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癫痫持续状态和糖尿病酮症酸中毒:肢端肥大症的罕见临床表现。

Status epilepticus and diabetes ketoacidosis: uncommon clinical presentations of acromegaly.

作者信息

Chamba Nyasatu G, Amour Ahlam A, Sadiq Abid M, Lyamuya Tecla R, Assey Emmanuel V, Sadiq Adnan M, Howlett William P

机构信息

Department of Internal Medicine, Kilimanjaro Christian Medical Centre, Moshi, Tanzania.

Kilimanjaro Christian Medical University College, Moshi, Tanzania.

出版信息

Endocrinol Diabetes Metab Case Rep. 2021 Apr 1;2021. doi: 10.1530/EDM-20-0156.

Abstract

SUMMARY

Acromegaly is a rare disease caused by hypersecretion of the growth hormone (GH). Most cases are caused by either pituitary microadenoma or macroadenoma. The GH producing tumors present with clinical manifestations of acromegaly due to excessive GH secretion or symptoms resulting from mass effects of the enlarging tumor. The physical changes are usually slow and, therefore, recognition of the disease is delayed. These adenomas are never malignant but can have significant morbidity and mortality. A subgroup of patients with acromegaly present with severe hyperglycemia resulting in diabetic ketoacidosis (DKA) which requires insulin. Rarely are pituitary tumors responsible for generalized convulsions except when they are too large. We hereby present two cases, the first is that of a 26-year-old female who presented with new onset status epilepticus, DKA with a 1-year history of diabetes mellitus (DM). On examination, she had clinical features of acromegaly. The second case is that of a 34-year-old female who presented with new onset status epilepticus, hyperglycemia with a history of recently diagnosed DM, and features of gigantism. In both cases, their diagnosis was confirmed by elevated serum GH and later by elevated insulin-like growth factor type 1 levels, and CT of the head demonstrating large pituitary macroadenoma. The importance of clinical history and examination, as well as investigations is vital in the recognition of acromegaly. The prognosis of acromegalic patients depends on early clinical recognition and tumor size reduction by either medical or surgical therapy.

LEARNING POINTS

Conditions such as status epilepticus and DKA may be clinical presentations in patients presenting with acromegaly. Seizures are rare in people with pituitary adenoma and typically occur when the tumor invades the suprasellar area due to mass effect on the brain. This article shows how best we were able to manage the acromegaly complications in a low resource setting. Hyperprolactinemia in acromegaly may be due to disruption of the normal dopaminergic inhibition of prolactin secretion due to mass effect of the macroadenoma, and around 25% of GH-secreting adenomas co-secrete prolactin.

摘要

摘要

肢端肥大症是一种由生长激素(GH)分泌过多引起的罕见疾病。大多数病例由垂体微腺瘤或大腺瘤所致。分泌GH的肿瘤因GH分泌过多而出现肢端肥大症的临床表现,或因肿瘤增大产生占位效应而出现相关症状。身体变化通常较为缓慢,因此疾病的识别会延迟。这些腺瘤从不恶变,但可导致显著的发病率和死亡率。一部分肢端肥大症患者会出现严重高血糖,进而导致糖尿病酮症酸中毒(DKA),这需要胰岛素治疗。垂体肿瘤很少导致全身性惊厥,除非肿瘤过大。我们在此报告两例病例,第一例是一名26岁女性,新发癫痫持续状态,有1年糖尿病(DM)病史并伴有DKA。检查时,她有肢端肥大症的临床特征。第二例是一名34岁女性,新发癫痫持续状态,有近期诊断的DM病史并伴有高血糖,以及巨人症特征。在这两例病例中,血清GH升高,随后胰岛素样生长因子1水平升高,头部CT显示垂体大腺瘤,从而确诊。临床病史、检查以及相关检查在肢端肥大症的识别中至关重要。肢端肥大症患者的预后取决于早期临床识别以及通过药物或手术治疗缩小肿瘤大小。

学习要点

癫痫持续状态和DKA等情况可能是肢端肥大症患者的临床表现。垂体腺瘤患者很少发生癫痫,通常是由于肿瘤对脑产生占位效应侵犯鞍上区域时才会发生。本文展示了在资源有限的情况下我们如何最好地处理肢端肥大症并发症。肢端肥大症中的高催乳素血症可能是由于大腺瘤的占位效应破坏了正常的多巴胺能对催乳素分泌的抑制作用,约25%分泌GH的腺瘤会共同分泌催乳素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bfa5/8185527/f66c3c091ffc/EDM20-0156fig1.jpg

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