Clinical data of patients analyzed in this series

Age of onsetAge at surgerySensitivity to diazoxideABCC8 paternal mutations
Focal PHHI (13)
 125 h1.5 months3165+1g>a
 2First hours2 months 2/3R842G
 31 h2 months 1/3R1494W
 41.5 months4 monthsR1421C
 55 days6 months 1/2Not tested
 6First hours7 monthsNot tested
 71 h5 monthsNot tested
 83 h3.5 monthsNot tested
 9First hours3 monthsNot tested
 10First hours1.5 months?4138delCGAC-4138insGTG
 113 months4.5 monthsNot tested
 122 days2 monthsNot tested
 136 h3.5 monthsG1555S
Insulinomas (8)
 145 years6 years+None found
 1511 years12 years+Not tested
 16?15 years?Not tested
 1729 years31 years?Not tested
 1829 years32 years?Not tested
 1943 years44 years+Not tested
 2079 years79 years?Not tested
 2180 years81 years+Not tested
  • Cases n°1–13 are FoPHHI and cases n°14–21 are insulinomas. Cases n°1, 2, 3, and 13 and the ABCC8 mutations have previously been published by Fournet et al. (13).