Endocrine Abstracts

In patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, life-long glucocorticoid (GC) treatment is often required to replace cortisol deficiency and to avoid the ACTH-dependent androgen levels increase. However, in these patients, the multiple daily doses required with conventional GCs can cause cortisol overexposure, leading to an increased risk of metabolic syndrome (MS), an impaired quality of life (QoL), and poor treatment compliance (TC). The current study aimed at investigating the impact of the switch from twice or thrice daily conventional GCs to once daily ‘dual release’ hydrocortisone (DR-HC) on metabolic and hormonal profile, QoL, depression status (DS), and TC in a cohort of patients with CAH. Twenty-three CAH patients (16F, 7M, 20–38 years), treated with HC (10–40 mg/day) or prednisone (6.25–12.5 mg/day) for at least 12 months, switched to DR-HC (10–40 mg/day) entered the study and were evaluated before and after 6–12 months of DR-HC. Metabolic and hormonal parameters were measured using routine assays and the MS was evaluated according with IDF criteria. QoL, DS, and TC were assessed using AddiQoL Questionnaire, Beck Depression Inventory II, and Morisky 8-items medication Adherence Questionnaire respectively. The change in metabolic and hormonal parameters in the same cohort of patients along the year between baseline and 12 months before the switch, while patients stably performed conventional GCs, was used as control. At 6-month follow-up, fasting plasma glucose (P=0.003) was significantly reduced, whereas at 12-month follow-up HDL (P=0.000) and LDL-cholesterol levels (P=0.024) were significantly improved as compared with baseline. A clear diagnosis of MS was performed in one patient at baseline, but this patient displayed no criteria for this diagnosis after 6 and 12 months. No significant change in morning plasma ACTH, UFC, and serum aldosterone, 17-OH progesterone, testosterone, DHEA-S, and androstenedione levels were observed and no clinical worsening of symptoms and signs related to hyperandrogenism were reported. Excluding from the analysis the four patients treated with prednisone at baseline, a significant increase in morning serum cortisol levels was registered after 6 months (P=0.016) but it was not confirmed after 12 months. Despite the unchanged fludrocortisone doses, both in the entire cohort (P=0.002) and in the subgroup of patients with salt wasting form (P=0.005) a significant decrease in renin levels was reported at 6-month follow-up, but it was not confirmed at 12-month follow-up. As control, no significant difference was observed in metabolic and hormonal parameters in the same cohort of patients between baseline and 12 months before the switch. Additionally, DS improved both after 6 months (P=0.04) and 12 months (P=0.07) whereas TC significantly, progressively, improved after 6 months (P=0.009) and after 12 months (P=0.000). In conclusion, the switch from conventional GCs to DR-HC significantly improves MS, DS, and TC, maintaining an optimal hormone control in patients with CAH due to 21-hydroxylase deficiency.