Paediatrics (RCH) - Research Publications
Now showing items 1-12 of 2924
Lipiodol flush under ultrasound guidance in Australia
BACKGROUND: Lipiodol tubal flushing is offered to select subfertile women primarily to confirm tubal patency and to increase pregnancy rates. AIMS: To investigate the safety of hystero-salpingo contrast sonography (HyCoSy) using Lipiodol flush (through frequency of adverse events and mean recalled pain score) and secondarily to quantify pregnancy rates. MATERIALS AND METHODS: Retrospective observational Phase 1 study of subfertile women in three centres across Australia between June 2017 and June 2019. Cases were identified from medical records, and women telephoned to assess adverse outcomes, procedure tolerability and confirm pregnancy outcomes within six months from procedure. RESULTS: A total of 325 cases were identified; 14 were excluded due to incomplete or abandoned procedure, 32 were lost to follow-up, leaving 279 for analysis. Fourteen women (5% overall) experienced mild vasovagal reactions, with one case of infection and no reports of anaphylaxis or allergy. There were 141 conceptions reported (51%) within six months after Lipiodol flush, and an ongoing pregnancy in 43% (119) of women. For women with ongoing pregnancies, 55% (78/119) conceived spontaneously, and 45% (63/119) via artificial reproductive technology. Mean recalled pain score was 5.7 (SD 3.2; range 0-10) at a single site. CONCLUSIONS: This Phase 1 study has indicated that Lipiodol flush using HyCoSy may be a safe and efficacious alternative to hysterosalpingography in the workup for infertility. The low adverse effect profile observed in this study coupled with a substantial ongoing pregnancy rate indicates that further investigation of Lipiodol under HyCoSy is warranted.
Periprocedural analgesia and sedation in air enema reduction for intussusception: A retrospective Australian cohort study
AIM: Periprocedural analgesia or sedation for air enema reduction (AER) of intussusception is a matter of debate. We set out to review Australian periprocedural pain management in AER. METHODS: Retrospective electronic medical record review of emergency department presentations of intussusception at an Australian children's hospital over 2 years for periprocedural analgesia and sedation and short-term outcomes. RESULTS: A total of 73 patients (mean age 23 months) had ultrasound-confirmed intussusception. Prior to AER, analgesia was administered to 61 of 73 (83.5%) patients. Opioids were administered in 48 of 73 (65.8%) and 8 of 73 (11.0%) received sedation. Thirteen of 73 (17.8%, 95% confidence interval 9.0-26.6) had spontaneously reduced; 60/73 that underwent primary AER had successful reduction in 54 (90.0%, 95% confidence interval 82.4-97.6). A total of seven patients required surgery. No AER attempts were complicated by bowel perforation. CONCLUSION: The use of periprocedural analgesia for AER in this Australian series was common, whilst sedation use was infrequent. No perforations occurred.
Rapid exome sequencing and adjunct RNA studies confirm the pathogenicity of a novel homozygousASNSsplicing variant in a critically ill neonate
Rapid genomic diagnosis programs are transforming rare disease diagnosis in acute pediatrics. A ventilated newborn with cerebellar hypoplasia underwent rapid exome sequencing (75 h), identifying a novel homozygous ASNS splice‐site variant (NM_133436.3:c.1476+1G>A) of uncertain significance. Rapid ASNS splicing studies using blood‐derived messenger RNA from the family trio confirmed a consistent pattern of abnormal splicing induced by the variant (cryptic 5′ splice‐site or exon 12 skipping) with absence of normal ASNS splicing in the proband. Splicing studies reported within 10 days led to reclassification of c.1476+1G>A as pathogenic at age 27 days. Intensive care was redirected toward palliation. Cost analyses for the neonate and his undiagnosed, similarly affected deceased sibling, demonstrate that early diagnosis reduced hospitalization costs by AU$100,828. We highlight the diagnostic benefits of adjunct RNA testing to confirm the pathogenicity of splicing variants identified via rapid genomic testing pipelines for precision and preventative medicine.
Blood transfusion following major orthopaedic surgery in cerebral palsy: a retrospective analysis
BACKGROUND: Progressive musculoskeletal pathology is ubiquitous among children with cerebral palsy (CP). Corrective surgery places them at risk of major blood loss and red blood cell (RBC) transfusion. Significant variability exists in uptake of perioperative patient blood management (PBM) strategies. This study aimed to examine factors contributing to RBC transfusion and assist in future development of care pathways. METHODS: A retrospective review at a tertiary paediatric hospital was undertaken to identify patients with CP undergoing either primary spinal fusion or single event, multilevel surgery (SEMLS) between 2010 and 2015. Solely soft tissue procedures were excluded. Data collected included demographics, Gross Motor Function Classification System level, surgical details, perioperative PBM and transfusion rates. Univariable analysis was performed to assess contributing factors to RBC transfusion. RESULTS: A total of 36 spinal fusion and 98 SEMLS patients were included. Preoperatively, 12% were anaemic, but only 19% had a ferritin checked. Overall, 49 patients (37%) received RBC transfusions. Intraoperative usage of tranexamic acid and cell salvage was 89% and 81%, respectively, for the spine cohort, and 22% and 3% for the SEMLS cohort. Successively higher Gross Motor Function Classification System levels, sodium valproate usage, longer surgical times, spinal fusion, pelvis instrumentation and more osteotomies were associated with RBC transfusion. CONCLUSION: More than one-third of CP patients who underwent major orthopaedic surgery received RBC transfusion. As expected, the more severely affected patients undergoing longer procedures were at highest risk. Significant improvements can be made in PBM to help optimize patients for surgery and minimize the need for transfusion.
Audit of paediatrician recognition of children's vulnerability to harm at the Royal Children's Hospital, Melbourne
AIM: Vulnerable children can be defined as those at risk of child abuse and neglect and long-term adverse health, neurodevelopmental and behavioural outcomes. This study examined whether a cohort of paediatricians and advanced trainees at the Royal Children's Hospital, Melbourne, recognised children's vulnerability. METHODS: We reviewed the clinical note in the electronic medical record (EMR) for 425 new patients presenting to five paediatric clinics between 1 July 2017 and 31 December 2017. We examined paediatrician documentation of adverse childhood experiences (ACE), risk and resilience factors, referrals for intervention to improve psychosocial well-being and the application of 'vulnerable child' alert flags in the EMR to indicate vulnerability to harm. Children were deemed vulnerable if the paediatrician explicitly stated it in the EMR, if the child had a 'vulnerable child' alert placed in their record or had an appropriate referral for management of neurodevelopmental trauma. RESULTS: Of the original cohort, 8% was documented as vulnerable, 21% had a referral for intervention and 2% had a 'vulnerable child' alert. Overall, paediatricians infrequently documented ACE, risk and protective factors. The odds of identifying vulnerability increased with each added risk factor recorded (odds ratio (OR) 2.6, P < 0.001, 95% confidence interval (1.9-3.5)), with an ACE score was >4 (OR 72, P < 0.001 (14.3-361)) and decreased with each added protective factor recorded (OR 0.6, P < 0.001 (0.5-0.8)). CONCLUSION: Paediatricians infrequently document ACE, risk and protective factors and rarely 'flag' children's vulnerability to harm. Identification of the vulnerable child is correlated with documentation of risk and resilience factors at the initial consultation.
Functioning, participation, and quality of life in children with intellectual disability: an observational study
AIMS: To investigate associations between functioning, community participation, and quality of life (QoL) and identify whether participation mediates the effects of functioning on QoL. METHOD: The caregivers of 435 children (211 females, 224 males; mean age 12y; SD 3y 11mo; age range 5-18y) with intellectual disability and autism spectrum disorder, cerebral palsy, Down syndrome, or Rett syndrome reported on their child's functioning (dependence for managing personal needs, mobility, communication, eye contact when speaking), frequency of participation, and QoL. Linear regression and mediation analyses were used to evaluate the relationships between child functioning, participation, and QoL. RESULTS: Children with greater dependency for managing personal needs and limited eye contact when speaking experienced poorer QoL. Less impaired functioning was associated with more frequent participation, which, in turn, was associated with a 3-point gain in QoL for each additional point in frequency of participation (coefficient=2.67, 95% confidence interval 1.56-3.78). The effect of impaired functioning on QoL was partially mediated by participation in children with greater dependency in managing personal needs and those with mildly impaired communication. INTERPRETATION: Greater levels of impairments with poorer functioning, notably a high level of dependence, were associated with poorer QoL. Poorer QoL can be partly explained by less frequent community participation.
Junior doctor perceptions of education and feedback on ward rounds
AIM: The literature suggests that feedback is wanted and needed in clinical medicine and specifically on ward rounds, yet it is often lacking. This study aimed to examine junior doctor perceptions of education and feedback on ward rounds in one clinical department at a tertiary paediatric hospital and the key influences on these perceptions. METHODS: Six semi-structured focus groups were conducted over a period of 9 months comprising of 20 participants (post-graduate year 1-5) in a general medical department of a tertiary paediatric hospital. Qualitative analysis was performed on focus group transcripts using an inductive approach and codes and themes were generated in an iterative fashion with checking of themes between two researchers. RESULTS: Feedback experiences were largely positive compared to previous rotations. Three overarching themes were identified which influenced trainee perceptions of education and feedback on ward rounds. These were: consultant influences (e.g. educational engagement), trainee influences (e.g. active seeking of feedback), and structural factors (e.g. organisational constraints). CONCLUSIONS: Despite positive feedback experiences, the need to improve feedback for our junior doctors is clear, but how to do this remains challenging when navigating work-learning tensions. The notion of the educational alliance between the consultant and trainee is a potential useful solution, but it requires deliberate effort and dedicated time to establish given our increasingly complex and busy clinical environments.
Acceptability of the culturally adapted ASQ-TRAK developmental screening tool to caregivers of Aboriginal children
AIM: The Ages and Stages Questionnaire-Talking about Raising Aboriginal Kids (ASQ-TRAK) culturally adapted developmental screening tool is widely used in Australian Aboriginal communities. However, there has been limited exploration of the tool's acceptability to caregivers. The aim of the study is to determine the acceptability of the ASQ-TRAK developmental screening tool to caregivers of Aboriginal children in urban, regional and remote South Australia. METHODS: Caregivers of Aboriginal children completed a survey regarding acceptability of the ASQ-TRAK. Convenience samples of caregivers were invited to a telephone interview. RESULTS: Ninety-two caregivers completed the survey (96% response). Acceptability (92%) and caregiver satisfaction (73%) were high. Families perceived the screen as easy to use and understand, strengths-based and providing valuable information about their child's development. CONCLUSIONS: The ASQ-TRAK tool was highly acceptable to caregivers in Aboriginal communities in South Australia. The study highlights the importance of culturally safe practice and supports ASQ-TRAK implementation. Broader use and further evaluation of the ASQ-TRAK in Aboriginal communities needs consideration.