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伴有 Werner 综合征的严重代谢紊乱:病例报告。

Severe metabolic disorders coexisting with Werner syndrome: a case report.

机构信息

Department of Endocrinology, The Second Hospital of Jilin University, Changchun, Jilin, China.

出版信息

Endocr J. 2021 Mar 28;68(3):261-267. doi: 10.1507/endocrj.EJ20-0448. Epub 2020 Oct 20.

DOI:10.1507/endocrj.EJ20-0448
PMID:33087645
Abstract

Werner syndrome, also called adult progeria, is a heritable autosomal recessive human disorder characterized by the premature onset of numerous age-related diseases including juvenile cataracts, dyslipidemia, diabetes mellitus (DM), osteoporosis, atherosclerosis, and cancer. Werner syndrome is a segmental progeroid syndrome whose presentation resembles accelerated aging. The most common causes of death for WS patients are atherosclerosis and cancer. A 40-year-old female presented with short stature, bird-like facies, canities with alopecia, scleroderma-like skin changes, and non-healing foot ulcers. The patient reported a history of delayed puberty, abortion, hypertriglyceridemia, and juvenile cataracts. A clinical diagnosis of WS was made and subsequently confirmed. We discovered two WRN gene mutations in the patient, Variant 1 was the most common WRN mutation, nonsense mutation (c.1105C>T:p.R369Ter) in exon 9, which caused a premature termination codon (PTC) at position 369. Variant 2 was a frameshift mutation (c.1134delA:p.E379KfsTer5) in exon 9, which caused a PTC at position 383 and has no published reports describing. Patients with WS can show a wide variety of clinical and biological manifestations in endocrine-metabolic systems (DM, thyroid dysfunction, and hyperlipidemia). Doctors must be cognizant of early manifestations of WS and treatment options.

摘要

Werner 综合征,也称成人早衰症,是一种遗传性常染色体隐性人类疾病,其特征是多种与年龄相关的疾病的过早发作,包括少年白内障、血脂异常、糖尿病(DM)、骨质疏松症、动脉粥样硬化和癌症。Werner 综合征是一种节段性早衰综合征,其表现类似于加速衰老。WS 患者最常见的死亡原因是动脉粥样硬化和癌症。一名 40 岁女性,表现为身材矮小、鸟样面容、白发伴脱发、硬皮病样皮肤改变和难以愈合的足部溃疡。患者报告有青春期延迟、流产、高甘油三酯血症和少年白内障的病史。临床诊断为 WS,并随后得到确认。我们在患者中发现了两个 WRN 基因突变,变体 1 是最常见的 WRN 突变,无义突变(c.1105C>T:p.R369Ter)在 9 号外显子中,导致 369 位的提前终止密码子(PTC)。变体 2 是 9 号外显子中的移码突变(c.1134delA:p.E379KfsTer5),导致 383 位的 PTC,且没有发表的报告描述过。WS 患者在内分泌代谢系统(DM、甲状腺功能障碍和高脂血症)中可以表现出多种多样的临床和生物学表现。医生必须意识到 WS 的早期表现和治疗选择。

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引用本文的文献

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Acta Med Litu. 2024;31(2):357-363. doi: 10.15388/Amed.2024.31.2.9. Epub 2024 Dec 4.
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WRN loss accelerates abnormal adipocyte metabolism in Werner syndrome.WRN缺失会加速沃纳综合征中异常的脂肪细胞代谢。
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