hrp0092p1-231 | Growth and Syndromes (to include Turner Syndrome) (1) | ESPE2019

Height and Weight Dynamics in Preschool Boys with Constitutional Delay of Growth and Puberty

Reinehr Thomas , Hoffmann Elisa , Rothermel Juliane , Lehrian Thersia , Binder Gerhard

Background: Constitutional delay of growth and puberty (CDGP) is one of the most frequent norm variants in children presenting with short stature. Knowing the height, growth, and weight pattern of CDGP in the first years of life is important to distinguish CDGP from growth hormone deficiency (GHD) or other diseases.Methods: We studied height and weight in the first 5 years of life in 54 boys with CDGP including measureme...

hrp0092p1-380 | Growth and Syndromes (to include Turner Syndrome) (2) | ESPE2019

A New Model of Adult Height Prediction Validated in Boys with Constitutional Delay of Growth and Puberty

Reinehr Thomas , Hoffmann Elisa , Rothermel Juliane , Lehrian Theresia , Binder Gerhard

Background: For children with retarded bone ages such as in constitutional delay of growth and puberty (CDGP) there are no specific methods to predict adult height based on bone age. Widely used methods such as Bayley-Pinneau (BP) tend to overestimate adult height in CDGP. Therefore, we aimed to develop a specific adult height prediction model for boys in pubertal age with retarded bone age >1 year.Methods</...

hrp0086p1-p206 | Diabetes P1 | ESPE2016

When to Screen for Coeliac Disease in Children with Type 1 Diabetes Mellitus: The Controversy

Ajanaku Ayo , Gorst Thomas , Ajanaku Deji , Chizo Agwu Juliana

Background: Routine screening for Coeliac disease (CD) beyond the first year of diagnosis with Type 1 Diabetes Mellitus (T1DM) is controversial due to a paucity of high-quality evidence. The UK guidelines (NICE) only recommend screening at diagnosis with T1DM or if subsequently symptomatic; whereas the International Society for Paediatric and Adolescent Diabetes (ISPAD) recommends routine screening every 1 to 2 years.Objective and hypotheses: We hypothes...

hrp0086p1-p232 | Diabetes P1 | ESPE2016

Extrahepatic Biliary Atresia in Combination with Toxic Cholestasis Due to Glibenclamide in a Case of Neonatal Diabetes

Kapellen Thomas , Flemming Gunter , Bartelt Heike , Wachowiak Robin , Kiess Wieland

Background: More than 20 gene loci are known to cause monogenic neonatal diabetes today. A definite mutation can be found in 65–70% of all cases. Mutations in the ATP sensitive potassium channel can frequently be treated by sulfonylurea. Glibenclamide is on of the drugs known to inhibit the bile salt export pump (BSEP). However most drug induced cholestasis cases are reported in adults.Objective and hypotheses: Glibenclamide is used frequently to tr...

hrp0086p1-p473 | Fat Metabolism and Obesity P1 | ESPE2016

Inherited Duplication (X) (p11.4) Associated with Obesity, Autoaggressive Behaviour and Delayed Speech Development

Doeing Carsten , Rahner Nils , Kummer Sebastian , Meissner Thomas , Mayatepek Ertan

Background: Obesity is a major feature in several syndromes. In patients with early-onset severe obesity, 7% harbour a single locus mutation.Objective and hypotheses: We report a 3.11 year old male patient with early onset obesity (BMI 29.9 kg/m2 >>P97), ongoing excessive weight gain, autoaggressive behaviour, and delayed speech development. No growth retardation or further dysmorphic signs. Early postnatal feeding difficulties require...

hrp0086p1-p595 | Growth P1 | ESPE2016

The Diagnostic Value of IGF-II, IGF-I and IGFBP-3 in Silver–Russell Syndrome

Binder Gerhard , Eggermann Thomas , Weber Karin , Schweizer Roland

Background: Recently we described a family with several members having intrauterine and postnatal growth failure as well as signs of Silver–Russell syndrome (SRS) who carried a heterozygote nonsense mutation of IGF2. The patients had low IGF-II serum levels, but normal IGF-I serum levels.Objective and hypotheses: We aimed to estimate the diagnostic value of the IGF-II, IGF-I and IGFBP-3 measurements in the assessment of children with SRS.<p clas...

hrp0082p1-d2-153 | Growth (1) | ESPE2014

Intima Media Thickness in Children Treated With GH

Knop Caroline , Wolters Barbara , Lass Nina , Wunsch Rainer , Reinehr Thomas

Background: The cardiovascular risk for children receiving treatment with GH has hardly been investigated. Therefore, we studied the relationships between GH treatment and carotid intima media-thickness (IMT), which is predictive for the cardiovascular diseases.Methods: We measured carotid IMT (four values) in 100 children (mean age 11.6±2.8 years, 63% male) treated with GH (GH deficiency 61%, SGA 31%, Turner syndrome 5%, SHOX deficiency 2%, Prader-...

hrp0084p1-23 | Diabetes | ESPE2015

Safety and Efficacy of Treatment with Long-Acting Lanreotide Autogel® in Early Infancy in Patients with Congenital Hyperinsulinism

Corda Heike , Meissner Thomas , Kummer Sebastian , Welters Alena , Teig Norbert

Background: Long-acting somatostatin analogues have been reported to be an effective treatment option to prevent severe hypoglycaemia in children with severe diffuse congenital hyperinsulinism (CHI). Possible side effects include gallstones, growth retardation and necrotizing enterocolitis (NEC), the latter occurring in particular cases of newborns treated with octreotide. So far only short-acting octreotide is being used in early infancy, requiring multiple injections daily o...

hrp0084p2-474 | Growth | ESPE2015

Growth Curves for Height, Weight, BMI and Head Circumference in Children with Achondroplasia

Merker Andrea , Hagenas Lars , Hertel Thomas , Neumeyer Lo

Background: Close monitoring of growth is vital when following children with achondroplasia yet existing growth curves suffer from a simple chart format and their clinical use is therefore limited. Also, references for body proportions; i.e. sitting height, relative sitting height and arm span, are lacking.Objective and hypotheses: The aim of this study was to construct age-specific growth curves for height, weight, body mass index, head circumference an...

hrp0084p3-936 | GH &amp; IGF | ESPE2015

Adherence to GH Treatment: Impact of Actual Height, Treatment Duration, and Puberty

Rothermel Juliane , Scheite Karl , Nazari Nadine , Hauffa Berthold , Reinehr Thomas

Background: Adherence to GH treatment is a challenge.Objective and hypotheses: We analysed the impact of treatment duration, treatment success, treatment indication, age, gender, pubertal stage, and height on treatment adherence (TA) to optimise treatment success.Method: Based on the easypod autoinjector used in the Saizen-online prospective, multicenter, open-label, noninterventional study we analyzed TA in 6 months periods. TA wa...