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重组人生长激素治疗一名患有胚胎性横纹肌肉瘤的年轻患者的难治性低血糖症

Recombinant Human GH in Managing Refractory Hypoglycemia in a Young Patient With Embryonal Rhabdomyosarcoma.

作者信息

Belamkar Aditya V, Shah Viral N, Patadia Palak, Abfall Avery, Priscu Adelina

机构信息

Department of Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA.

Division of Endocrinology & Metabolism, Indiana University School of Medicine, Indianapolis, IN 46202, USA.

出版信息

JCEM Case Rep. 2025 Jul 28;3(9):luaf137. doi: 10.1210/jcemcr/luaf137. eCollection 2025 Sep.

DOI:10.1210/jcemcr/luaf137
PMID:40727482
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12301714/
Abstract

Non-islet cell tumor hypoglycemia (NICTH) is a rare paraneoplastic syndrome associated with various malignancies, mediated by the overproduction of IGF-2. We describe a 24-year-old male with metastatic embryonal rhabdomyosarcoma who presented with severe symptomatic hypoglycemia. Workup confirmed suppressed insulin and ketone levels, with an IGF-2/IGF-1 ratio of 3.6 (<3). Hypoglycemia was initially managed with corticosteroids and dextrose-containing fluids without success. Since surgical debulking was not feasible due to tumor burden, recombinant human GH (rhGH) was considered. Somatropin was initiated at 1 mg daily and uptitrated to 2 mg daily over 2 days, resulting in resolution of hypoglycemia with progressive tapering of dextrose-containing fluids and steroid doses. He was discharged on rhGH 2.7 mg daily with no further hypoglycemic episodes. This case highlights the challenges of NICTH, emphasizing the need for individualized treatment strategies. While the IGF-2/IGF-1 ratio did not meet the classic threshold (>10), other laboratory testing was suggestive of a non-insulin, IGF-2-mediated pathway. The use of rhGH for NICTH is not fully understood; however, it may be an important tool in preventing hypoglycemia in patients with nonresectable malignancies.

摘要

非胰岛细胞瘤性低血糖症(NICTH)是一种罕见的副肿瘤综合征,与多种恶性肿瘤相关,由胰岛素样生长因子-2(IGF-2)过度产生介导。我们描述了一名24岁患有转移性胚胎性横纹肌肉瘤的男性,他出现了严重的症状性低血糖。检查证实胰岛素和酮水平受到抑制,IGF-2/IGF-1比值为3.6(<3)。低血糖最初采用皮质类固醇和含葡萄糖液体治疗,但未成功。由于肿瘤负荷大,手术减瘤不可行,因此考虑使用重组人生长激素(rhGH)。开始使用生长激素,每日1毫克,在2天内逐渐增加至每日2毫克,随着含葡萄糖液体和类固醇剂量逐渐减少,低血糖得以缓解。他出院时每日服用2.7毫克rhGH,未再发生低血糖发作。该病例凸显了NICTH的挑战,强调了个体化治疗策略的必要性。虽然IGF-2/IGF-1比值未达到经典阈值(>10),但其他实验室检查提示存在非胰岛素、IGF-2介导的途径。rhGH用于NICTH的机制尚未完全明确;然而,它可能是预防不可切除恶性肿瘤患者低血糖的重要工具。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e10e/12301714/4dcacafbfb8c/luaf137f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e10e/12301714/964d57cd6078/luaf137f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e10e/12301714/805589a10dcb/luaf137f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e10e/12301714/4dcacafbfb8c/luaf137f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e10e/12301714/964d57cd6078/luaf137f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e10e/12301714/805589a10dcb/luaf137f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e10e/12301714/4dcacafbfb8c/luaf137f3.jpg

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