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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...

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hrp0097t18 | Section | ESPE2023

Pubertal origin of growth retardation in Inborn Errors of Protein Metabolism: A longitudinal cohort study

Busiah Kanetee , Roda Célina , Crosnier Anne-Sophie , Brassier Anaïs , Wicker Camille , Ottolenghi Chris , Pontoizeau Clément , Souberbielle Jean-Claude , Piketty Marie-Liesse , Perin Laurence , Le Bouc Yves , Arnoux Jean-Baptiste , Imbard Apolline , Netchine Irène , de Lonlay Pascale

Background: Inherited amino-acid metabolism disorders (IAAMDs) require lifelong restriction of natural dietary proteins. Nutritional amino-acid mixtures (AMM) free of the poorly metabolised amino-acids by the enzyme block, improve nutrition. An adequate protein intake is crucial to ensure normal body development, notably during puberty. We aimed to: 1/ describe growth and puberty in IAAMDs 2/ investigate associations linking height, IGF1 and IGFBP3 with AAM an...

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ESPE Abstracts

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

Pubertal origin of growth retardation in Inborn Errors of Protein Metabolism: A longitudinal cohort study

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