Genomic Medicine Laboratory UILDM, IRCCS Fondazione Santa Lucia, Via Ardeatina 306-354, 00179 Rome, Italy.
Department of Biomedicine and Prevention, Tor Vergata University of Rome, Via Montpellier 1, 00133 Rome, Italy.
Int J Mol Sci. 2024 Oct 11;25(20):10949. doi: 10.3390/ijms252010949.
Rare diseases are heterogeneous diseases characterized by various symptoms and signs. Due to the low prevalence of such conditions (less than 1 in 2000 people), medical expertise is limited, knowledge is poor and patients' care provided by medical centers is inadequate. An accurate diagnosis is frequently challenging and ongoing research is also insufficient, thus complicating the understanding of the natural progression of the rarest disorders. This review aims at presenting the multimodal approach supported by the integration of multiple analyses and disciplines as a valuable solution to clarify complex genotype-phenotype correlations and promote an in-depth examination of rare disorders. Taking into account the literature from large-scale population studies and ongoing technological advancement, this review described some examples to show how a multi-skilled team can improve the complex diagnosis of rare diseases. In this regard, Facio-Scapulo-Humeral muscular Dystrophy (FSHD) represents a valuable example where a multimodal approach is essential for a more accurate and precise diagnosis, as well as for enhancing the management of patients and their families. Given their heterogeneity and complexity, rare diseases call for a distinctive multidisciplinary approach to enable diagnosis and clinical follow-up.
罕见病是具有多种症状和体征的异质性疾病。由于这些疾病的患病率较低(每 2000 人中有不到 1 人),医学专业知识有限,相关知识匮乏,医疗中心提供的患者护理也不足。准确诊断通常具有挑战性,且相关研究也不足,这使得人们难以理解最罕见疾病的自然进程。本综述旨在提出一种多模式方法,该方法支持多种分析和学科的整合,是阐明复杂基因型-表型相关性并促进深入研究罕见疾病的有价值的解决方案。本综述考虑了来自大规模人群研究的文献和正在进行的技术进步,描述了一些示例,以展示多技能团队如何能够改善罕见疾病的复杂诊断。在这方面,面肩肱型肌营养不良症(FSHD)是一个很好的例子,多模式方法对于更准确和精确的诊断以及增强患者及其家属的管理至关重要。鉴于其异质性和复杂性,罕见病需要独特的多学科方法来实现诊断和临床随访。