Mejia-Otero Juan D, Grishman Ellen K, Patni Nivedita
Division of Pediatric Endocrinology, Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas.
J Endocr Soc. 2019 Jun 5;3(7):1357-1360. doi: 10.1210/js.2019-00120. eCollection 2019 Jul 1.
Dumping syndrome-associated hypoglycemia is caused by an exaggerated hyperinsulinemic response to glucose absorption in the small intestine. Diazoxide acts on the ATP-sensitive potassium channels and prevents insulin secretion and, thus, should be beneficial for the treatment of hypoglycemia secondary to dumping syndrome. We report on the efficacy of diazoxide in a pediatric patient with dumping syndrome. A 6-year-old girl born at 32 weeks' gestation age with resultant short gut syndrome and liver failure, who had undergone liver, small bowel, and pancreas transplantation at 1 year of age, developed late dumping-like symptoms with postprandial hypoglycemia, headaches, tremors, and irritability. She experienced relief of symptoms with oral intake. An oral glucose tolerance test showed a fasting and 2-hour blood glucose of 3.9 and 2.8 mmol/L, respectively. A gastric emptying study confirmed the diagnosis of dumping. A diet with 2 g of fiber and cornstarch and antimotility medications failed to improve the dumping symptoms. Diazoxide was started orally at a dose of 3 mg/kg/d and was increased to 5 mg/kg/d, divided every 8 hours, after 1 month, with improvement of postprandial blood glucose values (3.6 to 5.0 mmol/L). No hypertrichosis, fluid retention, respiratory concerns, or other side effects were noted. Several duodenal dilations were performed, with resultant improvement of gastric emptying. She was eventually weaned from diazoxide, and no further episodes of substantial hypoglycemia occurred. In conclusion, diazoxide was efficacious and safe for the treatment of hypoglycemia secondary to dumping syndrome in children. It could be of particular use as a bridging therapy for children awaiting more definitive surgical interventions.
倾倒综合征相关的低血糖是由小肠对葡萄糖吸收的过度高胰岛素反应引起的。二氮嗪作用于ATP敏感性钾通道，阻止胰岛素分泌，因此对治疗倾倒综合征继发的低血糖应该有益。我们报告了二氮嗪治疗一名患有倾倒综合征的儿科患者的疗效。一名孕32周出生的6岁女孩，患有短肠综合征和肝功能衰竭，1岁时接受了肝脏、小肠和胰腺移植，出现了类似倾倒综合征的晚期症状，伴有餐后低血糖、头痛、震颤和易怒。口服摄入后症状缓解。口服葡萄糖耐量试验显示空腹血糖和2小时血糖分别为3.9 mmol/L和2.8 mmol/L。胃排空研究确诊为倾倒综合征。含2 g纤维和玉米淀粉的饮食以及抗蠕动药物未能改善倾倒症状。开始口服二氮嗪，剂量为3 mg/kg/d，1个月后增加至5 mg/kg/d，每8小时分服一次，餐后血糖值有所改善（3.6至5.0 mmol/L）。未观察到多毛症、液体潴留、呼吸问题或其他副作用。进行了几次十二指肠扩张，胃排空得到改善。她最终停用了二氮嗪，未再发生严重低血糖发作。总之，二氮嗪治疗儿童倾倒综合征继发的低血糖有效且安全。它作为等待更确切手术干预的儿童的过渡治疗可能特别有用。
