Maturity Onset Diabetes of the Young (MODY): French National Diagnosis and Care Protocol (PNDS, Protocole National de Diagnostic et de Soins)
Year: 2026
Type: article
Abstract: MODY (Maturity-Onset Diabetes of the Young) is characterized by autosomal dominant mode of inheritance, early onset of diabetes in the absence of autoimmunity directed to pancreatic β-cells, impaired insulin secretory capacity, however, maintained over time, and extra-pancreatic manifestations in some patients. Its prevalence has been estimated 0.6% to 6.5% of all diabetes in Europe and the USA. Pathogenic variants in the genes encoding glucokinase or transcription factors HNF1A or HNF4A are responsible for the majority of cases of monogenic forms of diabetes referred to as MODY. The objective of the French National Diagnosis and Care Protocol (PNDS, Protocole National de Diagnostic et de Soins) dedicated to GCK-MODY (formerly MODY2), HNF1A-MODY (MODY3), and HNF4A-MODY (MODY1) is to provide to health professionals a guide for optimal management and care of patients, based on a critical literature review and multidisciplinary expert consensus. The PNDS, written by members of the French National Reference Center for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS), is available on the French Health Authority website (in French). Thorough analysis of personal and family history, clinical examination and biochemical testing are key to raise the diagnosis, which has to be confirmed by molecular analysis. The attending physician, in conjunction with the national care network, will ensure that the patient receives optimal care through regular follow-up and screening. Overall, the management of patients with MODY requires the collaboration of several health care providers.
Authors: Danièle Dubois-Laforgue, Bruno Donadille, Cécile Ciangura, Chloé Amouyal, Jean-Baptiste Arnoux, Pascal Barat, Sabine Baron, Jacques Beltrand, Elise Bismuth, Clara Bouché, Jean-Claude Carel, Hélène Cavé, Sophie Christin-Maitre, Delphine Collin-Chavagnac, Christiane Damgé, Brigitte Delemer, Pierre Gourdy, Sophie Jacqueminet, Sonja Janmaat, Marc de Kerdanet, Laurence Kessler, Dominique Langin, Jean-Charles Nault, Jacky Nizard, Michel Polak, Rachel Reynaud, Cécile Saint-Martin, Igor Tauveron, René Valéro, Anne Vambergue, Marie-Christine Vantyghem, Camille Vatier, Marc Nicolino, Christine Bellanné-Chantelot, Corinne Vigouroux
Institutions: Inserm, Université Paris Cité, Hôpital Cochin, Assistance Publique – Hôpitaux de Paris, Institut Cochin, Sorbonne Université, Hôpital Saint-Antoine, Pitié-Salpêtrière Hospital, Hôpital Necker-Enfants Malades, Institut des Maladies Génétiques Imagine, Centre Hospitalier Universitaire de Bordeaux, Centre Hospitalier Universitaire de Nantes, Nantes Université, Institut Necker Enfants Malades, Hôpital Robert-Debré, Fondation de Rothschild, Maladies génétiques d’expression pédiatrique, Lyon 1 Université, Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement, Hospices Civils de Lyon, Laboratoire de recherche en cardiovasculaire, métabolisme, diabétologie et nutrition, Fédération Française des Diabétiques, Centre Hospitalier Universitaire de Reims, Centre Hospitalier Universitaire de Toulouse, Institut des Maladies Métaboliques et Cardiovasculaires, Université de Toulouse, Centre de Recherche Saint-Antoine, Fondation pour l’innovation en Cadiométabolisme et Nutrition, Centre Hospitalier Universitaire de Rennes, Hôpital Civil, Strasbourg, Université Sorbonne Paris Nord, Hôpital Avicenne, Assistance Publique Hôpitaux de Marseille, Hôpital de la Timone, Université Clermont Auvergne, Centre Hospitalier Universitaire de Clermont-Ferrand, Aix-Marseille Université, Hôpital de la Conception, Université de Lille, Hôpital Claude Huriez, European Genomic Institute for Diabetes
Language: English
Subfield: Surgery
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,990
OpenAlex ID: W7144352575