Searchable abstracts of presentations at key conferences in endocrinology

ea0035p825 | Paediatric endocrinology | ECE2014

Medication safety study investigating hydrocortisone individually and extemporaneously compounded capsules for paediatric use in congenital adrenal hyperplasia

Kauzor Daniela , Spielmann Sarah , Ross Richard , Blankenstein Oliver , Kloft Charlotte

Background: Treatment outcome with hydrocortisone for congenital adrenal hyperplasia (CAH) in neonates and children is highly variable. As there is no licensed formulation for children <6 years hydrocortisone capsules individually have to be compounded by local pharmacies (dose strength: 0.5–9.5 mg). The aim of this study was to characterise mass and content of these capsules in order to assess medication safety for effective and nontoxic dosing in terms of precision ...

ea0035p30 | Adrenal cortex | ECE2014

Development of oral hydrocortisone granules with taste masking for the treatment of neonates and infants with adrenal insufficiency

Ross Richard , Whitaker Martin , Digweed Dena , Spielmann Sarah , Huatan Hiep , Eckland David , Blankenstein Oliver , Krude Heiko

Background: Current standard treatment for adrenal insufficiency in neonates and infants is unsatisfactory as unlicensed adult dosage formulations are used. These are generally difficult to administer and may give rise to inconsistencies in dose as the content uniformity of the dosage form cannot be verified.Methods: Infacort is a newly-developed immediate release formulation of hydrocortisone that is provided in appropriate unit dosage units (0.5, 1, 2,...

ea0022p820 | Thyroid | ECE2010

Rapid solid-phase-extraction for the quantitative analysis of serum iodothyronine profiles

Hoefig Carolin , Wohlgemuth Franziska , Klein Jeannette , Blankenstein Oliver , Schweizer Ulrich , Kohrle Josef

A novel solid-phase-extraction (SPE) method followed by liquid chromatography tandem mass spectrometry (LC-MS/MS) was established for the simultaneous detection and quantification of thyroid hormone profiles in one sample of human or mouse serum.Recovery during preanalytical steps was monitored by addition of an internal deuterated standard (d5-T4) to 200 μl serum, which was deproteinized by adding a precipitating agent consisting of 1% f...

ea0016p587 | Paediatric endocrinology | ECE2008

Evaluation of Intergrated [18F]FDOPA–PET/CT for identification of focal forms of Congenital Hyperinsulinism (CHI)

Blankenstein Oliver , Mohnike Klaus , Hussain Khalid , Kuehnen Peter , Barthlen Winfried , Fuechtner Frank , Mohnike Wolfgang

Objective: CHI is the most frequent cause of severe hypoglycaemia in infants. Two distinct anatomical forms have been described which require different therapeutic strategies. We evaluated the predictive value and accuracy of integrated [18F]FDOPA-PET/CT as a new tool in identification of focal lesions in an observational study.Patients and methods: From 2005 to 2007, 73 infants and children from the UK (30) and Germany (42) with CHI were exam...

ea0073pep1.3 | Presented ePosters 1: Adrenal and Cardiovascular Endocrinology | ECE2021

Rationale for a reduced dexamethasone dosis in prenatal CAH therapy based on pharmacokinetic modelling

Reisch Nicole , Stachanow Viktoria , Neumann Uta , Blankenstein Oliver , Fuhr Uwe , Huisinga Wilhelm , Michelet Robin , Kloft Charlotte

ContextPrenatal dexamethasone (Dex) therapy is used in female foetuses with congenital adrenal hyperplasia (CAH) to suppress adrenal androgen excess and prevent virilisation of the external genitalia. The prenatal dexamethasone dose of 20 µg/kg per day has been used for decades in prenatal CAH and is associated with risks for the treated mother and potentially for the unborn child. Despite the high medical need, no prospective, clinical studies had ...

ea0058oc4.1 | Oral Communications 4 | BSPED2018

Hydrocortisone granules in capsules for opening: phase 3 trial in children with adrenal insufficiency and long-term safety data

Lewis Alexander , Neumann Uta , Wiegand Susanna , Krude Heiko , Digweed Dina , Voet Bernard , Ross Richard , Davies Madhu , Blankenstein Oliver

Introduction: Children with adrenal insufficiency requiring hydrocortisone rely on compounded adult medication. This study aimed to evaluate the absorption, palatability and safety of Alkindi® (hydrocortisone granules in capsules for opening).Methods: The phase 3 study was an open-label, single-dose study in a total of 24 children (aged 0–6 years) with adrenal insufficiency. Fasted children were given a single dose of Alkindi&#1...

ea0027oc3.2 | Oral Communications 3 | BSPED2011

Clinical, genetic, histological and radiological heterogeneity of focal forms of congenital hyperinsulinism

Ismail Dunia , Kapoor Ritika , Smith Virpi , Ashworth Michael , Blankenstein Oliver , Pierro Agostino , Flanagan Sarah , Ellard Sian , Hussain Khalid

Congenital hyperinsulinism (CHI) is a cause of severe and persistent hypoglycaemia due to unregulated insulin secretion from pancreatic β-cells. Mutations in the ABCC8 and KCNJ11 genes are the most common cause of medically unresponsive CHI. Histologically there are three major subgroups, focal, diffuse and atypical. The pathophysiology of focal CHI is complex and involves a two hit process with the patient firstly inheriting a paternal ABCC8/KCNJ11 m...

ea0022p264 | Developmental endocrinology | ECE2010

Baseline characteristics and serum IGF1 levels in GH deficient (GHD) patients treated in the transition phase from adolescence into adulthood: data from the NordiNet International Outcome Study (IOS)

Weber Matthias M , Blankenstein Oliver , Jorgensen Jens Otto L , Pedersen Birgitte T , Rakov Viatcheslav , Christiansen Jens Sandahl

Background: Data from several clinical studies have demonstrated the benefit of GH treatment in relation to bone mineral density and body composition in young adults who received GH treatment in childhood due to GHD1–3. There are recommendations to use a higher GH dose in the transition phase compared to the GH dose required during adulthood to mimic the endogenous GH secretion4,5.Methods: Data from NordiNet IOS have been analy...

ea0041ep14 | Adrenal cortex (to include Cushing's) | ECE2016

Adrenal crisis and sick day episodes among CAH patients: preliminary report based on international CAH (I-CAH) registry

Karunasena Nayananjani , Daniel Eleni , Bryce Jillian , Jiang Jipu , Faisal Ahmed S , Guran Tulay , Mendonca Berenice B , Bachega Tania A , Blankenstein Oliver , Koehler Birgit , Neumann Uta , Acerini Carlo , Krone Nils , Bonfig Walter , Mohnike Klaus , Elsedfy Heba , Ross Richard

Background: Congenital adrenal hyperplasia (CAH) is a rare condition that is associated with life long risk of adrenal crisis. Management of CAH demands a fine balance between excess glucocorticoid leading to adverse effects and too little glucocorticoid risking adrenal crises. Frequent occurrence of sick day episodes warrants dose adjustment and education regarding adrenal crisis. In a condition such as CAH it is difficult to collect sufficient data from small cohorts at a si...