Pediatric Gastroenterology and Hepatology, University Medical Center Groningen, University of Groningen, the Netherlands.
Pediatric Hepatology & Pediatric Liver Transplant Department, Centre de Référence de L'Atrésie des Voies Biliaires et des Cholestases Génétiques (AVB-CG), Filière de Santé des Maladies Rares Du Foie de L'enfant et de L'adulte (FILFOIE), Assistance Publique-Hôpitaux de Paris, Faculté de Médecine Paris-Saclay, CHU Bicêtre, Le Kremlin-Bicêtre, France.
Eur J Med Genet. 2021 Jul;64(7):104245. doi: 10.1016/j.ejmg.2021.104245. Epub 2021 May 13.
While rare diseases collectively affect ~300 million people worldwide, the prevalence of each disease concerns a relatively small number of patients. Usually, only limited data with regard to natural history are available. Multicenter initiatives are needed to aggregate data and answer clinically relevant research questions. In 2017, we launched the NAtural course and Prognosis of PFIC and Effect of biliary Diversion (NAPPED) consortium. In three years, NAPPED created a global network focused on rare genetic liver diseases in the Progressive Familial Intrahepatic Cholestasis (PFIC) spectrum. During these years, we have learned important lessons which we feel should be taken into account when initiating and leading a global consortium. First, it is essential to 'keep it simple' from the start. Research questions, case report forms (CRFs) and data acquisition should be limited and clear to stay focused and keep the workload low for new participants. Secondly, early rewards and research output are needed to keep momentum and motivation. Quick output can only follow a clean and simple design. Thirdly, the leading team should be in touch and accessible. Ideally, an involved PhD-candidate is appointed as primary contact person. Lastly, be inclusive and actively involve all participants the consortium's course. Global consortia are critical for personalized medicine in rare diseases. Also, they are essential for setting up trials to investigate generic drugs and personalized therapies. We hope to herewith stimulate others that are starting (or are planning to start) a global consortium, ultimately to help improve the care for patients with a rare disease.
虽然罕见病在全球范围内共影响约 3 亿人,但每种疾病的患病率都只涉及相对较少的患者。通常,只有关于自然病史的有限数据可用。需要多中心的倡议来汇总数据并回答临床相关的研究问题。2017 年,我们启动了先天性肝内胆汁淤积症(PFIC)和胆道分流术效果自然病程和预后(NAPPED)联盟。在三年的时间里,NAPPED 建立了一个全球性的网络,专注于 PFIC 谱中罕见的遗传性肝脏疾病。在这几年中,我们吸取了一些重要的经验教训,我们认为在发起和领导一个全球性的联盟时应该考虑这些经验教训。首先,从一开始就必须“保持简单”。研究问题、病例报告表(CRF)和数据采集应该是有限的和明确的,以保持专注并降低新参与者的工作量。其次,需要早期的回报和研究成果来保持动力和积极性。快速的产出只能遵循一个简洁明了的设计。第三,领导团队应该保持联系和可访问。理想情况下,应任命一名参与的博士研究生作为主要联系人。最后,要有包容性,积极参与联盟的整个过程。全球联盟对于罕见病的个体化医学至关重要。此外,它们对于开展调查通用药物和个体化治疗的试验也是必不可少的。我们希望借此鼓励其他正在发起(或计划发起)全球联盟的人,最终帮助改善对罕见病患者的护理。