RNF213 and familial isolated congenital asplenia: Although the association between RNF213 p.Arg4810Lys and ICAS was reported in numerous studies.6,7,26-29 Similar to previous reports, we found an association between ICAS and RNF213 p.Arg4810Lys.6,7 Several reports have demonstrated an association between ECAS and the RNF213 p.Arg4810Lys variant.