KCNQ1OT1 and Beckwith-Wiedemann syndrome: A subset of BWS patients with hypomethylation at the KCNQ1OT1 imprinting control regions (IC2 defect) have also been noted to have loss of methylation at other imprinted loci; hypomethylation of multiple maternally methylated imprinting control regions, including KCNQ1OT1 has also been demonstrated in cases of transient congenital diabetes [9].