Payal S Kubsad, H N Vani, Tejasvi Sheshadri, Raghupathy Palany
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引用次数: 0
Abstract
Introduction: Hyperinsulinemic hypoglycaemia (HH) is characterised by unregulated insulin secretion, leading to persistent non-ketotic hypoglycaemia with a lack of alternate fuel that induces a severe risk for brain damage and neurodevelopmental abnormalities. Octreotide, a somatostatin analogue, has been effectively administered as subcutaneous injections or depot preparations in diazoxide-unresponsive HH.
Methods: Children and infants with HH receiving short-acting octreotide injections were included. Anthropometric values, hypoglycaemic episodes, HbA1C, and side effects were noted from the records and were followed up for 12 months. Informed written consent was obtained from the parents before administration of a single dose of LAR (long-acting octreotide). Based on home-based glucose monitoring (HBGM), the dosage of LAR was modified, and short-acting octreotide was eventually withdrawn. The patients shared the injection's cost for cost-effectiveness. HH affects the quality of life (QoL) if not diagnosed and controlled adequately. A QoL questionnaire was given before starting LAR and after 6 months of receiving LAR, and the changes were noted accordingly.
Results: Twenty-two patients were diagnosed with HH, of which 11 infants and children were included in the study. Mutations were identified in 7 (63.63%) children. Daily octreotide could be tapered and stopped with the addition of sirolimus in one patient with an increasing dose of LAR to maintain euglycaemia. The hypoglycaemic episodes decreased with increasing dose of LAR with a decrease in the severity. Eight (72.7%) patients showed an improved lifestyle on LAR quantified through a QoL questionnaire.
Conclusion: LAR was found effective in reducing hypoglycaemic episodes with no adverse effects. The patient's parent's satisfaction was higher. Given its high cost, this trial achieved cost-effectiveness by sharing a single sitting of LAR injection.
简介高胰岛素血症性低血糖(HH)的特点是胰岛素分泌失调,导致持续性非酮症性低血糖,缺乏替代燃料,极易造成脑损伤和神经发育异常。奥曲肽是一种体生长抑素类似物,通过皮下注射或皮下注射制剂,可有效治疗对双氮醇无反应的 HH:方法:纳入接受短效奥曲肽注射的HH患儿和婴儿。从记录中记录人体测量值、低血糖发作、HbA1C 和副作用,并随访 12 个月。在注射单剂量 LAR(长效奥曲肽注射剂)前,已获得家长的知情书面同意。根据家庭血糖监测(HBGM)结果,对 LAR 的剂量进行了调整,最终停用了短效奥曲肽。患者共同承担注射费用,以实现成本效益。HH 如果得不到及时诊断和控制,会影响患者的生活质量(QoL)。在开始使用 LAR 之前和接受 LAR 6 个月之后,对患者进行了 QoL 问卷调查,并记录了相应的变化:22名患者被确诊为HH,其中11名婴幼儿被纳入研究。有 7 名儿童(占 63.63%)发现了基因突变。一名患者在增加 LAR 剂量以维持优格血症的情况下,每日使用奥曲肽的剂量可逐渐减少并停止使用,同时添加西罗莫司。低血糖发作随着 LAR 剂量的增加而减少,严重程度也有所减轻。八名患者(72.7%)在服用 LAR 后生活方式得到了改善,并通过 QoL 问卷进行了量化:结论:LAR 能有效减少低血糖发作,且无不良反应。患者家长的满意度较高。鉴于 LAR 的成本较高,该试验通过共用一次 LAR 注射剂实现了成本效益。
期刊介绍:
The Indian Journal of Endocrinology and Metabolism (IJEM) aims to function as the global face of Indian endocrinology research. It aims to act as a bridge between global and national advances in this field. The journal publishes thought-provoking editorials, comprehensive reviews, cutting-edge original research, focused brief communications and insightful letters to editor. The journal encourages authors to submit articles addressing aspects of science related to Endocrinology and Metabolism in particular Diabetology. Articles related to Clinical and Tropical endocrinology are especially encouraged. Sub-topic based Supplements are published regularly. This allows the journal to highlight issues relevant to Endocrine practitioners working in India as well as other countries. IJEM is free access in the true sense of the word, (it charges neither authors nor readers) and this enhances its global appeal.