PMID- 37564007 OWN - NLM STAT- Publisher LR - 20230811 IS - 1365-2265 (Electronic) IS - 0300-0664 (Linking) DP - 2023 Aug 11 TI - Mineralocorticoid replacement therapy in salt-wasting congenital adrenal hyperplasia. LID - 10.1111/cen.14959 [doi] AB - Patients with salt-wasting congenital adrenal hyperplasia (SW-CAH) usually show pronounced impairment of aldosterone secretion and, therefore, also require mineralocorticoid replacement. While a lot of research and discussion focusses on the glucocorticoid therapy in SW-CAH to replace the missing cortisol and to control adrenal androgen excess, very little research is dealing with mineralocorticoid replacement. However, recent data demonstrated an increased cardiovascular risk in adult CAH patients urging to reflect also on the current mineralocorticoid replacement therapy. In this review, we explain the role and function of the mineralocorticoid receptor, its ligands and inhibitors and its relevance for the therapy of patients with SW-CAH. We performed an extensive literature search and present data on mineralocorticoid therapy in SW-CAH patients as well as clinical advice how to monitor and optimise mineralocorticoid replacement therapy. CI - (c) 2023 The Authors. Clinical Endocrinology published by John Wiley & Sons Ltd. FAU - Lang, Katharina AU - Lang K AD - Endocrinology in Charlottenburg, Berlin, Germany. FAU - Quinkler, Marcus AU - Quinkler M AUID- ORCID: 0000-0003-4028-1671 AD - Endocrinology in Charlottenburg, Berlin, Germany. AD - Department of Endocrinology and Metabolism, Charite-Universitatsmedizin, Campus Mitte, Berlin, Germany. FAU - Kienitz, Tina AU - Kienitz T AD - Endocrinology in Charlottenburg, Berlin, Germany. LA - eng PT - Journal Article PT - Review DEP - 20230811 PL - England TA - Clin Endocrinol (Oxf) JT - Clinical endocrinology JID - 0346653 SB - IM OTO - NOTNLM OT - 17-hydroxy-progesterone OT - adrenal insufficiency OT - androstenedione OT - fludrocortisone OT - hydrocortisone EDAT- 2023/08/11 06:43 MHDA- 2023/08/11 06:43 CRDT- 2023/08/11 03:31 PHST- 2023/07/31 00:00 [revised] PHST- 2023/05/21 00:00 [received] PHST- 2023/08/03 00:00 [accepted] PHST- 2023/08/11 06:43 [medline] PHST- 2023/08/11 06:43 [pubmed] PHST- 2023/08/11 03:31 [entrez] AID - 10.1111/cen.14959 [doi] PST - aheadofprint SO - Clin Endocrinol (Oxf). 2023 Aug 11. doi: 10.1111/cen.14959.