NFKB1 and hyperinsulinemic hypoglycemia, familial, 4: It has also been described in genetically diverse IEIs including ataxia telangiectasia [26], NFKB1 haploinsufficiency [27], ADA2 deficiency [28], CD40 ligand deficiency [29], activated PI3K delta syndrome [30], STAT3 gain of function [31] and chronic granulomatous disease (CGD) [32].