FAMILIAL HYPERALDOSTERONISM: ABOUT A REVEALING SIBLING OF TWO AND SYSTEMATIC REVIEW

Introduction:Bilateral primary aldosteronism may be caused by genetic forms, including familial hyperaldosteronism (FH) types I to IV. The management of this particular condition refers to medical treatment in spite of surgical procedures. In this article, we report on the family case of a sibling through 2 index patients followed in our department.

Case Report: 59-year-old patient followed in our training for a secondary hypertension due to a confirmed left conn adrenal adenoma family history of a similar case in the sister with confirmed adrenal Conn adenoma causing hypertension diagnosed in the early forties in all the siblings and some other family members with a history of cerebral strokes. Our department postponed the surgical management planned for the sister in favour of a medical approach, which will be decided on the basis of the response to dexamethasone treatment in our two patients, in the absence of possibilities for genetic typing tests.

Discussion and Conclusion: There are 4 types of familial hyperaldosteronism, whose characteristics and genotype/phenotype correlation are very diverse. Type 1, as we suspected in our family, is characterised by a prevalence of stroke and a satisfactory response to corticosteroid therapy, particularly dexamethasone, as both a diagnostic and therapeutic test. Management has long depended on the attitudes of the medical teams, but the scientific societies of endocrinology have now issued well-coded diagrams and guidelines for screening and treatment of these cases, increasingly favouring a medical approach rather than the invasive, and often bilateral, surgery previously practised.