HNF4A and congenital isolated hyperinsulinism: A syndromic condition comprising CHI, renal tubular dysfunction (Fanconi syndrome) and transient or recurrent hepatic dysfunction (OMIM #616026) was reported in patients with a heterozygous mutation of HNF4A, a gene that is known to be associated with MODY1 (OMIM #125850), but initial presentation in infancy may be CHI (146–148): Flanagan 2010, Stanescu 2012, Hamilton 2014).