AP was common in community children. FAP was the most common FGID among healthy community children. The prevalence of AP by parental report is lower than the previously published prevalence of AP reported by children. Lack of awareness of children's symptoms may play a role in the low ratio of consultation for AP in symptomatic children. Future prospective studies should confirm our findings and investigate the factors influencing the medical consultation decision including parental awareness of children's symptoms.
Many childhood Wilms tumors are driven by mutations in the microRNA biogenesis machinery, but the mechanism by which these mutations drive tumorigenesis is unknown. Here we show that the transcription factor ) is a microRNA target gene that is overexpressed in Wilms tumors with mutations in microRNA processing genes. Wilms tumors can also overexpress through copy number alterations, and expression correlates with prognosis in Wilms tumors. overexpression accelerates growth of Wilms tumor cells in vitro and induces neoplastic growth in the developing mouse kidney in vivo. In both settings, transactivates (), a key Wilms tumor oncogene, and drives mammalian target of rapamycin complex 1 (mTORC1) signaling. These data link microRNA impairment to the PLAG1-IGF2 pathway, providing new insight into the manner in which common Wilms tumor mutations drive disease pathogenesis.
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