Endocrine Abstracts

SEVERE HYPERCALCEMIA CAUSED BY A VISIBLE PARATHYROID ADENOMA IN AN ADOLESCENT FEMALE

U.Das1, H Iassac1, H Kanchi1, DA Price1, GM Humphrey2 & C M Hall1

Departments of 1Endocrinology and 2Surgery, Royal Manchester Children's Hospital, Manchester, Hospital Road, Manchester M27 4HA.

A 16-year-old female collapsed following a 24-hour history of severe abdominal pain and vomiting. She was shocked, hypertensive (160/90mm Hg) with abdominal tenderness and a visible lump in the neck 11 (centimeter cubed). Investigations: amylase 636 units per Litre (<105), calcium 5.38 millimoles per Litre (2.2-2.7), parathormone [PTH] 215 picograms per millilitre (12-81), calcitonin 0.88 micrograms per Litre (0-0.08). Urinary epinephrine and nor-epinephrine were not elevated. Pituitary MRI was normal. Acute pancreatitis and renal calcification were identified by USS abdomen. CT and sestamibi neck scans suggested a parathyroid mass. Neuroendocrine tumour markers were acutely significantly elevated; GAWK 354 picomoles per Litre (<30), VIP 255 picomoles per Litre (<30), Gastrin 44 picomoles per Litre (<40), Proglucagon 55 picomoles per Litre (<5), Somatostatin 288 (<150) and PHN 19 (normal); postoperatively results were normal.

The pancreatitis was managed conservatively with antioxidants, hypertension with labetalol (stopped post operatively) and hypercalcaemia corrected within 4 days by rehydration and 2 infusions of 45 mg Disodium Pamidronate .

Histology showed an aggressive parathyroid adenoma with vascular invasion but no other malignant criteria and a normal thyroid gland.

'Hungry bone syndrome' developed intraoperatively (ionized calcium 0.95 millimoles per Litre [1-1.5]) and continuous intravenous infusions of 10% calcium gluconate (up to 21 millimoles per day) were required for 11 days. One year later, 100mmoles per day oral calcium is required to maintain eucalcaemia.

We report this case because this is the largest parathyroid adenoma recorded in a child associated with severe hypercalcemia requiring bisphosphonate therapy.