Abstract
Background: Pyloric stenosis is the most common condition requiring surgery in the first months of life. Case reports have suggested familial aggregation. However, to what extent this is caused by common environment or inheritance is unknown.
Aims: To investigate familial aggregation of pyloric stenosis from monozygotic twins to fourth-generation relatives according to sex and maternal and paternal contributions and furthermore to estimate disease heritability.
Methods: A population-based cohort study consisting of 1,999,738 children born in Denmark between 1977 and 2008. The cohort of children was followed for the first year of life during which 3362 children had surgery for pyloric stenosis.
The familial aggregation of pyloric stenosis was evaluated by rate ratios of pyloric stenosis.
Results: The incidence rate (per 1,000 person-years) of pyloric stenosis in the first year of life was 1.8 for singletons and 3.1 for twins. The rate ratios of pyloric stenosis were 182 (95% confidence interval [CI], 70.7-467) for monozygotic twins, 29.4 (95% CI, 9.45-91.5) for dizygotic twins, 18.5 (95% CI, 13.7-25.1) for siblings, 4.99 (95% CI, 2.59-9.65) for half-siblings, 3.06 (95% CI, 2.10-4.44) for cousins, and 1.60 (95% CI, 0.51-4.99) for half-cousins. We found no difference in the rate ratio for maternal and paternal relatives of a pyloric stenosis case and no difference according to own sex or sex of relative. The heritability of pyloric stenosis was 87%.
Conclusion: Onset of pyloric stenosis in Danish children showed strong familial aggregation and heritability.
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Krogh, C., Fischer, T., Skotte, L. et al. 288 Familial Aggregation and Heritability of Pyloric Stenosis. Pediatr Res 68 (Suppl 1), 148 (2010). https://doi.org/10.1203/00006450-201011001-00288
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DOI: https://doi.org/10.1203/00006450-201011001-00288