Weschle Lucas, Potratz Jenny, Rutsch Frank, Humberg Alexander, Oesingmann Sandra, Pascher Andreas, Busch Alexander S, Masjosthusmann Katja
Endocrinol Diabetes Metab Case Rep. 2025 Apr 29;2025(2). doi: 10.1530/EDM-25-0006. Print 2025 Apr 1.
Clinical management of congenital hyperinsulinism (CHI) remains a significant challenge due to its complex pathophysiology and the limitations of available therapies. Dasiglucagon, a synthetic glucagon analog, represents a novel approach to managing CHI, particularly in patients where conventional therapies fail. This report discusses a rare case of prolonged continuous subcutaneous dasiglucagon use in a neonate with CHI. Despite initial stabilization of glycemic levels, the patient developed necrolytic migratory erythema (NME), a rare dermatological condition associated with hyperglucagonemia, during dasiglucagon therapy. The patient further experienced severe malnutrition, zinc and amino acid deficiencies, and sepsis. Following the discontinuation of dasiglucagon therapy due to these severe side effects, the patient's skin and nutritional status improved markedly. However, glycemic control required subtotal pancreatectomy. This report underscores the potential of dasiglucagon in CHI management but highlights the importance of close monitoring during prolonged therapy.
NME, a rare but severe condition, appears to be associated with prolonged continuous subcutaneous dasiglucagon therapy, requiring early recognition and intervention. Close monitoring is essential during prolonged continuous subcutaneous dasiglucagon therapy to detect potential adverse effects, focusing on dermatological conditions, nutrient deficiencies or signs of infection. Multidisciplinary care is crucial to manage CHI with dasiglucagon, ensuring a comprehensive approach that addresses both glycemic control and potential side effects.
由于先天性高胰岛素血症(CHI)复杂的病理生理学以及现有治疗方法的局限性,其临床管理仍然是一项重大挑战。达西葡萄糖苷,一种合成的胰高血糖素类似物,代表了一种治疗CHI的新方法,特别是在传统治疗失败的患者中。本报告讨论了一例罕见的在患有CHI的新生儿中长时间持续皮下使用达西葡萄糖苷的病例。尽管血糖水平最初得到稳定,但患者在达西葡萄糖苷治疗期间出现了坏死性游走性红斑(NME),这是一种与高胰高血糖素血症相关的罕见皮肤病。患者还出现了严重营养不良、锌和氨基酸缺乏以及败血症。由于这些严重的副作用,达西葡萄糖苷治疗中断后,患者的皮肤和营养状况明显改善。然而,血糖控制需要进行次全胰腺切除术。本报告强调了达西葡萄糖苷在CHI管理中的潜力,但突出了在长期治疗期间密切监测的重要性。
NME是一种罕见但严重的疾病,似乎与长时间持续皮下使用达西葡萄糖苷治疗有关,需要早期识别和干预。在长时间持续皮下使用达西葡萄糖苷治疗期间,密切监测至关重要,以检测潜在的不良反应,重点关注皮肤病、营养缺乏或感染迹象。多学科护理对于使用达西葡萄糖苷治疗CHI至关重要,确保采取全面的方法来解决血糖控制和潜在副作用问题。