Table 2

Hyperinsulinism clinical features and management

Patient #	Diazoxide responsiveness	HI management	GIR	Surgery	Postsurgery management	Age off treatment (months)	Current age (months)
Surgery
1	Unknown	Deceased	15	ND	NA	NA	NA
2	–	Surgery	25	98%	Oct+continuous feeds	NA	Deceased
3	–	Surgery	44	2%	None	1	22
4	–	Surgery	22	90%	Diazoxide	7	15
5	–	Surgery	8	75%	none	9	78
6	–	Surgery	13	60%	Continuous D20	5	23
7	–	Deceased	50	ND	NA	NA	NA
8	–	Surgery	5	biopsies	Continuous D20	21	52
9	–	Surgery	32	99%	None	–	2.25
10	–	Surgery	14.5	91%	None	5	68
11	–	Surgery	22	95%	Continuous feeds	28	44
12	–	Surgery	18	85%	None	18	27
13	Unknown	Deceased	32	ND	NA	NA	NA
14	–	Deceased	24	ND	NA	NA	NA
Octreotide
15	–	Oct	11	–	NA	12	62
16	–	Oct	5.5	–	NA	7	17
17	–	Oct	7.7	–	NA	24	60
18	–	Oct	NK	–	NA	48	107
19	–	Oct	6.1	–	NA	17	45
20	–	Oct	13	–	NA	NA	Deceased
Diazoxide
21	+	Diaz	NK	–	NA	24	177
22	+	Diaz	11.5	–	NA	24	53
23	+	Diaz	NK	–	NA	1	75
24	+	Diaz	21	–	NA	–	16
25	+	Diaz	10	–	NA	12	63
26	+	Diaz	10	–	NA	NK	36
IC2
27	+	Low-dose diaz	12	–	NA	–	15
28	NT	Frequent feeds	NK	–	NA	13	22

D20, intravenous dextrose; Diaz, diazoxide; GIR, glucose infusion rate in mg/kg/min-; NA, not applicable; ND, patient died prior to surgery; NK, not known; NT, diazoxide not trialled; Oct, octreotide.