Mortality rates, cause and risk factors in people with spina bifida, register-based study over five decades.

AIM: Care for people with spina bifida can be improved. This may be done by evaluating mortality rates and causes of death. METHODS: Between 1973 and 2021, 1735 people with spina bifida appeared in registers of the Swedish population. Survival rates and causes of death were calculated according to age and decade. RESULTS: Over almost 50 years, the prevalence of spina bifida decreased from 5.2 to 1.2 per 10 000 births. Mortality fell sharply during the first year of life, with survival rising from 75% to 94%. For children aged 2-18 years and adults, mortality rates were low and differences between decades were minimal. Causes of childhood deaths were congenital abnormalities, hydrocephalus and infections, the latter two also in adults. Adult causes also included self-inflicted injuries and substance abuse, with suicidal or unclear intent, both more common than in the general population. Bladder malignancies were also more frequent, although after reconstructive bladder surgery, mortality rates were similar. CONCLUSION: Survival in the first year of life increased in children with spina bifida, whereas there was no difference in survival rates between adults born between 1973 and 1999. For adults, proactive prevention methods regarding self-inflicted injury, substance abuse and bladder cancer are warranted.

The Swedish PedsQL gastrointestinal symptoms scale and symptoms module showed good psychometric performance.

AIM: There is no validated symptom scale for Swedish children with gastrointestinal disorders. Our aim was to validate the Swedish version of the Paediatric Quality of Life Inventory (PedsQL) gastrointestinal symptoms scale and symptoms module. METHODS: Families were recruited from two hospitals in Gothenburg, Sweden, from 1 March 2021 to 31 October 2022. The instruments were completed by 115 children with functional, congenital or organic acquired gastrointestinal disorders and 149 of their parents. These were the gastrointestinal symptoms scales, symptoms module and the 4.0 Generic core scale. Data were analysed for feasibility, construct validity and reliability, including internal consistency, re-test reliability and child-parent agreement. RESULTS: Feasibility was good, with a failure to respond of ≤5%. Construct validity showed strong correlation in the PedsQL gastrointestinal symptoms module. The known-group validity agreed with the expectations associated with the disease characteristics (p < 0.05). Cronbach's alpha was 0.96, which indicated excellent internal reliability. The intraclass correlation coefficient for the child self-report and parent-proxy report was 0.74, which indicated good agreement. CONCLUSION: The Swedish PedsQL Gastrointestinal Symptoms Scales, the symptoms module provided acceptable measurement properties and can be used to evaluate symptoms of gastrointestinal disorders and quality of life during clinical work or research projects.