Endocrine Abstracts

We present a 66 year old man with a metastatic small bowel neuroendocrine tumour managed with resection of primary in 2011 and subsequent somatostatin analogue therapy. In 2018 disease progressed requiring right ureteric stenting due to extrinsic nodal compression. Three weeks later he presented with sweats, pyrexia and elevated inflammatory markers. Multiple blood and urinary cultures were clear. CT imaging, MRI spine and echocardiogram did not identify a source of sepsis. Ur...

21 year old man presented to the emergency department with hypotensive shock. He had been seeing the GP with complains of poor appetite, intermittent vomiting, increased thirst and postural dizziness for the last 6 months. Previous medical history included tonsillitis, gastritis and learning difficulties. The working differential was severe gastroenteritis with hypovolemic shock, and so he was given immediate fluid resuscitation. He remained hypotensive, and so was given empir...

Introduction: Children with chronic renal failure may grow poorly and have altered bone metabolism and body composition. Post-renal transplant (Tx), improvement in these parameters may be hindered due to glucocorticoid exposure.Aims & methods: To study changes in these parameters after Tx, anthropometry, bone mineral content (BMC) at total body (TB) and lumber spine (LS), lean mass (LM) and fat mass (FM) were analysed by DXA in 15 children (median ag...

Background: In XY disorders of sex development, a single measurement of serum Anti-Mullerian hormone (AMH) may replace the hCG stimulation test.Aim: To assess the level of concordance between serum AMH and hCG stimulated testosterone concentrations.Methods: About 284 children (M:F,154:130) with a median age of 8 years (10th, 90th centiles, 0.25,14) were used to establish a normal reference range for AMH. Clinical data were reviewed...

Background: PPNAD is a rare form of bilateral adrenal hyperplasia associated with Carney complex and PRKAR1A mutation. We report the experience of monitoring disease activity in two patients with PPNAD.Both patients (case 1: a 7.4-year-old boy and case 2: a 6.1-year-old girl) presented with rapid onset of cushingoid symptoms, weight gain and hypertension together with multiple freckles and lentigines.Biochemical analysis on both pa...

The eye is an organ vulnerable to a variety of external and internal stimuli that can abolish its vital function to allow accurate vision of images for survival. Inflammation is the key mediator of immune protection but its consequence leads to the disruption of the visual axis integrity. Due to this precise reason, through adaptation and evolution, the eye has developed mechanisms of immune tolerance and privilege. Glucocorticoids have potent immunosuppressive and anti-inflam...

Introduction: The hCG stimulation test is a useful indicator of functional testicular tissue and may also have a place in treating undescended testes (UDT). However, its utility is limited by the inconsistency in the regimens used. We have looked at the clinical and biochemical response in a group of children with UDT who had a prolonged hCG stimulation as part of their clinical management.Methods: The retrospective review in 17 cases with a median EMS (...

Three siblings of Pakistani origin presented shortly after birth with failure to thrive and hyperkalemia and were found to have isolated hyperreninaemic hypoaldosteronism. They were all well controlled on fludrocortisone therapy during childhood and adolescence. When reassessed in adult life off fludrocortisone treatment, hyperreninaemic hypoaldosteronism was confirmed in all subjects, but with significant hyperkalemia in only one case. None of the subjects developed orthostat...

Corticosteroids can be synthesised in tissues other than the adrenal cortex but the contribution of this extra-adrenal corticosteroidogenesis to circulating levels in man is not known. We studied this in a group 10 subjects taking chronic glucocorticoid replacement following bilateral adrenalectomy. In phase 1, they were maintained on cortisol alone (30 mg/day). In phase 2, cortisol was replaced by dexamethasone (2 mg/day) and in phase 3, they received both cortisol and dexame...

Although hydrocortisone is the standard replacement therapy for primary or secondary adrenal insufficiency (AI), there are limited data on dosage requirements and the value of salivary monitoring in this patient group. We assessed inter-individual variability in cortisol metabolism and relationships between plasma and salivary cortisol profiles in 27 patients. Ten (3 male) patients had primary AI and 17 (8 male) secondary AI. Salivary and plasma cortisol levels were measured a...