Issue 25 – Summer 2014

Issue 26 – Winter 2014

December 13, 2022

Issue 24 – Spring 2014

December 13, 2022

Published by giaro on December 13, 2022

Issue 25 - Summer 2014

Bringing The Latest In Paediatric Endocrinology To You

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<Welcome to issue 25 Dear FrienDs anD Colleagues, We are looking forward to meeting you at the 53rd annual Meeting of esPe in Dublin on 18–20 september, which is only a few weeks away now. We know that this esPe meeting will, yet again, be a memorable event in our academic lives, and will give us the chance to exchange knowledge with colleagues from all over the world and listen to leading experts. We wish every success to the President of the Dublin meeting, Hilary Hoey, and the organising Committee, who have compiled an excellent scientific programme. You can read more details from Professor Hoey on this page. Please mark your calendar for a trip to Dublin, and register if you have not already done so. on pages 4–5 you will also find exciting keynotes from a number of speakers at the esPe meeting, to peak your interest. We thank them for their contributions.

We are grateful to the Hellenic society of Pediatric and adolescent endocrinology for updating us with their activities in our regular column looking at the work of national societies (page 7). We are also delighted to have news from the 5th asPae Conference in Tanzania (page 6). There is much news about other esPe activities, including the very successful 1st esPe Diabetes and obesity school (below) and the esPe Visiting scholarship programme (page 7).

We are delighted to include an update from the Consensus group on rickets led by the esPe Bone and growth Plate Working group (page 3). experts from around the world have developed evidence-based guidelines on the prevention and management of nutritional rickets, a global problem. Page 3 also features a progress report from the accreditation and syllabus subcommittee on the revised esPe Training Programme. This issue’s interview is with Jan lebl, a senior member. on page 6 you can discover what esPe has brought to his academic life.

We also thank gary Butler for his review of a recently published book on auxology by Michael Hermanussen, which will evoke interest among our members. You can read his review on page 7. We, as your editorial Board, will do our best to continue to maintain the quality of the newsletter and try to enrich it with feedback from all members. as always, i thank my colleagues in the newsletter team for their support. We are pleased to welcome our new member, abel lópez-Bermejo from spain, to the editorial Board of the newsletter Yours sincerely, Professor Feyza Darendeliler editor, eSpe newsletter feyzad@istanbul.edu.t

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We are alreaDY HalFWaY through the year, and so many great esPe programmes and activities have taken place: the esPe Winter school, the esPe Diabetes/obesity school, the Consensus Conference on the Prevention and Management of rickets and the advanced seminar in Developmental endocrinology. The esPe research Fellowships and two Visiting scholarships have also already been awarded. To keep up to-date with all of esPe’s programmes and activities, including information on how to apply and deadline dates, please visit our website which is updated regularly with the latest information. The second half of the year promises to be just as busy, with two new schools taking place: the Caucasus & Central asia school, almaty, Kazakhstan in october and the esPe/asPeD school, abu Dhabi, uae, in november. The Maghreb Project also takes place in Morocco in november and, of course, esPe 2014 is in Dublin, ireland, on 18–20 september. esPe 2014 promises to be another excellent scientific and networking occasion for your diary. Please do come and visit us at esPe Connect, stand 1, where we would love almaty, Kazakhstan in october and the esPe/asPeD school, abu Dhabi, uae, in november. The Maghreb Project also takes place in Morocco in november and, of course, esPe 2014 is in Dublin, ireland, on 18–20 september. esPe 2014 promises to be another excellent scientific and networking occasion for your diary. Please do come and visit us at esPe Connect, stand 1, where we would love to meet you and answer any questions or feedback you have about your society. You can expect to find information about membership and esPe’s programmes and activities, and there will be plenty of seats to rest your weary feet! Come and meet your colleagues and the esPe team. Please don’t forget to encourage your colleagues to join esPe, with so many fantastic opportunities and membership categories to suit all medical professionals working in paediatric endocrinology. You can contact us via espe@eurospe.org, or find us on Facebook and Twitter if you have any queries. We look forward to meeting you in Dublin! Hannah Bonnell, Joanne Fox-Evans, Tracey-Leigh Meadowcroft eSpe tea

riCKeTs, HYPoCalCaeMiC seiZures and cardiomyopathy are common in countries with poor primary healthcare standards. These conditions have been successfully eradicated in most developed countries by adherence to universal vitamin D supplementation for infants. The Consensus Conference on Prevention and Management of rickets took place on 29–31 May 2014, in Birmingham, uK, with the aim of establishing global consensus recommendations on prevention and management of nutritional rickets. Wolfgang Högler, Chairman of the esPe Bone and growth Plate Working group, brought together 33 experts from around the world to develop evidence-based guidelines. The consensus conference was endorsed by esPe, Pes, sleP, JsPe, asPae, aPPes, aPeg, isPae, CsPeM and esPgHan*, and included endocrinologists and experts from the fields of nutrition, paediatrics, public health, epidemiology and health economics. The consensus group established definitions for rickets, vitamin D and calcium deficiency; intakes of vitamin D and calcium required for prevention and treatment of rickets and osteomalacia, including in women of child-bearing age, pregnancy and lactation; and identification of risk groups who benefit from screening and supplementation. The group also developed a number of recommendations. Women of child-bearing age, particularly during pregnancy, should meet their recommended intakes of calcium and vitamin D. all infants should be supplemented with 400 iu/day of vitamin D until 12 months of age, and calcium-rich foods should be introduced no later than 6 months. Food fortification with vitamin D is recommended to increase average population intakes to 400 iu/day, a level that would eradicate rickets and osteomalacia. nutritional rickets, a fully preventable disorder, is on the rise worldwide and should be as unacceptable to public health as polio or measles, and thus regarded as a global epidemic. The consensus group advocates eradication of rickets and osteomalacia through implementation of international vitamin D supplementation and food fortification programmes.

THe esPe aCCreDiTaTion anD sYllaBus suBCoMMiTTee was established under the
umbrella of the education and Training Committee (Chair Jan lebl) in 2011. since then, it has
been working to update the 1998 version of the esPe Training Programme. The updated syllabus
should revise, restructure and amend the current guidelines in the light of present practice and
new developments, should evaluate the feasibility of the current tutorial system approach in
view of national differences in paediatric training, and should also focus on minimum but
obligatory requirements.
after liaising with several colleagues from eu countries, obtaining feedback using a
questionnaire and advertisement through the website, esPe newsletter, e-alerts etc., the
subcommittee worked on updating the training syllabus in paediatric endocrinology based on
the recommendations of the european academy of Paediatrics (eaP) and the european Board of
Paediatrics, to present it to the esPe Council. The final version was prepared in 2013, put on the
esPe website for feedback and evaluation, presented at the last esPe meeting in 2013 and put
forward to eaP for approval.
Finally, the revised esPe Training Programme was approved at the last eaP Meeting in May
2014 in the Tertiary Care Working group and in the general assembly of eaP. The latest version
has been published at www.eurospe.org/education/training/esPe-syllabus_May-2013.pdf.

Following this approval, more important
issues will come onto the agenda, such as
methods to bring this training programme
into practice, recognition and accreditation
of the training centres and certification of
the trainees. The accreditation and syllabus
subcommittee will work on these items
before presenting them to members and
colleagues for finalisation.

Bringing you recent highlights from the world of research

good glycaemic control is crucial to prevent diabetes-related complications as well as hypoglycaemic episodes, seizure, coma and death. The Diabetes wireless artificial pancreas consortiuM (DreaM) was established by three diabetes centres in slovenia, germany and israel, with the aim of reducing the risk of hypoglycaemia while improving blood glucose control and reducing patients’ burden of diabetes management by using the MD-logic system. This is a wireless fully automated closed-loop system based on a fuzzy-logic theory algorithm, with learning capability, personalised system settings and safety alert module. since october 2010, in silico studies using the us Food and Drug administration-approved uVa (university of Virginia) simulator (n=300 virtual patients) and clinical studies have been conducted by the three centres, with more than 200 children, adolescents and adults with type 1 diabetes. after the successful conclusion of first feasibility in-hospital studies, a set of prospective randomised controlled, multicentre, multinational, cross-over studies was conducted in hospitals, at diabetes camps and at patients’ homes. We first used the glucositterTM as a solution for nocturnal blood glucose control, and recently a full 24-hour closed-loop system was tested successfully at patients’ homes. results showed the safety and efficacy of the MD-logic system both overnight and during the day. it achieved significantly less hypoglycaemia and tighter overnight glucose control than sensor augmented pump therapy. The MD-logic system may be safely integrated into lives of people with diabetes. The DreaM consortium is now evaluating the system over a longer study period at patients’ homes. Moshe Phillip Schneider Children’s Medical Center of israel, petah tikva, israel Novel therapies for congenital hyperinsulinism C Congenital hyperinsulinism (CHi) refers to a group of conditions characterised by dysregulated insulin secretion from pancreatic beta-cells. The inappropriate insulin secretion leads to severe hyperinsulinaemic hypoglycaemia. at a histological level, there are two major subtypes of CHi: focal and diffuse. The management of focal CHi has been revolutionised by the development of 18F-DoPa-PeT/CT scanning and laparoscopic surgery. Correct pre-operative localisation by 18F-DoPa-PeT/CT scanning and complete surgical removal of the focal lesion can cure the patient of hypoglycaemia. in contrast, the management of diffuse disease is complex and highly challenging for paediatric endocrinologists. until recently, a near-total pancreatectomy was the only option for those with diffuse CHi who did not respond to conventional medical therapies (such as diazoxide and octreotide). a near-total pancreatectomy inevitably leads to post pancreatectomy diabetes mellitus and pancreatic exocrine insufficiency. Thus there is an urgent need to develop novel therapies to avoid this procedure. Current research involves using (a) chaperone agents (such as sulphonylureas) to recruit defective KaTP channels to the beta-cell membrane surface, (b) glucagon-like peptide-1 receptor antagonists to lower caMP levels in defective beta-cells, (c) long-acting octreotide formulations (such as lanreotide), or more recently (d) mTor inhibitor drugs (such as sirolimus) in patients with severe diffuse disease. Khalid Hussain university College London, uK Childhood obesity – new treatments and the imperative for prevention The primary causes of the complex obesity pandemic are clear. energy intake has shifted to high sugar, high salt and high fat foods and drink, pedalled to our children by a food and drinks industry supported by regulatory inaction. Combined with this, physical activity levels have dropped and physical inactivity levels have soared. We know that childhood obesity is three times more common and more extreme in lower socio-economic groups. We are learning that the genes for diabetes and heart disease are switching on, and the genes for cancer defence are switching off, at as early as the age of 6, and in direct proportion to the degree of obesity. These facts, combined with the low self-image and esteem experienced by obese children, must drive the impetus for aggressive preventive measures to be more widely identified and adopted. The scale of rise in childhood obesity demands that treatments be improved. Current conservative weight management interventions can deliver up to 10% weight loss, with bariatric surgery delivering 30% weight loss – and experience in children is increasing. safer, more effective alternatives are needed. The recent recognition and understanding of how the immune system regulates body weight dynamically has opened up new possibilities for therapies. Currently oral bile salt preparations, analogues of glucagon-like peptide-1, fibroblast growth factor-21 and implantable upper gi stents are being studied, and all would be potentially suitable in children. Donal O’Shea St Vincent’s university Hospital and university College dublin, ireland

The McCune-albright syndrome (Mas) can be a disease of striking complexity, the management of which can be challenging. However, an understanding of the physiologic consequences of the underlying molecular and developmental biology makes the evaluation and treatment of this disease relatively straightforward. Mas arises from activating mutations in the ubiquitously expressed caMP-signalling protein, gsα. The mutations occur very early in development, prior to gastrulation. The timing and location of the mutational event dictate the ‘map’ of affected tissues. as such, a thorough phenotyping at presentation allows for the identification of all affected and (just as important) unaffected tissues to track over a patient’s lifetime. identifying affected and unaffected tissues, and understanding the effects of caMP signalling in a given tissue, explain the clinical presentation, help determine treatment, and allow for accurate prognosis (see figure). each patient is different, and the array of tissues affected ranges from few to many. in complex cases, care often involves the co ordinated efforts of multiple subspecialists. Patients suffer and parents are often frustrated by the fragmentation of care that can occur. a fairly comprehensive, evidenced-based literature exists to guide physicians. a concise distillation of this literature will be reviewed in this session, to equip the paediatric endocrinologist with the tools to understand Mas, and to co-ordinate and direct care of this often challenging and complex disease. Michael Collins Skeletal Clinical Studies unit, niH, Bethesda, Md, uSa Advances in therapy for CAH Managing McCune-Albright syndrome The most common form of congenital adrenal hyperplasia (CaH) is classic 21-hydroxylase deficiency, characterised by a defect in cortisol and aldosterone secretion, impaired development and function of the adrenal medulla, and adrenal hyperandrogenism. Treatment aims to provide adequate glucocorticoid and, when necessary, mineralocorticoid substitution, to prevent adrenal crises, and to suppress the excess secretion of androgens and steroid precursors from the adrenal cortex. However, current formulations of hydrocortisone are unable to simulate physiological cortisol secretion, and patients are at risk of developing iatrogenic Cushing’s syndrome and hyperandrogenism in tandem. Treatment optimisation has been attempted using thrice-daily dosing, which still fails to simulate the normal diurnal rhythm of cortisol secretion and results in temporary over- or under-replacement. Proof-of-concept studies using hydrocortisone infusions predict improvement in biochemical control and quality of life. The use of delayed- and extended-release hydrocortisone formulations offers the prospect of more physiological cortisol replacement. recent formulations use a scalable technology based on multiparticulates. The design configuration comprises an inert microcrystalline core coated with a drug layer and then further coated with polymeric layers that modify drug release. Following screening of several multiparticulate formulations, in vitro and in vivo, an optimal formulation has been chosen, which reproduces the overnight rise in cortisol. When given as a twice-daily regimen, this has provided cortisol exposure similar to that seen in physiological cortisol concentrations in a healthy reference population and also in dexamethasone-suppressed healthy volunteers after a single dose of 30mg hydrocortisone. other therapeutic alternatives that might be used in conjunction with substitution therapy include gonadotrophin-releasing hormone analogues, anti-androgens and aromatase inhibitors. Many of these agents still require further evaluation in patients with the classic form of the disease. Evangelia Charmandari aghia Sophia Children’s Hospital, athens, greece

ESPE is now more than 50 years old. Your Society has been uniting paediatric endocrinologists ever since its formation,
aiding communication and education and improving patient care. In this article, we look back at the Society's formative

THe esPe VisiTing sCHolarsHiP offers financial support to members of the society or to their collaborators/team members. This support can be used to gather information and experience regarding a specific research issue or on a laboratory technique in the field of paediatric endocrinology. esPe is very grateful to Pfizer for supporting this very important resource in recent years. it is especially valuable to early stage investigators who are eager to spend a short period of up to 6 weeks abroad in order to improve their skills in research by developing new research methods. The scholarship may be used to transfer knowledge and techniques to the home institution or to connect the researcher with a scientific unit for further collaboration using other funding mechanisms. For the Visiting scholarship, the funding needed for travel and accommodation must be specified, and will be reimbursed up to €2500. in 2013, nine proposals were received and five approved. The application process is straightforward, and applications may be submitted four times each year, on 31 January, 30 april, 31 July and 31 october (see www.eurospe.org/awards/ awards_visitingscholar.html for further details). The applications are evaluated by the Visiting scholarship Committee, currently comprising agnes linglart (Paris, France), Vallo Tillmann (Tartu, estonia), and olaf Hiort (lübeck, germany; Chair). Olaf Hiort Auxology: Studying Human Health and Development Michael Hermanussen (editor & Lead author), Schweizerbart, germany, 2013, 324 pp, €39.90, iSBn 978-3-510-65278-5 WHo KnoWs WHaT auxologY Means? if you are reading this review as a paediatric endocrinologist then you probably do. But i bet you think of textbooks on auxology as thick and dull, full of incomprehensible formulae and tables, and best employed as a doorstop. Well this is so completely different that perhaps it should have been called ‘everything sexy about growth’ to liven up interest! so, why is it different? Well it has lots of bright, easy to understand diagrams, photos, funny cartoons, and imaginary letters and conversations to break up the text, which is in bite-size chunks, and full of coloured words and bold phrases to aid skim-readers. although sometimes verging on being light on facts, you can generally get all the information you need and be pointed to the original sources in a huge bibliography if you want more detail. each topic is covered by a two-page spread which opens in front of you, and one section leads on from another. all the areas you expect to be covered are there, fully updated. other new ones, like face shape analysis, are completely fascinating. The many bright and clear diagrams make excellent teaching aids and make the book fun to read. For new readers there is short section on growth statistics for bunnies – easy to follow, but unfortunately parametric and regression analysis gets six pages of unadulterated formulae and text. it’s old fashioned auxology, but this is the only transgression. it is good as bedtime reading and a clinic manual with many useful tables and references. so you think you know all about growth as a paediatrician then? i think you would find some useful new knowledge on every page, so it is a recommended read. auxology is back in fashion! Gary Butler, London, uK eeπee, the Hellenic society of Pediatric and adolescent endocrinology, is a ‘young society’, founded in 1995 and currently numbering 51 members, led by President george P Chrousos. The eeπee is not organised around multisite collaborative working groups, unlike many other national societies, but concentrates on paediatric endocrinology training in the Division of endocrinology, Metabolism and Diabetes, First Department of Pediatrics at the university of athens, and in athens’ Children’s Hospital aghia sophia, as well as in other national centres. similarly, the performance of many special endocrine studies is centralised at the Choremeion research laboratory (university of athens), at the Biomedical research Foundation of the academy of athens and at other university, public health service and private laboratories. eeπee holds an annual meeting. The national accreditation in paediatric endocrinology is under development, as is the elaboration of national guidelines following the esPe syllabus. For further information contact george P Chrousos, eeπee President, at e.e.p.e.endo@gmail.com.

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Issue 25 – Summer 2014

Issue 26 – Winter 2014

Issue 24 – Spring 2014

Issue 25 - Summer 2014

Bringing The Latest In Paediatric Endocrinology To You

Editorial

Welcome

News

Intro

News

ESPE Update

News

Consensus recommendations on rickets

News

Accreditation and Syllabus Subcommittee

ESPE Activities

ESPE Activities

In anticipation of ESPE 2014...

ESPE Activities

Managing McCune-Albright syndrome

ESPE Review

ESPE Review

ESPE Visiting Scholarship

giaro

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