hrp0086p2-p62 | Adrenal P2 | ESPE2016

Final Height Data in a Cohort of Patients with Congenital Adrenal Hyperplasia Treated with Tailored Doses of Hydrocortisone

Shir Wey Pang Gloria , Rumsby Gill , Hindmarsh Peter C , Dattani Mehul T

Background: Patients with congenital adrenal hyperplasia (CAH) are glucocorticoid deficient and require cortisol replacement to maintain homeostasis and prevent adrenal crises. Hydrocortisone dosing needs to be individualized because of variable cortisol clearance rates. Patients are thus prone to be either over or undertreated, both of which compromises final height.Objective and hypotheses: Performing 24-h cortisol profiling serially may allow for more...

hrp0086p1-p14 | Adrenal P1 | ESPE2016

A Unique Case of Dual Opposing Pathologies

Viseras Irene Fernandez , Giri Dinesh , Bockenhauer Detlef , Deshpande Charu , Achermann John , Taylor Norman , Rumsby Gill , Senniappan Senthil , Ajzensztejn Michal

Background: We present a patient with co-existence of two rare conditions 3β-Hydroxysteroid dehydrogenase type 2 deficiency (HSD3B2) the rarest form of Congenital Adrenal Hyperplasia (CAH) and Bartter’s Syndrome (hypokalaemic alkalosis secondary to hyperaldosteronism).Case Report: A female infant (46XX) born at 34/40 weeks weighing 2.67 kg to non-consanguineous parents presented on day four of life with significant weight loss. Subsequent inves...