Table 1.

Clinical features of 12 girls with Turner syndrome and hyperinsulinism

Case	Birth weight1	Age at hyperinsulinism presentation	Diazoxide response	Pancreatectomy	Age at Turner syndrome diagnosis	Current age, years	Current status
1	3.1 kg at 37 weeks (50th–90th percentile)	2 months	−	90% at 8.5 months	10 years	44	diabetes at age 32
2	4.0 kg at 42 weeks (>90th percentile)	1 day	+	−	6 years	33	stopped diazoxide at 2 years
3	2.6 kg at 38 weeks (10th–50th percentile)	2 days	−	95% at 18 months	9 months	12	normoglycemic
4	3.6 kg at 39 weeks (>90th percentile)	4 months	−	−	5 months	11	overnight feeds stopped at 5.5 years
5	1.5 kg at 34 weeks (<10th percentile)	1 day	−	−	8 months	11	normoglycemic
6	2.5 kg at 39 weeks (10th–50th percentile)	5 months	+	−	12 months	7	diazoxide stopped at 5.5 years
7	3.2 kg at 40 weeks (50th–90th percentile)	2 days	−	50% at 3 months	prenatal	4	limited fasting and continued overnight feeds
8	3.2 kg at 38 weeks (50th–90th percentile)	1 day	−	−	9 months	3	tube feedings and lanreotide
9	2.3 kg at 37 weeks (10th–50th percentile)	1 day	+	−	prenatal	1	diazoxide
10	1.9 kg at 36 weeks (10th–50th percentile)	1 day	+	−	15 days	1	diazoxide
11	2.4 kg at 36 weeks (10th–50th percentile)	1 day	+	−	prenatal	1	limited fasting
12	2.8 kg at 39 weeks (50th–90th percentile)	7 days	+	−	prenatal	7/12	diazoxide

¹Birth weight percentiles based on an Italian nationwide survey of Turner Syndrome patients [17].