Mangla Pragya, Hussain Khalid, Ellard Sian, Flanagan Sarah E, Bhatia Vijayalakshmi
Department of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
Developmental Endocrinology Research Group, Clinical and Molecular Genetics, Institute of Child Health, University College London, London, UK.
J Pediatr Endocrinol Metab. 2018 Aug 28;31(8):943-945. doi: 10.1515/jpem-2018-0112.
Persistent hyperinsulinemic hypoglycemia of infancy (PHHI), also known as congenital hyperinsulinism, has been known to go into spontaneous remission, with patients developing diabetes in later life. A temporary phase of hyperglycemia is, however, rarely reported.
We describe a 16-month-old child, a known case of diazoxide responsive PHHI, presenting with mixed hyperglycemic hyperosmolar coma and ketoacidosis with rhabdomyolysis while on diazoxide treatment. The patient required temporary cessation of diazoxide and initiation of insulin infusion, followed by a relapse of hypoglycemia again necessitating diazoxide therapy.
Hyperosmolar coma with ketoacidosis is a rare side-effect of diazoxide therapy, documented even in patients with persistent hyperinsulinemic hypoglycemia of infancy.
婴儿持续性高胰岛素血症性低血糖症（PHHI），也称为先天性高胰岛素血症，已知可自发缓解，患者在以后的生活中会发展为糖尿病。然而，高血糖的临时阶段很少被报道。
我们描述了一名16个月大的儿童，已知为对二氮嗪有反应的PHHI患者，在接受二氮嗪治疗时出现混合性高血糖高渗性昏迷和酮症酸中毒伴横纹肌溶解。患者需要暂时停用二氮嗪并开始胰岛素输注，随后低血糖复发，再次需要二氮嗪治疗。
高渗性昏迷伴酮症酸中毒是二氮嗪治疗的一种罕见副作用，即使在婴儿持续性高胰岛素血症性低血糖症患者中也有记录。
