hrp0097p1-118 | Growth and Syndromes | ESPE2023

The effect of growth hormone therapy on body composition in girls with Turner syndrome – a 10-year follow-up.

Błaszczyk Ewa , Shulhai Anna-Mariia , Gieburowska Joanna , Gawlik Aneta

Background: Obesity and a thicker layer of subcutaneous adipose tissue are more common in Turner syndrome (TS), however, it is assumed that growth hormone (GH) treatment has beneficial effect on their body composition (BC). Objective: To investigate the effect of GH therapy on BC and its relation with metabolic syndrome components in girls with TS during a 10 year-follow-up.Patients and Methods: 21 girls with TS were des...

hrp0097p1-505 | Growth and Syndromes | ESPE2023

Changes in carbohydrate and lipid metabolism during 10-year follow-up of patients with Turner syndrome treated with growth hormone.

Błaszczyk Ewa , Shulhai Anna-Mariia , Gieburowska Joanna , Gawlik Aneta

Background: Disorders in carbohydrate and lipid metabolism are more common in girls with Turner syndrome (TS) than in general population. Metabolic disorders have been identified as risk factors for the development of cardiovascular diseases. Additionally, TS patients are usually treated many years with growth hormone (GH) that affects the parameters of carbohydrate-lipid metabolism. Therefore cardiovascular risk factors should be monitored in TS girls.<p ...

hrp0092p2-148 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Neonatal Hyperglycemia

Bochkova Larisa , Gumeniuk Olga

Hyperglycemia in newborns is most common in premature infants. At present, in clinical practice, the attitude towards this type of metabolic disorders is not well defined and controversial.Objective: To determine the feasibility of prescribing insulin for hyperglycemia in premature newborns.Patients and Methods: We observed 68 newborns with a birth weight of 1326 ± 119.8 g and a gestational ag...

hrp0094p2-130 | Diabetes and insulin | ESPE2021

Blood lipid concentrations of newborns and their mothers with gestational diabetes

Bochkova Larisa , Gumeniuk Olga ,

It is noted that with gestational diabetes mellitus, lipids cross the placenta in a limited amount. It is assumed that their concentration in maternal blood correlates with the concentration of lipids in the fetus. The purpose of this study was to compare the levels of the main indicators of the lipid profile, such as cholesterol, triglycerides and high and low density lipoproteins, in newborns and similar indicators in their mothers with gestational diabetes.<p class="abs...

hrp0086fc13.6 | Management of Obesity | ESPE2016

Treatment for Early Onset and Extreme Obesity in Two POMC Deficient Patients: Successful Weight Loss with the Melanocortin-4 Receptor Agonist Setmelanotide

Kuhnen Peter , Clement Karine , Gottesdiener Keith , Fiedorek Fred , van der Ploeg Lex , Wiegand Susanna , Blankenstein Oliver , Gruters Annette , Krude Heiko

Background: POMC deficiency is an extremely rare monogenetic obesity syndrome generally characterized by early onset hyperphagia, red hair and adrenal insufficiency. So far treatment of obesity and hyperphagia with MSH substitution failed, either due to ineffectiveness or side effects of available MC4R agonists.Objective and hypotheses: We hypothesized that the new MC-4R agonist setmelanotide might be a treatment option in POMC deficient patients.<p ...

hrp0082fc5.5 | Neuroendocrinology | ESPE2014

Characterization of IGFI Receptor Expression and Localization in Paediatric Gliomas Upon Diagnosis According to WHO 2007 Grading

Clement Florencia , Venara Marcela , Maglio Silvana , Martin Ayelen , Matho Cecilia , Petre Cesar , Lombardi Mercedes Garcia , Bergada Ignacio , Pennisi Patricia

Background: Gliomas are the most common subgroup of CNS tumours in children. Histologic grading is a means of predicting the biological behavior of these tumours and survival is strongly correlated with tumour gradation. The IGF system of ligands and receptors are known to play an important role in both normal and neoplastic growth. Recently, nuclear translocation of the type 1 IGF1R has been demonstrated in tumour tissues. Although the IGF1R expression has been described in C...

hrp0097p1-65 | Fat, Metabolism and Obesity | ESPE2023

Natural history of corpulence in patients carrying heterozygous pathogenic variants in the five major genes of the leptin-melanocortin pathway.

Nicaise Clarisse , Dubern Béatrice , Poitou Christine , Clement Karine , Tounian Patrick , Lemale Julie , COURBAGE Sophie , Le Bihan Johanne

Introduction: The leptin-melanocortin pathway plays a key-role in weight control. Pathogenic variants in the five major genes (LEP, LEPR, POMC, PCSK1, MC4R) are associated with early severe obesity. However, the specific associated phenotype with presence of mono-allelic variants and especially BMI trajectories are not well known.Objective: In order to identify specific profiles, we compared BMI trajectories during the f...

hrp0097p1-127 | Growth and Syndromes | ESPE2023

Effects and safety of growth hormone (GH) treatment in 6 children with pycnodysostosis

Renes Judith , Sas Theo , Clement-de Boer Agnes , Zwaveling-Soonawala Nitash , van der Kaay Danielle , Hokken-Koelega Anita

Background: Pycnodysostosis is a very rare autosomal recessive skeletal dysplasia caused by cathepsin K deficiency. It is characterized by extreme short stature resulting in an adult height in males typically <150 cm and in females <134 cm. Bone-fragility and frequent fractures are present. There are few case-reports on the effects of GH treatment.Objective: To evaluate the effect and safety of GH in 6 patients wit...

hrp0095p2-45 | Bone, Growth Plate and Mineral Metabolism | ESPE2022

Adult weight and BMI associate with higher bone mineral density in the lumbar spine and femoral neck in young adults born preterm in the surfactant era

Bruun Ella , Pätsi Pauli , Björkman Krista , Leskinen Markku , Tulppo Mikko , Kulmala Petri , Valkama Marita , Ojaniemi Marja

Background: Vitamin D is a steroid hormone which contributes to the maintenance of calcium homeostasis and bone mineralization. As bone mineral accretion occurs mostly during the third trimester of pregnancy, mineral storages are deficient especially in the extremely low birth weight (ELBW <1000g) preterm infants at birth. Recent studies suggest that ELBW is a risk factor for compromised bone mineral density at adulthood, but the role of neonatal morbidity ...

hrp0086p1-p26 | Adrenal P1 | ESPE2016

Sex-Specific Differences in Hypothalamus-Pituitary-Adrenal Axis Activity in Newborns with Very Low Birth Weight

van der Voorn Bibian , de Waard Marita , Rotteveel Joost , Hartmann Michaela , van Goudoever Johannes , Lafeber Harrie , Wudy Stefan , Finken Martijn

Background: Male preterm infants are at increased risk of neonatal mortality when compared to their female counterparts. The mechanisms explaining this male disadvantage are not fully elucidated yet.Objective and hypotheses: To compare glucocorticoid metabolite excretion in urine obtained at day 10 between male and female infants born with a very low birth weight (VLBW; i.e. <1500 g). We hypothesized that male preterm infants have impaired adrenocort...