ISSN 1662-4009 (online)

ESPE Yearbook of Paediatric Endocrinology (2020) 17 8.17 | DOI: 10.1530/ey.17.8.17


To read the full abstract: J Clin Endocrinol Metab. 2020; 105(1): dgz055. PMID: 31613957.

Mineralocorticoid (MC) replacement therapy along with glucocorticoid (GC) replacement is crucial to avoid life-threating adrenal crises in Addison´s disease (AD) and in classical CAH (1–3). MC is administered with the aim of achieving plasma renin concentration (PRC) within the upper limit of the reference range. Taking into account the complex regulation of PRC, this study explored the relationship between MC dose regimens and clinical and biochemical variables in clinical practice to determine whether those variables can be used to guide titration of MC dosing.

This retrospective observational study used data from the International CAH Registry (I-CAH) collected from 1982 to 2018, and data from local adrenal patient databases. After exclusions, data were available on 735 visits in 243 patients (204 SW-CAH, 39 AD). Seven variables were included in the multivariate models: serum sodium, serum potassium, mean arterial blood pressure (MAP), PRC, MC dose, age and body mass index (BMI). PRC levels were categorized as low, normal, or high according to the local reference ranges.

In univariate analyses, a positive relationship was observed between MC dose and PRC in adults and children. In children, but not in adults, a positive relationship was also observed between MC dose and BP. However, in multiple regression models, sodium was the only factor that predicted PRC in adults. In summary, these findings suggest that MC dose titration should be based not only on PRC normalization, but also on clinical data, such as BP and electrolyte concentration.

References:

1. Charmandari E, Nicolaides NC, Chrousos GP. Adrenal insufficiency. Lancet. 2014; 383(9935): 2152–2167.

2. Bancos I, Hahner S, Tomlinson J, Arlt W. Diagnosis and management of adrenal insufficiency. Lancet Diabetes Endocrinol. 2015; 3(3): 216–226.

3. El-Maouche D, Arlt W, Merke DP. Congenital adrenal hyperplasia. Lancet. 2017; 390(10108): 2194–2210.

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