Clinical Case Study

Hormonal counter-regulatory failure and severe hypoglycemia in a patient with type 1 diabetes and congenital adrenal hyperplasia

Elsemiek EEC Engwerda, Anita CTM Peeters, Nike MML Stikkelbroeck, Bas J Schouwenberg and Bastiaan E de Galan

Diabetic Hypoglycemia October 2012, Volume 5, Issue 2: page 9-12

Abstract

A 17-year-old Turkish girl was referred to the diabetes clinic because of poorly controlled type 1 diabetes and recurrent severe hypoglycemia. She was known to have adrenal insufficiency secondary to congenital adrenal hyperplasia (21-hydroxylase deficiency). It was hypothesized that the episodic severe hypoglycemia was the consequence of concomitant adrenomedullary dysfunction, with resultant impairment of epinephrine (adrenaline) response capacity. During a stepped hyperinsulinemic hypoglycemic glucose clamp (blood glucose nadir, 2.2 mmol/l; 40 mg/dl), plasma epinephrine and glucagon levels were low at baseline and failed to increase, whereas the norepinephrine (noradrenaline) and growth hormone responses were higher than normal. The generation of autonomic and neuroglycopenic hypoglycemia symptoms was not suppressed. This case demonstrates the importance of the epinephrine response to support glucose counter-regulation in people with type 1 diabetes and the independence of hypoglycemic symptom generation from secretion of adrenomedullary hormones.

Keywords: type 1 diabetes, hypoglycemia, congenital adrenal hyperplasia, counter-regulatory hormones, epinephrine, norepinephrine, hypoglycemia symptoms, hyperinsulinemic glucose clamp