Table 1.

Characteristics of carriers of p.R114W compared to carriers of other pathogenic HNF4A mutations.

	p.R114W mutation	Other pathogenic HNF4A mutations	P
Probands	30	100	N/A
Heterozygous family members	27	134	N/A
Birth weight corrected to 40 weeks gestation (g) (Mean (95% CI))	3476 (3160,3792) (n=23)	4147 (3985,4309) (n=84)	0.0004 *
Standard deviation scores of birth weight (Mean (95% CI))	-0.032 (-0.67,0.61) (n=23)	1.36 (1.03,1.68) (n=84)	0.0003 *
Birth weight corrected to 40 weeks gestation >4000g	5 (n=23)	45 (n=84)	0.009 †
Age of diagnosis of diabetes in probands (years) (Median (Q1,Q3))	21 (15,32) (n=30)	21 (16,28) (n= 96)	0.63 *
Age of diagnosis of diabetes in family members (years) (Median (Q1,Q3))	34 (20,50) (n=21)	24 (16,34) (n=95)	0.018 *
Duration of diabetes (years) (Median (Q1,Q3))	5 (0,14.5) (n=51)	7 (1,19) (n=191)	0.025 *
Initial treatment of diabetic patients (Diet/OHA/Ins)	10/19/16 (n=45)	47/50/59 (n=156)	N/A
Diabetic patients treated with sulfonylureas at some time during diabetes	21	70	N/A
Patients reported as being sulfonylurea sensitive	10 (47.6%)	51 (72.9%)	0.038 †
BMI of diabetic patients (Median (Q1,Q3))	22.65 (22.975,29.2) (n=40)	23.9 (21.945,27) (n=128)	0.97 *
Median HbA1c of diabetic patients (IFCC & DCCT)	65.0mmol/mol 8.1% (n=31)	57.4mmol/mol 7.4% (n=151)	0.97 *

^*t-test

^†fisher’s exact test