Endocrine Abstracts

Case history: A 45 year old lady with Von Hippel Lindau syndrome with a complex past medical history presented with symptoms of cauda equina compression. Past medical history includes cerebellar hemangioblastomas, bilateral retinal angiomas, right sided renal cell carcinoma and renal carcinoid tumour treated with partial and then completion nephrectomy, bilateral phaeochromocytoma treated with bilateral adrenalectomy, metastatic neuroendocrine tumour treated with Whipple’...

Introduction: Increased cardiovascular risk in hypopituitary patients was first documented by Rosen in 1990. Subsequent studies confirmed increased prevalence of cardiovascular and cerebrovascular disease in these patients. The exact mechanism for this is unclear. There is no clear consensus on how best to quantify or predict cardiac risk in hypopituitarism. QRISK2 cardiovascular disease risk algorithm provides estimates of 10-year cardiovascular disease (CVD) risk in patients...

Introduction: Patients with craniopharyngioma are characterised by a high incidence of hypopituitarism, visual failure and hypothalamic dysfunction. Standardised mortality is markedly elevated and controversy exists about optimal treatment.Aim: We aimed to examine the temporal trends in the treatment of craniopharyngioma at our centre. Also, we sought to examine treatment needs and long-term morbidity in this patient group.Methods:...

A 51 year old woman presented with severe Cushing’s syndrome. In addition to a typically Cushingoid appearance she demonstrated increased cutaneous pigmentation in her face and upper chest. Biochemical investigation confirmed elevated serum cortisol levels with loss of circadian variation, and failure to suppress with low dose dexamethasone (0.5 mg 6 hourly for 48 hours). Serum ACTH levels were undetectable. Cross-sectional imaging revealed bilateral macronodular adrenal ...

Background: The glands most commonly affected in MEN1 are parathyroid, pituitary and pancreas. Pancreatic neuroendocrine tumours (pNETs) are of foregut origin, but a number of other so-called ’foregut carcinoid tumours’ may also occur in MEN1, including tumours of bronchial, thymic, and gastrointestinal origin. Reported rates of prevalence of these latter tumours vary from 2% for thymic and bronchial carcinoids, to 10% for gastric carcinoids. Thymic carcinoids have b...

Background: Treatment options for Graves’ disease (GD) and multinodular goitre include antithyroid medication, near total thyroidectomy and radioactive iodine (RAI). RAI is an established treatment for GD in the adult population but is used less commonly in children and the adolescent population due to concerns with regards to safety.Methods: A review of a series of 14 adolescent patients receiving RAI between 2007 and 2013 in our department was per...

Introduction: Regimens for pre-operative α and β-blockade for patients with secretory phaeochromocytomas/paragangliomas vary widely between centres. The worldwide lack of availability of intravenous phenoxybenzamine (Goldshield) has removed a useful tool in the management of phaeochromocytoma crisis and has necessitated a change in our institution’s routine pre-operative strategy. We compare pre, peri and post-operative surrogate measures of blockade in a cohort...

Objective: (1) To review normative data for catecholamines using our cohort of hyperaldosteronism patients undergoing adrenal vein sampling. This adds to the previously published data of normative values in a cohort of patients with cortisol-producing adenoma. (2) To demonstrate the usefulness of the noradrenaline to adrenaline ratio in the interpretation of adrenal venous sampling results.Design: 38 patients underwent successful, bilateral adrenal venou...

Introduction: We describe two patients admitted to our institution with severe hypercalcaemia, resistant to conventional treatment, requiring haemofiltration. Patient 1 was admitted to hospital with profound hypercalcaemia (5.8 mmol/L) secondary to primary hyperparathyroidism (PTH >263 Pmol/L). The hypercalcaemia had been discovered on a surgical admission 3 months previously and was being managed with 0.9% normal saline and pamidronate infusions at an outside hospital. On...

The potential for primary tumour relapse is an important consideration during GH replacement therapy (GHR). We report 3 cases of relapse of intra cranial germ cell tumour (GCT) during GHR.Patient 1: An 11 year-old female presenting with visual loss and short stature due to a suprasellar malignant teratoma. She was successfully treated with bleomycin, etoposide and cisplatinum (BEP) and intrathecal chemotherapy. She suffered a first relapse two years late...