Bolmasova Anna V, Melikyan Maria A, Krylova Natalya A, Ionov Oleg V, Ryumina Irina I, Bockeria Ekaterina L, Pekareva Natalya A, Degtyareva Anna V
Kulakov Federal Rеseаrch Center for Obstetrics, Gynecology and Perinatology; Endocrinology research Centre.
Kulakov Federal Rеseаrch Center for Obstetrics, Gynecology and Perinatology.
Probl Endokrinol (Mosk). 2020 Sep 26;66(4):61-67. doi: 10.14341/probl12572.
Congenital hyperinsulinusm is rare disease characterized high secretion of insulin by pancreatic beta cells leading to the development of hypoglycemia. Persistent and transient forms of hyperinsulinism are distinguished. Transient hyperinsulinism are the most common cause of severe hypoglycemia in newborns. The etiology of this disease is not known. There are risk factors for the development of transient hyperinsulinism: asphyxia at birth, prematurity, maternal diabetes, low or large weight by gestation. Hypoglycemia with hyperinsulinism is severe. Therefore, early diagnosis and therapy especially during the neonatal period, are necessary.The article describes 3 clinical cases of transient hyperinsulinism in children with different gestational age and concomitant pathology. All children recevied insulinostatic therapy with diazoxide with a positive effect: euglycemia without glucose requirement . In all children, therapy was completed subsequently. At the time of publication of the article, the physical and psychomotor development of children is normal.
先天性高胰岛素血症是一种罕见疾病,其特征是胰腺β细胞分泌大量胰岛素,导致低血糖的发生。高胰岛素血症可分为持续性和短暂性两种类型。短暂性高胰岛素血症是新生儿严重低血糖最常见的病因。该病的病因尚不清楚。短暂性高胰岛素血症的发生有一些危险因素:出生时窒息、早产、母亲患糖尿病、孕期体重过低或过高。伴有高胰岛素血症的低血糖很严重。因此,早期诊断和治疗,尤其是在新生儿期,是必要的。本文描述了3例不同胎龄且伴有其他病症的儿童短暂性高胰岛素血症的临床病例。所有患儿均接受了二氮嗪的降胰岛素治疗,效果良好:血糖正常,无需补充葡萄糖。所有患儿随后均完成了治疗。在本文发表时,这些患儿的体格和精神运动发育均正常。