Royal Children Research Articles

Paediatric patients with myalgic encephalomyelitis/chronic fatigue syndrome value understanding and help to move on with their lives.

AimThe aim of this study was to document qualitative questionnaire feedback regarding management from a cohort observational study of young people with myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS).MethodsBetween 1991 and 2009, 784 paediatric patients, age 6‐18 years, were diagnosed with ME/CFS following referral to a specialised clinic at the Royal Children's Hospital, Melbourne. Over a 14‐year period, feedback was requested on up to seven occasions.Management included the following: symptom management and a self‐management lifestyle plan that included social, educational, physical and a pleasurable activity outside of home. They adjusted it by severity of illness, stage of education, family circumstances and life interests.ResultsQuestionnaires were returned from 626 (80%) with 44% providing feedback more than once. They reported that their management plan allowed them to regain control over their lives. They cited early diagnosis, empathetic, informed physicians, self‐management strategies and educational liaison as helping them to function and remain socially engaged. Ongoing support, particularly assistance to navigate the education system, was essential for general well‐being and ability to cope.ConclusionYoung people valued regaining the control over their lives that was lost through illness, support to maintain social contacts and assistance to achieve educational and/or life goals.

Ross Operation in Children: 23-Year Experience From a Single Institution

Data on the long-term outcomes in children after the Ross operation are limited. This study aimed to assess the long-term outcomes in children who underwent the Ross operation at a single institution. The study reviewed all children (n= 140) who underwent the Ross operation at the Royal Children's Hospital in Melbourne, Australia between 1995 and2018. Mean follow-up time was 8.9 years. Median age at operation was 7.4 years. The root replacement (n= 120, Ross-Konno; n= 38), root inclusion (n= 17), and subcoronary implantation (n= 3) techniques were used. Operative mortality was 5.0% (7 of 140; 3 neonates and 4 infants). There were 6 late deaths. Overall survival at 10 years was 96.2% in children older than 1 year of age and 78.9% in children younger than 1 year of age at operation (P= .003). Freedom from autograft reoperation was 86.0% at 10 years. Age younger than 1-year at operation was a risk factor for autograft reoperation (P= .02). Patients younger than 1 year of age at operation experienced a higher incidence of moderate or greater aortic insufficiency compared with patients who were older than 1 year of age (P= .006). In patients who had a poly-(p-dioxanone)-filament band placed around the sinotubular junction, freedom from moderate or greater aortic insufficiency at 10 years was 100%, compared with 83.1% in patients with no band (P= .09). In children older than 1 year of age, the Ross operation has excellent outcomes with no operative mortality and a low incidence of aortic insufficiency. In children younger than 1 year of age, the Ross operation is associated with higher operative mortality and a higher incidence of aortic insufficiency. Where possible, the Ross operation should be delayed beyond infancy. Poly-(p-dioxanone)-filament banding may reduce the incidence of aortic insufficiency after the Ross operation.

American Heart Association ideal cardiovascular health score and subclinical atherosclerosis in 22-35-year-old adults conceived with and without assisted reproductive technologies.

Is ART related with the association of American Heart Association (AHA) ideal cardiovascular health score and markers of subclinical atherosclerosis? The associations between AHA score and markers of subclinical atherosclerosis in ART and non-ART groups were similar in magnitude. Long-term consequences of ART on cardiovascular health are unknown. The study cohort for the cross-sectional analyses consisted of 172 ART-conceived and 78 non-ART conceived individuals of same age (range 22-35 years). Cardiovascular risk factor status was evaluated with American Heart Association (AHA) ideal cardiovascular health score consisting of seven factors (body mass index, blood pressure, total cholesterol, glucose, diet and physical activity, non-smoking). Carotid artery intima-media thickness (cIMT), arterial pulse-wave velocity (PWV) and retinal microvascular parameters were evaluated as markers of early atherosclerosis. Group comparisons in continuous variables were performed with t-tests. For categorical variables, comparisons were performed with chi-square tests. The relationships between AHA score and the markers of atherosclerosis were examined with linear regression analyses adjusted for age and sex. There was no difference in AHA ideal health score between the ART and non-ART groups; mean (SD) scores were 4.1(1.4) versus 4.0(1.5), respectively, P= 0.65. No differences were observed between groups for any individual ideal health metric (P always >0.2). AHA score was not associated with cIMT or retinal measures in either group (P always >0.05). An inverse association was observed between AHA score and PWV in the ART group (beta (95% CI) -0.18(-0.26 to -0.10)). A numerically similar relationship was observed in the smaller non-ART group (-0.19(-0.39 to 0.01)). Even though this cohort is among the largest ART studies with extensive cardiovascular data, the sample is still relatively small and the statistical power is limited. As the study population was still in early adulthood, we were not able to evaluate the associations with clinical cardiovascular events, but utilized non-invasive methods to assess early markers of subclinical atherosclerosis. These findings suggest that ART-conceived individuals do not have increased vulnerability for cardiovascular risk factors. This study was funded by a National Health & Medical Research Council Project Grant (APP1099641), The Royal Children's Hospital Research Foundation, Monash IVF Research and Education Foundation, and Reproductive Biology Unit Sperm Fund, Melbourne IVF. The authors have no conflicts of interest relevant to this article to disclose.

Training attention in children with acquired brain injury: a study protocol of a randomised controlled trial of the TALI attention training programme

IntroductionChildhood inattention has been linked with poor academic outcomes, and increased lifetime social, occupational and psychiatric morbidity. Children with an acquired brain injury (ABI) are particularly susceptible to attention deficits...

Study protocol for the Melbourne Infant Study: BCG for Allergy and Infection Reduction (MIS BAIR), a randomised controlled trial to determine the non-specific effects of neonatal BCG vaccination in a low-mortality setting

IntroductionBCG vaccination reduces all-cause infant mortality in high-mortality settings by more than can be attributed to protection against tuberculosis. This is proposed to result from non-specific protection against non-vaccine targeted...

STAGING ROMAN SLAVERY IN THE SECOND CENTURY BCE

In 167 BCE, L. Aemilius Paullus celebrated a triumph in Rome following the defeat of King Perseus of Macedon at the Battle of Pydna in the previous year. All of the accounts of the procession enumerate the incredible volume of booty that was paraded into Rome—wagons loaded with shields, weapons, statues of gods and men, golden bowls, livestock, luxury goods. Perseus himself, the defeated king, marched in this procession, as did his two sons and a daughter. Plutarch writes that ‘the children of Perseus were led alongas slaves’: τὰ τέκνα τοῦ βασιλέως ἤγετο δοῦλα (Aem.33.6), and that they were accompanied by their tutors who wept, taught the royal children to beg, and stretched out their hands to the Romans, who are here called ‘spectators’ (θεατάς,ib.). Perseus himself comes next, dressed in the black of mourning. Plutarch goes on to give a psychological picture of Perseus—he is dumbstruck and gaping, unable to process how his life had been turned upside down (34.1). Because Perseus could not face suicide, Plutarch says, ‘he converted himself into part of his own spoil’ (34.2).

Education during the Arab Renaissance and Its Path to the West

Islam was at the origin of schools, during the Umayyad and Abbasid periods, as every Moslem had to know how to read the Koran. In different regions, namely Spain, public schools were free, and education went beyond the teaching of the Scriptures. Propagating ideas read or heard was equivalent to an aptitude certificate. This approach led to the right of the author and was transmitted to universities in the West as well as the works of ancient philosophers and scientists. When The Christian Front Union disintegrated, alliances with Moslem princes were common. Hence, Arab teachers were in charge of educating the royal children of Aragon, and physicians were called at the bedside of patients among other functions. The school of translation established in Toledo by Bishop Raymond comprised a collection of scientific and literary Arab work that enlightened spirits of European countries. The fall of the Andalusias in the XV century marked the end of a perennial civilization. Yet, students are still taught had not Charles Martel beaten the Arabs, nothing would have survived.

Unmet Needs and Experiences of Adolescent Girls with Heavy Menstrual Bleeding and Dysmenorrhea: A Qualitative Study.

To identify and examine the key areas of need and explore the experiences of adolescent girls with heavy menstrual bleeding and/or dysmenorrhea. Qualitative interview study using semi-structured interviews. Gynaecology outpatient clinic at The Royal Children's Hospital, Melbourne, Australia. Adolescent girls (12-18years; mean age, 14.8±1.5years) presenting with heavy menstrual bleeding and/or dysmenorrhea at the clinic (N=30). In-depth semi-structured interviews were conducted between May and August 2018. Interview data were thematically analyzed using a grounded theory approach. Themes covered experiences and unmet needs of adolescent girls with heavy menstrual bleeding and/or dysmenorrhea. A total of 12 themes were identified, and covered the impact of symptoms, experiences, and/or unmet needs of these adolescents. Key themes highlighting experiences and unmet needs related to (1) coordination of healthcare, (2) day-to-day coping, (3) school, and (4) information surrounding menstrual issues. From these themes, 7 unmet needs emerged and were organized under 3 key areas of need: (1) treatment, management, and care, (2) improvements in the school environment, and (3) menstrual health as a gendered issue. Menstrual concerns can have a profound physical and psychosocial impact on adolescents. Effective school-based menstrual education programs may be key in reducing stigma, fear, and shame surrounding menstruation, in teaching positive management strategies and in encouraging adolescents to seek help for their menstrual concerns.

Who is prescribing psychotropic medications in a public developmental-behavioural clinical service and how often?

Psychotropic prescribing by paediatricians is increasingly common, and there is considerable variation in care provided. The aims of this study were to examine current rates of psychotropic prescribing in the developmental-behavioural outpatient clinics at the Royal Children's Hospital's Centre for Community Child Health, and to compare rates between paediatric consultants and advanced trainees. Data were extracted for appointment encounters from electronic medical records across 12 months in 2017. Patient demographics, provider and medication order data were analysed using Excel and STATA to calculate logistic regression, standard deviation, percentages and means. From 5069 encounters, there were 847 (16.7%) that included psychotropic prescribing. Advanced trainees prescribed psychotropic medications in only 4.4% of their encounters compared with consultants who prescribed these medications in 23% of encounters (P < 0.001). Stimulants were the most commonly prescribed psychotropic medication (62%), either alone (86.3%) or in combination (13.7%). Psychotropic prescribing rates were lower than expected. Advanced trainees may need more experience in psychotropic prescribing.

What are the health outcomes of trans and gender diverse young people in Australia? Study protocol for the Trans20 longitudinal cohort study

IntroductionBeing transgender is frequently accompanied by gender dysphoria, which often coexists with mental health concerns. Increased referrals of transgender and gender diverse (TGD) youth to gender clinics have been observed...

A SAB or two, a retrospective review. Staphylococcus aureus bacteraemia at the Royal Children's Hospital, Melbourne

A SAB or two, a retrospective review. Staphylococcus aureus bacteraemia at the Royal Children's Hospital, Melbourne

Anterior and posterior commissures in agenesis of the corpus callosum: Alternative pathways for attention processes?

Developmental absence (agenesis) of the corpus callosum (AgCC) is a congenital brain malformation resulting from disruption of corpus callosum formation, a structure that is crucial for the transfer and integration of information, including attention processes, across the brain. This study aimed to investigate previously proposed candidates for alternative inter-hemispheric pathways in AgCC by examining (1) white matter volume and microstructure of the anterior and posterior commissures in children with AgCC compared to typically developing controls (TDC), and (2) in children with AgCC, examine the associations of white matter volume and microstructure of the anterior and posterior commissures and any remaining corpus callosum with attention processes. Participants were 21 children with AgCC (13 complete, 8 partial) recruited from The Royal Children's Hospital, Melbourne, and 30 TDC aged 8–17 years. T1-and diffusion-weighted MR sequences were used to calculate volume and microstructural parameters. Neuropsychological testing assessed attention processes. We found the anterior commissure was significantly larger in volume in children with AgCC than TDC (p = .027), with reduced mean FA (p = .001) associated with increased mean RD (p < .001). In children with AgCC, we found microstructural properties of the anterior commissure associated with attentional processes, specifically, mean FA of the anterior commissure was associated with better divided attention (p = .03), and the association between alerting attention and mean AD and RD was found to be moderated by age (p = .027, p = .008) and the degree of corpus callosum agenesis (p = .025, p = .016). Furthermore, in partial AgCC, larger posterior commissure volume was associated with better orienting attention (p = .035). In conclusion, we provide evidence that the volume and microstructure of the anterior commissure are altered in children with AgCC, and this neuroplastic response might have an influence on attention processes.

Nontraumatic Pediatric Intracerebral Hemorrhage.

Nontraumatic Pediatric Intracerebral Hemorrhage.

Outcome of vein of Galen malformation presenting in the neonatal period

ObjectiveVein of Galenaneurysmal malformation (VGAM) is a rare but important congenital malformation presenting to neonatal intensive care units (NICUs), and with a change from surgical to endovascular management, survival for...

Thoracostomy in children with severe trauma: An overview of the paediatric experience in Victoria, Australia.

Thoracic trauma is a leading cause of paediatric trauma deaths. Traumatic cardiac arrest, tension pneumothorax and massive haemothorax are life-threatening conditions requiring emergency and definitive pleural decompression. In adults, thoracostomy is increasingly preferred over needle thoracocentesis for emergency pleural decompression. The present study reports on the early experience of thoracostomy in children, to inform debate regarding the best approach for emergency pleural compression in paediatric trauma. Retrospective review of Ambulance Victoria and The Royal Children's Hospital Melbourne, Trauma Registry between August 2016 and February 2019 to identify children undergoing thoracostomy for trauma, either pre-hospital or in the ED. Fourteen children aged 1.2-15 years underwent 23 thoracostomy procedures over the 31 month period. The majority of patients sustained transport-related injuries, and underwent thoracostomies for the primary indications of hypoxia and hypotension. Two children were in traumatic cardiac arrest. Ten children underwent needle thoracocentesis prior to thoracostomy, but all required thoracostomy to achieve the necessary definitive decompression. All patients were severely injured with multiple-associated serious injuries and median Injury Severity Score 35.5 (17-75), three of whom died from their injuries. Thoracostomy in our cohort had a low complication rate. In severely injured children, thoracostomy is an effective and reliable method to achieve emergency pleural decompression, including in the young child. The technical challenges presented by children are real, but can be addressed by training to support a low complication rate. We recommend thoracostomy over needle thoracocentesis as the first-line intervention in children with traumatic cardiac arrest, tension pneumothorax and massive haemothorax. [Correction added on 23 September 2019 after first online publication: in the second sentence of the conclusion, the words "under review process" were mistakenly added and have been removed.].

Childhood Hearing Australasian Medical Professionals network: Consensus guidelines on investigation and clinical management of childhood hearing loss.

Permanent hearing loss affects 1–3 per 1000 children in Australia1 ; this may be congenital or acquired, unilateral or bilateral, and ranges in severity from mild to profound. Hearing loss can be classified as sensorineural (SNHL), including auditory neuropathy, conductive and mixed (Table 1, Fig. 1). Australia has high-quality universal infant hearing screening, which facilitates early diagnosis and enables early intervention, hearing device fitting and timely planning for cochlear implantation if indicated.1,3 Although the National Framework for Neonatal Hearing Screening provides guidance on infant screening, and early intervention pathways are well established, aetiological investigation and medical management of newly diagnosed infants have varied. National guidelines are important to streamline management, ensure investigations are completed within relevant timeframes and reduce unnecessary stressors for families. Local and international evidence for the aetiological investigation of childhood hearing loss is heterogeneous and generally of low quality, with limited guidance on the structure or cost-effectiveness of investigations. A 2017 review recommended consensus guidelines to improve the management of hearing-impaired children,4 and both the British Association of Audiovestibular Physicians5–7 and the International Pediatric Otolaryngology Group8 have released guidelines. This position paper provides consensus recommendations for the aetiological investigation and medical management of children with SNHL, including auditory neuropathy, intended for general practitioners, paediatricians, otolaryngologists and genetic services in the Australian context.

Dr Ian Hamilton McDonald, MBBS, DA, FANZCA: The evolution of paediatric anaesthesia and intensive care at the Royal Children's Hospital, Melbourne.

Dr Ian Hamilton McDonald (1923-2019) was a pioneer of paediatric anaesthesia and intensive care at the Royal Children's Hospital, Melbourne. He first started working at the hospital in the 1940s, later doing further training from 1953-1955 at the Nuffield Department of Anaesthesia in Oxford under Sir Robert Macintosh. McDonald returned to Melbourne as assistant director supporting Dr Margaret (Gretta) McClelland as the director of anaesthesia, together pioneering the development of a major paediatric anaesthesia department. McDonald, along with Dr John Stocks (1930-1974), was intimately involved in pioneering prolonged nasotracheal intubation in children, following on from earlier work by Dr Bernard Brandstater (an Australian working in Beirut), and Drs Tom Allen and Ian Steven in Adelaide. Ian McDonald was an influential, highly respected and greatly loved paediatric anaesthetist who had a profound influence on the early days of paediatric anaesthesia in Australia.

Outcomes of Children With Chronic Intestinal Failure: Experience Over 2 Decades at a Tertiary Paediatric Hospital.

The aim of the study was to aid decisions on prognosis and transplantation; this study describes the outcome of children with intestinal failure managed by the multidisciplinary intestinal rehabilitation program at the Royal Children's Hospital, Melbourne. Retrospective review of children requiring parenteral nutrition (PN) for >3 months who were assessed for home PN between 1991 and 2011. A total of 51 children were included. Forty-two (82%) had short bowel syndrome (SBS), 5 (10%) had chronic intestinal pseudo-obstruction syndrome, and 4 (8%) had congenital enteropathies. Median small bowel length for patients with SBS was 45 cm (interquartile range 30-80) or 23.9% of the expected length for age (interquartile range 17.0%-40.6%). Overall survival rate was 84% (43/51). Mortality in children (n = 7) occurred after a median of 13.2 months (range 6.2-29.2) with intestinal failure-associated liver disease (IFALD) being the only predictor (P = 0.001). Out of 50 children 21 (42%) had IFALD. Children who were premature (P = 0.013), had SBS (P = 0.038), and/or frequent sepsis (P = 0.014) were more likely to develop IFALD. PN weaning occurred in 27 of 35 (77%) SBS survivors, after a median of 10.8 months (up to 8.2 years), with longer residual small bowel (P = 0.025), preservation of the ileocecal valve (P = 0.013) and colon (P = 0.011) being predictors. None of 5 (0%) patients with chronic intestinal pseudo-obstruction syndrome and 2 of 4 (50%) patients with congenital enteropathies weaned off PN. Overall sepsis rate was 7.3 episodes/1000 line days. Frequency of sepsis and longevity of central lines improved with time as patients grew older (both P < 0.001). Long-term PN with intestinal rehabilitation was effective in treating most children with intestinal failure. Children with severe refractory IFALD may have benefited from intestinal transplantation.

Outcomes of the arterial switch operation in patients with aortic arch obstruction

ObjectiveTransposition of the great arteries or Taussig–Bing anomaly with concomitant aortic arch obstruction is uncommon, with limited data on long-term outcomes after arterial switch operation and aortic arch obstruction repair. This study sought to determine outcomes of patients undergoing arterial switch operation and aortic arch obstruction repair at a single institution. MethodsFrom 1983 to 2015, 844 patients underwent an arterial switch operation for biventricular repair at The Royal Children's Hospital. Eighty-three (9.8%, 83/844) patients underwent an arterial switch operation and aortic arch obstruction repair. ResultsFifty-five (66%, 55/83) patients had transposition of the great arteries. and 28 (34%, 28/83) patients had Taussig–Bing anomaly. Fifty-nine (71%, 59/83) patients underwent arterial switch operation and aortic arch obstruction repair as a single-stage procedure, and 24 (29%, 24/83) patients underwent arterial switch operation and aortic arch obstruction repair as a 2-stage procedure. There were 5 early deaths (6.0%, 5/83). Follow-up was available for 74 (95%) of the 78 survivors. Median follow-up was 13.3 years (interquartile range, 7.3-19.3 years; range, 1-30 years). There were no late deaths. Freedom from reintervention was 77%, 71%, and 68% at 5, 10, and 20 years, respectively. Reintervention was more common compared with patients without aortic arch obstruction (P < .001). Reintervention for right-sided obstruction was more common compared with patients without aortic arch obstruction (P = .006). ConclusionsPatients with transposition of the great arteries or Taussig–Bing anomaly with associated aortic arch obstruction have a higher reintervention rate, especially for right-sided obstruction. Closer monitoring of this subgroup of patients is warranted.

The experiences of children with autism spectrum disorder, their caregivers and health care providers during day procedure: A mixed methods study.

Autism Spectrum Disorder is now diagnosed in more than 1% of children in Australia and USA. Children with autism spectrum disorder may have additional health care needs, require more specialized services for their health care, or experience more difficulties during hospital attendance. Customized care for these children may assist in decreasing potentially challenging behaviours during hospitalization. The purpose of this study was to explore the experiences of children with autism spectrum disorder and their caregivers during attendance for day procedures in two hospitals in Melbourne, Australia. Further, the perceptions of their health care providers were explored. Twenty-nine participants, including 14 health care providers and 15 caregivers of children with autism spectrum disorder, were interviewed within 72hours of their day procedure attendance at the Royal Children's Hospital and the Royal Dental Hospital in Melbourne, Australia. Interviews were recorded digitally, then transcribed and coded. Mixed quantitative and qualitative methods (content analysis) were used. Hospital attendance was often stressful. Participants identified a number of facilitating factors including good communication, clear explanations, and friendly attitudes of staff. Flexibility and individualized care of patients (such as avoiding unnecessary blood pressure measurements, and not changing into hospital gowns) were valued. Supportive aids (such as computers or special interest objects), use of social stories, and giving premedication were all considered helpful. Perceived barriers to care included prolonged waiting times for operation date as well as waiting on the day of operation, lack of private space, lack of noninvasive equipment such as cutaneous infrared thermometers, poor communication, and inadequate training of staff about autism spectrum disorder. Providing optimal care for children with autism spectrum disorder requires a multifaceted approach that may require changes to hospital work flow, staff training, better use of aids (such as tablet computers and social stories), and premedication. Good communication and flexibility are key areas of importance.