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2020.6 年 Werner 综合征管理指南。6. 与 Werner 综合征相关的皮肤溃疡:预防及非手术和手术治疗。

Management guideline for Werner syndrome 2020. 6. Skin ulcers associated with Werner syndrome: Prevention and non-surgical and surgical treatment.

机构信息

Department of Plastic, Reconstructive, and Aesthetic Surgery, Chiba University Graduate School of Medicine, Chiba, Japan.

Department of Diabetes, Metabolism and Endocrinology, School of Medicine, International University of Health and Welfare, Chiba, Japan.

出版信息

Geriatr Gerontol Int. 2021 Feb;21(2):153-159. doi: 10.1111/ggi.14096. Epub 2020 Nov 22.

Abstract

AIM

To provide guidelines on the diagnosis, treatment, and prevention of skin ulcers in Werner syndrome.

METHODS

This article was based on literature from 1996, when WRN was identified as a gene responsible for Werner syndrome, and we evaluated several authentic clinical cases of genetically diagnosed patients. There were 63 patients with Werner syndrome in the Japanese reports retrieved from Medical Online between January 1996 and December 2017. There were 56 patients with Werner syndrome in English reports written by Japanese authors and retrieved from PubMed during the same period.

RESULTS

Records on skin ulcers were found in 27 (43%) out of 63 patients and 22 (40%) out of 56 patients from the Japanese and English reports, respectively. The reported ulcers were often located at the distal one-third of the lower legs. There were 8 patients with callosities in the foot in the Japanese reports and 9 patients in the English reports. A skin ulcer in Werner syndrome is generally intractable. Weight-bearing ulcers or callosity should be critically assessed in surgical procedures because they have effects on patient pain and gait. By adopting a recently advanced technique to facilitate wound healing, the cases of ulcers that were difficult to treat and those requiring major operations can be closed with minimally invasive surgery.

CONCLUSIONS

Skin ulcers in Werner syndrome are refractory, and they lead to reduced quality of life of patients. A callosity in Werner syndrome is an important therapeutic target for the prevention of ulcers. Geriatr Gerontol Int 2021; 21: 153-159.

摘要

目的

为 Werner 综合征皮肤溃疡的诊断、治疗和预防提供指导。

方法

本文基于 1996 年 WRN 被确定为 Werner 综合征致病基因时的文献,同时评估了几个经基因诊断的患者的真实临床病例。从 1996 年 1 月至 2017 年 12 月期间在 Medical Online 上检索到的日本报告中,有 63 例 Werner 综合征患者,从同期 PubMed 上检索到的由日本作者撰写的英文报告中,有 56 例 Werner 综合征患者。

结果

在这两份报告中,分别有 27 例(43%)和 22 例(40%)患者记录到皮肤溃疡。报告中的溃疡常位于小腿下段的三分之一处。日本报告中有 8 例足部胼胝,英文报告中有 9 例。Werner 综合征的皮肤溃疡通常难以治愈。在手术中,负重性溃疡或胼胝应进行严格评估,因为它们会影响患者的疼痛和步态。采用最近先进的促进伤口愈合技术,可使难以治疗和需要大手术的溃疡病例通过微创手术闭合。

结论

Werner 综合征的皮肤溃疡难以治愈,会降低患者的生活质量。Werner 综合征的胼胝是预防溃疡的重要治疗靶点。老年医学与老年病学杂志 2021;21:153-159。

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