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Endocrine Abstracts (2023) 90 P676 | DOI: 10.1530/endoabs.90.P676

ECE2023 Poster Presentations Pituitary and Neuroendocrinology (123 abstracts)

Hyperosmolar Hyperglycaemic State (HHS) and severe decompensated heart failure as presenting features of ectopic ACTH Syndrome

David McDonnell 1 , Leanne Cussen 1 , Julie Martin-Grace 1 , Paul Cotter 2 , Liam Grogan 3 & Amar Agha 1


1Beaumont Hospital, Endocrinology Department, Dublin, Ireland; 2St. Lukes Hospital, Geriatric Medicine, Kilkenny, Ireland; 3Beaumont Hospital, Oncology, Dublin, Ireland


Ectopic ACTH syndrome is a rare condition occurring in five to ten percent of ACTH dependent hypercortisolism. We present the case of a fifty eight year old gentleman who presented with severe hyperosmolar hyperglycaemic state (blood glucose fifty seven mmol/l) and acute decompensated heart failure associated with elevated liver enzymes. Liver ultrasound followed by computed tomography of thorax abdomen and pelvis showed a lung tumour with liver metastases. Clinical suspicion of Cushing’s syndrome was confirmed by with an overnight dexamethasone suppression cortisol of three thousand nine hundred and forty two nmol/l. A twenty four hour urine free cortisol was markedly elevated at a hundred fold with an adrenocorticotropin hormone level of one thousand four hundred and forty six pg/ml (7.2-63.3). Liver biopsy confirmed small cell carcinoma. Following initial treatment of his hyperosmolar hyperglycaemic state and heart failure, he was commenced on four grams of metyrapone per day which normalised his cortisol day curve. His insulin therapy and diuretics were later stopped and he maintained euglycaemia without glucose lowering therapy. Following carboplatin/etoposide chemotherapy, his metyrapone dose was reduced to two grams/day due to hypocortislaemia and replacement Hydrocortisone was added. This is a case of very severe hypercortisolaemia secondary to ectopic ACTH syndrome presenting with life threatening complications. Prompt diagnosis and rapid cortisol control with high dose metyrapone resulted in marked clinical and biochemical improvement.

Volume 90

25th European Congress of Endocrinology

Istanbul, Turkey
13 May 2023 - 16 May 2023

European Society of Endocrinology 

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