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普萘洛尔成功治疗合并婴儿肝血管瘤的消耗性甲状腺功能减退症:病例报告及文献综述

Consumptive hypothyroidism complicating infantile hepatic hemangioma successfully treated with propranolol: a case report and literature review.

作者信息

Bettini Irene, Poletti Giulia, Rocca Alessandro, Di Natale Valeria, Gennari Monia, Lanari Marcello, Pession Andrea, Cassio Alessandra

机构信息

Specialty School of Pediatrics, Alma Mater Studiorum, University of Bologna, Bologna, Italy.

Pediatric Emergency Unit, IRCCS, Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.

出版信息

Ital J Pediatr. 2025 Jun 23;51(1):198. doi: 10.1186/s13052-025-02020-9.

Abstract

BACKGROUND

Consumptive hypothyroidism (CH) is a rare paraneoplastic syndrome, caused by the overexpression in vascular tumors of type 3 deiodinase (D3), converting thyroid hormones into inactive metabolites.

CASE PRESENTATION

We report the case of a 2-months-old male infant with diffuse infantile hepatic hemangioma (IHH). Thyroid function screening detected a CH. The patient was promptly treated with propranolol: after 2 weeks, a marked reduction in tumour size was observed and thyroid function was restored. No hormone replacement therapy was required. We then performed a literature review on PubMed/Medline: based on the title and abstract, we extracted 64 cases of CH secondary to IHH published between 2000 and 2023. 59.37% patients received propranolol, either alone (52.63%) or in combination with other treatments. 18.75% patients required surgical treatment or liver transplant. LT4 was administered in 92.85% of the patients. Patients who received propranolol required a lower dosage of LT4 than patients who received other treatments.

CONCLUSIONS

Thyroid function should be evaluated in all children with IHH to rule out CH. Early recognition of IHH and CH and prompt therapy with propranolol can effectively treat IHH and the subsequent hypothyroidism, sometimes even without the need of hormone replacement therapy.

摘要

背景

消耗性甲状腺功能减退症(CH)是一种罕见的副肿瘤综合征,由3型脱碘酶(D3)在血管肿瘤中过度表达引起,该酶将甲状腺激素转化为无活性代谢产物。

病例报告

我们报告一例2个月大的男性婴儿,患有弥漫性婴儿肝血管瘤(IHH)。甲状腺功能筛查发现了CH。患者立即接受普萘洛尔治疗:2周后,观察到肿瘤大小明显缩小,甲状腺功能恢复。无需激素替代治疗。然后我们在PubMed/Medline上进行了文献综述:根据标题和摘要,我们提取了2000年至2023年间发表的64例继发于IHH的CH病例。59.37%的患者接受了普萘洛尔治疗,单独使用(52.63%)或与其他治疗联合使用。18.75%的患者需要手术治疗或肝移植。92.85%的患者使用了左甲状腺素(LT4)。接受普萘洛尔治疗的患者比接受其他治疗的患者需要更低剂量的LT4。

结论

所有患有IHH的儿童都应评估甲状腺功能以排除CH。早期识别IHH和CH并及时使用普萘洛尔治疗可有效治疗IHH及随后的甲状腺功能减退症,有时甚至无需激素替代治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c90/12186321/896abb00b90c/13052_2025_2020_Fig1_HTML.jpg

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