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一名患有难治性库欣病患者的多处骨折

Multiple Bone Fractures in a Patient With Difficult-to-Treat Cushing's Disease.

作者信息

Correia Sara, Ramalho Diogo, Rocha Gustavo, Oliveira Maria J

机构信息

Endocrinology, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia, PRT.

Endocrinology and Diabetes, Centro Hospitalar de Vila Nova de Gaia/Espinho, Porto, PRT.

出版信息

Cureus. 2022 Sep 21;14(9):e29401. doi: 10.7759/cureus.29401. eCollection 2022 Sep.

DOI:10.7759/cureus.29401
PMID:36304359
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9586192/
Abstract

Osteoporosis at a young age should prompt clinicians to search for secondary causes, namely endogenous Cushing's syndrome.We report a case of a 33-year-old male with a history of spontaneous fracture of the 12th thoracic vertebra and florid features of Cushing's syndrome. The physical exam evidenced moon face, facial plethora, muscle atrophy of the upper and lower limbs, and accumulation of abdominal fat. Bone mineral density revealed osteoporosis in the lumbar spine and in the femoral neck. Scintigraphy showed bone fractures in several costal arches, dorsal columns, and sternum. Hypercortisolism was confirmed by blood work. Serum cortisol, adrenocorticotropic hormone and corticotropin (ACTH), and 24-hour urine cortisol values were elevated. Imaging with MRI sellar region was normal and bilateral catheterization of inferior petrosal sinuses was positive. The patient underwent transsphenoidal pituitary surgery (TPS) and a lesion in the right side of the pituitary was identified and resected. Postoperatively, the patient did not meet the remission criteria and we decided to initiate treatment with ketoconazole alongside pituitary radiotherapy. After two years of surgery, the patient presented with recurrent bone fractures, height loss (25 cm), intense fatigue, and difficulty walking without assistance. Due to severe disease, we performed bilateral adrenalectomy, which was essential to control hypercortisolism and improve the patient's quality of life.

摘要

年轻时发生骨质疏松应促使临床医生寻找继发原因,即内源性库欣综合征。我们报告一例33岁男性病例,该患者有第12胸椎自发性骨折病史及典型的库欣综合征表现。体格检查发现满月脸、面部充血、上下肢肌肉萎缩以及腹部脂肪堆积。骨密度检查显示腰椎和股骨颈骨质疏松。骨闪烁显像显示多个肋弓、脊柱胸段和胸骨有骨折。血液检查证实存在皮质醇增多症。血清皮质醇、促肾上腺皮质激素和促肾上腺皮质激素释放激素(ACTH)以及24小时尿皮质醇值均升高。蝶鞍区MRI成像正常,双侧岩下窦插管检查呈阳性。患者接受了经蝶窦垂体手术(TPS),术中发现并切除了垂体右侧的一个病变。术后,患者未达到缓解标准,我们决定在垂体放疗的同时开始使用酮康唑治疗。手术两年后,患者出现复发性骨折、身高降低(25厘米)、极度疲劳以及无辅助行走困难。由于病情严重,我们实施了双侧肾上腺切除术,这对于控制皮质醇增多症和改善患者生活质量至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf70/9586192/e30b645f668a/cureus-0014-00000029401-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf70/9586192/6c744b8d97fa/cureus-0014-00000029401-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf70/9586192/e30b645f668a/cureus-0014-00000029401-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf70/9586192/6c744b8d97fa/cureus-0014-00000029401-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf70/9586192/e30b645f668a/cureus-0014-00000029401-i02.jpg

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