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两个月大的婴儿因肾上腺皮质癌致库欣综合征,使用依托咪酯。

Using Etomidate in a Two-month-old Infant with Cushing Syndrome due to Adrenocortical Carcinoma.

机构信息

Division of Pediatric Endocrinology, Severance Children’s Hospital, Department of Pediatrics, Institute of Endocrinology, Yonsei University College of Medicine, Seoul, Korea

Division of Pediatric Cardiology, Severance Hospital, Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea

出版信息

J Clin Res Pediatr Endocrinol. 2022 Mar 3;14(1):102-106. doi: 10.4274/jcrpe.galenos.2020.2020.0164. Epub 2020 Nov 30.

Abstract

Cushing syndrome (CS) is a rare disease caused by hypercortisolemia. Although surgical treatment is the first-line treatment in CS, the appropriate medication for the patient’s condition should be selected when medical treatment is needed. Etomidate is an adrenal-blocking drug used to treat CS and the most suitable for severe hypercortisolemia and adrenocortical carcinoma (ACC), due to cardiovascular stability and an anti-tumorigenic effect. However, its use and safe recommended dosage in infants with CS is unreported. Here we describe the case of a 2-month-old girl treated with etomidate for CS caused by ACC. Even though radical mass excision was performed, severe hypercortisolemia persisted, resulting from metastatic lesions in the liver, and medical treatment was considered. The etomidate doses, no bolus dose and infusion rate of 0.03 mg/kg/hour, may be an appropriate dose for severe hypercortisolemia in infants. This case will help determine future treatment strategies for similar cases in infants.

摘要

库欣综合征(CS)是一种由皮质醇过多引起的罕见疾病。虽然手术治疗是 CS 的一线治疗方法,但在需要药物治疗时,应选择适合患者病情的药物。依托咪酯是一种用于治疗 CS 的肾上腺阻断药物,由于其心血管稳定性和抗肿瘤作用,最适合严重的皮质醇过多和肾上腺皮质癌(ACC)。然而,其在 CS 婴儿中的使用和安全推荐剂量尚未报道。在这里,我们描述了一例由 ACC 引起的 CS 的 2 个月大女孩接受依托咪酯治疗的病例。尽管进行了根治性肿块切除术,但由于肝转移病灶,严重的皮质醇过多仍然存在,因此考虑进行药物治疗。依托咪酯剂量、无推注剂量和 0.03mg/kg/h 的输注率可能是婴儿严重皮质醇过多的合适剂量。该病例将有助于确定未来类似婴儿病例的治疗策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b6/8900079/966ca8a2915e/JCRPE-14-102-g1.jpg

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