Archives of Disease in Childhood最新文献

Background: Kawasaki disease (KD) is a childhood vasculitis which causes coronary artery aneurysms (CAA). There is a paucity of data regarding KD in Aotearoa New Zealand. We aimed to provide up-to-date epidemiological and clinical data about KD in the Auckland region.

Methods: We conducted a retrospective population-based cohort study in the greater Auckland region between 2017 and 2021. Potential KD cases were identified from hospital discharge records, echocardiogram databases and intravenous immunoglobulin (IVIg) dispensing databases. Clinical records were reviewed and international diagnostic criteria were applied retrospectively.

Results: A total of 161 cases of KD were identified (66.5% complete, 33.5% incomplete), with 84% aged under 5 years. Overall incidence (per 100 000/year under 5 years) was 20.4; this was highest in Asian (43.9) and Pacific (17.7) children. There was no significant difference in incidence between New Zealand European (10.1) and Māori (8.3) children. The mean yearly number of cases reduced during the start of the COVID-19 pandemic (37.6 vs 24.0, p=0.01). All children received at least one infusion of IVIg, with 20.5% receiving a second infusion. Twenty-seven children (16.9%) developed CAA. CAA was more common in children under 1 year, non-response to first dose of IVIg and Pacific children.

Conclusion: Incidence of KD and rate of CAA were higher than previously reported, although case numbers reduced during the COVID-19 pandemic. There was a high incidence of KD among Pacific children, who were most likely to develop CAA. Research focusing on strategies to identify and treat those at highest risk of CAA remains a priority.

Objective: To describe the total birth prevalence of neural tube defects (NTDs) in Scotland in 2000-2021.

Design: National, population-based study.

Setting: Scotland, UK.

Participants: Babies with an NTD and pregnancy outcome of live birth, stillbirth (≥20 weeks' gestation) or termination of pregnancy (any gestation), as recorded in the Scottish Linked Congenital Condition Dataset.

Exposures: Year, maternal age group and maternal area-level deprivation quintile.

Main outcome measures: The main outcome measure was the total birth prevalence of all NTDs and separately anencephaly and spina bifida (per 10 000 total births). The association between exposures and outcomes was investigated using Poisson regression.

Results: In Scotland in 2000-2021, there were 1178 babies with a recorded NTD (436 anencephaly, 577 spina bifida) and 1 203 491 total births. The total birth prevalence of NTDs was 9.8 (95% CI 9.2, 10.4) per 10 000 total births. The prevalence was lower among babies born to mothers aged 30-39 years (compared with younger or older mothers) and those from less (compared with more) deprived areas. There was no evidence of change in the prevalence of NTDs (prevalence rate ratio (PRR) 1.01 (95% CI 0.997, 1.02), p=0.16) or spina bifida (PRR 0.99 (95% CI 0.98, 1.01), p=0.35) over the study period. The prevalence of anencephaly showed no change in 2000-2012, but increased in 2013-2021 (PRR 1.12 (95% CI 1.06, 1.19), p<0.001).

Conclusions: The observed trends in anencephaly likely reflect increasing detection in early pregnancy. Current strategies are failing to equitably reduce NTDs in Scotland. Monitoring of future trends is needed to assess the impact of mandatory folic acid fortification.

There are at least 12 paintings from the late 17th to the early 20th centuries titled, 'The Sick Child'. They were painted by well-known and obscure artists from Holland, Norway, France, England, Spain, North and South America. Most depict infants, and some older children, always in their homes, most with the mother at the bedside. The Sick Child paintings are a window into the human condition before hospital treatment was the norm, and childhood illness and death were an experience common to almost all families. They depict the mother-child bond of love and protection, complex human emotions, and the hardship and vulnerability of children and families of those eras. There remains a role for art in health and medicine, even in the 21st century.

Objective: Boys born with posterior urethral valves (PUV) often experience bladder development issues due to urethral obstruction during gestation. Despite early surgical intervention, bladder dysfunction can persist, leading to detrusor overactivity and vesical overdistension. Successful toilet training is key in managing bladder function and controlling intravesical and upper urinary tract pressures. This study aims to evaluate toilet training outcomes in children with PUV.

Study design: This single-centre ambispective cohort study included 142 children aged 4 years and older who underwent PUV ablation between January 2003 and December 2021. The patients were categorised into two groups: those with cognitive comorbidities (group A), including autism spectrum disorder, attention deficit hyperactivity disorder and trisomy 21, and those without (group B).

Results: Among the 142 children, 128 (90.1%) achieved daytime continence at a median age of 37 months (range 33-49 months) and 115 (81.0%) attained night-time continence at a median age of 42 months (range 34-60 months). Children in group A showed significantly lower rates of continence compared with group B (daytime: 74.2% vs 94.6%, p=0.0026; night-time: 67.7% vs 84.7%, p=0.0412). Additionally, children in group A completed toilet training significantly later than those in group B (daytime: 64 vs 36 months, p=0.0002; night-time: 60 vs 42 months, p=0.0139).

Conclusion: Boys with PUV can achieve daytime and night-time continence at ages comparable to those reported by their peers; the presence of cognitive comorbidities is associated with delays in achieving these milestones.