Department of Pediatric Critical Care and Intensive Care, Robert Debré Teaching Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.
Department of Pediatric Endocrinology, Gynecology, and Diabetology, Necker-Enfants Malades Teaching Hospital, Assistance Publique-Hôpitaux de Paris, IMAGINE Institute Affiliate, Paris, France.
Diabetes Care. 2020 Jun;43(6):1191-1199. doi: 10.2337/dc19-0324. Epub 2020 Apr 9.
Transient neonatal diabetes mellitus (TNDM) occurs during the 1st year of life and remits during childhood. We investigated glucose metabolism and socioeducational outcomes in adults.
We included 27 participants with a history of TNDM currently with ( = 24) or without ( = 3) relapse of diabetes and 16 non-TNDM relatives known to be carriers of causal genetic defects and currently with ( = 9) or without ( = 7) diabetes. Insulin sensitivity and secretion were assessed by hyperinsulinemic-euglycemic clamp and arginine-stimulation testing in a subset of 8 TNDM participants and 7 relatives carrying genetic abnormalities, with and without diabetes, compared with 17 unrelated control subjects without diabetes.
In TNDM participants, age at relapse correlated positively with age at puberty ( = 0.019). The mean insulin secretion rate and acute insulin response to arginine were significantly lower in TNDM participants and relatives of participants with diabetes than in control subjects (median 4.7 [interquartile range 3.7-5.7] vs. 13.4 [11.8-16.1] pmol/kg/min, < 0.0001; and 84.4 [33.0-178.8] vs. 399.6 [222.9-514.9] µIU/mL, = 0.0011), but were not different between participants without diabetes (12.7 [10.4-14.3] pmol/kg/min and 396.3 [303.3-559.3] µIU/mL, respectively) and control subjects. Socioeducational attainment was lower in TNDM participants than in the general population, regardless of diabetes duration.
Relapse of diabetes occurred earlier in TNDM participants compared with relatives and was associated with puberty. Both groups had decreased educational attainment, and those with diabetes had lower insulin secretion capacity; however, there was no difference in insulin resistance in adulthood. These forms of diabetes should be included in maturity-onset diabetes of the young testing panels, and relatives of TNDM patients should be screened for underlying defects, as they may be treated with drugs other than insulin.
短暂性新生儿糖尿病（TNDM）发生于 1 岁以内，儿童期缓解。我们研究了成年患者的糖代谢和社会教育结局。
我们纳入了 27 名有 TNDM 病史的患者，目前处于糖尿病缓解期（=24 例）或复发期（=3 例），以及 16 名已知携带致病基因突变且目前无糖尿病的非 TNDM 亲属。我们在 8 名 TNDM 患者和 7 名携带遗传异常的亲属中进行了亚组胰岛素敏感性和分泌功能评估，其中 4 人伴有糖尿病，4 人无糖尿病，将其结果与 17 名无糖尿病的无关对照者进行了比较。
TNDM 患者中，复发时的年龄与青春期年龄呈正相关（ = 0.019）。与对照组相比，TNDM 患者和伴糖尿病亲属的胰岛素分泌率和精氨酸刺激后的胰岛素急性反应均显著降低（中位数分别为 4.7 [四分位间距 3.75.7] vs. 13.4 [11.816.1] pmol/kg/min， < 0.0001；和 84.4 [33.0178.8] vs. 399.6 [222.9514.9] µIU/mL， = 0.0011），而不伴糖尿病的患者（分别为 12.7 [10.414.3] pmol/kg/min 和 396.3 [303.3559.3] µIU/mL）与对照组之间无差异。无论糖尿病病程如何，TNDM 患者的社会教育程度均低于一般人群。
与亲属相比，TNDM 患者的糖尿病复发更早，且与青春期相关。两组的教育程度均较低，且伴有糖尿病的患者胰岛素分泌能力降低；然而，成年期的胰岛素抵抗无差异。这些类型的糖尿病应纳入青年发病成年型糖尿病的检测项目，且 TNDM 患者的亲属应筛查潜在缺陷，因为他们可能需要用胰岛素以外的药物治疗。
