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Endocrine Abstracts (2018) 55 WH7 | DOI: 10.1530/endoabs.55.WH7

NHS Grampian, Aberdeen, UK.


Introduction: Symptomatic hypoglycaemia unrelated to the treatment of diabetes is rare. As symptoms of hypoglycaemia are non-specific, investigation should only be instigated if Whipple’s triad can be fulfilled- signs and symptoms of hypoglycaemia, a recorded low plasma glucose and resolution of symptoms after treatment.

Case history: A 37 year-old woman presented to the emergency department with fatigue and lightheadedness. She has modified her diet to alleviate her symptoms and gained over a stone. She has a daughter and family history of Type 1 and Type 2 diabetes but has no access to diabetes medications or insulin. She has no family history of Multiple Endocrine Neoplasia (MEN). Blood glucose recorded during admission was 1.6 mmol/l. After treatment, her symptoms resolved rapidly. Two fasting samples of glucose done by her own GP were 3.6 mmol/l and 1.8 mmol/l. Further investigations showed early morning cortisol of 465 nmol/l, calcium 2.71 mmol/l (2.2–2.6), parathyroid hormone of 13.2 pmol/l (1.3–6.85), IGF-1 48.9 nmol/l (14–37), normal prolactin and thyroid function. She was admitted for a 72-h fast and developed hypoglycaemia just after 12 h with blood glucose of 2.1 mmol/l. Insulin and c-peptide at the time were inappropriately elevated, 3.3 mu/l (<3) and 0.36 nmol/l (<0.2) respectively. Although insulin was only mildly elevated, proinsulin was markedly elevated at 72 pmol/l (<5). Serum sulfonylurea screen and insulin antibodies were negative. CT scan revealed well-defined low attenuation lesion in the superior aspect of the head of pancreas measuring 5×3 cm and incidental left adrenal adenoma. MRI pancreas showed multi-focal pancreatic lesion and PET scan showed metabolically active pancreatic lesion with no metastases. Further testing showed negative urinary metadrenalines, elevated chromogranin A 71 pmol/l (0–59) and chromogranin B 173 pmol/l (0–149) which is consistent with neuroendocrine tumour. She has multiple conditions compatible with a diagnosis of MEN-1 and genetic testing for MEN-1 was positive. She is currently awaiting total pancreatectomy and further work up of her primary hyperparathyroidism and pituitary imaging will be required.

Discussion: An insulinoma is the commonest endogenous cause of hyperinsulinaemic hypoglycaemia and second most common pancreatic islet cell tumour associated with MEN-1. Patients with insulinoma should receive counselling for potential lifelong requirement for insulin after pancreatectomy. MEN-1 is autosomal dominant with high penetrance and the finding of this mutation has implications on the patient and family members who will require screening for associated tumours.

Volume 55

Society for Endocrinology Endocrine Update 2018

Society for Endocrinology 

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