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DOI 10.1186/s13023-024-03370-z

Медицинская библиотека · Orphanet journal of rare diseases · 2024

Improving genetic testing pathways for transthyretin amyloidosis in France: challenges and strategies.

Hebrard Bérénice, Babonneau Marie-Lise, Charron Philippe, Consolino Emilie, Dauriat Benjamin, Dupin-Deguine Delphine, Fargeaud Dominique, Farrugia Agnès, Giguet-Valard Anna-Gaëlle, Guijarro Damien, Inamo Jocelyn, Jeanneteau Julien, Mazzella Jean-Michaël, Michon Claire-Cécile, Millat Gilles, Mouquet Frédéric, Oghina Silvia, Pereon Yann, Poinsignon Vianney, Pompougnac Julie, Proukhnitzky Julie, Schaefer Elise, Sturtz Franck, Trosdorf Mathilde, Auguste Anne, Canali Giorgia, Combes Alexandre, Funalot Benoît, Damy Thibaud

СтатьяПоддержка исследования: вне правительства СШАПеревод пока не подготовлен

Аннотация

Transthyretin amyloidosis (ATTR) is a severe and rare disease characterized by the progressive deposition of misfolded transthyretin proteins, causing irreversible organ damage. Transthyretin amyloidosis can present as a hereditary ATTR or acquired wild-type ATTR form. Genetic testing is critical for determining a hereditary predisposition and subsequently initiating appropriate family screening. In France, strict regulations govern genetic testing that aim to protect patients and their families affected by hereditary diseases such as ATTR. However, challenges persist in establishing an effective genetic testing pathway. A multidisciplinary group of French experts convened to discuss the challenges associated with an ATTR genetic diagnosis and to propose improvement strategies. Key challenges include the lack of pathway standardization, communication gaps between healthcare professionals (HCPs) and patients, and difficulties in complying with regulatory requirements. Concerns about patient data safety and outsourced testing quality further complicate matters. Proposed strategies included the development of stakeholder mapping tools for HCPs and patients, educational programs to improve literacy on genetic testing regulations, increase disease awareness among medical geneticists and genetic counselors, and strengthening HCP-patient communication through educational materials. These initiatives aim to streamline the genetic testing pathway, enhance compliance with regulations, and ultimately provide optimal support for patients and families with ATTR.

Для цитирования: Hebrard Bérénice, Babonneau Marie-Lise, Charron Philippe, Consolino Emilie, Dauriat Benjamin, Dupin-Deguine Delphine, Fargeaud Dominique, Farrugia Agnès, Giguet-Valard Anna-Gaëlle, Guijarro Damien, Inamo Jocelyn, Jeanneteau Julien, Mazzella Jean-Michaël, Michon Claire-Cécile, Millat Gilles, Mouquet Frédéric, Oghina Silvia, Pereon Yann, Poinsignon Vianney, Pompougnac Julie, Proukhnitzky Julie, Schaefer Elise, Sturtz Franck, Trosdorf Mathilde, Auguste Anne, Canali Giorgia, Combes Alexandre, Funalot Benoît, Damy Thibaud. Improving genetic testing pathways for transthyretin amyloidosis in France: challenges and strategies.. Orphanet journal of rare diseases. 2024. DOI: 10.1186/s13023-024-03370-z.

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