Children with Turner syndrome are operated on and admitted to hospital more often, especially in the first year of life, than their peers without congenital abnormalities. These children also have an increased risk of dying in the first five years of life.
Researchers from the European Registration Of Congenital Anomalies and Twins (EUROCAT) partnership wanted to better map the disease burden of children with Turner syndrome, among other things to enable good information to be provided to parents of these children. To this end, they collected data from 13 research cohorts of almost 6 million children from 10 different European countries, including the Netherlands (Birth Defects Research. 2023; online juni).
During the study period (1995-2014), 404 children were born alive with Turner syndrome (prevalence: 0.69 per 10,000 live births). In the first five years of life, 18 of these children died, usually before their first birthday (survival: 95.5% at the age of five). Most children with Turner syndrome were hospitalized during the first year of life (72.3% (95% CI: 59.8-81.6) vs. 31% (95% CI: 25.7- 36.5) in children without congenital abnormalities). The median length of stay was 5.6 days (95% CI: 3.5-7.7) compared to 3.0 days in children without congenital anomalies (95% CI: 2.3-3.7). Children with Turner syndrome were more likely to undergo surgery in the first year of life (18.7% (95% CI: 13.9-23.9) vs. 0.79% (95% CI: 0.55-1 ,11) in children without congenital abnormalities). They were also often operated on at the age of 1 to 4 years (30.8% (95% CI: 17.6-44.7) vs. 6.25% (95% CI: 4.01-9 ,16) in children without congenital abnormalities).
The authors note that the disease burden of Turner syndrome is greatest in the first year of life. They hypothesize that the congenital heart defects associated with the syndrome are an important explanation for the operations and mortality. It should be noted that the diagnosis was made at a very young age in the children examined. If Turner syndrome is diagnosed during adolescence, the disease burden in early childhood appears to be more limited.
2023-11-30 05:00:00
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