SAT-470 A Case of Acromegaly with Concomitant Hypercortisolism Presenting with Diabetic Ketoacidosis

Background: Acromegaly is a rare disorder with an annual incidence of 4-6 new cases per 1 million population.(1) Impairment in glucose metabolism has been previously described in such disorder, but diabetic ketoacidosis is rare. Case: A 25 year old, male, presented at our emergency room with seizures. Laboratory data showed hyperglycemia (blood glucose 418 mg/dl), ketonuria (urine ketone +2), and pure high anion gap metabolic acidosis consistent with severe Diabetic Ketoacidosis. Hba1c was 12%. A normal fasting C-peptide 1.68 ng/ml (1.10-4.40 ng/ml) indicate residual insulin secretion, or maybe an inappropriately normal response in the presence of severe hyperglycaemia hyperglycemia. Features of acromegaly were noted and hormonal evaluation showed elevated IGF-1 level at 484.50 ng/ml (83-344 ng/ml), supporting the diagnosis. Random growth hormone was elevated at 5.7 ng/ml (<3 ng/ml). GH suppression testing with oral glucose load was not done due to unstable glucose levels. An elevated 24 hour urine cortisol 6208.0 nmol (100.0-379.0 nmol/24h) may be associated with uncontrolled diabetes, or an underlying subclinical Cushing’s disease contributing to further insulin resistance. Other hormones of the anterior pituitary reveal normal thyroid function, central hypogonadism and hyperprolactinemia at 828.50 uIU/ml (58.0-475.0 uIU/ml) likely due to hypophyseal stalk compression by the tumor. Cranial MRI with contrast showed an enhancing sellar mass measuring 23.7 x 22.3 x 18.0 mm (AP x T x CC) which extends inferiorly to the upper portion of the prepontine cistern. Resolution of ketoacidosis required 360 units of regular insulin per day, without achieving target blood glucose levels. Blood glucose level was successfully controlled with insulin and oral hypoglycemic agents. There was incomplete resection of the mass following transphenoidal surgery. Conclusion: This case highlights the concomitant presence of increased counter regulatory hormones (GH and cortisol) and IGF-l levels in Acromegaly leading to increased insulin resistance, which are uncommon, but important causes of Diabetic Ketoacidosis. Reference: Melmed, S. (2016). Williams textbook of endocrinology. Elsevier Health Sciences.