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肢端肥大症并发暴发性垂体卒中:临床特征分析及文献复习。

Acromegaly complicated with fulminant pituitary apoplexy: clinical characteristic analysis and review of literature.

机构信息

Department of Endocrinology, Key Laboratory of Endocrinology of National Health Commission, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, China.

Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, China.

出版信息

Endocrine. 2023 Jul;81(1):160-167. doi: 10.1007/s12020-023-03379-7. Epub 2023 May 17.

Abstract

PURPOSE

To retrospectively summarize the clinical features of acromegaly complicated with fulminant pituitary apoplexy and analyze the prognostic factors to guide early identification and timely treatment of such patients.

METHODS

A retrospective analysis was carried out to summarize the clinical manifestations, hormone changes, imaging, treatment and follow-up of ten patients with acromegaly complicated with fulminant pituitary apoplexy admitted to our hospital from February 2013 to September 2021.

RESULTS

The mean age of the ten patients (five males and five females) at the time of pituitary apoplexy was 37.1 ± 13.4 years old. There were nine cases with sudden severe headaches and five cases with visual impairment. All patients had pituitary macroadenomas, of which six cases with Knosp grade ≥3. The level of GH/IGF-1 hormone after pituitary apoplexy was lower compared with pre-apoplexy, and 1 patient reached biochemical remission spontaneously. Seven patients underwent transsphenoidal pituitary surgery after apoplexy and one patient was treated with long-acting somatostatin analog. The biochemical remission rate was 37.5% in eight patients immediately after treatment and 50% at the last follow-up. Patients with Knosp grade ≥3 were less likely to achieve biochemical remission than those with Knosp grade <3 (16.7% vs. 100%, p = 0.048), and patients who achieved biochemical remission had a smaller maximum tumor diameter [20.1 (20.1,28.0) mm vs. 44.0 (44.0,60) mm, p = 0.016].

CONCLUSION

Acromegaly complicated with fulminant pituitary apoplexy remains a diagnostic and therapeutic challenge.

摘要

目的

回顾性总结肢端肥大症并发暴发性垂体卒中的临床特征,并分析其预后因素,以指导此类患者的早期识别和及时治疗。

方法

对 2013 年 2 月至 2021 年 9 月我院收治的 10 例肢端肥大症并发暴发性垂体卒中患者的临床表现、激素变化、影像学、治疗及随访进行回顾性分析。

结果

10 例垂体卒中患者(男 5 例,女 5 例)发病时的平均年龄为 37.1±13.4 岁。9 例患者突发剧烈头痛,5 例患者视力障碍。所有患者均存在垂体大腺瘤,其中 Knosp 分级≥3 级者 6 例。垂体卒中后 GH/IGF-1 激素水平较卒中前降低,1 例患者自发达到生化缓解。7 例患者在卒中后接受经蝶窦垂体手术,1 例患者接受长效生长抑素类似物治疗。治疗后即刻生化缓解率为 37.5%,末次随访时为 50%。Knosp 分级≥3 级患者的生化缓解率低于 Knosp 分级<3 级患者(16.7%比 100%,p=0.048),且达到生化缓解的患者肿瘤最大直径更小[20.1(20.1,28.0)mm 比 44.0(44.0,60)mm,p=0.016]。

结论

肢端肥大症并发暴发性垂体卒中仍然是一个诊断和治疗上的挑战。

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