TABLE 2

AIR and diazoxide sensitivity in affected family members

Calcium AIR (μU/ml)Leucine AIR (μU/ml)Glucose AIR (μU/ml)Tolbutamide AIR (μU/ml)Diazoxide responsiveness
Patient
 Ib3002510Not tested
 IIc371262Partial
 IIIaNot testedNot tested4417Partial
Control subjects (95% CI)
 Recessive KATP-HI (SUR1 g3992-9a/g3992-9a and g3992-9a/delP1388) (n = 9)14–340–95–23−2 to 3No
 GDH-HI (n = 5)−8 to 415–7063–17023–36yes
Normal control subjects (95% (I) (n = 9)−1 to 2−4 to 822–6226–80yes
  • HI, hyperinsulinism.