Child health

Allergic bronchopulmonary aspergillosis (ABPA) is an uncommon condition which may complicate asthma and cystic fibrosis; it is seldom considered life-threatening. We report a well 8-year-old boy with cystic fibrosis and normal lung function who progressed to respiratory failure over several days, attributable to ABPA. He recovered with non-invasive ventilation and oral corticosteroid and antifungal medications, regaining normal lung function within 2 months. To our knowledge, such an acute severe presentation of ABPA in a previously well child has not been reported before. Clinical record An 8-year-old boy with pancreatic insufficient, homozygous ΔF508 cystic fibrosis (CF) was transferred from a district hospital with a 7-day history of progressive cough, wheeze and tachypnoea, despite 4 days of broad-spectrum antibacterial therapy (intravenous flucloxacillin and cefotaxime with oral roxithromycin), frequent nebulised salbutamol, oral prednisone (2 mg/kg/day) and chest physiotherapy. There were no other systemic symptoms such as rash, myalgia, arthralgia, diarrhoea or headaches. Initial sputum cultures isolated only normal respiratory flora. The provisional diagnosis was an atypical lower respiratory tract infection, attributed to a viral infection or Mycoplasma pneumoniae. Cystic fibrosis had been diagnosed at newborn screening. His height and weight had tracked along the 10th percentile. Lung function had been normal. Previous sputum cultures had grown Staphylococcus aureus but never Pseudomonas aeruginosa or Burkholderia cepacia. He had not previously wheezed. On arrival, he was mildly dyspnoeic on supplemental mask oxygen at 6 L/minute with a blood oxygen saturation (Spo2) of 94%. He was tachypnoeic (44 breaths per minute), tachycardic (112 beats per minute), afebrile and normotensive. He had bilateral expiratory wheeze with basal crackles. Within 24 hours of transfer, our patient’s condition deteriorated, and he required more frequent nebulised salbutamol and developed signs of respiratory fatigue. At this point an arterial blood gas analysis in 12 L of mask oxygen showed: pH, 7.3; partial pressure of oxygen (Po2), 58 mmHg; partial pressure of carbon dioxide (Pco2), 62.7 mmHg; bicarbonate (Hco3) level, 30 mmol/L; and base excess, 2.7. He was transferred to the paediatric intensive care unit (PICU) for respiratory support with mask continuous positive airway pressure (CPAP). Subcutaneous emphysema developed over the chest wall and neck during the first 48 hours of his PICU admission. A full blood count showed leukocytosis (19.6 × 109/L), and his initial mycoplasma complement fixation test titre was low (< 4). The mycoplasma IgM test result was subsequently negative. Immunofluorescence and culture of nasopharyngeal secretions to isolate a viral pathogen were negative. Total serum IgE titre was 1664 IU (normal range, 0–180 IU) and the skin prick test was positive for Aspergillus fumigatus. The provisional diagnosis was changed to allergic bronchopulmonary aspergillosis (ABPA). Prednisone therapy was continued, and antifungal treatment with oral itraconazole (100 mg twice daily) was added to his therapy. His respiratory status gradually improved and he was weaned off the nasal mask CPAP after 5 days, avoiding endotracheal intubation. He had clinically apparent subcutaneous emphysema, a small pneumomediastinum, but no pneumothoraces during his PICU stay. The chest radiograph before discharge from PICU (Box 1) showed increased perihilar opacities and left lower lobe infiltrates with resolution of the pneumomediastinum and subcutaneous emphysema. His improving spirometry measurements with treatment are shown in Box 2. He was discharged on Day 13 of admission with marked improvement in symptoms and an FEV1 (forced expiratory volume in 1 second) 66% of predicted. A provisional diagnosis of ABPA was confirmed by the significantly elevated serum IgE titre, positive skinprick tests for aspergillus, positive IgG aspergillus precipitins (× 4) and clinical findings consistent with the diagnostic criteria outlined in Box 3.1-5 He received decreasing doses of oral corticosteroids over 3.5 months, as well as oral itraconazole. Within 2 months, his spirometry results had returned to normal. Predictably, he became transiently cushingoid, developed mild untreated hypertension (maximum recorded blood pressure, 116/84 mmHg), and gained 4 kg in weight, but had no glycosuria. He was weaned from corticosteroids and itraconazole, and the side effects resolved within 3 months of discontinuing corticosteroids. Ten months later, he remains well, with normal lung function. DiscussionAlllergic bronchopulmonary aspergillosis (ABPA) is an uncommon condition that can complicate asthma and cystic fibrosis (CF),6,7 and is seldom considered life-threatening.7 The rapid deterioration in the condition of our previously well patient highlights the spectrum of disease severity that can occur in ABPA, and is a reminder that not all deteriorations in respiratory function in patients with CF are attributable to Pseudomonas aeruginosa or Burkholderia cepacia.7,8 Furthermore, this patient’s prompt clinical response to systemic corticosteroids with a return to normal lung function within 2 months suggests that his prognosis will not be adversely affected.9 The presence of Aspergillus species in the sputum cultures of patients with CF has been reported in up to 57% of patients,9 yet the prevalence of ABPA is reported to be between 2% and 14%.1,6 The diagnostic criteria are listed in Box 3. ABPA is a hypersensitivity reaction to the inhalation of aspergillus spores manifesting as chronic wheeze, pulmonary infiltrates and systemic immune activation.5 This results in elevated IgE, IgG and IgA titres.9 Interestingly, although aspergillus grows in the bronchial mucus, this is not an invasive disease.3-5,9 The exact mechanism by which bronchial wall damage evolves and how this gives rise to bronchiectasis and fibrosis is poorly understood.10 Aspergillus fumigatus infection often occurs months before a clinical diagnosis of ABPA is considered. Moreover, as about 40%–50% of school-aged patients with CF have aspergillus in their sputum, comparatively few develop ABPA.3 The reasons for this are not clear, but presumably relate to genetic predisposition, host defences and environmental exposure to aspergillus. Most children with CF who develop ABPA have relatively mild symptoms, respond to treatment over weeks and can often avoid hospital admission altogether.7 Patients with ABPA complicating CF more commonly follow a course of gradually worsening lung function (because of progression of their CF related bronchiectasis) with recurring relapses of ABPA, particularly in summer and autumn, when spore levels in the environment are at their highest.9,10 Treatment of ABPA has centred on the use of systemic corticosteroids for periods of 2–6 months, reducing from doses of 1.0 mg/kg/day of prednisone.4 The adjunctive use of oral antifungals has been advocated for the treatment of ABPA complicating CF11,12 and asthma.6 The response can be dramatic, as in our patient (Box 2). Itraconazole in combination with inhaled corticosteroids was recently shown to be useful for reducing eosinophilic airway inflammation, reducing systemic immune activation and reducing severe exacerbations over a period of 16 weeks in adults with ABPA complicating asthma.13 The use of itraconazole in children is less well studied. However, a recent case series of patients aged 9 to 33 years with CF and ABPA, treated with inhaled budesonide (800–1600 μg/day) and itraconazole (400–600 mg/day), showed a high prevalence of biochemical adrenal insufficiency on adrenocorticotropin testing. This was attributed to an increased systemic budesonide concentration through a reduced or inhibited metabolism (potentially caused by itraconazole), leading to inhibited steroidogenesis.14 This reminds us to use caution when treating patients who take inhaled steroids with courses of itraconazole for exacerbations of ABPA. In conclusion, we are unable to find another case report in which a previously well child with ABPA presented with severe acute respiratory failure. This case highlights the importance of considering a diagnosis of ABPA in highly unusual presentations which may complicate cystic fibrosis. 1 Chest x-ray 3 days after admission to intensive care Shows hyperinflated lung fields, perihilar inflammatory changes, emergence of an interstitial infiltrate in the left lower lobe and a small right-sided pleural effusion. 2 Relationship between IgE levels and FEV1 (forced expiratory volume in one second) before, during and after admission Inverse relationship between IgE levels and FEV1 over time (non-linear scale), showing the drop in lung function at the peak of the disease. 3 Classic case criteria for the diagnosis of allergic bronchopulmonary aspergillosis (ABPA) in patients with cystic fibrosis1-5 Clinical deterioration (increased cough, wheezing, exercise intolerance, increased sputum, decrease in pulmonary function) Immediate cutaneous reactivity to aspergillus or presence of serum IgE from A. fumigatus Total serum IgE concentration >1000 IU/L Precipitating antibodies to A. fumigatus or serum IgG from A. fumigatus Abnormal chest x-ray (infiltrates, mucus plugging, or a change from earlier films) Adapted from the ABPA Consensus Conference of the Cystic Fibrosis Foundation4

Emma Skowronski BMedSci · Dominic A Fitzgerald PhD, FRACP

There are strong associations between childhood sleep disorders and behavioural, concentration and mood problems. Sleep disorders caused and maintained by behavioural factors (eg, sleep-onset association disorder) are common in young children, and have a significant impact on families. Evaluation should include a medical history, a physical, neurological and developmental examination, a description of any nocturnal events or daytime effects of the child’s disturbed sleep, and a good understanding of the family situation and parental management of the child. Management involves recognising the developmental age of the child and the family dynamics, and educating and supporting families in applying behavioural techniques to establish good sleep hygiene. Children with parasomnias (eg, night terrors) also benefit from good sleep hygiene, while those with respiratory or neurological causes of sleep disturbance should be referred for specialist treatment.

Helen S Heussler MB BS, FRACP

Re: MJA Practice Essentials – Paediatrics “Investigation and treatment of upper-airway obstruction: childhood sleep disorders I”, by Kennedy JD and Waters KA (Med J Aust 2005; 182: 419-423). In printed version of this article, the last two paragraphs were missing from the Case study on page 422. The complete Case study is given below. The html version of this article was correct on publication and the pdf version was corrected on 27 April 2005. Case study — a 5-year-old boy with obstructive sleep apnoea syndrome (OSAS) A mother brings her 5-year-old son to you, as she is concerned about his loud snoring. He has been snoring for 2 years, but it is gradually becoming louder. Although she is unaware of whether or not her son has obstructive apnoeas, she is anxious about his breathing during sleep. She often sits up to watch him breathe before retiring herself. The mother reports that the boy’s teacher at school says that her son has a poor concentration span and is easily distracted in class. Both the father and grandfather snore loudly but neither has sought medical advice or investigation. Management On examination, you find that the boy has relatively small tonsils and you initially reassure his mother. However, as his symptoms persist, you refer him to a paediatric respiratory physician. An overnight home oximetry study shows only mild desaturations that appear to be associated with movement. A lateral x-ray of the neck confirms adenoidal hypertrophy but a patent postnasal space. A polysomnogram shows an obstructive respiratory disturbance index of nine per hour. Based on the data from the polysomnogram, the boy undergoes adenotonsillectomy. After the operation, the boy’s snoring resolves, his behaviour and concentration improve, and his mother describes him as a “different boy”. At the 1-year follow-up, the boy’s mother reports that his snoring has returned. You resume frequent clinical monitoring, and subsequently refer him for repeat polysomnography, as his history once again suggests the presence of OSAS. If polysomnography confirms that OSAS has returned, and a lateral neck x-ray shows that his adenoids have not regrown, the use of night-time continuous positive airway pressure (CPAP) support will be considered.

J Declan Kennedy MD, FRCP, FRACP · Karen A Waters MB BS, FRACP, PhD

The most important need is for a change of attitude and approach Transition is “the purposeful, planned movement of adolescents and young adults with chronic physical and medical conditions from child-centred to adult-orientated health care systems”.1 The importance of transition of young people with chronic health conditions from paediatric to adult care is finally being recognised, but it needs to be addressed in a coordinated and integrated way. In Australia, as internationally, there are some well established and potentially effective transition programs. However, little is known about the efficacy of such programs, as there is little published evaluation.2 What is clear, both anecdotally and with some modest support in the literature, is that the journey faced by young people with chronic and disabling conditions is a complex one.3,4 The majority will face obstacles that arise from the lack of infrastructure and precedent in this specialised area of health care. Current approaches are rarely ideal, the most likely options being abrupt transfer to adult services, staying in the paediatric setting longer than is appropriate, or leaving medical supervision altogether. 5 The study at the Royal Children’s Hospital, Melbourne, reported by Lam and colleagues6 in this issue of the Journal (page 381) revealed a doubling in admissions of young adults aged 18 and over between 1992 and 2001, along with an overall increase in the numbers of adolescents admitted. In a more detailed analysis of a cohort of 247 young adults admitted during 2001, the authors also examined disease complexity and discovered a paucity of transition planning, particularly in surgical units. While some medical services (eg, Endocrinology, Respiratory Medicine) appeared to effect transition of adolescents with complex health issues efficiently, this was definitely not the norm. With increasing prevalence of some diseases and improved survival rates for previously fatal childhood conditions, pressures on all tertiary care facilities continue to rise. In paediatric hospitals, one possible outcome is pressure to improve facilities for adolescents. A psychosocial survey of Australian hospitals undertaken in 2004 by the Association for the Welfare of Child Health showed an increase in the number of “adolescent units” since the previous survey in 1994. 7 Hospitalised and ambulatory young people require developmentally appropriate health care supported by psychosocial services — needs that are usually better served in dedicated adolescent facilities. While the impact of this trend on transition practices is yet to be determined, the broader challenge is to consider transition needs in the coordinated planning of health care services. Until transition to adult care is recognised by the adult health care system as requiring a demonstrable change in attitude and resources, little real progress will be possible. Older adolescents deserve to be treated more as adults than as children. Keeping young adults in the paediatric system is working against this goal on many levels. A sense of maturity and hope for the future are implied in “moving on”, 8 but one of the prerequisites for an effective transition program is “an interested and capable adult service”. By allowing young adults to stay on in the paediatric system (which carries its own set of problems, including the inappropriate collocation of young children with “adults”), the development of such services is effectively stifled, and adult physicians and surgeons are not encouraged to develop their skills in the area. The article by Lam et al asks, “Why are they there?” It has been proposed that paediatric services may hold onto patients because of mistrust of adult services9 or through failure to promote independence and autonomy in health-care seeking. 10 Some other probable reasons are less strongly supported by the literature. One is that paediatric services are family-focused, while adult services treat patients as independent adults. This is problematic for young adults who still require family involvement because of the nature or severity of their disease or disability. Doctors and other staff in adult services may have limited knowledge and understanding of childhood chronic illnesses in young people who survive into adulthood, or of developmental issues in adolescents. Furthermore, young people are “diluted” in the adult health care system and their special needs may be largely overlooked in individual services. There are inherent difficulties in discovering, accessing and negotiating adult services for young people and their carers. The implications of “failed transition” for young people range from a lack of continuity of care and reliance on crisis services to “falling through the gap”, with significant adverse health consequences. 11 We agree with Lam and colleagues that the solution to the problems of young adults in children’s hospitals lies more in a greater focus on the infrastructure supporting transition than in admission policies per se. Unfortunately, there are no established, evaluated transition programs described in the literature on which such an infrastructure could be based. In New South Wales, the Transitional Care for Young People with Chronic Childhood Illnesses Group (part of the Greater Metropolitan Clinical Taskforce) is developing a state-wide strategy to address transition, which may serve as a blueprint for a national process (Box). Data collection, “gap” identification and the use of transition coordinators based in adult hospitals are all part of this initiative. Australia urgently needs to develop a national policy on transitional care that articulates the critical role of transition coordinators as well as the coordination of transitional care between paediatric and adult services. Success will also depend on educating health professionals and families about the value of coordinated transition; developing appropriate attitudes and expertise, particularly in adult services; comprehensively evaluating transition programs; examining health outcomes and cost–benefit issues; and involving consumer advisory groups.12,13 While additional resources may be needed, the overwhelming need is for a change of attitude and approach. Transition plan for young people and their families/carers*† * Based on a strategy being developed by the Transitional Care for Young People with Chronic Childhood Illnesses Group of the Greater Metropolitan Clinical Taskforce. †This model will require adjustment to meet the needs of special groups.

David L Bennett FRACP, FSAM · Susan J Towns FRACP · Kate S Steinbeck FRACP

Objective: To measure the pattern of admissions of young adults to a children’s hospital.Design and setting: Ten-year audit (1992–2001) of admissions of young adults aged 18 years and over to the Royal Children’s Hospital (RCH), Melbourne, with a detailed chart review of the 2001 cohort to assess disease complexity and transition planning.Outcome measures: Number of admissions, disease complexity, transition planning.Results: There was a significant increase in the number of young adults admitted over 10 years, from 308 in 1992–1993 to 659 in 2000–2001. The greatest increase was in admissions to surgical units. There was significant variation in admission practices between units over time. Many young adults required multidisciplinary care: 57% had more than three medical/surgical units involved in their care, and 34% had two or more allied health units involved. Fifty-one per cent of surgical inpatients and 28% of medical inpatients had no documented plan for transition to adult care. Only 30% of medical and 17% of surgical inpatients in 2001 had been transferred to adult services by 2002.Conclusions: Both disease complexity and failure of transition planning appear to have contributed to the increased admission of young adults to the RCH. While greater support of transition planning is needed, there are also concerns about the lack of appropriate services within the adult sector for young adults with complex, multidisciplinary healthcare needs.

Pei-Yoong Lam MB BS, FRACP · Bronwyn B Fitzgerald MB BS · Susan M Sawyer MB BS, MD, FRACP

Always take a history of snoring and sleep disturbance when reviewing children in primary care, as there is evidence that episodes of hypoxia and arousal during sleep may result in deficits in memory, attention and behaviour, in addition to the well known sequelae of growth failure, developmental delay and cor pulmonale. Check for changes in behaviour affecting school progress. To investigate for possible obstructive sleep apnoea syndrome (OSAS), clinical examination, lateral neck x-ray (adenoidal hypertrophy) and overnight oximetry (desaturation episodes) are useful screening tests, but oximetry is best used in conjunction with polysomnography. A negative oximetry test does not exclude OSAS. Polysomnography is the best method for detecting and assessing the severity of OSAS in children, and is especially helpful for prioritising treatment and evaluating the risk of perioperative complications of adenotonsillectomy. Adenotonsillectomy is thought to “cure” (ie, symptoms disappear and overnight respiratory parameters are corrected) in about 80% of children with OSAS. The remaining 20% need ongoing evaluation and treatment. Further research is needed to determine the “true” prevalence of OSAS; what degrees of severity of upper-airway obstruction lead to morbidity requiring treatment; and whether the deficits in neurocognitive function associated with sleep-disordered breathing are fully correctable.

J Declan Kennedy MD, FRCP, FRACP · Karen A Waters MB BS, FRACP, PhD

Environmental strategies must be complemented by behavioural approaches to help children learn to use roads safely In Australia, pedestrian injury is the leading cause of death among 1–14-year-olds.1 In 2000, 38 child pedestrians in this age group died2 and about 1140 (29 per 100 000) were hospitalised, often with lengthy stays, because of injuries sustained when hit by a vehicle.1 These rates decrease with age and are lowest for 10–14-year-olds.1 The most recent comparison with other OECD countries shows that Australia has the 13th lowest pedestrian fatality rate for 0–14-year-olds,3 with slightly more pedestrian deaths among 0–5-year-olds than the median rate for all OECD nations (1.03 per 100 000 versus 0.89 per 100 000).4 The primary predictors of this child pedestrian trauma relate to the interaction between the characteristics of the child and the design and nature of the road environment to which the child is exposed. A Western Australian case–control study of child pedestrians aged 1–14 years identified four key environmental and behavioural factors that independently predicted the likelihood of child pedestrian injury.5 These comprised the volume of traffic encountered by the child, presence of visual obstructions, availability of footpaths on the child’s street of residence, and the child’s behaviour. Predictors also vary according to the age of the child. Whereas 1–2-year-olds are more likely to be hit by a reversing vehicle, the most common cause of pedestrian trauma in 3–9-year-olds is mid-block “dart-out” (entering the road between intersections and not seeing, or misjudging, a gap in traffic).6,7 Pedestrians aged under 10 years are particularly vulnerable because of their small physical size and underdeveloped abilities for dealing with traffic situations, both cognitive (attention focus, interpreting traffic signs) and perceptual (locating sounds, judging speed, peripheral vision).6 Given these limitations, children under the age of 10 do not have the ability to cross roads without adult help. Ten to 14-year-olds are also vulnerable, but more because of their failure to apply safe pedestrian skills than because of their lack of skills. Further, road trauma in this age group may also be associated with general delinquency and problem behaviour.8 Many of these predictors of pedestrian trauma can be prevented or modified and are therefore amenable to intervention.7 While debate continues about the merits of environmental (passive) versus behavioural (active) intervention strategies to reduce this road trauma, evidence suggests that all are necessary, and no single strategy is sufficient.6 A multifaceted approach that combines strategies targeting the behaviour of all road users (including education, training and publicity), the road environment and vehicle design have been found to be the most effective.3 Strategic approaches involving public health, education, health promotion, urban planning, engineering and motor vehicle design are required. Consequently, while efforts are needed to make the road environment safer for pedestrians by reducing the speed and volume of traffic to which they are exposed, it is also necessary for pedestrians (particularly children) to learn how to use these road environments safely. Yet research into behavioural approaches to pedestrian safety has lagged behind environmental research.9 Behavioural programs for children need to be developmentally appropriate and include modelling and training by an adult in a social context and road environments relevant to the child. Programs also need to be interactive and involve problem-solving with consistent and prompt feedback from a caring adult, rather than merely following rules.10 The use of didactic knowledge-only strategies (such as rote learning of rules) is inappropriate, as younger children are not able to generalise this learning to real roads.11 Roadside training and, to a lesser extent, realistic simulations appear to improve visual timing and gap selection, to increase the ability to identify safe and dangerous crossing locations, and to enhance learning of appropriate strategies for crossing at parked cars. Such training has produced positive results with children as young as 5 years.12,13 Several new approaches to children’s pedestrian safety education are being tested. These include programs targeting younger children and adults who care for children, and use of new technologies. Two Australian reviews recommend targeting 0–5-year-olds, arguing that, with good quality pedestrian safety training, young children could demonstrate a rudimentary conception of danger, which improves with age.14,15 Some Australian jurisdictions have developed curricula and materials based on these approaches, such as the Victorian “Starting Out Safely” program. Engaging parents and helping them recognise their important role in their children’s pedestrian safety has the potential to significantly enhance children’s safety on and near roads.14 Parents provide the best role models and one of the only means for children to receive the necessary personalised one-on-one training and to practise crossing real roads. Technologies that use interactive simulations (eg, visual reality computer) coupled with real road experience and “pretend” road practice (the “pretend” road is set up parallel to a real one) can enable children to practise their skills, receive consistent and instant feedback and repetition, and be introduced with careful control to the complexity of traffic.16 While reviews of the impact of behavioural and environmental programs on preventing road trauma in children have demonstrated mixed success, it is apparent that programs need to take a comprehensive preventive approach with modifications to the road environment, enforcement, engineering and education. While much still needs to be done to determine the optimal mix and “dose” of these approaches to reduce child pedestrian trauma, every effort must be made to keep children safe near traffic and roadways that are becoming increasingly busy and complex.

Donna S Cross EdD · Margaret R Hall PhD

Early diagnosis of language disorders and autism is important, and early intervention for autism and some language disorders makes a difference. Developmental surveillance of children to detect these disorders should be a routine part of medical practice. The persistence and pervasiveness of communication and socialising deficits differentiate children with autism from those with specific developmental language disorders. Hearing and vision assessment is essential in any communication disorder. Interventions, targeted to identified areas of need, should encompass communication enhancement, behavioural therapy, educational modification, parent education and family support. Pharmacological interventions have an important but discrete role in autism, but there are no magic bullets. It is important to remember that the normal childhood illnesses occur in children with developmental disorders. Parents should be directed to reliable websites on the Internet, and given information and books to read as well as phone numbers of relevant services (eg, autism associations). There is a need for increased government financial support for early intervention programs.

John Wray FRACP · Helen Knott BApplSc(S · Natalie Silove FRACP, MMed, FCP(SA)

A child with atopy produces IgE antibodies after exposure to common environmental allergens. The atopic diseases (eczema, asthma and rhinoconjunctivitis) are clinical syndromes each defined by a group of symptoms and signs. Not all children with atopy will have atopic disease or develop symptoms after exposure to an allergen. Both genetic and environmental factors determine the development of atopic disease. The presence of specific IgE antibodies to environmental allergens is determined with skin prick or radioallergosorbent testing in children with atopy. Test results should be interpreted in the context of the clinical history and further investigations (eg, allergen avoidance or challenge). Management of atopic disease is frequently symptomatic, but it is important to avoid identified allergen triggers. Immunotherapy may be considered in selected school-age children with severe rhinoconjunctivitis. Preventing atopic disease in high-risk infants and hindering progression of disease in children with established disease are the areas of active research.

Michael S Gold MD, FCP, FRACP · Andrew S Kemp PhD, FRACP

Objective: To evaluate the effect of a behaviour modification program, taught to parents in a single visit to a trained nurse, in improving sleep performance in newborn infants.Design: Randomised controlled trial.Setting and participants: 268 families with normal newborn infants in the community, recruited between October 1996 and March 1997 from birth notices published in a South Australian daily newspaper.Intervention: A 45-minute consultation with a nurse 2–3 weeks after the birth, including a tutorial discussion on normal sleep patterns in newborn infants, supported by retained written material and, for infants with weight gain < 30 g daily, referral to their usual postnatal care provider.Main outcome measures: Hours of daytime sleep (0600–1800), night sleep (1800–0600) and total sleep per 24 h; and number of daily records with total sleep ≥ 15 h per 24 h, assessed by 7-day sleep diary at ages 6 and 12 weeks.Results: 268 families returned at least one sleep diary (137/171 intervention, 131/175 control), recording 3273 days. Two intervention infants were referred for low weight gain. Total sleep time was 15 h or more per 24 h on 62% of recorded days in the intervention group, compared with 36% in the control group (P < 0.001). At 6 weeks of age, intervention infants slept a mean 1.3 h per day more than control infants (95% CI, 0.95–1.65), comprising a mean 0.5 h more night sleep (95% CI, 0.32–0.69) and 0.8 h more daytime sleep (95% CI, 0.56–1.07). At 12 weeks, intervention infants slept a mean 1.2 h per day more (95% CI, 0.94–2.14), comprising 0.64 h more night sleep (95% CI, 0.19–0.89) and 0.58 h more daytime sleep (95% CI, 0.39–1.03). There was no significant difference in crying time between the groups.Conclusions: A single consultation supported by written material in the first 3 weeks of a child’s life improves sleep performance at 6 weeks of age. This improvement is maintained at 3 months.

Brian G Symon MD · John E Marley MD · A James Martin MD · Emily R Norman MB BS

Constipation is common in children, with prevalences ranging from 0.3% up to 28%. In most children, constipation is functional (ie, without objective evidence of a pathological condition). Painful defecation has been proposed as the primary precipitant of functional faecal retention in early childhood. Faecal soiling is often secondary to constipation, and may occur during spontaneous relaxation of the sphincters precipitated by rectal distension. Management in general practice combines behavioural modification techniques with prolonged courses of laxatives. Treatment is usually successful, but may take up to 6–12 months. Significant numbers of children with initially good responses to therapy for constipation relapse in the long term. Long-term relapse is more frequent in children under 4 years at onset of symptoms and in whom there is a history of faecal soiling associated with constipation.

Anthony G Catto-Smith MD, FRACP

Bedwetting (nocturnal enuresis) is common. It occurs in up to 20% of 5 year olds and 10% of 10 year olds, with a spontaneous remission rate of 14% per year. Weekly daytime wetting occurs in 5% of children, most of whom (80%) also wet the bed. Bedwetting can have a considerable impact on children and families, affecting a child’s self-esteem and interpersonal relationships, and his or her performance at school. Primary nocturnal enuresis (never consistently dry at night) should be distinguished from secondary nocturnal enuresis (previously dry for at least 6 months). Important risk factors for primary nocturnal enuresis include family history, nocturnal polyuria, impaired sleep arousal and bladder dysfunction. Secondary nocturnal enuresis is more likely to be caused by factors such as urinary tract infections, diabetes mellitus and emotional stress. The treatment for monosymptomatic nocturnal enuresis (bedwetting with no daytime symptoms) is an alarm device, with desmopressin as second-line therapy. Treatment for non-monosymptomatic nocturnal enuresis (bedwetting with daytime symptoms — urgency and frequency, with or without incontinence) should initially focus on the daytime symptoms.

Patrina H Y Caldwell FRACP, PhD · Elisabeth Hodson MRCP, FRACP · Jonathan C Craig FRACP, PhD · Denise Edgar RGN, BN

Ursula Bayliss,* Christopher Cowell,† James Hong,‡ Veronica Wiley,§ Bridget Wicken¶ * Clinical Nurse Consultant, §Principal Scientist, ¶ Clinical Director, NSW Newborn Screening Programme, † Head, Institute of Endocrinology & Diabetes, The Children's Hospital at Westmead, Westmead, NSW 2145; ‡ Paediatrician, North Gosford Medical Centre, North Gosford, NSW. bridgetwATchw.edu.au To the Editor: We report an acute presentation of congenital hypothyroidism in a child almost 6 years old. The condition was not detected by newborn screening. Screening of all neonates started in New South Wales in July 1977, with thyroid stimulating hormone (TSH) being measured in dried blood spots taken from a heel-prick blood sample (currently at 2–3 days of age). A whole-blood TSH level of 40 mIU/L or above triggers a request for full thyroid function testing, whereas with a level of 20–39 mIU/L a second sample is requested. We have screened over 2.3 million babies and detected 690 babies with congenital hypothyroidism. Ten babies with dyshormonogenesis or ectopic thyroid tissue had normal results and were missed by the screening test. Since screening started, “juvenile hypothyroidism” not associated with thyroid antibodies has all but disappeared. A healthy girl aged 5 years 11 months presented with acute dysphagia and drooling. There were no previous dysphagic symptoms. Initially, epiglottitis was suspected; however, at endoscopy a lingual thyroid was visualised at the base of her tongue, and this was confirmed by a technetium scan. She had normal growth and development, with both height and weight at the 50th centiles, a pulse rate of 90 beats/min, and normal deep tendon reflexes. The whole-blood TSH level at newborn screening on Day 3 was 40 mIU/L (reference range [RR], < 20 mIU/L). Thyroid function testing at another hospital on Day 10 showed a serum TSH level of 16.6 mIU/L and a serum free thyroxine (FT4) level within the normal range (12 pmol/L; RR, 11–30 pmol/L). These results were interpreted as normal, whereas, in fact, the TSH level was above the reference range for 10 days of age (< 10 mIU/L), although within the reference range for 2–7 days. On the patient’s admission for treatment of acute dysphagia, the TSH level was 10.9 mIU/L and the FT4 level was 18 pmol/L. A diagnosis was made of compensated hypothyroidism secondary to the ectopically placed lingual thyroid. Thyroxine treatment was commenced on diagnosis, and regular follow-up arranged. Three months after the start of treatment, the results of thyroid function tests (FT4, 17 pmol/L; TSH, 2.7 mIU/L) were within the normal range. Acute presentation of a lingual thyroid is most unusual.1 This case emphasises that further investigations must be performed when thyroid function test results are equivocal. Unfortunately, the thyroid status was considered normal because the FT4 value was within the normal range. All babies whose TSH results remain elevated while the FT4 levels are normal should have a thyroid scan, as we recommend when reporting results.

Ursula Bayliss · Christopher Cowell · James Hong · Veronica Wiley · Bridget Wicken

Aim: To ascertain the incidence of autism spectrum disorders in Australian children.Setting: New South Wales (NSW) and Western Australia (WA), July 1999 to December 2000.Design: Data were obtained for WA from a prospective register and for NSW by active surveillance.Main outcome measures: Newly recognised cases of autism spectrum disorders (defined as autistic disorder, Asperger disorder and pervasive developmental disorder not otherwise specified [PDD-NOS]) in children aged 0–14 years; incidence was estimated in 5-year age bands (0–4 years, 5–9 years, 10–14 years).Results: In WA, 252 children aged 0–14 years were identified with autism spectrum disorder (169 with autistic disorder and 83 with Asperger disorder or PDD-NOS). Comparable figures in NSW were 532, 400 and 132, respectively. Most children were recognised with autistic disorder before school age (median age, 4 years in WA and 3 years in NSW). Incidence of autistic disorder in the 0–4-years age group was 5.5 per 10 000 in WA (95% CI, 4.5–6.7) and 4.3 per 10 000 in NSW (95% CI, 3.8–4.8). Incidence was lower in older age groups. The ratio of all autism spectrum disorders to autistic disorder alone was 1.5:1 in WA and 1.3:1 in NSW, and rose with age (1.8:1 and 2.9:1 in 10–14-year-olds in WA and NSW, respectively).Conclusions: These are the first reported incidence rates for autism for a large Australian population and are similar to rates reported from the United Kingdom. Ongoing information gathering in WA and repeat active surveillance in NSW will help to monitor any future changes.

Katrina Williams PhD, FRACP, FAFPHM · Megan Helmer MHlthSc(CDM) · Craig M Mellis MPH, MD, FRACP · Marshall Tuck MPH · Emma J Glasson PhD · Carol I Bower MSc, PhD, FAFPHM · John Wray FRACP

Obesity in children and adolescents has reached alarming levels — 20%–25% of children and adolescents are overweight or obese, and 4.9% of boys and 5.4% of girls are obese. Rates of obesity have increased significantly in Australia from 1985 to 1995, with the prevalence of overweight doubling and obesity trebling. Body mass index (related to reference standards for age and sex) is recommended as a practical measure of overweight and obesity in children, and is used in monitoring individual progress in clinical practice. Obesity in childhood and adolescence may be associated with a range of medical and psychological complications, and can predispose individuals to serious health problems in adult life, including type 2 diabetes, hypertension, dyslipidaemia and non-alcoholic steatohepatitis. Obesity interventions for which there is some evidence include family support, a developmentally appropriate approach, long-term behaviour modification, dietary change, and increased physical activity and decreased sedentary behaviour. Prevention of obesity in children and adolescents requires a range of strategies involving changes in both the microenvironment (eg, housing, neighbourhoods, recreational opportunities) and the macroenvironment (eg, food marketing, transport systems, urban planning).

Jennifer A Batch MB BS, MD, FRACP · Louise A Baur MB BS, PhD, FRACP

Emma J Best,* Monica M Lahra,† Pam Palasanthiran‡ * Paediatric Infectious Diseases Fellow, † Microbiology Registrar, Neonatal Medicine, Royal Prince Alfred Hospital, Sydney, NSW. ‡ Infectious Diseases Physician, Sydney Children’s Hospital, Level 4, High Street, Randwick, NSW 2031; PalasanthiranpATsesahs.nsw.gov.au To the Editor: Pasteurella multocida is an oral commensal of domestic pets known to be an opportunistic human pathogen after traumatic animal contact. The most common infections in humans are skin and pulmonary infections. This report outlines a case of P. multocida meningitis, which has not previously been reported in Australia. A 19-day-old girl presented with a 12-hour history of fever and poor feeding. Her temperature was 39.5°C, and she was irritable, with no localising signs or skin lesions. A full septic screen was performed. Cerebrospinal fluid (CSF) showed a neutrophilic pleocytosis and gram-negative coccobacilli. She was treated with intravenous cefotaxime and gentamicin. Within 24 hours both CSF and blood cultures showed growth of gram-negative bacilli. The initial Gram stain, growth on chocolate agar and positive oxidase and catalase tests were suggestive of a Haemophilus species. However, further biochemical tests revealed the organism to be P. multocida. The infant made an excellent clinical recovery, with normal neurological and growth assessments at 6 and 12 months. The family owned two cats but reported no contact between their baby and the pets. A single tonsillar swab performed on each cat by a veterinarian 10 days after the baby’s presentation failed to isolate Pasteurella species. The family elected to keep the pets. Pasteurella meningitis occurs at extremes of age, in the immunocompromised (associated with liver cirrhosis, renal disease and haematological malignancies) and after traumatic head injury.1 Infants aged under 1 year account for almost half the cases of P. multocida meningitis. On review of the literature, we found 37 reported cases of P. multocida infection in infants (Box).1-5 In more than three-quarters of these cases, there was known contact with household animals — in more than half of these contact was non-traumatic (licking or presumed handling of the pet). Molecular studies in one of the cases with no history of traumatic contact confirmed that P. multocida isolates from pet and infected child were indistinguishable.2 This infection is unusual, and, given the popularity of household pets, the risk appears low. However, this case highlights the relative immunocompromise of newborn infants, and is a reminder of the importance of hand hygiene and preventing contact between newborn infants and pets. Details of 38 case reports of invasive Pasteurella multocida infection in infants (including current case)1-5 Mean age (range) 2.6 months (1 day– 11 months) Type of infection Meningitis 30 (79%) Puerperal sepsis, chorioamnionitis 7 (18%) Bacteraemia (postnatal) 1 (3%) Nature of animal contact Traumatic (scratch, bite) 9 (24%) Non-traumatic 22 (58%) Unknown 7 (18%) Type of animal (n = 31) Cat 16 (52%) Dog 11 (35%) Both 4 (13%)

Emma J Best · Monica M Lahra · Pam Palasanthiran

Megan L Counahan,* Ross M Andrews,† Rick Speare‡ * Surveillance Manager, Communicable Diseases Section, Department of Human Services, Level 17/120 Spencer Street, Melbourne, VIC 3000; † Senior Research Fellow, Centre for International Child Health and Clinical Epidemiology and Biostatistics Unit, Murdoch Children’s Research Institute, Melbourne, VIC; ‡ Professor, School of Public Health and Tropical Medicine, James Cook University, Townsville, QLD. megan.counahanATdhs.vic.gov.au To the Editor: For parents to treat head lice (pediculosis) effectively in their children, it is necessary for them first to recognise it is present. We conducted a school-based screening program involving 1838 children from 16 randomly selected primary schools in Victoria between May and October 2001 (participation rate, 55.2%).1 As part of this program, we compared a written report from parents on their child’s pediculosis status against results of our examination (7–10 days later). We examined the scalp and hair of each child for lice (“crawlers”) or viable louse eggs (“active infestation”) and dead or hatched louse eggs (“inactive infestation”) using white hair conditioner, which makes lice and eggs easier to see with the naked eye, and a fine-toothed head lice comb. This is a validated, accurate and sensitive diagnostic technique.2 Parents were unaware of the proposed screening date, and the study team was unaware of the parents’ reports. We compared parental report about pediculosis against results of our screening for 1179 children who could be matched with completed questionnaires. Overall, 149 children (12.6%) had active pediculosis, but parents reported head lice in only 36 children (3.0%) (Box 1 and Box 2). These comprised 24 of the 149 children with confirmed pediculosis (16%), and another 12 children who did not have pediculosis when examined. The positive predictive value (PPV) of parental report was 66.6%, indicating that parental reporting was not a reliable indicator of pediculosis. An implication of the low PPV is that some children may have been unnecessarily treated with insecticide for an infestation they did not have. On the other hand, a substantial proportion of children with head lice had not been identified by their parents and could contribute to ongoing transmission within schools. While it was possible they were infected subsequent to completion of the questionnaire, this seemed unlikely, as 72% were found to have multiple louse eggs, indicating a longer duration of infestation than the 7–10 days since the questionnaire was completed. Our study clearly demonstrates that parental reporting of head lice in their children is unreliable. We suggest several possible reasons: parents did not see the head lice, did not recognise them, or used a diagnostic technique with a lower sensitivity than the method we chose, such as examining dry hair. It is also possible that parents were inhibited from reporting pediculosis by the possible repercussions, such as exclusion of the child from school. Indeed, children whose parents failed to answer the question about pediculosis had a higher prevalence of head lice than those whose parents answered. Nevertheless, parents’ management of pediculosis is likely to improve if a sensitive detection method is used. To improve the sensitivity of parental diagnosis and control of head lice we recommend that parents be instructed to screen their children weekly using hair conditioner and combing. 1 Screening results compared with parental report Pediculosis by parent report Pediculosis on examination Yes No Total Yes 24 12 36 No 99 969 1068 Unsure 26 49 75 Total 149 1030 1179 2 Sensitivity and specificity of parental report versus screening Pediculosis prevalence By parental report 3.0% (36/1179) By screening 12.6% (149/1179) Sensitivity 16.1% (24/149) Specificity* 98.8% (1018/1030) Positive predictive value 66.6% (24/36) Negative predictive value* 89.0% (1018/1143) * Specificity and negative predictive value were calculated after grouping “unsure” and “no” responses.

Megan L Counahan · Ross M Andrews · Rick Speare

In most cases, childhood leukaemia has a fetal origin, but multiple molecular events are required after birth for pre-leukaemic cells to progress to leukaemia. Cure rates for acute lymphoblastic leukaemia (ALL) now approach 80%. A high level of minimal residual disease detected by polymerase chain reaction in patients with ALL in remission has profound prognostic importance and is the focus of a major Australian study attempting to prevent relapse in these children. Greater awareness of the late effects of chemotherapy has led to changes in the treatment protocols for ALL, with improvement in neurocognitive outcomes and reduced rates of second malignancies. Pharmacogenetics is a new field of research that aims to enhance treatment efficacy by assessing the individual’s metabolism of and response to chemotherapeutic agents. Targeted therapies currently being developed show some promise of being able to further improve cure rates. Adolescents with ALL have a better prognosis if treated with paediatric rather than adult protocols.

David S Ziegler MB BS · Glenn M Marshall MB BS, FRACP · Luciano Dalla Pozza MB BS, FRACP · Keith D Waters MB BS, FRACP

CorrectionRe: “Prescribing of amino acid formula”, by Andrew S Kemp in the 15 November 2004 issue of the Journal (Med J Aust 2004; 181: 574-575). The author’s position and address were omitted. Dr Kemp is Professor of Paediatric Allergy, The Children’s Hospital at Westmead, Locked Bag 4001, Westmead, NSW 2145. andrewk5ATchw.edu.au The html and pdf versions of his letter published in the eMJA were corrected on 21 December 2004.

Andrew S Kemp

Gwendolyn L Gilbert,* Heather F Gidding,† Josephine Backhouse,‡ Peter B McIntyre§ * Director, ‡ Serology Project Officer, Centre for Infectious Diseases and Microbiology, Institute of Clinical Pathology and Medical Research, PO Box 533, Wentworthville, NSW 2145. † Epidemiologist, § Director, National Centre for Immunisation Research and Surveillance of Vaccine Preventable Diseases, University of Sydney, Westmead, NSW. LyngATicpmr.wsahs.nsw.gov.au To the Editor: Varicella vaccine was licensed in Australia in 2000. It is safe and efficacious and can prevent significant acute morbidity, significant out-of-pocket expenses for parents of affected children and, in Australia, prevents an estimated 450 admissions to hospital and one death per year.1 In September 2003, the National Health and Medical Research Council (NHMRC) recommended giving varicella vaccine to all children at 18 months of age.2 The net effect of childhood immunisation on varicella morbidity will depend on vaccination coverage. Modelling of Australian (unpublished National Centre for Immunisation Research and Surveillance of Vaccine Preventable Diseases data) and UK3 seroprevalence data suggests that, for a range of vaccine efficacy estimates, 80% coverage is required before morbidity is reduced in adults. Accurate Australian coverage data are unavailable, as varicella vaccine is not funded under the National Immunisation Program.2 National serosurveillance can provide a practical alternative estimate of vaccine uptake. The first Australian national serosurvey of vaccine-preventable diseases, for which sera were collected between July 1996 and February 1999, established baseline seroprevalence for future assessment of the effects of changes in the vaccination schedule. It showed that the incidence of varicella was highest in 5–9-year-old children,4 indicating that infant vaccination would provide optimal protection. The second national serosurvey (with sera collected in 2002) is under way, with methods identical to those of the first.4 We compared varicella IgG levels in children aged 1–5 years, testing 459 sera in the first serosurvey and 380 in the second (see Box). The proportion with protective or equivocal antibody levels increased between the two serosurveys. The difference was statistically significant only in 3–4-year-olds, which is consistent with the greatest uptake when children enter childcare. Although there is some variation in varicella incidence over time, the changes are consistent with only modest vaccine uptake in the time that varicella vaccine has been available in Australia. The only other available estimates of varicella vaccine uptake are from GP consultations5 and reports to the Australian Childhood Immunisation Register (ACIR), which also indicate it is modest. For example, only 6.2% of children aged 4 years were reported to the ACIR to have received varicella vaccine (Brynley Hull, Epidemiologist, National Centre for Immunisation Research and Surveillance of Vaccine Preventable Diseases, personal communication). This is probably an underestimate, as there is no incentive for notifying varicella vaccination. However, a low vaccination uptake is consistent with use only in the private sector, and could lead to an increase in adult morbidity (despite an overall reduction in infection rates) because of a higher average age of infection (unpublished National Centre for Immunisation Research data). Unless vaccine is provided in the routine immunisation program at no cost to parents, uptake is unlikely to meet the 80% target required to reduce disease burden in all ages.3 Varicella IgG seroprevalence in preschool children in the 1st and 2nd national serosurveys 1st serosurvey, July 1996 to February 1999 2nd serosurvey, 2002 Age group No. Positive Negative Equivocal* No. Positive Negative Equivocal* Increase in positive and equivocal (95% CI) P 1 to < 3 years 138 27 (19.6%) 111 (80.4%) 0 152 34 (22.4%) 118 (77.6%) 0 2.8% (−6.6 to 12.2) 0.6 3 to < 5 years 214 72 (33.6%) 140 (65.4%) 2 (0.9%) 152 73 (48.0%) 79 (52.0%) 0 13.4% (3.3 to 23.6) 0.01 5 years 107 61 (57.0%) 46 (43.0%) 0 76 43 (56.6%) 33 (43.4%) 0 −0.4% (−15.0 to 14.1) 1.0 Total 459 160 (34.9%) 297 (64.7%) 2 (0.4%) 380 150 (39.5%) 230 (60.5%) 0 4.2% (-2.4 to 10.8) 0.2 *Sera giving equivocal results by enzyme immunoassay were retested and most were resolved by immunofluorescence (IF). These results represent the few that were still equivocal by IF and are probably low-titre positive results.

Gwendolyn L Gilbert · Heather F Gidding · Josephine Backhouse · Peter B McIntyre

Ailsa Goulding,* Andrea M Grant,† Peter L Davidson‡ * Professorial Research Fellow, † Assistant Research Fellow, Department of Medical and Surgical Sciences, ‡ Research Fellow, Injury Prevention Research Unit, University of Otago, Dunedin, New Zealand. Ailsa. GouldingATstonebow.otago.ac.nz To the Editor: The interesting article on playground safety and arm fractures by Sherker and Ozanne-Smith1 documents a steady increase in hospitalisation rates for arm fractures among Victorian children between 1987 and 2002. It would appear that the increase over this 15-year period was about 45%. These figures may represent the tip of the iceberg, as few children with arm fractures are admitted to hospital and most are treated on an outpatient basis. Increases of similar magnitude in forearm fractures in adolescence have also been reported recently in the United States.2 It would seem that children are becoming more vulnerable to such fractures. This is a concern, as fractures of the distal forearm are extremely common during growth. Indeed, about a quarter of all fractures during childhood and adolescence occur at this site. One factor that might be contributing to this rising incidence of arm fractures during growth is increasing childhood adiposity. Childhood obesity has increased sharply in Australian children over recent years.3 Obese children fall with more force, and, although they may have more bone for chronological age than children of healthy bodyweight, adaptive increases in bone mass are not enough to accommodate their high bodyweight gain, placing them at a biomechanical disadvantage during falls on the outstretched arm.4 Our studies of consecutive series of girls and boys with distal forearm fractures indicate that a high proportion of these children are overweight. Moreover, in a 4-year prospective study of 170 girls, we found that high bodyweight at baseline increased the risk of new fractures.5 In their study, Sherker and Ozanne-Smith measured the heights and weights of 402 children under 13 years of age who had broken their arm falling from playground equipment between 2000 and 2002. We wonder how many of these children were overweight or obese for their age. Examination of the body mass index values would provide this information, and we ask that the authors report these data for both girls and boys using international cut-off points. We would like to know whether or not overweight is contributing to rising rates of arm fracture in Australian playgrounds.

Ailsa Goulding · Andrea M Grant · Peter L Davidson

Shauna Sherker,* Joan Ozanne-Smith† * Postdoctoral Research Fellow, NSW Injury Risk Management Research Centre, University of New South Wales, Sydney, NSW; † Chair of Injury Prevention, Accident Research Centre, Monash University, Melbourne, VIC. Shauna. SherkerATunsw.edu.au In reply: The prevalence of obesity has increased dramatically among Australian children, particularly over the past 2 decades.1 However, the role of obesity as a risk factor for fall-related arm fracture remains unclear. Using standard definitions for child overweight and obesity,2 our study population3 (Box) did not demonstrate quite as high a prevalence of obesity as had been previously reported for Victorian schoolchildren.1 This case series was part of a larger, yet to be published case–control study, the results of which indicate no significant difference in body mass index between children who fell from playground equipment and fractured their arm (cases) and those who fell and landed on their arm with no significant injury (controls). The negative public health effects of increasing prevalence of obesity among Australian children highlight the need to promote safe and enjoyable physical activity. Playground equipment provides a very popular means of physical activity for children. Improving playground safety standards to minimise the risk of arm fracture — a traumatic, costly and preventable childhood injury — has never been more urgent. Children with arm fracture caused by falls from playground equipment, showing proportion of children who were normal weight, overweight or obese.* * Based on unpublished data from Sherker and Ozanne-Smith.3

Shauna Sherker · Joan Ozanne-Smith

Child health will only improve when local structures are further strengthened, enabled and supported Most countries in the western Asia-Pacific region have made consistent gains in child survival over the past 25 years (Box 1).1,2 Notable exceptions to this positive trend are Papua New Guinea (PNG) and East Timor. Sadly, a static child mortality rate such as has occurred in PNG, where the population has doubled over the past 25 years, means that, in this new century, about twice as many children are dying per year as in the mid-1970s. The health inequities between Australia and its nearest neighbours are many, and breathtaking in magnitude. In this issue of the Journal, McGain et al (page 687) document 87 deaths from snakebite at Port Moresby General Hospital (PMGH) (the largest hospital in PNG) over a 10-year period.4 This hospital serves a population of about 500 000 people. By comparison, throughout the whole of Australia since 1981 there have been an average of 2.6 snakebite deaths per year.5 McGain et al point out that lack of antivenom is a major reason for the high mortality, and that the cost of antivenom in real terms is 40 times greater in PNG than in Australia. The reasons for this include a per-capita gross national income that is 2.9% of that in Australia,2 price mark-up with privatisation of overseas distribution from Australian suppliers, and an Australian government subsidy for antivenoms sold in Australian hospitals that does not apply elsewhere. It is not difficult to suggest some feasible solutions to this problem: an Australian government subsidy for overseas developing-country purchasers, and/or direct supply from the manufacturer to the PNG Department of Health. However, snakebite is just one small piece of a very large puzzle of poor child health outcomes in PNG and the Asia-Pacific, and lack of antivenom is only one factor in high death rates from snakebite. Other factors are more systemic: limited access to health services, limitations in the quality of health systems, inadequate manpower, and poor management and financing. These problems are greater in rural areas, where 85% of the population lives; they have a major impact on all causes of avoidable child mortality, and are more difficult to solve than the lack of snake antivenom. PerspectiveAt PMGH, for every child who dies from snakebite, more than 50 die from other conditions that have been eradicated or controlled in Australia. In a 12-month period in 2001–2002 there were 238 child deaths at PMGH, of which four were from snakebite. Of 195 deaths in which the cause could be certified, 29 were from measles, 35 from meningitis (about a third of which were caused by Haemophilus influenzae type b [Hib]), 14 from HIV, 7 from tuberculosis, 66 from pneumonia and 11 from acute gastroenteritis.6 Throughout PNG, about a third of Hib isolates are resistant to available antibiotics (principally chloramphenicol),7,8 and rates of HIV are rising rapidly. The persistence of these infections in PNG despite the existence of effective methods of prevention or control should be a cause for concern and action in Australia as well as in PNG. Two-thirds of all child deaths are associated with moderate to severe malnutrition. On the role of aid and economic developmentAustralia currently allocates $435 million in aid to PNG (representing 20% of Australia’s official development assistance and 0.26% of its gross national income9 — far short of the benchmark of 0.7% agreed to by rich nations at the Earth Summit in 1992, and only achieved by Scandinavian countries and The Netherlands10). Much of Australia’s aid is now tied to strengthening law-making and law-enforcement facilities and financial management, but a proportion is allocated to social services (principally health and education). Aid programs in PNG often find themselves “between a rock and a hard place”. Sustainable development cannot occur in an environment of poor governance. When existing systems are not functioning well, one outcome, sometimes occurring by default and sometimes by design, has been the development or evolution of “parallel projects”, which circumvent existing government structures to achieve a flow of services or information to the periphery. There is a tension between this project approach and the building of genuine long-term capacity (ie, the resources and structures that enable self-sustainability) within government programs. However, if inequity within PNG and between PNG and Australia is to be reduced, aid allocated to social services must be spent in ways that will strengthen local systems so that services reach the most marginalised communities. One example of this dilemma is the Women’s and Children’s Health Project, funded by the Australian government and launched in PNG in 1997 (funding will cease at the end of 2004). The project has allocated $10 million a year to improve child and family health services. Credit must be given for its achievements, such as improvements to the vaccine “cold chain” (previously a major limitation on the quality of vaccines distributed in remote areas) and training and capacity support in some rural areas. However, results have generally been disappointing. Only a small proportion of the aid money has filtered down to the villages and settlements where child mortality is highest. Much has been consumed by large infrastructure costs in Port Moresby. In an attempt to tick off activities as completed achievements, weak and sometimes frustratingly inefficient government systems have often been circumvented using a “parallel project” mentality, rather than taking the much slower approach of working with and strengthening existing local structures. In a thought-provoking but pessimistic review, Professor Helen Hughes, Senior Fellow at the Centre for Independent Studies, has argued that aid has failed the Pacific nations.11 She believes that aid has created an ambiguity of independence, an environment in which government funds are spent on consumption rather than economic development, elevated exchange rates, and provided fodder for political corruption. These factors, plus high tariffs, have hindered manufacturing for domestic markets and export of agricultural products; reduced employment opportunities, skill development and entrepreneurship outside the government sector; and encouraged dependent welfare states. A partial solution suggested by Hughes11 is to make receipt of aid conditional on achieving certain goals, under an agreement of mutual obligation. This would require removing aid from government budgets, with mutual agreement between recipient and donor countries on its use, mutual monitoring, and disbursement subject to regular account auditing. Successful examplesPapua New Guinea (PNG) needs human capacity to provide a quality health service. In some areas of endeavour, this has been achieved. The Paediatric Society of PNG is one example of the slow and successful development of indigenous technical and professional capacity. This is a story of committed engagement by many paediatricians over four decades, building on the foundation laid by the late Professor John Biddulph. Progress has been based on the principles of quiet example and mentorship, working together at the front line of healthcare and grappling with everyday problems. In the past decade there has been increasing development of a few subspecialty areas and extraclinical skills, such as public health, research, evidence-based understanding, policy development, advocacy and child health nursing capacity. Australian public hospitals and individual paediatricians have played key enabling roles in this development, and, in turn, their support has been greatly assisted by AusAID through the PNG Medical Officer, Nursing and Allied Health Professional program and its predecessors. The outcomes are impressive. Locally trained PNG paediatricians now provide services in most of the 20 provinces, and contribute substantially to all areas of public child health, policy and service delivery.12 The PNG standard treatment manual,13 along with the National Government Health Plan, is a blueprint for a quality child health service in a resource-poor setting, and has been reproduced in many other developing countries and in internationally adopted strategies. These advances have only been achieved through the work of vital national child health institutions, the PNG Paediatric Society and the Department of Child Health at the University of PNG, with aid projects providing background support at various stages. Limiting factors to progressDespite some successful programs, many activities in PNG have not resulted in health gains where they are needed. Support areas of the health service remain weak: health and human resources management at all levels, drug and vaccine procurement, distribution and stock management, and health financing. Primary care, the most essential form of healthcare in rural areas but the least robust and most vulnerable level of the health service, has suffered the most because of these deficiencies.14 There needs to be a similar concentration on building capacity and commitment in these areas, improving efficiency, and minimising waste of resources and squandering of funds.15 The beginnings of progress in some of these areas have occurred. Health management is stronger in some provincial health services and hospitals now than it was 10 years ago, partly as a result of structural reforms and support and mentoring for management capacity provided by the AusAID-funded Health Sector Support Program. More needs to be done, but sustainable change will only occur slowly, tailoring strategies to individual situations — an approach that is at odds with some aid projects, whose designers often propose a “one size fits all” formula for rolling out the latest Big Idea, with little critical evaluation of outcomes. Equity and conditionality as principles of aidConditional aid, as Hughes suggests,11 might be a useful strategy, providing direct funding to carefully selected high-priority areas, with an agreement that certain process milestones will be reached. Potential examples might be Australian government funding of Hib vaccine, conditional upon completion of the national supplemental immunisation activities16 and achieving coverage of over 80%; or subsidising the purchase of snake antivenom, nevirapine and ceftriaxone, conditional upon improvements in drug procurement and national distribution systems. A further condition to ensure commitment and sustainability would be the understanding that the PNG government would take over responsibility for funding after a mutually agreed period of time. These targeted interventions would have broad benefits to the health service, would enable the implementation of new (to PNG) and highly effective interventions, and would enhance equity within PNG and between our two countries. However, there are some risks with conditional agreements. The withholding of interventions if conditions are not met would continue to hurt the people who are innocent of any waste or corruption — nurses and doctors who struggle every day to provide good healthcare, and the patients who suffer from the effects of a lack of quality services. Support should be given to local training institutions rather than aid projects running unsustainable training programs. A portion of the aid budget could be provided to build key areas of capacity by financially supporting individuals or groups committed to collaboration in ways that are appropriate to Melanesian society. AusAID and the PNG Health Department are currently proposing a step in this direction by establishing a Capacity Building Service Centre, which will place more emphasis on engaging locally successful individuals to act as mentors, build capacity, and result in significant changes to external contracting. The approaches outlined above are complementary and would reduce inequity between Australia and its Pacific neighbours. In some ways, they would be a departure from some current large aid projects, whose economic benefits often spin back to the donor country, and whose resources are consumed by project infrastructure that duplicates government institutions. I can see little place in a country like PNG for health projects that are managed by overseas private consortia. International tendering for health projects in a country that has no structured health management organisations can scarcely improve equity. This model has worked better for some aid development projects, such as road and water supply contracts, in which local engineering companies have won contracts, thus contributing to local development, employment and economic growth. However, the idea that health aid should be corporatised in a country that desperately needs an effective public health system is fundamentally flawed. No easy answersThere are no easy answers to how Australia can best assist regional countries. Ongoing engagement remains necessary at many levels — between governments, professional societies, institutions, and individuals. Without this there can be no mutual understanding, which provides the basis for progress and is crucial for regional peace. Some of the best examples of success suggest that sustained, quiet and modest-budget collaboration by committed groups or individuals who treat each other as equals will be the most effective strategy. As yet I have not addressed the problems of child mortality in the worst-affected country in the region — East Timor. A sustained collaborative approach would be of great benefit to this small country (Box 2). The view of PNG as a “failed state” is wrong. Progress is being made in many areas. Now is not the time for Australia to abandon PNG or our closest Pacific neighbours, but to learn from institutions and areas that have achieved much, to support them to do more, and to tailor approaches to specific situations. Australia also has much to learn from Pacific countries — quiet persistence, patience and a sense of community are qualities that might help us have a more realistic view of what progress really means. 1 Trends in mortality in children under 5 years (per 1000 live births) in the Asia-Pacific region over the past 50 years1-3 * In a Demographic Health Survey (DHS) in East Timor in 2003, the mortality rate in 2003 among children under 5 years of age was estimated to be 107 per 1000 live births. Mortality rate estimates from the previous eras, represented on the graph, are also based on retrospective data from the 2003 DHS, so the accuracy of these trends is uncertain. Reliable data from previous years are not available. 2 Increasing East Timor’s capacity to meet its child health needs The problems of capacity in East Timor are even deeper than in Papua New Guinea (PNG), and the child health system is in a much more embryonic stage of development. Currently, there are no East Timorese paediatricians, which is a major impediment to sustainable progress, local leadership, autonomy and direction. However, collaboration between the East Timorese Ministry of Health, the University of PNG and the Royal Australasian College of Physicians (RACP) will hopefully see East Timorese doctors trained in child health, largely in PNG, with some additional clinical experience in rural hospitals in Australia, over the next 5–10 years. This will provide training in settings that are similar to those of East Timor, foster personal and institutional connections between two developing countries, promote a developing-country university as a regional centre for high-quality specialist training, and minimise the risk of “brain drain” that would exist if specialist RACP Fellowship training were done in Australia.

Trevor Duke MD, FRACP, FJFICM

To the Editor: We report an unusual case of Ross River virus (RRV) disease in a 7-year-old child. The patient presented to her general practitioner with fever, rash and pain in the lower limbs. Swelling of the joints of the hands and left knee was found, with a widespread rash that covered the trunk, limbs and face. The rash comprised lesions of varying types, including maculopapular, vesicular and petechial lesions (Box). Rash in a child with Ross River virus disease The general practitioner transferred the patient to the state tertiary paediatric service. No antibiotics were given before transfer. On arrival at Princess Margaret Hospital for Children, Perth, the patient was unwell, with a fever of 38°C. A provisional diagnosis was made of septicaemia (probably meningococcal), and treatment was begun with intravenous ceftriaxone. Extensive investigations were performed, but results of all initial serological, polymerase chain reaction and culture investigations were negative. Rheumatology review was requested because of the prominent arthritic component of the illness. This revealed widespread polyarthritis, and the illness was felt to be a reactive or post-infectious process. The child’s family raised the possibility of RRV disease, as her grandmother had had this disease several years previously, and the child had stayed overnight at her grandmother’s home in a coastal lake area 2 weeks before disease onset. The area had abundant mosquitoes, as well as kangaroos, which are vertebrate amplifiers for RRV.1 Serological tests for RRV were performed 3 days after admission, and were negative for IgG and positive for IgM. Repeat serological testing during convalescence showed a fourfold rise in IgG titre (from 80 to 320), confirming the diagnosis of RRV disease. The patient’s rash decreased over several days. She had persistent synovitis in the left knee at review 3 weeks after admission. At review at 8 weeks all symptoms and signs had resolved, and she had full function. This case highlights the fact that, while RRV disease with severe symptoms and arthritic manifestations is uncommon in children, it nevertheless should still be considered in the differential diagnosis of children with a febrile and arthritic disease.1,2 This child’s illness appears to have been a reactive vasculitis and polyarthritis, which, while well recognised with other infections, is not well described in association with RRV disease in children. RRV arthritis is caused by joint infection, and treatment is currently based on empirical anti-inflammatory regimens. During the recent RRV disease epidemic in Western Australia, 1174 notifications for RRV disease were received between 1 October 2003 and 31 March 2004. Of these, 21 patients were aged 15 years or younger. Thus, while RRV disease is an infrequent illness in children, it does occur, and should be considered in the differential diagnosis of a child who presents with a febrile illness, rash and joint symptoms from an area with known autochthonous transmission of RRV.

Kynan T Feeney · Kevin J Murray · Amanda J Whittle · Gary K Dowse

Trial: Hatakka K, Savilahti E, Pönkä A, et al. Effect of long term consumption of probiotic milk on infections in children attending day care centres: double blind, randomised trial. BMJ 2001; 322: 1327-1329. QuestionIn children attending daycare centres (population), does long term consumption of probiotic milk (intervention) prevent infections (outcome)? Trial detailsObjective: To examine whether long term consumption of a probiotic milk could reduce gastrointestinal and respiratory infections in children in daycare centres. Design: Randomised, double-blind, placebo-controlled study over 7 months. Setting: 18 daycare centres in Helsinki, Finland. Participants: 571 healthy children aged 1–6 years; 282 in the intervention group (mean age, 4.6 years; SD, 1.5 years) and 289 in the control group (mean age, 4.4 years; SD, 1.5 years). Intervention: Milk with or without Lactobacillus GG. Average daily consumption of milk in both groups was 260 mL. Main outcome measures: Number of days with respiratory and gastrointestinal symptoms, absences from daycare because of illness, respiratory tract infections diagnosed by a doctor, and courses of antibiotics. Results: Children in the Lactobacillus group had fewer days of absence from daycare because of illness (4.9 days [95% CI, 4.4–5.5] versus 5.8 days [95% CI, 5.3–6.4]; absolute difference, 16% [P = 0.03]). Corresponding age-adjusted findings were 5.1 days (95% CI, 4.6–5.6) for the Lactobacillus group versus 5.7 days (95% CI, 5.2–6.3) for the control group; age-adjusted difference, 11% (P = 0.09). There was also a relative reduction of 17% in the number of children having respiratory infections with complications and lower respiratory tract infections (unadjusted absolute reduction, 8.6%; 95% CI, -17.2% to -0.1%; P = 0.05; age-adjusted odds ratio, 0.75; 95% CI, 0.52–1.09; P = 0.13) and a 19% relative reduction in courses of antibiotic for respiratory infection in the Lactobacillus group (unadjusted absolute reduction, -9.6%; 95% CI, -18.2 to -1.0; P = 0.03; adjusted odds ratio, 0.72; 95% CI, 0.50–1.03; P = 0.08). Conclusions: Lactobacillus GG may reduce respiratory infections and their severity among children in daycare. The effects of the probiotic Lactobacillus GG were modest but consistently in the same direction. CommentaryRationale for the trialChildren attending daycare centres are more likely to suffer gastrointestinal and respiratory infections than children cared for at home or in small family groups.1 There are public health and economic consequences, including direct medical costs and the indirect cost of parents taking time off work to care for sick children.2 Studies support the use of probiotics to reduce the incidence of antibiotic-associated diarrhoea3,4 and to hasten recovery from rotavirus diarrhoea.5 Probiotic bacteria may have a beneficial effect on the host immune response by altering intestinal microbial balance. Trial methodsThis was a randomised, double-blind, placebo-controlled trial carried out in 18 daycare centres in children aged 1–6 years over 7 months, which included winter. Daycare staff, parents, children and investigators were blinded or unaware of treatment allocation. Randomisation was effectively concealed, as children were allocated to intervention or control groups by a computer-generated block-randomisation procedure, with stratification on the basis of age and daycare centre. The randomisation procedure was successful in equalising baseline characteristics between placebo and active groups, apart from the control group having slightly younger children and more children with more than five recent infections. There was excellent follow-up, with nearly 90% of children completing the study, although reasons for withdrawal were not stated. Groups were analysed on an intention-to-treat basis. A random selection of 100 faecal samples was assessed to confirm compliance. Outcome measures included days of respiratory or gastrointestinal symptoms, days of absence from daycare because of illness, and number of upper respiratory tract infections complicated by lower respiratory tract infections. In summary, this trial was well designed and conducted. The average compliance in both groups was 60%. Unfortunately, there was a difference in age distribution between the two groups after randomisation. The investigators decided before the study that a minimum clinically relevant beneficial effect would be a 20% difference between the groups. Thus, based on previously reported episodes of illness in children attending daycare, they used a power calculation to estimate that, to detect a 20% difference with a power (or sensitivity) of 90% with 95% confidence, they needed to enrol 250 children per group. They were able to achieve this goal. New informationThe authors claim that milk containing Lactobacillus GG slightly reduced the incidence of respiratory infections and antibiotic treatment in children. The effect of probiotics was modest when adjustments were made for age. Of the measured variables — days of any illness, days of respiratory or gastrointestinal symptoms, and absence because of illness — only for absence because of illness was there a difference between the intervention and control groups that approached statistical significance, although there was a trend towards less illness for the other variables in the Lactobacillus group. It is useful to look at the tables of results in the article in question. Table 2 presents the raw data, and it is clear from scanning both the unadjusted and age-adjusted results that the effect is modest at best. The unadjusted days of absence because of illness was 4.9 days in the probiotic group and 5.8 days in the control group (ie, an improvement of 0.9 days); when adjusted for age, the difference between the probiotic group and the control group was 0.6 days. This raises the question of whether a reduction of 0.6 days in duration of absence because of illness is actually clinically useful. When the episodes of illness were diagnosed by a doctor (Table 3 of the article), the overall episodes of illness and courses of antibiotics prescribed for infections were significantly reduced for the probiotic group. Of all antibiotic courses prescribed, there were 119 in the probiotic group versus 144 in the control group, with an absolute percentage reduction of 8.0% (95%CI, -16.6 to 1.0). The P value was 0.07, thus approaching, but not reaching, statistical significance. However, the age-adjusted results (reported as odds ratios) are less impressive and the statistical significance of these findings is further reduced, with a 95% confidence interval crossing unity, and a P value of 0.17 — not significant. The results are thus entirely consistent with a chance difference. Implications for clinical practice This is the first large, high quality study to examine this interesting research question.6 There were no reported harmful effects of the Lactobacillus GG; costs were not fully explored in the report. The author of the accompanying editorial stated that probiotics “show promise but bigger studies are needed”.7 This is not entirely correct, as the study was adequately powered to detect a 20% difference and it failed to report a convincing clinical effect. However, it is likely that further clinical research will be undertaken in this and related areas, such as the use of probiotics to hasten recovery from acute gastroenteritis in children and the prevention of antibiotic-associated diarrhoea.

Mark G Coulthard MB BS, FRACP · Craig M Mellis MD, MPH, FRACP