Evidence map›Paper›PMID 42227416›Full record

ReviewOrphanet journal of rare diseases2026

Maturity Onset Diabetes of the Young (MODY): French National Diagnosis and Care Protocol (PNDS, Protocole National de Diagnostic et de Soins).

Danièle Dubois-Laforgue, Bruno Donadille, Cécile Ciangura, Chloé Amouyal, Jean-Baptiste Arnoux, Pascal Barat, Sabine Baron, Jacques Beltrand, Elise Bismuth, Clara Bouché and 25 more

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In one paragraph

Review in Orphanet journal of rare diseases, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

35 authors.

Danièle Dubois-LaforgueAssistance Publique-Hôpitaux de Paris, Cochin Hospital, Department of Diabetology and Clinical Immunology, Paris-Cité University & INSERM U1016, Cochin Institute, Paris, France.
Bruno DonadilleAssistance Publique-Hôpitaux de Paris, Saint-Antoine Hospital, National Reference Center for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS), Endocrinology, Diabetology and Reproductive Endocrinology Department, Sorbonne University, European Reference Network for Rare Endocrine Conditions (Endo-ERN Assistance-Publique Consortium), Paris, France. bruno.donadille@aphp.fr.ORCID http://orcid.org/0000-0002-5507-4782
Cécile CianguraAssistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Diabetology Department, Sorbonne University, Paris, France.
Chloé AmouyalAssistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Diabetology Department, Sorbonne University, Paris, France.
Jean-Baptiste ArnouxAssistance Publique-Hôpitaux de Paris, Necker-Enfants Maladies Hospital, Reference Center for Inherited Metabolic Diseases, Paris-Cité University, G2M Network, Imagine Institute, Paris, France.
Pascal BaratUniv. Bordeaux, CHU Bordeaux, Paediatric Endocrinology and Diabetology Department, Bordeaux, France.
Sabine BaronNantes University, University Hospital, Paediatric Endocrinology Department, Nantes, France.
Jacques BeltrandAssistance Publique-Hôpitaux de Paris, Necker Enfants Malades Hospital, Paediatric Endocrinology, Gynecology and Diabetology Department, Reference Center for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS); European Reference Network for Rare Endocrine Conditions (Endo-ERN Assistance-Publique Consortium) Paris-Cité University & INSERM U106 Cochin Institute and U1163 Imagine Institute, Paris, France.
Elise BismuthAssistance Publique-Hôpitaux de Paris, Robert Debré Hospital, Pediatric Endocrinology and Diabetes Department, Reference Center for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS), Paris-Cité University, Paris, France.
Clara BouchéRothschild Foundation, Diabetology Department, Paris, France.
Jean-Claude CarelAssistance Publique-Hôpitaux de Paris, Robert Debré Hospital, Pediatric Endocrinology and Diabetes Department, Reference Center for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS), Paris-Cité University, Paris, France.
Hélène CavéAssistance Publique-Hôpitaux de Paris, Robert Debré Hospital, Molecular Genetics Department, Paris-Cité University, Paris, France.
Sophie Christin-MaitreAssistance Publique-Hôpitaux de Paris, Saint-Antoine Hospital, National Reference Center for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS), Endocrinology, Diabetology and Reproductive Endocrinology Department, Sorbonne University, European Reference Network for Rare Endocrine Conditions (Endo-ERN Assistance-Publique Consortium), Paris, France.
Delphine Collin-ChavagnacHospices Civils de Lyon HCL, Biochemistry and Molecular Biology Department, Lyon, and CarMeN Laboratory, Claude Bernard Lyon 1 University, Inserm, INRAE, Pierre-Bénite, France.
Christiane DamgéPatient Association Fédération Française des Diabétiques, Paris, France.
Brigitte DelemerReims University, Reims University Hospital, Endocrinology-Diabetology Department, Reims, France.
Pierre GourdyToulouse University, Toulouse University Hospital, Endocrinology-Diabetology-Nutrition Department and INSERM, I2MC UMR1297, Toulouse, France.
Sophie JacqueminetAssistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Diabetology Department, Sorbonne University, Paris, France.
Sonja JanmaatAssistance Publique-Hôpitaux de Paris, Saint-Antoine Hospital, National Reference Center for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS), Endocrinology, Diabetology and Reproductive Endocrinology Department, Sorbonne University, European Reference Network for Rare Endocrine Conditions (Endo-ERN Assistance-Publique Consortium), Paris, France.
Marc de KerdanetRennes University Hospitals, South Hospital, Paediatric Endocrinology Department, Rennes, France.
Laurence KesslerStrasbourg University, Strasbourg Civil Hospital, Endocrinology-Diabetology Department, Strasbourg, France.
Dominique LanginToulouse University, Toulouse University Hospital, Genetics Department, Toulouse, France.
Jean-Charles NaultAssistance Publique-Hôpitaux de Paris, Avicenne Hospital, Hepatology Department, Paris 13 University, Bobigny, France.
Jacky NizardAssistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Obstetrics Department, Sorbonne University, Paris, France.
Michel PolakAssistance Publique-Hôpitaux de Paris, Necker Enfants Malades Hospital, Paediatric Endocrinology, Gynecology and Diabetology Department, Reference Center for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS); European Reference Network for Rare Endocrine Conditions (Endo-ERN Assistance-Publique Consortium) Paris-Cité University & INSERM U106 Cochin Institute and U1163 Imagine Institute, Paris, France.
Rachel ReynaudAssistance Publique-Hôpitaux de Marseille, Timone Children Hospital, Pediatric multidisciplinary department, Marseille, France.
Cécile Saint-MartinAssistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Medical Genetics Department, Sorbonne University, Paris, France.
Igor TauveronClermont-Ferrand University Hospital, Endocrinology Department, Clermont Auvergne University, Clermont-Ferrand, France.
René ValéroAix Marseille University, Assistance Publique Hôpitaux de Marseille, INSERM-INRAE-C2VN, University Hospital La Conception, Department of Nutrition, Metabolic Diseases and Endocrinology, Marseille, France.
Anne VambergueLille University, Claude-Huriez University Hospital, Department of Diabetes and Nutrition, EGID Center, Lille, France.
Marie-Christine VantyghemUniversity Hospital of Lille, Department of Diabetes and Nutrition, EGID Center, University of Lille, Lille, France.
Camille VatierAssistance Publique-Hôpitaux de Paris, Saint-Antoine Hospital, National Reference Center for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS), Endocrinology, Diabetology and Reproductive Endocrinology Department, Sorbonne University, European Reference Network for Rare Endocrine Conditions (Endo-ERN Assistance-Publique Consortium), Paris, France.
Marc NicolinoLyon University, CHU de Lyon HCL-GH Est, Mère Enfant Hospital, Endocrinology Department, Lyon, France.
Christine Bellanné-ChantelotAssistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Medical Genetics Department, Sorbonne University, Paris, France.
Corinne VigourouxAssistance Publique-Hôpitaux de Paris, Saint-Antoine Hospital, National Reference Center for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS), Endocrinology, Diabetology and Reproductive Endocrinology Department, Sorbonne University, European Reference Network for Rare Endocrine Conditions (Endo-ERN Assistance-Publique Consortium), Paris, France.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

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.

Indexed as

Diabetes Mellitus, Type 2FranceGlucokinaseHepatocyte Nuclear Factor 1-alphaHepatocyte Nuclear Factor 4HumansGlucokinaseHepatocyte Nuclear Factor 1-alphaHepatocyte Nuclear Factor 4HNF1A protein, humanDiabetesGCKGlucokinaseHNF1AHNF4AMaturity onset diabetes of the YoungMODY

Identifiers

PMID42227416

What Socratic holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.