Starship Children's Health

Neonatal cardiac surgery (NCS) is associated with a risk of neurodevelopmental impairment. We reviewed outcomes of a national pre-school screen, the Before School Check (B4SC), of children who had NCS compared to the national cohort to determine whether NCS children had higher rates of abnormality and whether the B4SC could be useful to identify at-risk NCS children. B4SC results of children who had NCS between 2006 and 2014 were compared to controls. Sub-analyses were performed by cardiac subtype and timing of diagnosis. Outcome measures were the Parental Evaluation of Developmental Status (PEDS), vision and hearing screening, and the Strengths and Difficulties Questionnaire parent version (SDQ-P). Multivariable analysis adjusted for ethnicity, socio-economic positioning, and sex. Of 508 NCS children, 390 (76.8%) had B4SC outcomes completed. The 371 without a syndrome or chromosomal anomaly, were compared with controls (n = 543 971). NCS children were more likely to have abnormal PEDS (27.9% versus 19.8%, p < 0.001), vision (10.5% versus 5.6%, p < 0.001) and hearing (18.0% versus 9.0%, p < 0.001). There was no difference on SDQ-P. Adjusted for confounding variables, children with single ventricle (SV) had higher rates of abnormal PEDS (RR 1.9, CI 1.4-2.5), whilst SV and 2V with prenatal diagnosis had increased risk of an abnormal vision screen (RR 3.1, CI 1.8-5.5, and 2.2, CI 1.4-3.4). Abnormal hearing screen was increased in all NCS groups. NCS children have higher rates of abnormal PEDS, hearing and vision screening on the B4SC. The B4SC may help to identify at-risk NCS children who may benefit from intervention.

Hospital discharge codes can be used to identify possible Adverse Events of Special Interest (AESI) following COVID-19 vaccinations. We sought to estimate the positive predictive value (PPV) and level of certainty of ICD-10-AM and SNOMED coding for meeting Brighton Collaboration case definitions of AESIs in Aotearoa, New Zealand. Our expert panel identified 24 ICD-10-AM codes and 2 SNOMED codes expected to identify 9 AESIs. We sought codes likely to be specific rather than sensitive. Medical record reviews were conducted by an experienced coder, adjudicated by medical specialists, for the level of certainty that each case met the target case definition. Admissions coded to an explicit alternative diagnosis were classified as Not a Case. Our data covered over 3 million people. Reviews were conducted on 761 medical records, randomly selected from admissions principally in calendar years 2016 to 2019. We report the PPV of each code with respect to its level of certainty of meeting the case definition. Only Guillain-Barré had a single code specific to a single AESI. Several neurological conditions had overlapping codes, conditions and case definitions. PPVs for individual codes to meet case definitions ranged from 11% to 100%. Level of certainty of each code and PPV are specified. PPVs of codes for each AESI need to be assessed on a case-by-case basis. Many codes are arguably insufficiently 'accurate' to identify a target AESI. We did not assess PPVs for combinations of codes. Our method did not allow us to estimate the number of cases missed by the coding.

Most children with intussusception are successfully treated with radiologic enema reduction. Our recent nationwide study revealed significant variation in enema reduction success and the requirement of secondary operative intervention. This may be due to variation in the management of intussusception. As such, radiologic enema management patterns across Aotearoa New Zealand (AoNZ) were investigated. A snapshot survey of all AoNZ radiologists who had managed paediatric intussusception in the preceding 5 years was performed. Survey answers were collected anonymously via REDCap between April 1, 2023, and July 1, 2023. Network sampling was used to attain respondent saturation. Responses were recorded from 24 of the 26 radiologists who had recently managed paediatric intussusception. An institutional management guideline was available in 88% of cases. Pneumatic reduction via Foley catheter under fluoroscopic guidance was used preferentially by 23 clinicians (96%). Respondents described a wide range of catheter retention strategies and reduction hardware. There was significant variation in reported initial (0-120 mmHg) and peak (100-130 mmHg) reduction pneumatic pressures. If partially successful, respondents attempted further reduction after 0-5 min with a maximum of 3-8 attempts per sitting. Most clinicians confirmed reduction with small bowel insufflation (92%), but seven utilised post-reduction ultrasound (29%). Almost all AoNZ centres relied on pneumatic reduction under fluoroscopic guidance and rarely utilised hydrostatic or ultrasound-guided reduction. Reduction hardware and catheter retention strategies were diverse. There was inter-centre variation in pneumatic pressure limits, reduction timing, and periprocedural care. Intussusception care could benefit from a multidisciplinary, standardised, national approach.

Abdominal pain is a common pediatric presentation in the emergency department (ED), and acute appendicitis (AA) is the most common surgical diagnosis. This study describes the management of suspected AA in a tertiary Australian pediatric ED. A single-center prospective observational study was performed between November 2022 and May 2023. Children aged 5 to 15 years presenting to the ED with acute abdominal pain and clinician suspicion of AA were included. Clinical gestalt was measured using a 5-point Likert Scale before and after blood tests. Diagnosis of AA was confirmed on histopathology, and non-AA was confirmed with follow-up at 30 to 60 days. The study enrolled 481 children; AA was diagnosed in 146 (30.6%). Patients with AA were older (11.1 vs. 10.0y) and more likely to be male (69.2% vs. 48.1%) compared with those without AA. Blood tests were performed in 449 (93.3%) children, with higher rates in AA cases (100% vs. 90.4%). Gestalt improved with blood test results, with overall diagnostic accuracy increasing from 65.9% pre-blood tests to 88.5% post-blood tests. Ultrasound (US) was performed in 361 (75.1%) children with a diagnostic accuracy of 90.6% and was frequently used even in patients deemed low-risk post-blood tests (65.7%). One hundred and fifty (31.2%) of suspected AA cases underwent surgery, with 3 negative appendectomies and 1 ovarian cystectomy without appendectomy. Median (IQR) hospital length of stay was significantly longer for AA cases than non-AA cases [50.8 (35.3 to 95.0) h vs. 7.1 (4.6 to 16.8) h]. This study reviews the management and outcomes of suspected AA in a high-volume Australian ED. It shows the importance of blood tests in improving diagnostic accuracy of clinician gestalt and highlights the potential overuse of US in low-risk patients. Future research should explore a more structured diagnostic approach to increase diagnostic accuracy, optimize resource utilization, and improve patient outcomes.

To evaluate the real-world user experience of young people (7-25 years) with type 1 diabetes and previously very elevated glycaemia (≥69 mmol/mol) transitioning from the MiniMed™ 780G advanced hybrid closed-loop (AHCL) system with Guardian™ 4 continuous glucose monitoring systems (CGM) and 3-day infusion sets to the upgraded system with Simplera Sync™ all-in-one CGM and 7-day Extended™ wear infusion sets (EIS). Qualitative interviews were conducted with 11 participants from the CO-PILOT trial extension phase who had used the upgraded system for at least 4 weeks. Thematic analysis was used to identify aspects of the technology transition that reduced, continued, or did not affect the burden of diabetes care and technology. Participants reported several burden-reducing aspects of the upgraded system, including easier, independent insertion of both sensors and infusion sets, improved device design (slimmer profile, one-piece disposable design), reduced frequency of equipment changes, and decreased psychosocial burden. Aspects with ongoing burden included premature sensor failures, connection issues between pump and sensor, adhesion problems, and concerns about alarm functionality. Trust in the AHCL system remained high across both systems for most participants. The transition from Guardian™ 4 and 3-day infusion sets to Simplera Sync™ and EIS was overall positive, with participants reporting enhanced user experience, greater convenience, and improved autonomy. However, challenges with premature sensor failure and alarm functionality were identified at times. These findings highlight the importance of continued technological advancement while maintaining multiple options to accommodate individual needs and preferences.

Introduction Paediatric patients with cerebral palsy (CP) have higher rates of hip displacement. Surgical strategies vary with the likelihood of repeat procedures remaining a key outcome. We compared paediatric patient characteristics (age, GMFCS, ethnicity, region) with regards to the primary surgical hip procedure and any subsequent surgical procedure, including soft tissue release (STR), femoral osteotomy ± STR, and femoral plus pelvic osteotomy ± STR. Methods Health data for 1,149 patients with cerebral palsy born between 2002-2018 and included on the New Zealand Cerebral Palsy Register were matched to data from the National Minimum Dataset (NMDS, 2002-2023) using the National Health Index (NHI). Procedures were grouped using national coding data and analysed by demographics, GMFCS level, CP type, and region of domicile. Results Overall, 251 of the 1149 patients (22%) had had hip surgery within the first 15 years of life. 182 of these patients (73%) were GMFCS IV–V and had quadriplegic CP. Overall, 72 of the 182 (40%) non-ambulatory children had ≥1 repeat operation, with 44 having one repeat operation, 28 having 3 operations and 8 having 4 operations. Repeat procedure rates were highest in the STR group (45%), followed by femoral (40%) and combined osteotomies (29%). Femoral osteotomy was the most frequent repeat procedure (60%). The median time from index procedure to second operation was 3.8 years. Regional variation was observed in choice of index procedure. Conclusion Patients with non-ambulatory CP undergoing STR alone had an approximately 45% chance of further surgery, slightly higher than those undergoing femoral osteotomies as the index procedure. Patients undergoing femoral and pelvic osteotomy primarily had the lowest rate of repeat surgery. These findings highlight expected revision rates at a national level and could be useful in counselling families on outcomes for this group of patients.

To analyse trends in community systemic antibiotic dispensing to children and young people (0-14 years) in New Zealand between 2010 and 2019. Dispensing data were retrieved from the National Pharmaceuticals Collection and population data from the Te Whatu Ora Populations Web Tool. Dispensing rates were described using the number of defined daily doses/1000 inhabitants per day (DID) and courses dispensed/1000 inhabitants/year. Total antibiotic dispensing declined over the study period, both in terms of courses dispensed (annual percentage change [APC] -3.4% per year) and DIDs (APC -0.67% per year). Amoxicillin/clavulanic acid showed the greatest decline in dispensing (APC -9.5% per year), while cefalexin had the most significant increase (APC +53.7% per year). The highest dispensing rates were observed among children aged 0-4 years, Pacific patients and those in the lowest socioeconomic quintile. Counties Manukau had the highest regional dispensing rates. Antibiotic stewardship, as per the WHO AWaRe guidelines, improved: Access antibiotics increased from 83% to 91%, while Watch antibiotics decreased from 17% to 9% of total dispensing. These findings suggest an overall improvement in prescribing practices, likely due to the adoption of antimicrobial stewardship programmes. Further study is under way to understand the reasons for and any risks associated with, high community rates of cefalexin dispensing.

An international panel of 27 experts (pediatric and movement disorder neurologists, psychiatrists, and parent representatives) from all continents participated in a Delphi process to establish international consensus guidelines for the evaluation, diagnosis, and management of children with Sydenham chorea (SC) based on best evidence and expert opinion. In total, 88 recommendations reached consensus. Practitioners should identify key signs of SC (chorea and hypotonia), screen for behavioral, mobility, swallowing, speech, and cognitive impairments, and acute rheumatic fever (ARF) features including carditis. Etiological evaluation will differ according to population ARF risk. At all times, patients, families, and educators should receive support, information, and guidance to minimize the impact of SC on academic and social functioning. Antibiotic treatment is recommended at first presentation. Long-term secondary antibiotic prophylaxis should follow international or local guidelines, and measures to reduce pain and distress associated with intramuscular antibiotics will aid in adherence. Immunotherapy (corticosteroids) is recommended in moderate to severe SC. In those with inadequate recovery, intravenous immunoglobulin or plasma exchange should be given. In SC relapse, repeat clinical assessments, etiological investigation, and antibiotics plus corticosteroid therapy should be considered. This consensus guideline will standardize the evaluation and management of patients with SC and direct future research to improve the lived experience and outcomes of patients and families.

Abstract Diffuse intrinsic pontine glioma (DIPG) is an aggressive pediatric brain tumor with no curative therapies. Standard of care is focal radiation therapy (RT), with a 2-year survival rate &amp;lt;10%. Patients who receive re-irradiation have post-progression median overall survival of 6 months. These tumors were historically not biopsied due to their eloquent location and poor prognosis. However, in the past few years, with advanced neurosurgical techniques, tissue diagnosis has been pursued when feasible which led to greater understanding in tumor biology. Unfortunately, outcomes have not yet reflected these advances. Here, we describe the case of a long-term survivor of DIPG following targeted therapy. The patient presented at 5 years of age with headaches and left cranial nerve VI palsy and had neuro-radiological findings consistent with “typical DIPG”. A biopsy was not obtained, and the patient was treated with focal RT 60 Gy. He had a good response to RT with reduction in symptoms and tumor size. Thirty months following initial diagnosis, he developed an asymptomatic solid nodule at the anterior fornix with stable appearance of the original pontine mass. A biopsy of the lesion was performed, with histopathology confirming metastatic relapse diffuse midline glioma, H3 K27M-mutant. Next-generation sequencing of the tumor was positive for the following alterations: H3F3A K28M, EP300 L415P (subclonal), and ATM R3008H.The patient was treated with further RT to the metastatic lesion. Subsequently, he was started on Olaparib (PARP inhibitor) based on somatic ATM mutation, via the compassionate use program of AstraZeneca. The patient remains alive with a Lansky performance score of 100% on continuing Olaparib, six years from relapse. This case highlights the importance of molecular testing and utility of precision therapy in brain tumors. Ongoing research effort is needed to understand the mechanism of response, and patient cohorts that may benefit from specific targeted therapies.