Assessing recovery of adrenal function in glucocorticoid-treated patients: Our strategy for screening and management.

Background: Long-term glucocorticoid (GC) use is the most frequent cause of adrenal insufficiency through suppression of the hypothalamic-pituitary-adrenal axis. There are no guidelines for predicting adrenal insufficiency (AI) and minimizing its risk. Methods: This is a prospective observational study carried out in the Endocrinology-Diabetology and Nutrition department between 2014 and 2021. All patients had received GC therapy for longer than 3 weeks before switching to hydrocortisone substitution, for various indications. These patients were admitted to our department for stimulation tests. We assessed the prevalence of AI, predicting factors, screening and management. Results: In our study the GC-induced AI was found in 49% of patients. We found a strong correlation between the basal morning serum cortisol, body mass index and the peak cortisol level after stimulation tests, while no correlation was found between adrenal function and age, sex, indication of GC therapy, duration of corticosteroid therapy, cumulative dose and daily dose. Patients with GC induced AI took a mean of 12 ± 12,18 months to recover. Adrenal function recovery rate was higher in patients tested by short Synacthen than in those tested by Insulin Hypoglycemia. Conclusions: We demonstrated the positive correlation between serum cortisol peak levels after stimulation and body mass index. The study supports that basal cortisol level, the duration of corticoid cessation and the type of stimulation test can predict the response of cortisol to stimulation tests.

A rare case report about a congenital adrenal hyperplasia by 21-hydroxylase lock in its pure virilizing form discovered in adolescence.

Introduction: Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is an autosomal recessive disease. The diagnosis of the classic virilizing form must be made at birth. Case presentation: We report the case of a 16-year-old female patient, who consulted for primary amenorrhea and absence of breast development, in whom the clinical examination found a male morphotype, signs of virilization with a peniform hypertrophy of the clitoris. Pelvic ultrasound confirmed the presence of the uterus and ovaries. A syacthen test on 17 hydroxy-progesterone was performed confirming the diagnosis of congenital adrenal hyperplasia by partial 21-hydroxylase deficiency. The treatment was based on hydrocortisone and spironolactone with a decrease in hairiness and a breast development after 3 months. Discussion: The principal aim of the management at adolescent age is to block hyperandrogenism and to prevent or manage the complications of classic form and its treatment. The treatment must be completed by a feminization surgery which constitutes a great challenge given the necessity of participation of a gynecologist and a pediatric surgeon experienced in the surgery of anomalies of sexual development. Conclusion: This rare case of anomaly of sexual development discovered at an adolescent age with all the obstacles and difficulties of its management allows to put the point on the necessity of a good clinical examination at birth and the early management of any anomaly of sexual development.