hrp0084fc9.6 | Beta cell disorders | ESPE2015

Pharmacokinetics of a New Suspension of Glibenclamide for Use in Young Patients and Infants with Neonatal Diabetes

Beltrand Jacques , Busiah Kanetee , Berdugo Marianne , Treluyer Jean-Marc , Elie Caroline , Polak Michel

Background: Sulfonylurea therapy allows a better metabolic control than insulin in patients with neonatal diabetes secondary to mutation in potassium channel. Its galenic form (tablets) is not suitable for children, as the dosage can’t be easily modulated and as it induces large pharmacokinetics (PK) variations when administer to young children.Objective and hypotheses: To measure relative biodisponibility of a new galenic form of glibenclamide and ...

hrp0094fc7.3 | Fetal Endocrinology and Multisystem Disorders | ESPE2021

European Training Requirements for Competency in Paediatric Endocrinology and Diabetes 2021

Busiah Kanetee , Peet Aleksandr , Tornese Gianluca , Weintrob Naomi , Patel Leena , Hamza Rasha ,

Background: A Europe-wide training program in Paediatric Endocrinology and Diabetes (PED) was initiated by Professor Leo Van den Brande in 1976. PED was recognised as a subspecialty in 1996 by the European Board of Paediatrics (EBP), which is a section of the Union of European Medical Specialists (UEMS). UEMS aims to ensure high standards for training across Europe and the minimum requirements are set out in the European Training Requirements (ETR). The first ...

hrp0092p1-424 | Thyroid (2) | ESPE2019

Transition for Patients with Chronic Thyroid Diseases

Ratnasabapathy Piriya , Bouthors Thérèse , Antoniou Maria-Christina , Stoppa-Vaucher Sophie , Elowe-Gruau Eglantine , Busiah Kanetee , Hauschild Michael

Background: Children and adults with chronic thyroid disorders (TD) need continuous monitoring as periods of inadequate thyroid hormone substitution can impact metabolism, puberty and fertility. Transition from pediatric to adult-oriented care is often characterized by discontinuity in care resulting in poor health outcomes and impaired quality of life. Transition care (TC) for young adults with TD remains largely unknown.Objecti...

hrp0086fc5.4 | Management of Disorders of Insulin Secretion | ESPE2016

Persistent Beneficial Metabolic Effect after Five Years in a Cohort of 28 Subjects with Neonatal Diabetes owing to Potassium Channel Mutation and Transferred from Insulin to Sulfonylureas

Hoarau Marie , Fauret Anne-Laure , Busiah Kanetee , Godot Cecile , Colas Sandra , Treluyer Jean-Marc , Scharfmann Raphael , Cave Helene , Polak Michel , Beltrand Jacques

Background: Sulfonylureas (SU) have proven to be effective in patients with monogenic diabetes owing to potassium channel mutation. They allow the discontinuation of insulin and a good metabolic control. Long-term data arguing for a persistent beneficial effect of SU are missing.Objective and hypotheses: SU provide a good metabolic control maintained over time in patients with neonatal diabetes.Method: From a French cohort of 34 pa...

hrp0084p2-469 | Growth | ESPE2015

Major Improvement in Parental Perception of their Children’s Height-Specific Quality of Life after 1 Year of GH Treatment: Our Experience with the QoLiSSY Questionnaire

Gonzalez-Briceno Laura Gabriela , Viaud Magali , Flechtner Isabelle , Dassa Yamina , Samara-Boustani Dinane , Thalassinos Caroline , Pauwels Christian , Beltrand Jacques , Busiah Kanetee , Pinto Graziella , Polak Michel

Background: Short stature may be a source of social and affective stress in children and their parents, and thus impact negatively on their quality of life (QoL). Treatment by GH may improve QoL through normalisation of height.Objective: To evaluate height-specific QoL in short stature children after 1 year of GH treatment.Methods: Prospective study. Inclusion criteria were: having started GH treatment at Hôpital Necker-Enfant...

hrp0092lb-11 | Late Breaking Posters | ESPE2019

Height in Inborn Errors of Metabolism Requiring Hypoprotidic Diet: A Longitudinal Follow Up Study About 213 Patients

Busiah kanetee , Roda Célina , Brassier Anaïs , Pontoizeau Clément , Ottolenghi Chris , Piketty Marie , Crosnier Anne-Sophie , Perin Laurence , Le Bouc Yves , Netchine Irène , De Lonlay Pascale

Background: Protein intake is crucial for growth. Many inherited metabolic diseases (IMD) require a strict controlled protein diet.Aim: to evaluate growth, pubertal status and body composition in IMD requiring a strict controlled protein diet.Patients & Méthods: Longitudinal follow up cohort study. We recorded data before 4years (early childhood, n=189); betwe...

hrp0086fc5.3 | Management of Disorders of Insulin Secretion | ESPE2016

Glibentek, a New Suspension of Glibenclamide for Patients with Neonatal Diabetes, is as Effective and more Convenient than Crushed Tablets

Beltrand Jacques , Godot Cecile , Busiah Kanetee , Djerada Zoubir , Baron Sabine , Tallec Claire Le , Tessier Raphael , Ribault Virginie , Cartigny Maryse , Bruel Henri , Gozalo Claire , Treluyer Jean-Marc , Elie Caroline , Polak Michel

Background: Glibenclamide has proven to be efficient for patients with neonatal diabetes owing to potassium channel mutations. Anyway its pharmaceutical form is not suitable for young children or infants. The tablets dosage is too high for most infants and must be crushed and diluted before administration. We developed a suspension of glibenclamide (EMA Orphean drug designation january 2016) fitting recommendations of drug administration to allow a precise dosage and designed ...

hrp0086rfc5.4 | Management of Disorders of Insulin Secretion | ESPE2016

“Transient” Neonatal Diabetes In Adulthood: Metabolic Outcomes

Busiah Kanetee , Baz Baz , Lebourgeois Fleur , Djoudi Malek Ait , Bachere Nadege , Bourron Olivier , Ythier Hubert , Pouvreau Nathalie , Bellanne-Chantelot Christine , Vialettes Bernard , Gourdy Pierre , Hartemann Agnes , Robert Jean-Jacques , Cave Helene , Polak Michel , Gautier Jean-Francois

Background: “Transient” Neonatal Diabetes Mellitus (TNDM) is a rare genetic beta cells dysfunction leading to hyperglycaemia that resolves in early childhood. About 80% of patients relapse during adolescence or adulthood. Glucose homeostasis had not been investigated in adulthood.Objective and hypotheses: To investigate insulin secretion and insulin sensitivity in adults affected with TNDM or in their 1st degree mutated relatives.</p...

hrp0082fc9.6 | Beta cells | ESPE2014

Sulfonylurea Therapy Corrects Hypotonia, Attention Deficits, Improves Complex Neuropsychological Functions and Motricity in Patients with Neonatal Diabetes Secondary to Mutation in Potassium Channel Subunits, Through a CNS Effect

Beltrand Jacques , Vaivre-Douvret Laurence , Busiah Kanetee , Fournier Emmanuel , Boddaert Nathalie , Vera Myriam , Bahi-Buisson Nadia , Bui-Quoc Emmanuel , Ingster-Moati Isabelle , Flechtner Isabelle , Simon Albane , Scharfmann Raphael , Cave Helene , Elie Caroline , Polak Michel

Background: Sulfonylurea therapy (SU) allows a better metabolic control than insulin in patients with neonatal diabetes secondary to mutation in potassium channel subunits (ND-K). Most of these patients have neurological and neuromotor developmental impairments whose changes under SU has not been studied in a systematic and prospective way in a large cohort.Objective and Hypotheses: To demonstrate the beneficial effect of SU on neuropsychological functio...

hrp0082fc9.5 | Beta cells | ESPE2014

Transient Neonatal Diabetes in Adulthood: Metabolic and Neurodevelopmental Outcomes

Le Bourgeois Fleur , Busiah Kanetee , Abu-Amara-Olivieri Sawsan , Bachere Nadege , Bertrand Anne-Marie , Bourron Olivier , David Paul , De Boisvilliers Fabienne , Deumier Bernard , deVries Liat , Jelliman Stephanie , Le Tallec Claire , Martin-Dessila Amelie , Nimri Paul , Paoli Anne , Perrin Mireille , Stuckens Chantal , Ythier Hubert , Pouvreaux Nathalie , Bellane-Chantelot Christine

Background: Transient neonatal diabetes mellitus (TNDM) is a rare genetic β-cell dysfunction leading to hyperglycaemia that resolves in early childhood. About 80% of patients relapse during adolescence or adulthood. Some of these patients suffer from neurodevelopmental defect. Long-term outcome has been poorly investigated.Objective and Hypotheses: To investigate metabolic and neurologic outcomes in adults affected with TNDM.M...