has been used to treat a genetic disease in Australia. Only partial immunological reconstitution was achieved, most likely because of the relatively low dose of gene-corrected CD34+ cells re-infused, although viral infection during the early phase of T-cell reconstitution and the infant’s NK+ phenotype may also have exerted an effect.

Samantha L Ginn BSc(Hons), PhD · Julie A Curtin PhD, FRACP · Christine M Smyth MSc, PhD · Margot Latham BSc · Sharon C Cunningham BSc(Hons), PhD · Maolin Zheng BSc(Hons), MSc · Linda Hobson BPharm(Hons) · Peter B Rowe MD, FRACP · Ian E Alexander PhD, FRACP · Belinda Kramer BSc(Hons), MSc · Melanie Wong PhD, FRACP · Alyson Kakakios FRACP · Geoffrey B McCowage FRACP · Debbie Watson BSc(Hons) · Stephen I Alexander FRACP · Alain Fischer MD, PhD · Marina Cavazzana-Calvo PhD · Salima Hacein-Bey-Abina PhD