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A Journal on Internal Medicine and Pharmacology

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Gazzetta Medica Italiana Archivio per le Scienze Mediche 2014 April;173(4):237-40

language: English

Hypoglycemia unmasking rare disease entity

Antonopoulou M., Loney-Hutchinson L. M.

Department of Endocrinology, SUNY Downstate Medical Center, Brooklyn, NY, USA


The aim of this paper was to document a case of hypoglycemia in a 24-year-old female patient with special consideration to alarming clinical signs that can point to rare disease diagnosis. We present the diagnostic approach of a female patient with hypoglycemia. A 24-year-old female of Caribbean descent, presented with a syncopal episode due to hypoglycemia. Physical exam was unremarkable except for central obesity and multiple pedunculated skin lesions in the abdomen and back. Past medical history was positive for a macroadenoma secreting prolactin and the patient has been off bromocriptine for one month. Secondary adrenal insufficiency was suspected and morning cortisol level was remarkably low. The patient was placed on steroid replacement and dextrose drip, but she still became severely hypoglycemic. Insulin level and C-peptide was inappropriately normal for the low glucose level. Corrected calcium level was also found to be elevated. Multiple Endocrine Neoplasia syndrome type 1 (MEN 1) was suspected and indeed the patient was diagnosed with hyperparathyroidism and insulinoma that was treated surgically. Post pancreatectomy, ACTH stimulation test was performed that showed normal response. Recurrent hypoglycemias in patients with insulinomas blunt the counterregulatory hormone response and neuroglycopenic symptoms, which can return to normal after surgical treatment. During spontaneous hypoglycemia a seemingly low plasma cortisol is not sufficient evidence of adrenocortical insufficiency. Multiple collagenomas should raise suspicion for MEN 1 syndrome.

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