Position statement on the diagnosis and management of congenital pituitary deficiency in adults: the French National Diagnosis and Treatment Protocol (NDTP) - Institut de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition
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)

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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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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