Journal of paediatrics and child health最新文献

Empathy abounds in paediatrics and is deemed a valuable trait that enhances child and family care. Concurrently, research indicates there has been a decline in paediatric training applications, both medical and nursing, and there are challenges to workforce retention related to empathy exhaustion. While the cause is unclear and likely multifactorial, there may be a correlation with empathy levels, requiring analysis by policy makers and governing bodies. Empathy is a disposition, generally understood as cognitive or affective, and I propose here that clinician empathy exists on a continuum. At the affective extreme, there may be an intense emotional transference or pathological empathy response-I use the portmanteau 'empathological' to describe this. Further, that this response may be associated with negative sequelae, and compromise child and family care when complexity, uncertainty, and tragedy co-occur. Identifying the appropriate empathy dose and duration is therefore key to mitigate harm to all parties. Developing strategies to harness empathy by judiciously employing reason and moral theory could be protective. To help understand empathy bounds and balance, I outline the moral foundations of clinical empathy and weigh its benefits and burdens in clinical settings. I conclude that reasoned empathy, which draws on specific elements of Paul Bloom's analysis of rational compassion, allows for engaging empathetically with children and families without paralysing moral action by overly deeply relating to tragic circumstances. Attending to a form of reasoned empathy could ultimately inform healthcare staff selection and training to sustain a healthier paediatric workforce, and lead to better care for sick children.

Background: Paediatrics obstructive sleep apnoea syndrome (OSAS) is often underdiagnosed by medical practitioners due to its subtle and nonspecific symptoms in children. This study aimed to determine the prevalence of OSAS and identify clinical predictors of its occurrence and severity.

Methods: Medical records of children (2-18 years) who underwent polysomnography (PSG) at a sleep laboratory in Myanmar during 2012-2023 were reviewed retrospectively. Children with incomplete PSG data, a prior PSG record in the same sleep laboratory, or a history of adenotonsillectomy were excluded from the study.

Results: This study included 349 children with mean ± SD age 6.8 ± 2.8 year. OSAS was identified in 82.2% (289/349). Boys, overweight/obesity and tonsil grade-4 were independently associated with OSAS and boys, overweight/obesity, lower resting SpO₂, witnessed apnoea, abnormal daytime sleepiness and tonsil grade-4 were independently associated with severe OSAS on multivariable logistic regression. Among 2-8-year-old children, there was a significant mild positive correlation between AHI and tonsil grades (r = 0.29, p < 0.001) and between AHI and BMI (r = 0.21, p < 0.001). Among 9-18-year-old children, there is a moderate positive correlation between AHI and BMI (r = 0.34, p = 0.003); however, the correlation between AHI and tonsil grades was not significant r = 0.08 (p = 0.51).

Conclusion: Male sex, overweight/obesity and tonsil grade-4 can predict OSAS and its severity in children. Lower SpO₂ levels, witnessed apnoea and excessive daytime sleepiness can also predict severe OSAS. Although the degree of obesity is correlated with OSAS severity in both older and younger children, the degree of tonsil grades is correlated with its severity only in younger children.

Background: South African children face a double burden of malnutrition from undernutrition and rising obesity. Simple, accurate methods to estimate fat-free mass, a key health indicator, are needed, as bioelectrical impedance analysis is limited by cost, availability and lack of local validation.

Objectives: To develop and validate prediction equations for fat-free mass using simple anthropometric measurements in children aged 6-9 years.

Methods: In this cross-sectional study, anthropometric and bioelectrical impedance data were obtained from 117 children. Bioelectrical impedance-derived fat-free mass was used as reference in multivariable regression models. Four equations were externally validated in 75 Black prepubertal children, using dual-energy X-ray absorptiometry-derived fat-free mass as standard. Relationships, mean differences, and agreement were assessed using Pearson's correlation, independent t-tests and Bland-Altman plots, respectively.

Results: Fourteen prediction equations, containing five to nine variables, were developed (R² range: 0.88-0.92) in the sample of children (51% Black; 55% boys; 7.9 ± 0.8 years). Four equations were strongly correlated with dual-energy X-ray absorptiometry-derived fat-free mass (r > 0.95; p < 0.001) in the validation sample (8.5 ± 1.3 years), and three yielded estimates with acceptable agreement (mean difference: 0.16-0.94 kg; limit of agreement: ±5 kg).

Conclusion: Fat-free mass of prepubertal children can be predicted using simple anthropometric measurements, allowing assessment of body composition in low-resource settings.

Aim: To evaluate neonatal nutrition practices in Australia and New Zealand (NZ) in 2024 and compare these with previous surveys, international recommendations and British Dietetic Association workforce standards. The survey aimed to investigate progress towards standardisation, variations in practice and priorities for neonatal dietetic service development.

Methods: A two-part online survey was distributed to members of the Australasian Neonatal Dietitians Network (ANDiN) and dietitians working in Australasian neonatal units. Part 1 gathered site-level data; Part 2 focused on individual dietitians' roles. Responses were analysed descriptively and compared with the 2018 ANDiN survey. Dietitian full-time equivalent (FTE) allocations were benchmarked against (BDA) service recommendations.

Results: About 39 neonatal units (26% NZ, 74% Australia) and 66 dietitians responded. Growth monitoring was near-universal, with 86% using Fenton 2013 charts and 91% using z-scores. However, 32% transitioned to WHO charts at 40 weeks, earlier than recommended. Parenteral nutrition hang time practices varied significantly. Donor breastmilk was available in 77% of units and probiotics were used in 89%. Only 23% of units met the recommended dietitian FTE and 16% reported ≤ 0.1 FTE. While 61% of dietitians attended ward rounds weekly or more, one-third never attended. Research participation remains low at 21%.

Conclusions: Progress is evident in standardised growth assessment and nutrition practices. However, wide variation remains in feed strategies, parenteral nutrition protocols and workforce capacity. Greater alignment with consensus guidelines and workforce benchmarks is needed. Enhancing neonatal dietitian integration, research engagement and resourcing is critical to supporting equitable, high-quality neonatal nutrition care.

Aim: To evaluate the diagnostic yield and clinical triage performance of a structured, multistep algorithm in children referred for suspected inborn errors of immunity (IEI) due to recurrent infections.

Methods: This single-centre study included 705 children (0-18 years) referred for recurrent infections. All were screened using JMF and/or MENA criteria. Of these, 132 met at least one criterion and underwent stepwise immunologic evaluation, including advanced testing when indicated.

Results: Of 705 children referred with recurrent infections, 132 (18.7%) met screening criteria and underwent structured immunologic evaluation. Inborn errors of immunity were diagnosed in 50 patients (7.1%), with a 71% diagnostic confirmation rate. Pathogenic variants were detected in 74%, immunoglobulin abnormalities in 78% and all showed lymphocyte subset disturbances. The most common classifications were antibody deficiencies (32%) and syndromic combined immunodeficiencies (28%). Half received intravenous immunoglobulin, and no mortality occurred during follow-up.

Conclusion: The structured diagnostic algorithm based on JMF and MENA criteria improved IEI diagnosis and enabled effective prioritisation of children presenting with non-infectious immune phenotypes. This model reduced unnecessary testing, supported efficient allocation of limited resources and facilitated timely diagnosis. The approach offers a practical, cost-effective solution particularly applicable in regions with high consanguinity rates and limited access to advanced immunologic diagnostics.

Aim: To systematically review clinical and genetic testing approaches to VACTERL association, a non-random co-occurrence of congenital anomalies involving the vertebrae, anus, cardiac system, trachea-oesophagus, renal system, and limbs. The review will examine investigation strategies used in clinical practice and evaluate the diagnostic yield of genetic testing in affected individuals.

Methods: A systematic search of PubMed, MEDLINE, EMBASE, Web of Science, and CINAHL was conducted from database inception to 1 December 2024. Eligible studies included English-language human studies reporting genetic testing in individuals with VACTERL association. Grey literature and studies limited to management were excluded. Risk of bias and certainty of evidence were assessed using Joanna Briggs Institute tools and the Grading of Recommendations Assessment, Development, and Evaluation.

Results: A total of 65 articles met inclusion criteria-32 observational studies, 20 case reports, 9 case series, and 4 expert opinions. Findings were tabulated and narratively synthesised. Reported diagnostic yields were 2%-31% for chromosomal microarrays and 5%-22% for whole-exome sequencing.

Conclusions: Definitions of VACTERL and diagnostic approaches vary widely. Limitations of the evidence base include study heterogeneity, reliance on retrospective designs, outdated technologies, and lack of meta-analyses. Prospective studies are needed to develop protocols. In the interim, imaging, complete blood count and film, chromosomal microarray, chromosomal breakage studies, and exome or genome sequencing should be considered for patients with two or more VACTERL features, or selected individuals with an isolated feature. This recommendation is based on the implications of a molecular diagnosis for management. Key diagnostic elements and differential diagnoses are summarised.