Transient diabetes mellitus with ABCC8 variant successfully treated with sulfonylurea: Two case reports and review of literature

Table 1 Clinical features of transient neonatal diabetes mellitus patients with ABCC8 variants (including 2 patients in this case report)

Case	Age	DKA	PH	HCO3-	BE	HbA1c	C-peptide	Neurological symptoms	Mutation	Zygosity	Inheritance	Treatment	Ref.
1	36 d	Severe	7.08	3.3	−26	7.6	0.17	No	Arg1183Trp	Het	Paternal	Insulin → SU remission in 24 mo	Ngoc et al[29], 2021
2	50 d	Moderate	7.19	8	−19	7.58	0.41	Convultion	Glu1141Gly	Het	Paternal	Insulin → SU remission in 6 mo	Ngoc et al[29], 2021
3	35 d	Yes	NA	NA	NA	NA	NA	No	Arg216Cys	Hom	Both parents	Insulin → SU remission in 14 mo	Nayak et al[30], 2021
4	270 d	No	Normal	NA	NA	NA	NA	No	Leu1295Phe	Het	Paternal	Insulin remission in 48 mo	Nayak et al[30], 2021
5	4 d	No	Normal	NA	NA	NA	0.06	NA	Arg1380Cys	NA	NA	Insulin remission in 4 mo	Torbjörnsdotter et al[31], 2020
6	96 d	NA	NA	NA	NA	7.2	NA	Developmental delay	Arg653Gln	Het	NA	NA	Balamurugan et al[32], 2019
7	12 d	NA	NA	NA	NA	4	0.33	NA	Ile395Phe	NA	NA	Insulin → SU unresponsive remission in 5.5 mo	Li et al[28], 2018
8	60 d	NA	NA	NA	NA	7.2	< 0.5	NA	Arg877Gln	NA	NA	Insulin remission in 4 mo	Li et al[28], 2018
9	105 d	NA	NA	NA	NA	7.9	0.42	NA	Gly1255Ser	NA	NA	Insulin remission in 3.5 mo relapse in 8 yr	Li et al[28], 2018
10	35 d	No	7.459	22.5	−0.4	4.3	0.6	No	Gly832Cys	Het	De novo	Insulin → SU remission in 1 yr and 10 mo	Yamazaki M et al[33], 2017
11	74 d	Severe	7.06	3.4	−24.8	0.88	9.52	No	Leu1294Phe	Het	De novo	Insulin → SU remission in 1 mo	This study
12	230 d	Severe	7.12	2.1	−27	1.44	12.63	No	Leu1294Phe	Het	De novo	Insulin → SU remission in 12 mo	This study

HbA1c: Glycosylated hemoglobin; NA: Not available; SU: Sulfonylurea.