Optic neuropathy from hypovitaminosis A in a series of children with severe dietary restrictions.

AIM: Hypovitaminosis A is a leading cause of preventable childhood blindness, especially in developing nations. Vitamin A is a fat-soluble essential micronutrient that serves vital functions in the visual system and in regulating bone resorption. We report on a series of four children with mixed nutritional and compressive optic neuropathy and provide a review of the literature. METHODS: A retrospective observational study of four males (ages 9-12), three with autism spectrum disorder who presented with loss of vision and multiple vitamin deficiencies including hypovitaminosis A. RESULTS: Patients presented with unexplained visual loss or a change in visual behaviour. All patients had severely restricted diet comprising of predominantly carbohydrates. Two of the four cases demonstrated optic nerve pallor at initial presentation with marked optic atrophy developing in all patients over time. Electrophysiology available in two patients demonstrated optic nerve dysfunction with preserved retinal function. Extensive investigations revealed profound deficiency in multiple vitamins including vitamin A (<0.1-0.2 µmol/L, normal = 0.9-1.7 µmol/L). Three patients also had low vitamin B12 (90-111 pmol/L, normal = 170-800 pmol/L) with normal folate. All four cases had radiological evidence of skull base thickening indicative of low vitamin A. Genetic testing did not find any relevant pathogenic variants. CONCLUSIONS: Hypovitaminosis A is a crucial form of nutritional deprivation that results in significant visual loss with potential hyperostosis and optic nerve compression exacerbating nutritional optic neuropathy. Additional micronutrient deficiencies usually co-exist and may contribute. Extra vigilance in vitamin replacement is required of clinicians with patients with autism who have restricted diets.

Typhoid fever in children in Goroka, Papua New Guinea.

BACKGROUND: Typhoid is endemic in many low-income countries, including in Papua New Guinea. This study aimed to describe the burden and clinical features of typhoid in children in a provincial hospital, to describe environmental conditions that lead to typhoid, and to document the antibiotic sensitivity of Salmonella spp. in the Eastern Highlands Province. METHODS: A combined retrospective and prospective study of children admitted to with clinical features of typhoid to the Goroka Hospital throughout 2022. RESULTS: The study included 98 children, of which 54% were female. The median age was 8 (IQR 5-10.6) years. Over 60% of the patients were from Goroka District, the peri-urban area encompassing the town and surrounds. Ninety-four percent (92) of the patients used a pit latrine as a toilet and only 28% had access to treated water. Neuropsychiatric symptoms were common (60%), as was leukopenia (48%), thrombocytopenia (52%) and anaemia (42%). Thirty-seven patients had positive blood cultures for Salmonella typhi; all isolates were sensitive to third-generation cephalosporins, pefloxacin, ampicillin, trimethoprim and sulfamethoxazole, and only 54% sensitive to chloramphenicol. The median duration of hospitalisation was 6 days (IQR). There were no deaths. CONCLUSION: Prompt public health actions are needed to reduce the burden of typhoid infection in the Papua New Guinea. The conjugate typhoid vaccine should be considered in the highlands region, where typhoid is most endemic.

Epidemiology and assessment of Hepatitis B positive children in Western Australia.

AIM: To describe the characteristics of patients with chronic hepatitis B (CHB) presenting to a tertiary paediatric hospital in Perth, Western Australia. Review of implementation of previous follow-up recommendations for the cohort was also undertaken. METHOD: A retrospective data analysis of all individuals aged between 0 and 17 years presenting to the tertiary children's hospital who were hepatitis B surface antigen (HBsAg) positive over 8 years (2013-2020). Demographic features, clinical progress and follow up are described, including proportion transferred to adult services. RESULTS: Seventy-four patients were identified to have CHB; mean age at diagnosis 11 years; standard deviation 4 years; 41 (55%) male. Cultural and ethnolinguistic diversity was high; 74% (n = 55) were from refugee-like backgrounds. Many did not demonstrate English proficiency (23/40; 75%) and 7 (10%) Australian born including 4 patients who were Aboriginal. Most patients (58%) with CHB were in the hepatitis B e antigen-positive chronic infection phase with no intervention provided. Seventeen children had undergone liver ultrasonography and one underwent liver biopsy; none received antiviral treatment. Follow up was concerning; 28 (38%) had at least one clinic non-attendance, 24 (32%) lost to follow-up and interpreter utilisation was poorly documented. Thirty-nine (53%) were transferred to adult services with only 56% attending follow-up. CONCLUSION: CHB burden is higher in those from culturally and ethnolinguistically diverse backgrounds. There is a significant loss to follow-up and suboptimal transfer to adult services. Improved recall, education and referral processes are necessary to overcome language, socioeconomic and cultural barriers. Although childhood complications are infrequent, longitudinal monitoring is crucial to prevent long-term complications and adult morbidity.

Social media use and paediatric practice: Hippocratic help, hype or harm?

Social media is increasingly a part of our personal and professional life and is here to stay. Here, I reflect on issues surrounding the use of social media (SMU) in the digital health context by clinicians in Australian paediatric health care. I aim to briefly highlight some inherent multifactorial and contextual ethical considerations which mainly relate to professionalism; including boundaries, obligations to patients and families within the therapeutic alliance, and balancing the help, hype and harm of SMU in clinical practice. I conclude that digital health and SMU are ubiquitous, and can be beneficial if used circumspectly with Hippocratic principles that have been updated for the modern era and are grounded in ancient moral codes. Unfettered SMU however, without adherence to ethical and legal guidelines is problematic, and may expose patients, families and clinicians to significant risk of harm and moral vulnerability. Justifiable, explicit, consistent and regularly reviewed boundaries, both professional and personal, are ethically advisable. These should reflect, and adapt to, the rapidly evolving nature of social media as imprudent digital health and SMU without proportionate limits, may undermine still relevant Hippocratic tenets, and the primacy of doing no harm.

Mixed-methods pilot study exploring the influence of the novel Paediatric Anaesthetic Drug Solution tool on clinician cognitive load during simulated paediatric rapid sequence intubation in the emergency department.

AIM: Rapid sequence intubation (RSI) in children is a low-incidence, high-risk event associated with cognitive overload and potential errors producing unfavourable outcomes. Cognitive aids, such as charts, algorithms and flow diagrams, are prompts that externalise and structure mental processes to reduce cognitive load, thereby reducing errors. The Paediatric Anaesthetic Emergency Drug Solution (PAEDS) approach combines a colour-coded chart and medication box with a simplified mathematical system of volume-based dosing; the effect of which on cognitive load during a simulated RSI has not previously been described. METHODS: A randomised, cross-over trial was conducted with 26 multi-disciplinary emergency medicine clinicians (doctors and nurses) allocated to four groups, performing four high-fidelity RSI simulations, two mandating the use of the PAEDS approach. This mixed methods study followed the pragmatic ontology using grounded theory methodology. Qualitative data were collected from nine individual interviews by a process of thematic analysis via an inductive approach, to allow for appropriate open and axial coding to occur. Quantitative data collected included cognitive loading using the raw NASA-Task Load Index as well as time to intubation and drug dosage details to assess for safety. RESULTS: Qualitative results showed that the PAEDS approach reduced cognitive loading through the use of both the labelled medication box and colour-coded medication charts. The PAEDS approach also showed improved perceived time pressure without feeling rushed, and with no recorded drug errors. Differences in the quantitative data for total cognitive load, error and time were not statistically significant, likely due to sample size. CONCLUSION: The PAEDS approach is a multifaceted system which is not inferior to current practice, with some components described as an improvement. Further research on a larger sample size needs to be conducted to assess the aspects of the PAEDS approach both collectively and independently.

Prevalence of type 2 diabetes risk factors, including overweight and obesity, among youth attending hospital-based paediatric care in Western Melbourne.

AIM: To determine the prevalence of risk factors for type 2 diabetes in overweight and obese adolescents attending hospital-based paediatric care in Western Melbourne. METHODS: One hundred overweight and obese adolescents (aged 10-17 years) who attended an outpatient clinic at Sunshine Hospital between May 2019 and May 2020 were randomly selected following a retrospective chart review of 10-17 years old for whom a height and weight had been documented. Additional risk factors for type 2 diabetes were ascertained via structured telephone interview. Data were analysed to determine the overall prevalence of risk factors for type 2 diabetes, and to evaluate for associations between each parameter with body mass index and the number of risk factors. RESULTS: Of the 487 adolescents who had height and weight data recorded, 45% were overweight or obese. 77% of those who were interviewed had an additional risk factor for type 2 diabetes. No association between the number of risk factors and body mass index standard deviation score was found. Additionally, there was no association between the number of risk factors for type 2 diabetes and either family history of type 2 diabetes or ethnicity. CONCLUSIONS: This paediatric subpopulation had a high rate of risk factors for type 2 diabetes in addition to overweight and obesity, and are at risk of premature mortality and chronic morbidity should they develop type 2 diabetes.

Impact of parental health literacy on the health outcomes of children with chronic disease globally: A systematic review.

AIM: Health literacy is the ability to understand and interpret health information and navigate the health-care system. Low health literacy is associated with poorer health knowledge and disease management, increased chronic illness, underutilisation of preventative health services and increased hospitalisations. The aim of the study is to review the available literature on the relationship between parental health literacy and health outcomes for children with chronic disease in high-income countries (HIC) and low- and middle-income countries (LMIC). METHODS: We systematically searched Medline, EMBASE and Web of Science, and assessed study quality using the Newcastle Ottawa Scale. We reviewed all relevant studies, and identified themes using thematic analysis. RESULTS: Of 1167 studies assessed, 49 were included in the review. All studies were of adequate quality. Twenty-two were from LMIC and 27 from HIC. Six themes were identified: Parental health literacy, parental education, socio-economic conditions, identity and culture, family factors, and health behaviours. In both HIC and LMIC, lower parental health literacy was associated with poorer child health outcomes. Disease-specific knowledge was found in a number of papers to directly impact parent health behaviour and child health outcomes, and may mitigate the effects of low parental health literacy. CONCLUSION: There is a clear link between parental health literacy, health behaviour and health outcomes for children with chronic disease. Disease-specific knowledge as a target for health-care interventions holds promise for application in low-resourced settings with parents (particularly mothers) who have lower health literacy, where disease-specific education may improve child health outcomes, although more research is required to determine how we can best facilitate these programmes.

Time to change the reference ranges of children's physiological observations in emergency care? A prospective study.

AIM: High heart and respiratory rates are key indicators in many published guidelines to identify and treat serious bacterial infection and sepsis in children, but the credibility of evidence underpinning what is considered abnormal is questionable. This study established the distribution of heart and respiratory rates of children using a large data set to inform debate on what the 'normal' range of these should look like. The primary aim was to compare the distribution of heart and respiratory rates measured in children recruited from non-tertiary emergency care settings with those published by Advanced Paediatric Life Support (APLS). The secondary aim was to compare the distribution of this study's data set to other national guidance on what constitutes a severe (high-risk) measurement and previously published data sets. METHOD: Prospective study using anonymised patient data, extracted from electronic patient records of children and young people 0-16 years, recruited from three Emergency Departments and one Urgent Care Centre in Northwest England, UK. RESULTS: Heart and respiratory rates, including the reporting of values at certain centiles and comparisons of averages. Distribution of heart and respiratory rate were consistently higher than those used by the APLS guidance, resulting in a large proportion exceeding the 'severe' cut-offs proposed. This varied greatly by age. CONCLUSIONS: This study's data set suggests normal heart rate ranges proposed by the APLS and others is too low and therefore 'abnormal' measurements encompass too large a proportion. The respiratory rate of this data set was more consistent with the guidelines and other published data sets.

Towards a broader concept of wellbeing in evaluating paediatric quality of life.

Paediatric health-care professionals have a primary duty to promote the best interests of their patients. This is reiterated in article 3 of the United Nations Convention on the Rights of the Child and is predicated on promoting children's health and wellbeing. However, there is ambiguity over what standard applies when evaluating whether a paediatric health-care decision supports good outcomes. Values like 'best interests', 'doing no harm' or 'quality of life' may be indeterminate or vague and clinicians may have difficulty in conceptualising what exactly constitutes 'a good life' for children. This uncertainty leads to the question: how do we best evaluate paediatric health decisions and outcomes? Patient-reported outcome measures (PROMs) are questionnaires that aim to achieve this by attaining data on a patient's quality of life and wellbeing. While PROMs originated with adult cohorts, they have since been applied to paediatric populations. Children are vulnerable due to their interdependency; and this raises ethical tensions regarding the potential benefits of such data, respect for autonomy and assent/consent of the individual child in clinical settings. These inherent tensions should be balanced by realising a collective good for children. PROMs should be a robust data collection source that facilitates substantive justice, both procedurally and in distributing limited health resources via accurate quality-adjusted life-years generation. This article aims to (i) overview the traditional and emerging paediatric PROMs; (ii) outline the tensions of using PROMS for children in a clinical setting and (iii) analyse the ability of traditional and emerging PROMs to promote justice in paediatric resource allocation.

Post-operative steroids in boys with histologically proven balanitis xerotica obliterans reduce the need for subsequent meatal dilatation.

AIM: Post-circumcision topical steroids may reduce the number of patients with balanitis xerotica obliterans (BXO) who require subsequent meatal dilatation. However, their use has only been investigated in the presence of an abnormal urethral meatus. The aim of this study is to determine if post-operative topical steroids reduce the need for subsequent meatal dilatation in patients with histologically proven BXO independent of the appearance of the urethral meatus. To determine if post-operative topical steroids reduce the need for subsequent meatal procedures in patients with histologically proven BXO. To determine if the appearance of the meatus at circumcision affects the need for subsequent meatal procedure. METHODS: We performed a retrospective review of all paediatric patients with histologically confirmed BXO post circumcision. We excluded all those with 'chronic inflammation' or 'early BXO'. Patients were divided into groups based on whether post-operative topical steroids were prescribed. The appearance of the urethral meatus at circumcision was recorded. Primary outcome was progression to subsequent meatal dilatation. Statistical significance was calculated using Fisher's exact test. A P value < 0.05 was considered significant. RESULTS: One hundred and seventeen patients with histologically confirmed BXO were identified between October 2012 and December 2017. Fifty-nine patients were prescribed post-operative topical steroids (50%). Nine patients required subsequent meatal dilatation procedure (8%) and only one of these had been prescribed post-operative topical steroids (P = 0.0165). Abnormal meatus was noted in 24 (21%) patients. Three patients in this group required subsequent meatal dilatation compared with 6 in the group with a normal meatal appearance (P = 0.385). CONCLUSIONS: Post-operative topical steroids can reduce the need for subsequent meatal dilatation in boys with BXO. The appearance of the urethral meatus at circumcision does not predict the need for subsequent meatal dilatation.

Virtual health care for children: Parental willingness to adopt virtual health-care technologies.

AIM: To understand parental awareness and appetite for virtual health modalities, including asynchronous communication and remote monitoring. To understand which socio-demographic characteristics are associated with greater awareness and appetite for virtual health. METHODS: Nationally representative survey of Australian parents, recruited via an online panel in February 2021 as part of the Royal Children's Hospital National Child Health Poll. Participants were randomly selected from the consumer panel. RESULTS: 1981 (59.4% response rate) parents completed the survey. 39.9% were aware of virtual health care, defined by digital technology and medical devices to support remote monitoring. Higher levels of awareness were associated with being male, having previous teleconsultation experience, higher education attainment and employment. Most parents reported an appetite for asynchronous communication using an app or website to either provide information prior to a consult (65.9%), provide a photo of a rash or similar (61.7%) or receive medical advice (58.1%). Appetite for wearable devices was similar at 59.9%. CONCLUSIONS: Whilst awareness of virtual healthcare was low, appetite exists amongst Australian parents to use technology to support their child's health care. Health-care models, which incorporate asynchronous communication or symptom monitoring through apps, are likely to be acceptable and offer an accessible and sustainable alternative to traditional face-to-face health care.

The impact of a multidisciplinary care package for vaccination in needle phobic children: An observational study.

AIMS: Children with severe needle phobia find vaccination extremely distressing and can remain unvaccinated, which puts them at an increased risk of contracting and transmitting vaccine preventable disease. Referral to a specialist or hospital service may occur when they cannot be safely vaccinated in the community, but engagement of allied health services can be inconsistent. The aim of the study was to assess the impact of a multidisciplinary, consumer-oriented model of care on vaccinations for needle phobic children. METHODS: Needle phobic children aged between 6 and 16 years attended multidisciplinary consultation, as part of a care package, to assess previous experiences and determine the level of intervention that was required to support vaccination. A multidisciplinary case meeting followed this appointment and an individualised plan formulated for each patient. The main outcome of the project was rate of successful vaccination. RESULTS: The care package resulted in a successful vaccination rate of 83% (n = 20) with 69 vaccines administered across three clinics. Of those successful, 90% required multiple injections per visit. The majority of patients indicated moderate to high level of anxiety. Supportive care was escalated and de-escalated as tolerated. CONCLUSIONS: Results demonstrate the diversity of patients presenting with needle phobia and indicate an individualised, collaborative approach is preferable to a 'one size fits all' model of care. The study highlights a need for the development of guidelines that streamline the assessment and individualisation of procedural anxiety plans to meet patient needs and embed these processes into standard care.

Characteristics and outcomes of SARS-CoV-2 infection in Victorian children at a tertiary paediatric hospital.

AIM: Victoria experienced two 'waves' of COVID-19 between March and September 2020 and more cases than any other jurisdiction in Australia. Although world-wide reports of COVID-19 reflect that children are less likely to experience severe disease compared with adults, hospitalisations and deaths have been reported. We report testing and outcomes of children with SARS-CoV-2 infection presenting to a tertiary paediatric hospital in Melbourne. METHODS: We conducted a prospective cohort study at The Royal Children's Hospital (RCH), including all children and adolescents (aged 0-18 years) who presented and were tested for SARS-CoV-2 over a 6-month period, between 21 March 2020, up to the 21 September 2020. Detailed epidemiological and clinical data were recorded. RESULTS: A total of 19 708 tests for SARS-CoV-2 were performed in 14 419 patients. One hundred and eighty patients tested positive for SARS-CoV-2 (1.2%). 110 (61%) were symptomatic, 60 (33%) were asymptomatic and 10 (6%) were pre-symptomatic. Close contacts of a positive case were associated with a higher risk of a testing positive for SARS-CoV-2 (120/2027 (6%) vs. 60/14589 (0.4%), RD 5.5 (95% CI 4.5 to 6.5), P < 0.001). Eighteen (10%) SARS-CoV-2-positive patients were admitted to hospital with one patient requiring intensive care. All patients recovered fully with no deaths. CONCLUSION: In Victorian children presenting to a tertiary hospital, SARS-CoV-2 infection caused predominantly mild or asymptomatic infection, with most children not requiring hospitalisation.

Early detection of hearing loss for infants in Western Australia: Comparison to international benchmarks.

AIM: To assess the degree to which timely audiological assessment of congenital hearing loss is achieved at our institution - Perth Children's Hospital, Western Australia, and to review cases which breached this timeframe in order to address barriers to timely assessment. The benchmark used to determine timely assessment is that set out by The Joint Committee on Infant Hearing (JCIH) in which diagnostic audiological testing occurs by three months of age for those who do not pass newborn hearing screening. METHODS: A retrospective chart review of infants who underwent diagnostic auditory assessment at Perth Children's Hospital between 2016-2019. A total of 151 children were identified as meeting the inclusion criteria and their medical files were reviewed. Time to first dABR was the time point for whether testing was achieved within the 3 month timeframe. RESULTS: Of the 151 children who underwent dABR assessments, 1 was identified as having breached the 90 day time limit (tested on day 91) for which no valid reason for delay could be identified. The timely delivery of dABR assessments in 99.3% of cases within this cohort compares favourably with the literature. CONCLUSIONS: Conclusion Timely diagnostic audiological assessment is achievable for children with congenital hearing loss. The reasons for patients breaching this timeframe are explored in the paper along with factors which may help avoid delays.

Rapid onset functional tic-like behaviours in children and adolescents during COVID-19: Clinical features, assessment and biopsychosocial treatment approach.

AIM: To report the prevalence and clinical characteristics of children with rapid onset functional tic-like behaviours during the COVID-19 pandemic. METHODS: Single centre, retrospective cohort study of children (<18 years) referred to the tic clinic from January 2018 to July 2021. We calculate the prevalence of newly diagnosed functional tics, and compare the clinical features to chronic tic disorder/Tourette syndrome (CTD/TS). RESULTS: A total of 185 new patients were referred to the tic clinic between 2018 and 2021. There was a significant increase in the percentage of functional tics in 2020 and 2021 (2% in 2018, 5.6% in 2019, 10.6% in 2020 and 36% in 2021). Differences between functional tics (n = 22) and CTD/TS (n = 163) include female predominance (100 vs. 28%, P < 0.0001), later age of onset (mean age 13.8 vs. 6.8 years, P < 0.0001) and higher rates of anxiety/depression (95 vs. 41%, P < 0.0001). The functional tic group were more likely to present with coprolalia-like behaviours (77 vs. 10%, P < 0.0001), complex phrases (45 vs. 0.6%, P < 0.0001), copropraxia (45 vs. 2%, P < 0.0001), self-injury (50 vs. 4%, P < 0.0001), hospitalisation/emergency visits (36 vs. 2%, P < 0.0001) and school absenteeism (56 vs. 7%, P < 0.0001). A total of 18.2% of patients with functional tics reported preceding exposure to social media content involving tics. CONCLUSIONS: There is an increase in adolescent females presenting with rapid onset functional tic-like behaviours during the COVID-19 pandemic. We highlight differences in clinical features between the functional tic group and CTD/TS to aid diagnosis and management in the community. Based on our findings, we propose a mixed model of neuropsychiatric vulnerability and social media contagion in this group of adolescents with functional tics.

Paediatric ultrasound-guided vascular access: Experiences and outcomes from an emergency department educational intervention.

AIM: This quality assurance project aims to describe the provision of an ultrasound-guided vascular access education package to paediatric emergency department staff. It subsequently aims to measure clinician and departmental responses to this educational intervention to support future effective education provision. METHODS: Participants were opt-in emergency department staff. Staff were required to be approved to insert intravenous cannulae in the department. A minimum of 50% were non-rotational staff. The educational package consisted of a theory phase (pre-learning video, information document), a practical phase (intensive 90-120 minute individualised session using a mix of live subjects/training equipment), and an embedding phase (education group available for procedural supervision). Data collection was via de-identified, encoded self-reported survey data and logbooks. RESULTS: Twenty-three staff were enrolled for training. Sixteen (69.9%) were non-rotational. Prior to the education intervention, 18 trainees (78.3%) had placed no successful ultrasound-guided peripherally inserted venous cannulae. By 15 weeks following training, six participants (28.6%) had achieved a predetermined competency benchmark; 61.9% had placed at least one successful ultrasound-guided cannula. Difficult intravenous (IV) access predictors were present in 46.3% of patients throughout the data collection period, with infants overrepresented in this group (64.9% with difficult IV access predictors). IV access attempts by staff with prior ultrasound experience increased from 11.0 to 81.8% post-education intervention. CONCLUSIONS: A low-resource brief educational intervention around ultrasound-guided vascular access is achievable. Several barriers to education uptake were presented. Targeting the group of trainees with a high degree of motivation led to the highest yield of benchmark competency acquisition.

Factors contributing to neonatal readmissions to a level 4 hospital within 28 days after birth.

AIM: To identify maternal and neonatal factors associated with neonatal readmissions. METHODS: A case controlled, cross-sectional, retrospective review of neonatal readmissions within 28 days from birth to a level 4 hospital in Western Sydney was conducted from January to December 2018. Maternal and neonatal factors for readmission were assessed. A control group of 122 neonates were randomly selected. Comparative statistics and logistic regression analysis were used to analyse the data. RESULTS: Of the 3914 neonatal discharges following birth, there were 129 neonatal readmissions (3.3%). Following regression analysis, gestational age (odds ratio 0.82, 95% confidence interval 0.7-0.97, P = 0.02) and intrapartum intravenous (IV) fluids (odds ratio 2.78, 95% confidence interval 1.66-4.67, P < 0.001) were associated with readmission. The majority of readmissions were feeding-related (72.9%). Of these readmissions, 29.8% had feeding concerns noted by nursing or midwifery staff during the initial hospital stay. During the initial hospital stay following birth, neonatal feeding issues were significantly associated with primiparous mothers (P = 0.005). Mothers who did not receive IV fluids during labour were also more likely to experience feeding issues (P = 0.015). CONCLUSION: Our findings indicate that hospital discharge prior to established feeding patterns may be associated with an increased incidence of neonatal readmission. The factors associated with neonatal readmission are earlier gestational age and intrapartum IV fluid administration. These findings suggest that more comprehensive feeding assessment prior to discharge, flexibility of discharge timing and increased community support may reduce neonatal readmission.

How can advance care planning tools help young people's voices be heard?

The integration of the child or young person (CYP) in conversations around advance care planning (ACP) is an important area of paediatric practice. These discussions provide CYP with the opportunity to voice their values, goals and preferences, enabling health-care professionals to ensure they are aligned with their planned course of treatment. This process, often undertaken within the context of the child's family, empowers children to participate in decisions and experience the dignity of self-determination. It also facilitates an increased understanding between the CYP and those involved in their care. The objectives of this paper are to highlight the importance of these conversations for paediatricians and identify barriers, both real and perceived, that may prevent them from engaging with a CYP about their preferences for future care including focusing more on discussion with the parents, and concern about or not having the skills to engage the child in such sensitive conversations. Triggers that can prompt clinicians to undertake ACP are also discussed. A further complexity is that after seeking their perspective, it may become apparent that the CYP may hold different views and perspectives to their parents and/or clinician. This review article will especially examine elements of end-of-life conversations that are critical to support the important goal of listening to the voice of the CYP. This includes considerations around timing, legal aspects, ethical tensions that arise when amplifying a child's voice, clinician/team-member roles, clinical process considerations, and the use of specific interventions and ACP tools to facilitate these conversations with CYP.

Managing behavioural and developmental paediatric conditions in rural outpatient clinics: An insight to the challenge ahead.

AIM: The aim of this study is to characterise the nature and caseload of general paediatric outpatient clinics in rural Queensland and to compare the findings with national data. METHODS: A retrospective clinical audit of medical records in paediatric clinics at two rural hospitals was undertaken over a 6-month period. Data extracted included demographics, diagnostic category and onward referral outcomes. The same diagnostic categories were used as the national Children Attending Paediatricians Study (CAPS) to facilitate comparison. RESULTS: A total of 346 records were reviewed, 56 (16.2%) documented Aboriginal and Torres Strait Islander descent. Compared to national data, significantly more children with behavioural/developmental diagnoses were seen in the rural clinics (CAPS 33.8%; rural 59.2%; P < 0.001). In contrast, children presenting with medical conditions (CAPS 48.2%; rural 28.6%; P < 0.001) and mixed medical/developmental conditions (CAPS 17.9%; rural 12.1%; P = 0.006) were seen less frequently in the rural clinics. Referral rates from the rural sites were lower than the rates reported by CAPS for children with behavioural/developmental diagnoses (CAPS 35.9%, rural 24.9%; P = 0.002) and mixed diagnoses (CAPS 40.6%, rural 19.0%; P = 0.005), while there was no difference in referral rates for children with medical diagnoses (CAPS 16.1%, rural 18.2%; P = 0.575). CONCLUSIONS: Rural paediatricians' caseloads are dominated by developmental/behavioural conditions, however children with these conditions are less likely to be referred to allied health and psychology services. The reasons for lower referral rates and specific pressures upon rural health services need to be investigated in future studies to provide better health services for rural children.

Young boy with a long history of splenomegaly and cytopenia.

A 15-year-old boy was admitted with a history of cytopenia (white blood cell count 3.170/µm, platelets 90.000/µm) associated with splenomegaly, found during investigations for recurrent mild jaundice due to Gilbert's syndrome.He was in good general health, without systemic symptoms; therefore, the leading causes of asymptomatic splenomegaly were excluded. Coagulation, liver tests and abdomen ultrasound (US) were normal, showing a hepatopetal portal flow to the colour-Doppler. There was no sign of haemolysis on haematology investigations. The C reactive protein, immune globulins levels and erythrocyte sedimentation rate were normal, excluding both an infective and an immune regulation disorder. We excluded the haematological malignancy and lymphoproliferative disorders through a peripheral blood smear and a bone marrow biopsy.His history was remarkable for neonatal sepsis, which required umbilical venous catheter during hospitalisation in a neonatal intensive care unit (NICU). The patient follow-up was interrupted for a while, probably due to his good health condition.At age 17 years, the child accessed our emergency department. for a minor trauma to the limbs, and his physical examination was unremarkable, except for the splenomegaly. We repeated the abdomen US, with colour flow Doppler (figure 1).