Volume 82 | ESPE2014 | Next issue

ESPE 2014

Dublin, Ireland
18 Sep 2014 - 20 Sep 2014

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Dublin, Ireland; 18-20 September 2014. Further information

Poster Presentations

Adrenals & HP Axis (1)

hrp0082p1-d3-12 | Adrenals & HP Axis (1) | ESPE2014

Adrenal Rest Tumors in Patients with Primary Adrenal Insufficiency

Abali Zehra Yavas , Saka Nurcin , Erol Oguz Bulent , Aydin Banu Kucukemre , Guran Tulay , Bas Firdevs , Poyrazoglu Sukran , Bundak Ruveyde , Darendeliler Feyza

Background: Gonads containing adrenal rests may enlarge in response to chronic overstimulation by ACTH in diseases with ACTH hypersecretion such as poorly controlled congenital adrenal hyperplasia (CAH), Addison’s disease and Nelson’s syndrome. Testicular adrenal rest tumors (TART) are present in childhood in CAH patients and frequency in adults may be up to 50–95%. Ovarian adrenal rest tumors (ART) are less frequently detected. TART is decribed less in patients...

hrp0082p1-d3-13 | Adrenals & HP Axis (1) | ESPE2014

Assessment of Blood Pressure and Left Ventricular Parameters in Children with Classical CAH-due to 21 Hydroxylase Deficiency

Abdelghaffar Shereen , Ibrahim Reem , Abdelaziz Osama , Hafez Mona , Mamdouh Mona , Alashmawy Abeer , Elmougy Fatma , Abdelaty Sahar , Ibrahim Amany , Mehawed Hend , Ghaly Isis

Background: Patients with congenital adrenal hyperplasia (CAH), due to 21 hydroxylase (OH) deficiency may develop an adverse cardiovascular risk profile as reported by few previous studies.Objective and Hypotheses: Blood pressure (BP) and echo parameters in children with CAH due to 21-OH deficiency were evaluated.Method: This cross-sectional study included 53 children. BP, echocardiographic left ventricular functions as well as gro...

hrp0082p1-d3-14 | Adrenals & HP Axis (1) | ESPE2014

Clinical, Biological and Genetic Characteristics of 48 Pediatric Patients with Micronodular Adrenal Hyperplasia (MAH)

Rothenbuhler Anya , Gourgari Evgenia , Lodish Maya , Stratakis Constantine

Background: Micronodular adrenal hyperplasia (MAH) is a rare disease.Objective: Describe the clinical, biological and genetic characteristics of micronodular adrenal hyperplasia (MAH) in children.Method: Retrospective study based on medical records (from the NIH and Bicêtre Hospital) of 47 pediatric patients (age <18 years at first signs of disease) with ACTH-independent Cushing syndrome (AICS) due to MAH proven histologic...

hrp0082p1-d3-15 | Adrenals &amp; HP Axis (1) | ESPE2014

Testicular Adrenal Rest Tumors and Sertoli Cell Function in Adolescents with Cah

Chugunov Igor , Kareva Maria , Orlova Elizaveta , Kuznecova Elvira , Bogolubov Sergey

Background: Infertility is one of the major problems in adult males with congenital adrenal hyperplasia (CAH), associated with the development of testicular adrenal rest tumors (TART). Sertoli cell dysfunction could be diagnosed not only in adults but in adolescence.Objective and Hypotheses: To study Inhibin B and AMH levels in adolescents with CAH and TART and its diagnostics value in access of Sertoli cell function.Method: We stu...

hrp0082p1-d3-16 | Adrenals &amp; HP Axis (1) | ESPE2014

A Novel Mutation in Exon 5 of TP53 Gene in a Familial Adrenocortical Carcinoma

Simsek Enver , Binay Cigdem , Tokar Baran , Dundar Emine , Demiral Meliha

Background: Adrenocortical carcinoma (ADCC) is a rare cancer in children and differs significantly in epidemiology, clinical characteristics, and biologic features from their counterparts in adults. Germline mutations of the TP53 tumor suppressor gene are associated with cancer predisposition in families with the Li-Fraumeni syndrome.Aim: To report a young girl with family a history of ADCC who presented with severe virilisation secondary to ADC...

hrp0082p1-d3-17 | Adrenals &amp; HP Axis (1) | ESPE2014

Aldosterone/Renin Ratio as Key Player in the Diagnosis of Primary Hypoaldosteronism in Newborns and Infants

Ruecker Beate , Lang-Muritano Mariarosaria , Spanaus Katharina , Welzel Maik , Lallemand Dagmar , Phan-Hug Franziska , Konrad Daniel , Katschnig Claudia , Holterhus Paul-Martin , Schoenle Eugen

Background: Primary hypoaldosteronism (PA) is a rare inborn disorder with life-threatening symptoms in newborns and infants due to an aldosterone synthase defect. As plasma aldosterone concentration (PAC) can remain in the normal range, interpretation of the laboratory findings could be difficult and might lead to delayed initiation of therapy.Objective and Hypotheses: This study aims to show that PAC/PRC (plasma renin concentration) rat...

hrp0082p1-d3-18 | Adrenals &amp; HP Axis (1) | ESPE2014

Zenhale Inhaled Corticosteroid Therapy: Useful Second Line Therapy for Asthma in Children but be Wary of Adrenal Suppression

Perry Rebecca , Schwarz Wendy , Stosky Karen , Dawrant Jonathan , Pacaud Daniele , Noseworthy Mary , Anselmo Mark

Background: Children with Asthma who do not respond to first-line therapy may need inhaled corticosteroid-long-acting beta agonist combination (ICS-LABA) therapy. Adrenal insufficiency (AI) due to adrenal suppression is a recognized but relatively uncommon side effect of ICS. An increase in suspected cases of AI associated with one particular ICS-LABA, mometasone-formoterol (Zenhale) was observed at a tertiary care Asthma clinic over a 6-month period.Obj...

hrp0082p1-d3-19 | Adrenals &amp; HP Axis (1) | ESPE2014

Development of Scotland Wide Process for Management of Acute Adrenal Insufficiency

Wardhaugh Barbara S , Reid Jacquie

Background: The Scottish Paediatric Endocrine Managed Clinical Network is committed to providing equity of care across Scotland. A key role identified by the nurses group is developing information leaflets supporting patient care. It was recognized that there was no local unified approach to the management of adrenal insufficiency, especially in the home, community and acute setting. Of particular concern was the lack of a pathway of care from home to hospital and involvement ...

hrp0082p1-d3-20 | Adrenals &amp; HP Axis (1) | ESPE2014

Salivary Cortisol as a Diagnostic Tool of Hypercortisolism in Primary Pigmented Nodular Adrenocortical Disease (ppnad)

Moreira Ayrton , Elias Paula , Bittar Marcelo , Martinelli Carlos Eduardo , Antonini Sonir , Castro Margaret

Background: Hypercortisolism due to PPNAD may be cyclical, atypical and may develop suddenly or progressively.Objective and Hypotheses: The performance of salivary cortisol (SF) in this rare cause of Cushing’s syndrome (CS) is lacking.Method: Ten patients (nine F/one M) with PPNAD (two sporadic; eight Carney complex) were evaluated. Among these, six had CS family history, while in two the diagnosis was confirmed by germline PR...

hrp0082p1-d3-21 | Adrenals &amp; HP Axis (1) | ESPE2014

How to Interpret Cortisol Responses to Acth in Patients with Non-Classic Congenital Adrenal Hyperplasia

Martin M Belen Roldan , Barcelli Yoko Oyakawa , Castellanos Raquel Barrio , Frias Maria Martin , Martin Daniel Alonso , Blanco Milagros Alonso

Background: Recent clinical guidelines recommend that all patients with non-classic congenital adrenal hyperplasia (NCCAH) on glucocorticoid therapy (GC) need to be informed about stress doses and suggest the use of GC in the subgroup of patients with low cortisol response during periods of stress even though they are not on GC.Objective and Hypotheses: To study the response of cortisol to ACTH 250 μg i.v. (Synacthen®, Novartis) in p...

hrp0082p1-d3-22 | Adrenals &amp; HP Axis (1) | ESPE2014

Clinical Utility of Urinary Steroid Metabolite Ratios in Children Undergoing Investigations for Suspected Disorders of Steroid Synthesis

Lucas-Herald Angela , Rodie Martina , Lucaccioni Laura , Rankin Karen , Shaikh Guftar , McNeilly Jane , Shapiro David , Ahmed Faisal

Background: Calculation of a urinary steroid metabolite ratio (uSMR) may be a useful method of improving diagnostic yield when investigating disorders of steroid hormone synthesis. Our aim was to investigate the range of uSMR in children with suspected disorders of steroid hormone synthesis.Methods: Ten ratios were calculated on steroid metabolite data analysed by GC-MS in urine samples collected between 2008 and 2010 from 94 children who were undergoing...