Article Dans Une Revue Annales d'Endocrinologie = Annals of Endocrinology Année : 2024

Position statement on the diagnosis and management of congenital pituitary deficiency in adults: the French National Diagnosis and Treatment Protocol (NDTP)

1 Service de pédiatrie multidisciplinaire [Hôpital de la Timone Enfants - APHM]
2 LA CONCEPTION - Hôpital de la Conception [CHU - APHM]
3 CHU Pitié-Salpêtrière [AP-HP]
4 SU - Sorbonne Université
5 IHU ICAN - Institut de Cardiométabolisme et Nutrition = Institute of Cardiometabolism and Nutrition [CHU Pitié Salpêtrière]
6 Hôpital Européen [Fondation Ambroise Paré - Marseille]
7 MMG - Marseille medical genetics - Centre de génétique médicale de Marseille
8 Service de Génétique Moléculaire Pharmacogénétique et Hormonologie [CHU Bicêtre]
9 Université Paris-Saclay
10 MITOVASC - MitoVasc - Physiopathologie Cardiovasculaire et Mitochondriale
11 Endo-ERN - European Reference Network on rare endocrine conditions
12 CHU Angers - Centre Hospitalier Universitaire d'Angers
13 AP-HP Hôpital universitaire Robert-Debré [Paris]
14 Service d’endocrinologie et nutrition [AP-HP Ambroise-Paré]
15 UVSQ - Université de Versailles Saint-Quentin-en-Yvelines
16 CHU Tenon [AP-HP]
17 CHU Saint-Antoine [AP-HP]
18 Centre de Référence des Maladies Endocriniennes Rares de la Croissance [APHP Robert Debré]
19 UPCité - Université Paris Cité
20 Service Endocrinologie - Diabétologie [CHU Caen]
21 LILLE - Endocrino - Service Endocrinologie, diabétologie, maladies métaboliques et nutrition
22 NeuroDiderot (UMR_S_1141 / U1141) - Maladies neurodéveloppementales et neurovasculaires
23 MarMaRa - Institut Marseille Maladies Rares
24 Service d'endocrinologie, diabète, maladies métaboliques [Hôpital de la Conception - APHM]
Nathalie Chabbert-Buffet

Résumé

Pituitary deficiency, or hypopituitarism, is a rare chronic disease. It is defined by insufficient synthesis of one or more pituitary hormones (growth hormone, TSH, ACTH, LH-FSH, prolactin), whether or not associated with arginine vasopressin deficiency (formerly known as diabetes insipidus). In adult patients, it is usually acquired (notably during childhood), but can also be congenital, due to abnormal pituitary development. The present study focuses on congenital pituitary deficiency in adults, from diagnosis to follow-up, including special situations such as pregnancy or the elderly. The clinical presentation is highly variable, ranging from isolated deficit to multiple deficits, which may be part of a syndromic form or not. Diagnosis is based on a combination of clinical, biological (assessment of all hormonal axes), radiological (brain and hypothalamic-pituitary MRI) and genetic factors. Treatment consists in hormonal replacement therapy, adapted according to the period of life and the deficits, which may be progressive. Comorbidities, risk of complications and acute decompensation, and the impact on fertility and quality of life all require adaptative multidisciplinary care and long-term monitoring.

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Dates et versions

hal-04689015 , version 1 (05-09-2024)

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Sarah Castets, Frédérique Albarel, Anne Bachelot, Gilles Brun, Jérôme Bouligand, et al.. Position statement on the diagnosis and management of congenital pituitary deficiency in adults: the French National Diagnosis and Treatment Protocol (NDTP). Annales d'Endocrinologie = Annals of Endocrinology, 2024, Annales d'Endocrinologie = Annals of Endocrinology, 85 (4), pp.327-339. ⟨10.1016/j.ando.2024.03.001⟩. ⟨hal-04689015⟩
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