Moore Megan M, Bailey Abby M, Flannery Alexander H, Baum Regan A
1 University of Kentucky College of Pharmacy, Lexington, KY, USA.
2 Department of Pharmacy, University of Kentucky HealthCare, Lexington, KY, USA.
J Pharm Pract. 2017 Aug;30(4):468-475. doi: 10.1177/0897190016645036. Epub 2016 Apr 24.
Rabson-Mendenhall syndrome is a rare genetic disorder resulting from mutations in the insulin receptor and is associated with high degrees of insulin resistance. These patients are prone to complications secondary to their hyperglycemia including diabetic ketoacidosis (DKA). We report the case of a 19-year-old male with Rabson-Mendenhall syndrome presenting with DKA who required doses of up to 500 U/h (10.6 U/kg/h) of insulin. The patient's insulin infusion was originally compounded with U-100 regular insulin, although to minimize volume, the product was compounded with U-500 insulin. The DKA eventually resolved requiring infusion rates ranging from 400 to 500 U/h. Although numerous opportunities for medication errors exist with the use of U-500 insulin, this case outlines the safe use of concentrated intravenous insulin when clinically indicated for patients requiring extremely high doses of insulin to control blood glucose.
拉布森 - 门登霍尔综合征是一种由胰岛素受体突变引起的罕见遗传性疾病,与高度胰岛素抵抗有关。这些患者容易出现继发于高血糖的并发症,包括糖尿病酮症酸中毒(DKA)。我们报告了一例19岁患有拉布森 - 门登霍尔综合征的男性患者,该患者因DKA就诊,需要高达500 U/h(10.6 U/kg/h)的胰岛素剂量。患者最初使用的胰岛素输注液是用U - 100常规胰岛素配制的,不过为了尽量减少液体量,后来改用U - 500胰岛素配制。DKA最终得到缓解,所需输注速率为400至500 U/h。尽管使用U - 500胰岛素存在许多用药错误的机会,但该病例概述了在临床表明需要极高剂量胰岛素来控制血糖的患者中安全使用浓缩静脉胰岛素的情况。