Results of the Prolonged Use of Subcutaneous Continuous Infusion of Hydrocortisone in a Man with Congenital Adrenal Hyperplasia

Abstract

This is a case report study of a young man with Congenital Adrenal Hyperplasia (CAH) who has been treated during 2 years by a subcutaneous continuous infusion hydrocortisone (SCIH) to optimize his treatment. Hydrocortisone was delivered via an insulin infusion device. We also studied the evolution of testicular adrenal rest tumors (TARTs) and the quality of life through SF36 survey. Four rates were determined, with a total of 47 mg per day. Biochemical parameters were normalized at 2 months. The SF36 questionnaire showed a progress of well-being. The weight decreased to 106 kg, that is, −5 kg (height: 1.71 m). Unfortunatly, there was no change of the TARTs. Two episodes of dermohypodermitis, with abscess at the infusion site, were observed. This case demonstrates the feasibility of prolonged SCIH therapy in patients with CAH, reporting positive effects on quality of life and on BMI.

Glucocorticoid Regimens in the Treatment of Congenital Adrenal Hyperplasia: A Systematic Review and Meta-Analysis

“…Five of the 23 studies examined the influence of glucocorticoid regimens on quality of life [11, 12, 18, 20, 30]. Mallappa et al [11] found no difference between subjects’ baseline glucocorticoid regimen and MR-HC in terms of the 36-item short-form health survey (SF-36), health-related quality of life in Addison disease, and global fatigue index scores.…”

“…In contrast, SF-36 scores were significantly lower in individuals treated with prednisolone or dexamethasone compared with those in the CaHASE cohort treated with hydrocortisone [20]. CSHI was associated with improved SF-36 and global fatigue index scores in two studies [12, 30].…”

Long‐term use of continuous subcutaneous hydrocortisone infusion therapy in patients with congenital adrenal hyperplasia

“…Previous studies evaluating CSHI therapy in CAH and Addison's disease have been restricted to case reports or to clinical trials of short duration . Merza et al investigated the effect of CSHI therapy on disease biomarkers for 24 hours and demonstrated improvements in 17‐OHP levels in two patients with CAH and ACTH (morning) levels in two Addison's disease patients.…”

“…After 18 months, patients continued to report improved HRQoL as the main reason for wishing to continue with CSHI therapy. Overall, the significant improvements in HRQoL associated with CSHI therapy seen short term were maintained long term.Previous studies evaluating CSHI therapy in CAH and Addison's disease have been restricted to case reports or to clinical trials of short duration [32][33][34][35][36]. Merza et al34 investigated the effect of CSHI therapy on disease biomarkers for 24 hours and demonstrated improvements in 17-OHP levels in two patients with CAH and ACTH (morning) levels in two Addison's disease patients.…”

Novel treatments for congenital adrenal hyperplasia

“…Continuous intravenous or subcutaneous HC infusion via a programmable pump more closely replicates the normal circadian rhythm of cortisol compared to conventional therapy in patients with adrenal insufficiency [48][49][50][51][52][53][54]. In 21OHD, continuous HC infusion lowers morning ACTH and 17OHP levels to near normal [48].…”