Continuous subcutaneous octreotide infusion in congenital hyperinsulinism: practical application and insights in infancy and early childhood

先天性高胰岛素血症的持续皮下奥曲肽输注:婴幼儿期的实际应用及启示

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Abstract

PURPOSE: Octreotide is second-line treatment for patients with congenital hyperinsulinism (CHI) unresponsive to diazoxide. Short-acting octreotide is administered via subcutaneous (SC) injection or continuous infusion using a pump, but limited data exist on pump therapy. We present two cases of CHI managed with an octreotide infusion via pump with or without concurrent continuous glucose monitoring (CGM), providing practical guidance. CASE PRESENTATION: Octreotide 500mcg/1ml ampoule was used. The starting dose was 5mcg/kg/day, with titration to optimize glycaemia, and delivered as a continuous basal rate using a pump. CGM was used by Patient 2.Patient 1: CHI due to a paternal ABCC8 mutation was detected at 16 weeks of age, with diffuse uptake on 18F-DOPA PET. Unresponsive to diazoxide, SC octreotide injections commenced, transitioning to an infusion (13mcg/kg/day) with good response, and complete cessation of therapy at four years.Patient 2: Neonate with CHI due to Beckwith Wiedemann Syndrome developed side-effects from diazoxide and was commenced on SC octreotide injections. Effective at 30mcg/kg/day, he transitioned to an infusion at 4.5 months followed by long-acting octreotide at 12 months.Both patients tolerated the infusion with no adverse effects. CONCLUSION: This report highlights the practical use of octreotide infusion via pump. When combined with CGM, it allows for tailored dosing to meet individual needs while enhancing patient convenience.

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