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

Clinical Endocrinology
Ashwini MallappaDeborah P Merke

Abstract

In a phase 2 short-term (6 months) study of patients with congenital adrenal hyperplasia (CAH), continuous subcutaneous hydrocortisone infusion (CSHI) was found to be a safe, effective and well-tolerated method of replacing cortisol with improved disease and patient-related outcomes. To evaluate the safety and efficacy of long-term CSHI. Single-centre, open-label, phase 2 extension study. Five adults with classic CAH. Biomarkers of disease control, metabolic indices and health-related quality-of-life (HRQoL) estimates. Six of eight patients chose to continue on long-term CSHI therapy. Compared to baseline, eighteen months of CSHI resulted in decreased (P = 0.043) 0700-hour ACTH, 17-hydroxyprogesterone, androstenedione and progesterone; increased whole-body lean mass (P = 0.024); and improved HRQoL, especially symptoms of adrenal insufficiency (P = 0.003). Findings at six and eighteen months did not differ, and improvements achieved in androgen control, lean body mass and HRQoL after 6 months of CSHI were maintained at eighteen months. The hydrocortisone dose appeared to decrease with time [6 vs 18 months: 38.3 ± 8.8 vs 33.6 ± 12.2 mg/day (P = 0.062)], especially in women receiving oral contraceptives. Reduction of testicular ad...Continue Reading

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Citations

May 7, 2019·Current Opinion in Pediatrics·Ahmed Khattab, Ian Marshall
Sep 19, 2019·The Journal of Clinical Endocrinology and Metabolism·Alberto S TresoldiKrishnarajah Nirantharakumar
Aug 11, 2020·The Journal of Clinical Endocrinology and Metabolism·Phyllis W Speiser
Jul 12, 2019·Current Opinion in Pediatrics·Ahmed Khattab, Ian Marshall
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Mar 31, 2021·Current Opinion in Endocrinology, Diabetes, and Obesity·Muhammad Fahad Arshad, Miguel Debono

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