hrp0089rfc15.4 | Growth and syndromes | ESPE2018

Characteristics, Effectiveness and Safety Data from Clinically Relevant Subgroups of Patients with Severeprimary IGF-I Deficiency (SPIGFD): Results from the European Increlex® Growth Forum Database (EU-IGFD) Registry

Woelfle Joachim , Polak Michel , Perrot Valerie , Sert Caroline , Bang Peter

Background: The EU-IGFD registry was established to monitor the safety and effectiveness of recombinant human IGF-I (rhIGF-I) (mecasermin (rDNA origin) injection; Increlex®) for short stature in children with SPIGFD, including those with Laron syndrome (LS).Objective: To report patient characteristics, effectiveness and safety data in clinically relevant patient subgroups.Methods: Data were compiled from this ongoin...

hrp0089p1-p184 | Growth & Syndromes P1 | ESPE2018

Characteristics, Effectiveness and Safety Data for Patients with Growth Failure Treated with Recombinant IGF-I (rhIGF-I) and Achieving Adult or Near-adult Height (AH): Results from the European Increlex® Growth Forum Database (EU-IGFD) Registry

Polak Michel , Woelfle Joachim , Perrot Valerie , Sert Caroline , Bang Peter

Background: The EU-IGFD registry comprises data for children with severe primary IGF-I deficiency (SPIGFD) receiving rhIGF-I (mecasermin [rDNA origin] injection; Increlex®) for growth failure.Objective: To report patient characteristics, effectiveness and safety data for children receiving rhIGF-I for SPIGFD and achieving AH.Methods: Patients from this ongoing observational study (NCT00903110) were included in analy...

hrp0089p2-p337 | Pituitary, Neuroendocrinology and Puberty P2 | ESPE2018

A Case of Testotoxicosis Due to a Constitutive Mutation of the LH Receptor Initially Presented as a Central Precocious Puberty at 3 Years Old

Porquet-Bordes Valerie , Pienkowski Catherine , Roux Nicolas de

Background: A thirty-four months old boy was referred for precocious puberty. He was the first child of healthy non-consanguineous parents. His family history was unremarkable. He had no exposure to oestrogenic endocrine-disrupting chemicals. He had presented secondary sexual characteristics for five months: pubic hair, enlarged testicular volume to 6 ml (Tanner stage P2A1G2) and enlarged penile size. He had a deepening voice and aggressive behavior. He had a significant growt...

hrp0086rfc6.7 | Syndromes: Mechanisms and Management | ESPE2016

Characteristics of Responders and Poor-responders to Increlex® Therapy – Data from Children Enrolled in the European Increlex® Growth Forum Database (EU-IGFD)

Bang Peter , Polak Michel , Woelfle Joachim , Perrot Valerie , Sert Caroline

Background: The post-authorization registry, EU-IGFD, was initiated in Dec-2008 to collect data in children with growth failure receiving Increlex® (Mecasermin [rDNA Origin] Injection).Objective and hypotheses: To report patient characteristics, safety and effectiveness data in poor-responders (i.e. with change in year 1 Height SDS <0.3).Method: European, multicentre, open-label, observational study; eCRF data collection.<...

hrp0084p3-693 | Diabetes | ESPE2015

Blood vs Urine Ketone Monitoring in a Pediatric Cohort of Patients with Type 1 Diabetes: a Crossover Study

Goffinet Line , Barrea Thierry , Vandooren Valerie , Lysy Philippe

Background: Diabetes ketoacidosis (DKA) is the most severe complication in type 1 diabetes (T1D) but patient education and ketone monitoring may help decrease its frequency. However, the influence on glucose homeostasis of systematic ketone monitoring and of the nature of monitoring (urine vs blood) is unclear.Objective and hypotheses: To determine whether the use of blood ketone monitoring, as compared to urine ketone testing, decreases the duration of ...

hrp0095p1-581 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2022

A case series of granulosa cell tumors of the ovary

Giuca Diandra , Dumitru Niculina , Scurtu Cristian , Vladoiu Suzana , Dumitrica Alina , Boboc Madalina , Procopiuc Camelia , Gherlan Iuliana

Introduction: Granulosa cell tumors (GSTs) of ovary are very rare, usually large gonadic neoplasms arising from the mesenchyme and sex cords. The main clinical appearance of the juvenile GCTs is represented of signs of excessive estrogen (Precocious puberty - 75% of cases) or testosterone secretion (rare).Aim: To present the different clinical presentation and outcome in a case series of a rare tumor patients.<p clas...

hrp0089p2-p241 | Growth &amp; Syndromes P2 | ESPE2018

Turner Syndrome and Autoimmune Thyroid Disease: Pecularities of Evolution in 93 Turner Syndrome Patients

Dumitrescu Cristina , Gherlan Iuliana , Radomir Lidia , Vintila Madalina , Brehar Andreea , Caragheorgheopol Andra , Purice Mariana , Procopiuc Camelia

Turner Syndrome (TS) is a relatively common chromosomopathy and according to epidemiological studies the prevalence of Autoimmune thyroiditis (AIT) in TS fluctuates from 10% to 21% versus 1.3% in the general population.Objective: – to retrospectively evaluate thyroid autoimmune disorders and thyroid function in a group of 93 TS patients.– to compare the prevalence of AIT and thyroid dysfunction in subgroups of TS accordin...

hrp0089p2-p359 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P2 | ESPE2018

Clinical, Hormonal and Metabolic Profile in Adolescent Girls Treated with Gonadotropin Releasing Hormone Agonist for Idiopathic Central Precocious Puberty

Procopiuc Camelia , Dumitrescu Cristina , Vintila Madalina , Caragheorgheopol Andra , Brehar Andreea , Radomir Lidia , Gherlan Iuliana

Background: Gonadotropin-releasing hormone analog (GnRHa) is the gold standard treatment for central precocious puberty (CPP). In recent years, increased prevalence of polycystic ovary syndrome (PCOS) has been reported in girls treated with GnRHa for CPP. Attributes of PCOS overlap normal pubertal changes, making the diagnosis of PCOS during adolescence controversial.Aim: To assess the metabolic profile, the prevalence of PCOS and describe its phenotypes...

hrp0086p1-p36 | Adrenal P1 | ESPE2016

Early Onset Hypertension with Primary Hyperaldosteronism through Mutation in the Calcium Channel CACNA1H – Case Report

Dumitrescu Cristina , Chirita Corina , Procopiuc Camelia , Gherlan Iuliana , Olaru Maria , Lifton Richard P , Nelson-Williams Carol

Background: The genetic causes of primary hyperaldosteronism are still being discovered.Results: We present the case of a 17-years-old girl who was found by accident with severe hypertension (TA 180/100 mmHg, bilateral). Her personal history was unremarkable. Her father had hypertension and a paternal aunt had died at 55 due to a stroke. Both her sisters and mother had normal blood pressure. The cardiological examinationshowed concentric left ventricle h...

hrp0082p2-d3-553 | Puberty and Neuroendocrinology (2) | ESPE2014

Inhibin B in the Diagnosis of Delayed Puberty

Procopiuc Camelia , Gherlan Iuliana , Dumitrescu Cristina , Brehar Andreea , Vladoiu Suzana , Procopiuc Livia , Caragheorgheopol Andra

Background: Existing tests are not 100% accurate in differentiating between isolated hypogonadotropic hypogonadism (HH) and constitutional delay of puberty (CDP) in boys. Inhibin B is a glycoprotein produced by the Sertoli cells and is measurable even before puberty. Its level increases at the beginning of puberty under the influence of FSH.Objective and hypotheses: The levels of inhibin B (InhB) could differentiate between HH and CDP. We aimed to establ...