Table 1. Clinical characteristics of the nine MODY probands investigated.

Proband	P01a	P02	P03	P04	P05	P06	P07	P08	P09
Sex	F	M	F	F	F	F	M	M	M
Age of diabetes diagnosis (y)	38b	24	25	24	19	11	25	28	14
Number of generations with DM	3	3	3	4	3	4	3	3	3
Current status
Age (y)	68	52	38	31	55	16	35	35	29
BMI	30.1	21.4	29.9	27.2	28.3	31.5	24.3	24.6	25.3
Insulin dose (U/kg/day)	2.2	0.4	0	0.8	0.7	2.0	0.8	NA	0.5
OHA	Yes	No	Yes	Yes	No	Yes	No	No	No
Glycosylated hemoglobin (%)	7.5	6.1	9.0	6.7	7.1	8.4	7.2	9.3	6.4
Other clinical features
Hypertension	Yes	No	No	No	Yes	Yes	Yes	No	No
Hypercholesterolemia	Yes	No	No	No	Yes	Yes	No	No	No
Retinopathy	Yes	No	No	No	Yes	No	Yes	No	No
Nephropathy	Yes	No	No	No	No	No	No	No	No
Arteriosclerosis	Yes	No	No	No	No	No	No	No	No
Polyneuropathy	Yes	No	No	No	Yes	No	No	No	No

None of the individuals had hearing loss or acantosis nigricans. Data regarding hepatic steatosis, renal cysts, polycystic ovarian disease and triglyceride status were not available in all individuals.

^aP01 also had partial lipodystrophy with reduced subcutaneous fat in extremities and excess of abdominal subcutaneous fat. There was no evidence of hypertriglyceridemia, hepatic steatosis, acanthosis nigricans or polycystic ovarian disease.

^bAn affected family member was diagnosed at age 28 years.

Abbreviations: BMI, body mass index; DM, diabetes mellitus; NA, not available; OHA, Oral Hypoglycemic agents; U, international unit (used for insulin doses); y, year; kg, kilo gram body weight.