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患有遗传性代谢紊乱疾病的女性所面临的多方面挑战。

The multifaceted challenges faced by women in the field of inherited metabolic disorders.

作者信息

Lenzini Livia, Bianconi Sara, Gugelmo Giorgia, Gragnaniello Vincenza, Messerotti Benvenuti Simone, Fadini Gian Paolo, Vitturi Nicola

机构信息

Division of Metabolic Diseases, Department of Medicine, Padova University Hospital, University of Padova, Padua, Italy.

Hospital Psychology Unit, Padua University Hospital, Padua, Italy.

出版信息

Orphanet J Rare Dis. 2025 Mar 5;20(1):104. doi: 10.1186/s13023-025-03604-8.

Abstract

Inherited metabolic disorders (IMDs) are heritable conditions that affect up to 125:100,000 people worldwide. In addition to severe disabling forms that require continuous and costly assistance in both pediatric and adult patients, some IMDs can have mild forms, with the first clinical signs starting in adolescence or very late in adulthood. In the complex field of IMDs, featuring multifaceted challenges that span from scientific discoveries to patient care, women play a central role in contributing to clinical practice, research, patient advocacy, care, and education. In this narrative review, we focused on the involvement of women in the field of IMDs, highlighting not only their extensive contributions but also the undervaluation of the psychological and emotional tolls paid by women dealing with these diseases. Moreover, from a female-centered perspective, we explored the condition of an adult patient with an IMD to highlight the importance of changing the current approach to the clinical management of these diseases toward a more gender-focused approach.

摘要

遗传性代谢疾病(IMDs)是一种遗传性疾病,全球每10万人中就有多达125人受其影响。除了严重致残形式,这类疾病在儿童和成年患者中都需要持续且昂贵的医疗支持,还有一些IMDs表现为轻症形式,最初的临床症状始于青春期或成年晚期。在IMDs这个复杂领域,面临着从科学发现到患者护理等多方面的挑战,女性在临床实践、研究、患者宣传、护理和教育等方面发挥着核心作用。在这篇叙述性综述中,我们聚焦于女性在IMDs领域的参与情况,不仅强调她们的广泛贡献,还指出女性在应对这些疾病时所付出的心理和情感代价被低估。此外,从以女性为中心的视角出发,我们探讨了一名成年IMD患者的情况,以强调改变当前这些疾病临床管理方法、转向更注重性别的方法的重要性。

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