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肾上腺切除术促成了ALECT2淀粉样变性的早期诊断。

Adrenalectomy Leading to Early Diagnosis of ALECT2 Amyloidosis.

作者信息

Hu Andrew C, Passman Jesse E, Baloch Zubair, Wachtel Heather

机构信息

Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.

Department of Surgery, University of Pennsylvania Health System, Philadelphia, PA, USA.

出版信息

Sage Open Pathol. 2025 Apr 22;18:30502098251331780. doi: 10.1177/30502098251331780. eCollection 2025 Jan-Dec.

Abstract

The diagnosis of amyloidosis requires high clinical suspicion and is often made only after significant clinical symptoms arise. Here, we present a case of amyloidosis diagnosed in a patient with an enlarging incidental adrenal cyst. Imaging and biochemical evaluation suggested that the cyst was benign and non-functional. Adrenalectomy was performed to treat symptoms of mass effect, and surgical pathology led to the diagnosis of leukocyte cell-derived chemotaxin 2 (ALECT2) associated adrenal amyloidosis. Patients with ALECT2 amyloidosis may have subclinical involvement of the adrenal glands, subcutaneous fat, and other tissues before developing the more common manifestation of renal amyloidosis. Early identification of patients with ALECT2 amyloidosis can assist in prevention of renal morbidity. Prevention of chronic kidney disease (CKD) should be among the main goals of managing patients with ALECT2 amyloidosis.

摘要

淀粉样变性的诊断需要高度的临床怀疑,通常只有在出现明显临床症状后才能确诊。在此,我们报告一例在偶然发现肾上腺囊肿增大的患者中诊断出的淀粉样变性病例。影像学和生化评估表明该囊肿为良性且无功能。为治疗占位效应症状进行了肾上腺切除术,手术病理诊断为白细胞衍生趋化因子2(ALECT2)相关的肾上腺淀粉样变性。ALECT2淀粉样变性患者在出现更常见的肾淀粉样变性表现之前,可能已有肾上腺、皮下脂肪和其他组织的亚临床受累。早期识别ALECT2淀粉样变性患者有助于预防肾脏疾病。预防慢性肾脏病(CKD)应是管理ALECT2淀粉样变性患者的主要目标之一。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/49d8/12161887/b2b9c7b48ac9/10.1177_30502098251331780-fig1.jpg

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