Figure 1.

Pedigree of the family. The members developed either hypoglycaemia, diabetes or both are indicated as affected and shown with black-filled boxes. IV.4: Insulin dependent diabetes since 35 years-old, developed diabetic nephropathy (chronic renal failure) (reportedly), IV.5: Had insulin dependent diabetes and diabetic nephropathy (reportedly), IV.6: Diabetes and bilateral visual loss was reported, V.1: Father, 41 years old, apparently healthy with normal glucose and HbA1c (5.6%) levels, V.2: Mother 37 years old, developed insulin dependent diabetes during pregnancy and has been on insulin treatment since 24 years old, changing the treatment to SU therapy is in progress (see the section of the case report concerning sulphonylurea treatment), V.3: 40 years old, had insulin dependent diabetes mellitus since 32 years-old, VI.1: Born at term, macrosomic birth weight (4750 gram; 2.8 SDS), hypoglycaemia was detected during the neonatal period, died at 1-month during hospitalization with unknown etiology, VI.2: Index patient, VI.3: 9.5 years-old, born at term, birth weight was 3750 gram (0.4 SDS), had transient hypoglycemia during the neonatal period, latest blood glucose and HbA1c levels were normal, VI.4: 6.5 years old, born at seven months of gestation from a twin pregnancy, birth weight was 1250 gram (1.05 SD), HbA1c is normal

M: mutated, WT: wild type, SDS: standard deviation score, SU: sulphonylurea, HbA1c: haemoglobin A1c