Endocrine Abstracts

Background: Patients taking hydrocortisone replacement for primary or secondary adrenal failure require individual adjustment of their dose. Previous observations in our department suggest that total serum cortisol levels achieved following an afternoon or evening dose of 5 mg hydrocortisone are almost as high as those that result from a 10 mg dose in the early morning; and that the ‘area under the cortisol curve’ (AUC) generated by an evening 5 mg dose is broader th...

Background: FGD is an autosomal recessive disorder causing glucocorticoid deficiency. Mutations in the ACTH receptor (MC2R) or the MC2R accessory protein (MRAP) cause FGD types 1 & 2 respectively. All the reported MRAP mutations result in abolition of a functional protein. This is reflected clinically as type 2 patients present early, no patient described to date has presented later than 1.6yrs. In contrast FGD type 1 mutations are usually missense and patients have a medi...

The GH receptor antagonist pegvisomant entered clinical trials in 1997 and was (UK) marketed in 2005. There is little long-term experience with this novel drug. We report the experience from two centres that used common protocols to manage 58 patients (36 male, median age 53, range 27 78) since 1997. Before commencing pegvisomant, patients had IGF-I above the upper limit of normal (ULN) of age-related reference ranges (median 1.8 xULN, range 1.2–4.1 xULN) and were uncontr...

Introduction: Histology is a poor predictor of the malignant potential of phaeochromocytomas and paragangliomas (PPGL) and only the presence of distant metastasis confirms malignancy. This study reviews patterns of recurrence, treatment and mortality in patients with malignant PPGL presenting to our department at UCLH over 30 years.Materials and Methods: In our series of 128 patients with PPGL, 26 (20%) with maligna...

Background: Radioiodine treatment (RIT) for benign functioning thyroid disease is an effective treatment for patients with hyperthyroidism. The safety, preparation, administration and follow-up of treatment demands time and organisation. The planning of this elective treatment is affected by the patient’s availability which makes adequate and timely treatment of the subsequent hypothyroidism challenging. A quality improvement project was therefore set up to mitigate this....

Introduction: Histology is a poor predictor of the malignant potential of phaeochromocytomas and paragangliomas (PPGL) and only the presence of distant metastasis confirms malignancy. This study reviews patterns of recurrence, treatment and mortality in patients with malignant PPGL presenting to our department at UCLH over 30 years.Materials and Methods: In our series of 128 patients with PPGL, 26 (20%) with maligna...

Background: Radioiodine treatment (RIT) for benign functioning thyroid disease is an effective treatment for patients with hyperthyroidism. The safety, preparation, administration and follow-up of treatment demands time and organisation. The planning of this elective treatment is affected by the patient’s availability which makes adequate and timely treatment of the subsequent hypothyroidism challenging. A quality improvement project was therefore set up to mitigate this....

Introduction: Primary hyperparathyroidism is a common endocrine disorder, surgery is curative. Patients with severe hypercalcemia receive cinacalcet or intravenous bisphosphonates as bridging. Intra-operative-parathyroid-hormone (IOPTH) measurement improves surgical accuracy. Bisphosphonates may increase PTH, while cinacalcet reduces it. The main aim was to assess the effect of zoledronate and cinacalcet on IOPTH.Method: Patients over 15-yo who underwent...

Background: Klinefelter syndrome (KS), karyotype 47XXY, affects 1 in 650 males. Subjects develop primary gonadal failure requiring life-long testosterone replacement. Many different testosterone formulations are available and long-term monitoring is necessary to avoid secondary polycythaemia.Objective: To investigate the effect of testosterone formulations used in KS subjects and estimate frequency of association with secondary polycythaemia.<p class...

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...