Department of Endocrinology, Puerta del Mar University Hospital, Cádiz, Spain.
Department of Endocrinology, Mater Misericordiae University Hospital, Dublin, Ireland.
Diabet Med. 2021 Jun;38(6):e14488. doi: 10.1111/dme.14488. Epub 2021 Jan 12.
To determine the fetal and maternal outcomes in pregnant women with Glucokinase-Maturity onset diabetes of the young (GCK-MODY).
We studied the obstetric and perinatal outcomes in 99 pregnancies of 34 women with GCK-MODY. The mutation status of the offspring was known in 29 and presumed in 33. Clinical outcomes were determined and compared between affected (n = 39) and unaffected (n = 23) offspring.
59% of pregnancies were treated with diet alone and 41% received insulin. Birthweight, percentage of large for gestational age (LGA) and caesarean section (CS) in GCK-unaffected offspring was significantly higher than in GCK-affected offspring (4.0 ± 0.7 vs. 3.4 ± 0.4 kg, p = 0.001), 15 (65%) vs. 5(13%) (p = 0.00006) and 17 (74%) vs. 11 (28%) (p = 0.001), respectively. We observed an earlier gestational age at delivery on insulin in unaffected offspring (38.3 ± 1.0 vs. 39.5 ± 1.5 weeks, p = 0.03) with no significant change in LGA (9 (82%) vs. 6 (50%); p = 0.12), and a higher rate of CS (8 [73%] vs. 3 [11%]; p < 0.001), and no change in small for gestational age (0 [0%] vs. 4 [14%]; p = 0.30) in affected offspring.
Insulin therapy in unaffected offspring did not reduce LGA and was associated with earlier gestational age at delivery. Insulin treatment in GCK-affected offspring was associated with an increased incidence of CS, but did not adversely affect fetal outcome. Fetal genotype determines birthweight rather than treatment. Pre-pregnancy diagnosis of GCK-MODY, use of continuous glucose monitoring and non-invasive fetal genotyping may enable further investigation of targeted therapy in this condition.
确定患有葡萄糖激酶-年轻发病型糖尿病（GCK-MODY）的孕妇的胎儿和母体结局。
我们研究了 34 名 GCK-MODY 女性的 99 例妊娠的产科和围产期结局。29 例后代的突变状态已知，33 例推测。确定并比较了受影响（n=39）和未受影响（n=23）后代的临床结局。
59%的妊娠仅用饮食治疗，41%用胰岛素治疗。GCK 未受影响后代的出生体重、大于胎龄儿（LGA）的百分比和剖宫产（CS）率明显高于 GCK 受影响后代（4.0±0.7 与 3.4±0.4kg，p=0.001）、15（65%）与 5（13%）（p=0.00006）和 17（74%）与 11（28%）（p=0.001）。我们观察到未受影响后代在使用胰岛素时分娩的孕龄更早（38.3±1.0 与 39.5±1.5 周，p=0.03），但 LGA 无显著变化（9（82%）与 6（50%）；p=0.12），CS 发生率更高（8[73%]与 3[11%]；p<0.001），GCK 受影响后代的小于胎龄儿发生率无变化（0[0%]与 4[14%]；p=0.30）。
未受影响后代的胰岛素治疗并未降低 LGA，且与更早的分娩孕龄相关。GCK 受影响后代的胰岛素治疗与 CS 发生率增加相关，但对胎儿结局无不利影响。胎儿基因型决定出生体重，而不是治疗方法。GCK-MODY 的孕前诊断、连续血糖监测和非侵入性胎儿基因分型可进一步探讨该疾病的靶向治疗。
