ISSN 1662-4009 (online)

ey0021.8-6 | Important for Clinical Practice | ESPEYB21

8.6. Glucose. pattern in children with classical congenital adrenal hyperplasia: evidence from continuous glucose monitoring

A Galderisi , D Kariyawasam , A Stoupa , AN Quoc , G Pinto , M Viaud , S Brabant , J Beltrand , M Polak , D Samara-Boustani

Brief Summary: This study investigated daily glucose patterns in young children with classic congenital adrenal hyperplasia (CAH) and their relation with hormonal circadian rhythm.Comment: The aim of treatment in classical CAH is to provide adequate glucocorticoid and – when necessary – mineralocorticoid replacement to prevent adrenal crises and suppress excess adrenal androgen production. Glucocorticoids regulate glucose homeostasis, and patie...

ey0018.2-8 | Neonatal diabetes mellitus | ESPEYB18

2.8. Long-term Follow-up of Glycemic and Neurological Outcomes in an International Series of Patients With Sulfonylurea-Treated ABCC8 Permanent Neonatal Diabetes.

P Bowman , F Mathews , F Barbetti , MH Shepherd , J Sanchez , B Piccini , J Beltrand , LR Letourneau-Freiberg , M Polak , SAW Greeley , E Rawlins , T Babiker , NJ Thomas , E De Franco , S Ellard , SE Flanagan , AT Hattersley , Neonatal Diabetes International Collaborative Group

Diabetes Care. 2021 Jan;44(1):35–42. doi: 10.2337/dc20-1520. PMID: 33184150.The key findings from this cohort of patients with ABCC8 neonatal diabetes mellitus (NDM) are: A) good glycaemic control is maintained over the long-term without any serious adverse events (including severe hypoglycaemia) despite high doses of sulphonylurea, B) some patients show improvements in neurologica...

ey0017.2-9 | Neonatal Diabetes Mellitus | ESPEYB17

2.9. Long-term metabolic and socio educational outcomes of transient neonatal diabetes: A longitudinal and cross-sectional study

F Le Bourgeois , Baz B Beltrand J , Riveline JP Julla JB , A Simon , I Flechtner , M Ait Djoudi , AL Fauret-Amsellem , Y Vial , R Scharfmann , J Sommet , P Boudou , H Cave , M Polak , JF Gautier , KTNDM Long-Term Follow-Up Study Group Busiah

To read the full abstract: Diabetes Care. 2020 Apr 9. pii: dc190324. doi: 10.2337/dc19-0324. [Epub ahead of print]. PMID: 32273272TransientNeonatalDiabetes (TNDM) develops in the first six months of life, and then remits only to relapse again during adolescence and adulthood. The three main genetic causes of TNDM are: 1) 6q24 abnormalities, 2) activating mutations in genes encoding the ATP-sensi...

ey0016.2-8 | Neonatal Diabetes Mellitus | ESPEYB16

2.8. Effectiveness and safety of long-term treatment with sulfonylureas in patients with neonatal diabetes due to KCNJ11 mutations: an international cohort study

P Bowman , AE Sulen , F Barbetti , J Beltrand , P Svalastoga , E Codner , EH Tessmann , PB Juliusson , T Skrivarhaug , ER Pearson , SE Flanagan , T Babiker , NJ Thomas , MH Shepherd , S Ellard , I Klimes , M Szopa , M Polak , D Iafusco , AT Hattersley , PR Njolstad , Neonatal Diabetes International Collaborative Group. Collaborators (113)

To read the full abstract: Lancet Diabetes Endocrinol. 2018 Aug;6(8)This study describes a 10-year follow-up of a large international multicenter cohort of patients with KCNJ11 permanent neonatal diabetes. It addresses key questions relating to long-term efficacy and safety of sulfonylureas in these patients.The discovery that mutations in the KCNJ11</...

ey0021.1-13 | New Treatments and Hopes | ESPEYB21

1.13. Long-term weight gain in children with craniopharyngioma

S Rovani , V Butler , D Samara-Boustani , G Pinto , L Gonzalez-Briceno , Quoc A Nguyen , G Vermillac , A Stoupa , A Besancon , J Beltrand , C Thalassinos , I Flechtner , Y Dassa , M Viaud , MB Arrom-Branas , N Boddaert , S Puget , T Blauwblomme , C Alapetite , S Bolle , F Doz , J Grill , C Dufour , F Bourdeaut , S Abbou , L Guerrini-Rousseau , A Leruste , K Beccaria , M Polak , D Kariyawasam

Brief Summary: This single-centre retrospective cohort study offers valuable insights into the trajectory of weight gain in a paediatric craniopharyngioma cohort over a mean follow-up period of 10.4 years, reinforcing the necessity for targeted interventions to address this issue.Craniopharyngioma poses a substantial clinical challenge in paediatric patients, primarily due to the risk of hypothalamic involvement. A particularly severe long-term consequen...

ey0021.9-13 | Growth Problems in Cancer Survivors | ESPEYB21

9.13. GH and childhood-onset craniopharyngioma: when to initiate GH replacement therapy?

A Nguyen Quoc , K Beccaria , Briceno L Gonzalez , G Pinto , D Samara-Boustani , A Stoupa , J Beltrand , A Besancon , C Thalassinos , S Puget , T Blauwblomme , C Alapetite , S Bolle , F Doz , J Grill , C Dufour , F Bourdeaut , S Abbou , L Guerrini-Rousseau , A Leruste , S Brabant , I Cavadias , M Viaud , N Boddaert , M Polak , D Kariyawasam

Brief Summary: This retrospective, observational, single centre study compared the risk of tumor progression or recurrence in 71 patients with childhood-onset craniopharyngioma (CP) who started GH replacement therapy (GHRT) with different latency from the end of tumor treatment.GHRT latency was defined as the time from end of CP treatment (last debulking procedure or radiotherapy, or from CP diagnosis for the 5 patients without any debulking procedure or...