Searchable abstracts of presentations at key conferences in endocrinology
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46th Meeting of the British Society for Paediatric Endocrinology and Diabetes

Birmingham, UK
07 Nov 2018 - 09 Nov 2018

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Birmingham, UK - 7-9 November 2018

ea0058p001 | Adrenal | BSPED2018

A Cochrane Review of glucocorticoid replacement regimens in the treatment of 21-hydroxylase deficiency congenital adrenal hyperplasia

Ng Sze , Stepien Karolina

Congenital adrenal hyperplasia (CAH) is an autosomal recessive condition which leads to glucocorticoid deficiency. During childhood, aims of treatment are to prevent adrenal crisis and to achieve optimal adult height and normal puberty. In adults, aims of treatment are to prevent adrenal crisis, ensure normal fertility and avoid long-term consequences of glucocorticoid use. Current regimens with glucocorticoids cannot optimally replicate the normal physiological cortisol level...

ea0058p002 | Adrenal | BSPED2018

Borderline peak plasma cortisol following Synacthen stimulation – single-centre analysis of three years’ data

Burn Sarah , Colyer Sharon , Dimitri Paul , Wright Neil , Krone Nils , Elder Charlotte

Introduction: Diagnostic cut-offs for plasma cortisol on Short Synacthen Test (SST) are controversial, made more complicated by modern assays and paediatric normative values extrapolated from adult data. Some advocate a division between biochemical and clinical AI, with different cut-offs and management strategies. For asymptomatic children, with a low-index of suspicion, and borderline SST results our department has evolved a tendency to advise hydrocortisone replacement in t...

ea0058p003 | Adrenal | BSPED2018

Differences in hydrocortisone absorption during the 24 hour period in patients with adrenal insufficiency

Hindmarsh Peter , Charmandari Lia , Honour John

Hydrocortisone therapy should be individualised in patients with adrenal insufficiency to avoid over and under replacement. We assessed hydrocortisone absorbtion at different times of day which may impact on treatment regimens. We assessed the oral absorption of hydrocortisone in 48 patients (21M) aged between 6.1 and 20.3 years with congenital adrenal hyperplasia due to P450c21 deficiency. Hydrocortisone dosing ranged between 11.5 and 22.6 mg/m2 per day in three or...

ea0058p004 | Adrenal | BSPED2018

Adrenocortical function in infants admitted to PICU

Pintus Donatella , Das Urmi , Dharmaraj Poonam , Didi Mohammed , Ramakrishnan Renuka , Senniappan Senthil , Thorburn Keith , Blair Joanne

Background: Hypocortisolaemia is common in neonates1 and infants following cardiac surgery.2 In critically ill children with other pathologies, hypocortisolaemia may result from accelerated cortisol metabolism and reduced protein binding. However, the timing and frequency of normalisation of cortisol concentrations following infantile critical illness is poorly described.Objective: To describe the natural history of hypocortisolaemi...

ea0058p005 | Adrenal | BSPED2018

Sphingosine-1-phosphate lyase (SGPL1) deficiency is associated with mitochondrial dysfunction

Prasad Rathi , Maharaj Avinaash , Bradshaw Teisha , Williams Jack , Guran Tulay , Braslavsky Deby , Brugger Britta , Metherell Lou

Introduction: Loss of function mutations in SGPL1, a key component of sphingolipid metabolism, are associated with accumulation of sphingolipid intermediates giving rise to a multisystemic disease incorporating primary adrenal insufficiency (PAI) and progressive renal and neurological disease. Sphingolipid accumulation is implicated in mitochondrial pathology.Objective: To investigate the impact of SGPL1 deficiency on mitochondrial morphology/ f...

ea0058p006 | Adrenal | BSPED2018

Questionnaire survey identifies timing of last dose of hydrocortisone as important determinant of side effects

Hindmarsh Peter , Honour John

The aim of cortisol replacement in adrenal insufficiency is to mimic the normal cortisol circadian rhythm. Timing of the last dose varies. Paediatric practice doses as close to midnight or after as possible compared to no dose after 18.00 h in adults. Using a detailed questionnaire, we ascertained side effect prevalence in 226 patients with adrenal insufficiency (77 CAH, 82 Addison, 67 hypopituitarism) and compared frequency of problems with timing glucocorticoid replacement. ...