Raisingani Manish, Brar Preneet Cheema
Department of Pediatrics, Division of Pediatric Endocrinology, University of Arkansas for Medical Sciences, 1 Children's Way, Slot 512-6, Little Rock, AR 72202, USA.
Department of Pediatrics, Division of Pediatric Endocrinology and Diabetes, New York University School of Medicine, New York, NY, USA.
J Pediatr Endocrinol Metab. 2019 Nov 26;32(11):1241-1245. doi: 10.1515/jpem-2019-0066.
Background Prolonged neonatal hyperinsulinism (PHI) causes hypoglycemia in the neonatal period and is associated with perinatal stress. Even though diazoxide is an effective treatment option for PHI, it has serious adverse effects making an argument for safe yet expeditious wean off of diazoxide while ensuring normoglycemia. The objective of this study was to characterize clinical course, dose requirement and duration of treatment with diazoxide in our cohort of infants diagnosed with PHI. Methods A retrospective chart review of infants diagnosed with PHI during a 6-year period was done documenting the diagnostic workup and the duration of treatment with diazoxide. Results PHI was diagnosed (n = 20; mean ± standard deviation [SD]) at 14.3 ± 22.4 days. Elevated insulin (8.3 ± 8.4 mIU/L), normal cortisol (15.5 ± 6.6 μg/dL [6-21]), normal growth hormone (18.8 ± 15.7 ng/mL [0.1-6.2]) and inappropriate low serum free fatty acids (0.3 ± 0.2 mmol/L [>1.5]) levels were measured during hypoglycemia (plasma glucose <50 mg/dL). Detectable insulin at the time of hypoglycemia was measured in 17 of 20 infants while the same number (17/20) of infants had a positive glucagon stimulation test (GST). The dose of diazoxide was 10 ± 3.7 mg/kg/day and duration of treatment was 44.9 ± 27.9 days. Conclusions This study illustrates that the duration of treatment with diazoxide in infants with PHI can be shorter than previously reported in the literature. We speculate that active tapering of diazoxide started within a week after discharge from hospital as well an outpatient tapering of diazoxide based on glucose monitoring were possible reasons for this outcome.
背景 持续性新生儿高胰岛素血症（PHI）在新生儿期会导致低血糖，且与围产期应激有关。尽管二氮嗪是治疗PHI的一种有效选择，但它有严重的不良反应，这就需要在确保血糖正常的同时安全且迅速地停用二氮嗪。本研究的目的是描述我们诊断为PHI的婴儿队列中二氮嗪的临床病程、剂量需求和治疗持续时间。方法 对6年间诊断为PHI的婴儿进行回顾性病历审查，记录诊断检查和二氮嗪治疗持续时间。结果 PHI在14.3±22.4天被诊断（n = 20；平均值±标准差[SD]）。低血糖期间（血浆葡萄糖<50mg/dL）测量到胰岛素升高（8.3±8.4mIU/L）、皮质醇正常（15.5±6.6μg/dL[6 - 21]）、生长激素正常（18.8±15.7ng/mL[0.1 - 6.2]）以及不适当的低血清游离脂肪酸水平（0.3±0.2mmol/L[>1.5]）。20名婴儿中有17名在低血糖时检测到可检测的胰岛素，同时相同数量（17/20）的婴儿胰高血糖素刺激试验（GST）呈阳性。二氮嗪剂量为10±3.7mg/kg/天，治疗持续时间为44.9±27.9天。结论 本研究表明，PHI婴儿中二氮嗪的治疗持续时间可能比文献中先前报道的要短。我们推测，出院后一周内开始积极逐渐减少二氮嗪剂量以及基于血糖监测的门诊逐渐减少二氮嗪剂量是导致这一结果的可能原因。
