Endocrine Abstracts

Glucocorticoids form the mainstay of many treatment modalities in paediatrics ranging from short term use in asthma to longer term use as anti-inflammatory agents in nephrotic syndrome and rheumatoid disorders. Long term use although effective brings with it the problems of adrenal suppression.When considering weaning a patient from glucocorticoid use consideration needs to be given to the type of glucocorticoid used, duration of treatment, dosage used a...

Convention places 17OHP measures as the way to monitor replacement therapy in congenital adrenal hyperplasia due to P450c21 deficiency. One case several years ago led to questioning of this approach as the 17OHP measures suggested inadequate replacement with hydrocortisone using a general dosing regimen of 12 mg/m2/day and questions about compliance. 24 h cortisol profiles showed high 17OHP concentrations with low cortisol concentrations. Careful studies ...

Paediatric diabetes is essentially a hospital based service with opportunities for care delivery in settings such as the home, school and community based organisations. The delivery of care (as defined in part by HbA1c) varies throughout the UK. Factors influencing this include varying staffing levels and skill sets along with social deprivation and ethnic diversity and attaining a standard of equitable care is a major challenge. The fundamental issue is not whether care is de...

Hypoglycaemia is the main factor limiting the use of intensive insulin regimens. The frequency is 0.1 – 0.3 episodes/person per day for symptomatic episodes and 1/year for severe ones. There is also an estimated mortality of 2–4% of people with type 1 diabetes mellitus (T1DM). Severe hypoglycaemia increases in frequency with duration of insulin treatment. The symptoms and signs of hypoglycaemia can be separated into those due to neuroglycopenia (cognitive impairment,...

Classical congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency leads to glucocorticoid and mineralocorticoid deficiency. Management should be viewed as a process of care which requires input from an Inter-disciplinary Team. Glucocorticoid therapy should take the form of hydrocortisone in a starting dose of 10–12 mg/m2 per day (divided into three or four doses) and the dose should be titrated to blood or urine profiles of cortisol and 17 hydroxy...

The newborn screening programme for congenital hypothyroidism was introduced in the United Kingdom in 1981 and has virtually abolished cases of untreated congenital hypothyroidism (CH). Congenital hypothyroidism is a common (1 in 3–4000 life births) condition in which the thyroid gland has either failed to develop (agenesis) developed in an abnormal position (ectopic gland) or is present but does not respond to thyroid stimulating hormone (TSH) (dyshormonogenesis). In the...

Hydrocortisone therapy needs to be individualised in congenital adrenal hyperplasia (CAH) patients to avoid over and under replacement. Plasma cortisol concentration is determined by absorption and half-life of cortisol influence glucocorticoid exposure. Terminal plasma half-life is the time required to divide the plasma concentration by two and is important when absorption may vary.To ascertain a role for this measure we have studied 48 patients (21M) a...

Androstenedione and 17OHP are often used as measures of cortisol replacement in congenital adrenal hyperplasia (CAH) rather than cortisol itself. Very little is known of the dose response relationships between cortisol and A4 and 17OHP.We have studied the relationship between 24 h serum cortisol, 17OHP and A4 in 33 (18M) children with CAH due to P450c21 deficiency. 24 h serum cortisol and 17OHP profiles were constructed using 20 min sampling intervals an...

Introduction: Insulin requirements change with age, in part related to changes in Growth Hormone secretion. Little is known of the impact of age on the circadian variation in insulin secretion. We have studied changes in insulin basal rates as a proxy for insulin sensitivity in CYP with well controlled T1DM.Methods: Insulin pump settings for total daily dose (TDD) and sensitivity ratio were obtained from 22 CYP with T1DM. Basal insulin requirements were ...

Conventional hydrocortisone dosing does not mimic the normal cortisol circadian rhythm making treatment optimisation difficult in patients with adrenal insufficiency. We described the first use of a continuous variable subcutaneous hydrocortisone infusion (CSHI) via an insulin pump to replace cortisol in a patient with congenital adrenal hyperplasia (CAH) to mimic the normal plasma cortisol circadian rhythm. We report the long term experience of CSHI in seven patients with adr...