Abnormal thyroid function tests (TFTs) are found in a wide range of settings in hospitalised infants. This retrospective descriptive study reviewed management decisions and outcomes of these infants, identifying factors influencing clinicians' decision to treat with thyroxine.
Data were collected for all infants referred to the on-call endocrinology service at a tertiary Australian centre for thyroid dysfunction between 1 July 2019 and 30 June 2021. Electronic medical records were reviewed for birth and neonatal factors, TFTs, relevant investigations, treatment and disposition.
Of 124 infants referred to the service over a 2-year period, 43% (52 out of 120) were premature, with a high rate of comorbidities, including jaundice, hypoglycaemia, respiratory distress and cardiac anomalies. About 57% (69 out of 121) of referrals to the service were from neonatal intensive care units. Investigations to evaluate hypothyroidism included nuclear thyroid scans in 40% (46 out of 114) and ultrasound in 37% (41 out of 112). Levothyroxine treatment was initiated in 66% (77 out of 117) of infants, with 63% (73 out of 117) assigned a presumptive diagnosis of transient congenital hypothyroidism. At the end of the study period, 70% (54 out of 77) remained on thyroid replacement.
Our study demonstrates the challenge clinicians face when deciding whether to treat premature and medically unwell infants with abnormal TFTs. Further prospective studies are required to determine predictors of permanent CH in this complex population.
The aim of this study was to describe long-term outcomes of kidney replacement therapy (KRT) in Australians with prune belly syndrome in comparison to a control group of congenital kidney disease.
We identified all Australians treated with KRT between 1977 and 2021 with a diagnosis of PBS from the Australia and New Zealand Dialysis and Transplant Registry.
We identified 37 males (no females) who commenced KRT at a median age of 17 years (range 1–45). At initiation of KRT treatment, 54% of patients were on haemodialysis, 30% on peritoneal dialysis and 16% received a pre-emptive kidney transplant. Forty-eight kidney transplants (35 first, 11 second and 2 third grafts) occurred, of which 48% were from deceased donors. Median age at first transplant was 21 years (range 2–47). Graft survival at 1, 5 and 10 years for first grafts was 91%, 71% and 51%, respectively (range 6 days to 36 years). Three men reported parenthood at median age 35 years. There were 10 deaths reported at a median age of 37 years (range 17–49). Reported aetiology was cardiac death (50%), malignancy (20%), dialysis cessation (10%) and uncertain cause (20%). Compared to an age and gender-matched control group of people with congenital kidney dysplasia, Australians with PBS had equivalent peritoneal dialysis technique survival, but slightly better transplant graft and overall survival.
Prune belly syndrome has marked variation in outcomes from KRT, but overall, these were equivalent or better than a matched control group with congenital kidney disease, including use of peritoneal dialysis (despite lack of abdominal wall musculature).
To assess the quality and rigour of Neonatal Hypoglycaemia guidelines used in the major Australian and New Zealand neonatal care centres. To compare and highlight any major differences in management guidelines between centres.
All level III NICUs in Australia and New Zealand were invited to participate. The AGREE II (Appraisal of Guidelines, Research & Evaluation) was used to critically appraise the guideline for the management of neonatal hypoglycaemia. Recommendations regarding definition, treatment, method of testing and admission criteria were compared from the guidelines provided.
Neonatal Hypoglycaemia guidelines were received from 19 of the 29 invited hospitals; two guidelines were excluded as the hospitals providing these guidelines did not provide care for inborn neonates. None of the 17 guidelines received a standardised score of 50% or higher on all six domains of the AGREE II tool. The mean scores of each of the AGREE II domains were as follows: Scope and Purpose 76%; Stakeholder Involvement 41%; Rigour of Development 20%; Clarity of Presentation 66%; Applicability 30% and Editorial Independence 0.1%. The glycaemic threshold defining hypoglycaemia varied between 2.0 and 2.6 mmol/L in the guidelines. True blood glucose using either a glucose oxidase method or blood gas analyser was recommended as the first line test in 35% of the guidelines. Fifteen of the 17 guidelines recommended buccal gel as first-line treatment of hypoglycaemia.
Neonatal Hypoglycaemia guidelines are of varying methodological quality. There are inconsistences in the management of hypoglycaemia across neonatal units in Australia and New Zealand.
Autism spectrum disorder (ASD) is a common neurodevelopmental disorder. This study aims to investigate the impact of an ASD diagnosis on children and their carers from a regional/rural Australian perspective.
A three-part survey development study included: (i) Semi-structured individual ASD carer interviews to identify common themes; (ii) survey development and testing; and (iii) online survey circulation to wider group of carers, for data collection and analysis.
Transcripts from eight carer interviews guided the development of 65 survey questions. The survey was circulated to 316 carers of children diagnosed with ASD. Of the 101 respondents, 95% were female, 86% regional and 12% were rural inhabitants. The average child's age at diagnosis was 6.64 years. Most carers (93%) reported that diagnosis of ASD met their goal, for some an improved understanding of their child's behaviour (39%) and allowing access to therapy (16%), government disability funding (National Disability Insurance Scheme) (19%) and learning support (9%). Some (44%) reported no downsides to an ASD diagnosis; however, 38% reported fears of discrimination, particularly with future relationships (5%) and employment (14%). Barriers included waiting times (16%), costs of appointments (9.9%) and difficulty navigating through the health system (5.9%). Only five participants reported having no costs associated with appointments.
Carers had positive attitudes and experiences regarding their child's ASD diagnosis. The benefits outweighed the perceived harms. Barriers to accessing services included waiting times, out-of-pocket expenses and travel distance.
We report the epidemiology, treatment and outcomes of acute flaccid myelitis (AFM), including the first two cases of enterovirus (EV) D68 myelitis, in Singapore.
Retrospective observational study from a paediatric hospital in Singapore, from January 2012 to December 2022. Clinical, laboratory, neuroimaging and outcome data were analysed.
Eight patients were identified (all male). Median age at presentation was 3 (interquartile range (IQR) 2.2–10.0) years. Seven (87.5%) patients were clustered in two time periods (July 2018–January 2019 and April–August 2022). Presenting features were a preceding febrile respiratory illness (n = 6) or hand-foot-and-mouth disease (n = 2), upper limb weakness (n = 5, 62.5%) and neurogenic bladder (3, 37.5%), while the spinal cord lesions were predominantly in the cervical region (7, 87.5%). Five (62.5%) and six (75.0%) patients had elevated cerebrospinal fluid (CSF) white cells (median 7.5/mm3 (IQR 2.8–40.3)) and protein (median 0.6 g/L (IQR 0.4–0.7)), respectively. CSF was negative for pathogens. Two (50%) of four patients with EV detected in respiratory/rectal swabs had EVD68 (2022 cluster). All received intravenous methylprednisolone, six (75%) had additional intravenous immunoglobulin and either plasma exchange therapy (n = 1) or intravenous tocilizumab (n = 1). Median modified Rankin Scale (mRS) at acute illness was 4 (IQR grades 3–5), with an improvement (median 2 (IQR 1.8–2.3) mRS grades) on follow-up (median duration 3.7 (IQR 1.4–4.1) years). One patient (12.5%) had a full recovery and seven (87.5%) have moderate disability (mRS 2–3).
Disability risk in AFM is high despite aggressive immunotherapy. We report the first two confirmed cases of EV D68 AFM in Singapore and Southeast Asia.
To evaluate real-world glycaemic outcomes in children with type 1 diabetes (T1D) commencing advanced hybrid closed loop therapy (AHCL) and to explore these outcomes based on the cohort's clinical and socioeconomic characteristics.
A single-centre, population-based retrospective study in children commencing AHCL (Smart Guard, Control IQ, CamAPS) with minimum 70% data from two-weeks CGM pre-AHCL was conducted between December 2021 and June 2023 in Western Australia. CGM metrics (time in range (TIR) 3.9–10 mmol/L, time below range (TBR) < 3.9 mmol/L, glucose management indicator (GMI)) were analysed at baseline, monthly and 6 months. HbA1c at baseline and 6 months were also collected. The proportion meeting glycaemic targets of TIR > 70%, TBR < 4% and GMI < 7.0% were determined. Change in TIR from baseline to 6 months was examined by the following characteristics: %TIR, age group and Index of Relative Socioeconomic Disadvantage (IRSD) of residential postcode.
CGM data of 309 children, mean (SD) age 12.4 (3.2) years were analysed. Glycaemia improved from baseline to 6 months with (mean) TIR +8% (95% CI 7, 9; P ≤ 0.001), GMI −0.3% (95% CI −0.3, −0.2; P < 0.001) and (median) TBR −0.3% (95% CI −0.4, −0.1; P < 0.001). Proportion meeting glycaemic targets increased from 13.3% at baseline to 30.6% at 6 months. Improvement in TIR did not differ based on age group or IRSD Quintile. Greater increase in TIR was seen in those with lowest TIR at baseline (+20.9%, −0.2%; P < 0.001 for baseline TIR < 40%, >70%). There was a 0.27% reduction in HbA1c in 6 months (n = 116) (P < 0.001).
AHCL improves glycaemia, irrespective of age and socioeconomic characteristics, with greatest changes seen in those with lowest baseline TIR.
To investigate the long-term quality of life and surgical outcome of female patients with congenital adrenal hyperplasia (CAH).
Questionnaires for health-related quality of life (HRQoL) outcome (WHO-5, SF-36), lower urinary tract symptoms (ICIQ-FLUTS) and sexual outcome (ICIQ-FLUTSsex) were administered to adult CAH patients. Paediatric CAH patients and their parents were invited to complete WHO-5, PedsQL-4.0-SF15-Generic Core Scales and ICIQ-CLUTS.
Six (46%) adults and 7 (54%) children with 21-hydroxylase-deficiency (age ranged from 8 years to 53 years) were recruited. Eleven (85%) had undergone a feminising genitoplasty. In the adult group, no statistically significant difference was identified in the HRQoL and ICIQ-FLUTS scores. Mean overall score of ICIQ-FLUTSsex was lower but not significantly in adult patients compared to controls (7.3 ± 2.9 vs. 2.9 ± 3.4, P = 0.06). A statistically significant negative correlation (r = −0.98, P = 0.02) was found between age at first operation and role limitations due to emotional problems in SF-36. No statistically significant difference was identified in the self-reported HRQoL and ICIQ-CLUTS scores in the paediatric group compared to controls. However, parent's report showed a statistically significant difference in school functioning (73.8 ± 18.9 vs. 91.2 ± 18.7, P = 0.02) and emotional functioning (80.3 ± 15.9 vs. 94.7 ± 12.7, P = 0.007).
Our data did not show an impaired HRQoL nor long-term lower urinary tract symptoms in CAH patients' self-reports. Healthcare team should pay more attention to adult patient's sexual function.
This study sought to assess the clinical efficacy of honey suppositories in managing functional constipation in pre-school children, with a focus on those exhibiting Yin deficiency and intestinal dryness according to traditional Chinese medicine principles. The investigation aimed to determine whether honey suppositories could serve as a safer and more efficacious alternative to standard treatments.
A randomised controlled trial was conducted involving pre-school children diagnosed with functional constipation. They were allocated to either a control group or a treatment group. While both groups received standard care, the treatment group also received honey suppositories. The efficacy of the treatment was evaluated based on the ‘Guiding Principles for Clinical Research of New Chinese Medicines’, and statistical analysis was performed using SPSS 25.0.
Following treatment, the treatment group demonstrated significantly lower constipation scoring system scores and higher bowel movement frequencies compared with the control group (P < 0.05). The clinical efficacy of the treatment group was notably superior, with a higher rate of complete recovery and effectiveness observed (P < 0.05). Additionally, no significant differences in baseline characteristics were found between the groups, confirming the comparability of the study cohorts.
Honey suppositories offer a promising therapeutic intervention for paediatric functional constipation, with significant clinical benefits over standard care.
Children with cerebral palsy (CP) can develop neuromuscular hip dysplasia (NHD) and radiographic surveillance is recommended, guided by gross motor function classification system (GMFCS) level. This study evaluated the clinical practice of hip surveillance for NHD in a children's hospital and risk factors for abnormal first and subsequent X-rays.
Health data were extracted for 159 participants with CP, 98 male, 52 GMFCS level IV or V (birth years 2008–2018) and linked to electronic radiology datasets.
The median age at diagnosis of CP was 18 months (1–96 months). Thirty-eight participants had X-rays prior to diagnosis and 10 (6%) had no X-ray. Seventy-nine of 111 children classified as GMFCS levels II to V (71%) met both 2008 and 2020 Australian Hip Surveillance Guidelines (AHSG) having the first hip X-ray by 24 months of age. Sixteen participants (11%) had abnormal first hip X-ray (subluxation or migration percentage >30% in 14; MP 90%–100% or dislocation in 2). Univariate analyses showed NHD (MP > 30%) or dislocation at first X-ray was associated with GMFCS IV or V (OR = 6.98 (2.12–22.94), P = 0.001); >4 months between diagnosis and first hip X-ray (OR = 5.60 (1.52–20.59), P < 0.0009) and more common in NZ Māori children than non-Māori children (OR = 3.71 (1.25–11.01), P = 0.012).
Surveillance for NHD did not follow guidelines in almost a third of children, with delays in screening associated with greater risk of NHD at first X-ray. Inequities found for Indigenous NZ Māori children with CP require further investigation and stakeholder consultation.
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