TABLE 1

Clinical features of affected family members

PatientBirth weightAge at diagnosisFasting duration at diagnosis (h)SurgeryDiazoxide treatment
IbNormal40 years21
IIb2.2 kg at 32 weeks5 months2yesyes
IIcNormal8 months<8yes
IIIa5.3 kgAsymptomatic18
IIIb3.9 kg2 days4yes