hrp0086p1-p193 | Diabetes P1 | ESPE2016

Recombinant Human Insulin-Like Growth Factor 1 (rh IGF1) Treatment of a Case of Leprechaunism: A Two and a Half Year Follow-Up

Valle Aline , Lefevre Christine , Weill Jacques , Cartigny Maryse

Background: Leprechaunism, due to a mutation of both alleles of insulin receptor gene, is a most severe and precociously life-threatening condition, difficult to treat.Objective and hypotheses: In leprechaunism rhIGF1 may replace insulin through I the insulin-like metabolic properties of its own receptor.Method: rh IGF1 was continuously subcutaneously administered through a.pump from the age of four months and adjusted according to...

hrp0086p2-p501 | Fat Metabolism and Obesity P2 | ESPE2016

Efficacy of the Treatment for Childhood Obesity in Specialist Care: Age Over 10 Years at Baseline and Acanthosis Nigricans Predict a Worse Outcome

Valle Marketta Dalla , Laatikainen Tiina , Nykanen Paivi , Jaaskelainen Jarmo

Background: Treatment of pediatric obesity is challenging and especially the long-term outcome has been modest in many previous studies.Objective and hypotheses: We wanted to evaluate the efficacy of obesity treatment in specialist care and to find out factors, which have a major impact on the outcome.Methods: A total of 654 children (302 girls) aged 2–18 years treated for obesity between 2005 and 2012 in three specialist clin...

hrp0086p2-p95 | Adrenal P2 | ESPE2016

Early Adrenarche: A Common Query but not Easily Resolved

Sarasua Ainhoa , Diez-Lopez Ignacio

Introduction: Early/advanced adrenarche is a frequent reason for consultation in Children Endocrinology.Objectives: To evaluate the number and characteristics of cases of early/advanced adrenarche referred for assessment to the Children’s Consultation Endocrinology in the period between January 2015 and December 2015.Results: In this period are rated 522 first consultations of which 39 (7%) have been prematurely adrenarche. In...

hrp0089p2-p053 | Bone, Growth Plate & Mineral Metabolism P2 | ESPE2018

Follow-up on Bone Health in Children with Acute Lymphoblastic Leukemia (ALL)

Brenzoni Luciana , Cassinelli Hamilton , Bergada Ignacio

Background: Acute lymphoblastic leukemia (ALL) is the most common paediatric cancer. Skeletal morbidity has been recognized as a complication of ALL and its treatment, occurring at diagnosis, during chemotherapy and/or years later.Aim: To describe the adversely effect on bone health, in terms of vertebral fractures (VF) and bone mineral density (BMD), in the follow-up of children with ALL.Design, patients and methods: Descriptive a...

hrp0089p3-p139 | Fat, Metabolism and Obesity P3 | ESPE2018

Lymposomal Acid Lipase Deficit in Patients with Hypercholesterolemia

Diez-Lopez Ignacio , Sarasua Ainhoa , Lorente Isabel

The deficit of lysosomal acid lipase (LAL) is an infrequent (1: 40,000–300,000 prevalence), autosomal recessive, monogenic pathology. It can aggressively (Wolman’s disease): malabsorption and severe dyslipidemia with survival less than one year of life. The cholesterol ester storage disease (CESD) presents with dyslipidemia, liver disease and early cardiovascular disease.Goals: Descriptive study of the prevalence of LAL deficiency and carriers ...

hrp0086p2-p96 | Adrenal P2 | ESPE2016

Assessment of Clinical Effectiveness and Safety of Using Flutamide in Children with Pre-menarche Hierperandrogenismo

Diez-Lopez Ignacio , Sarasua Ainhoa , Lorente Isabel

Today we have an increase in patients with early-ahead forecast pubarche with impaired bone carving for advancement but organicidad criteria (HSC) but if dysfunction or adrenal hyperandrogenism and/or ovarian There are several anti-androgen drugs, all without usual pediatric use (use ‘of label’). Low-dose flutamide 62.5 mgr/day has been postulated as a treatment in these cases, but its use is restricted due to the risk of side effects (gynecomastia and liver involvem...

hrp0086p2-p284 | Diabetes P2 | ESPE2016

The Implementation of a System of Continuous Monitoring of Blood Glucose and Open (FREE STYLE FREE) Improves Metabolic Control of Affected Children DMtipo1

Diez-Lopez Ignacio , Saraua Ainhoa , Lorente Isabel

It is known that more checks of blood glucose, metabolic control of patients with type 1 suffering Dm improvement. Improved technology has made it possible to develop devices interstitial glucose control both blinded (retrospective) and open (in real time). The FREE STYLE free device is the latest appearance on the Spanish/European market. Although initially not seem indicated in <18 th, these are the a priori more could benefit from an improvement of metabolic control.</p...

hrp0084p2-416 | GH &amp; IGF | ESPE2015

Effects of GH Treatment on the Heart in Children with GH Deficiency

Gomez-Guzman Elena , Canete Mara Dolores , Valle-Jimenez Miguel , Caballero-Villarraso Javier , Canete Ramon

Introduction: GH deficiency (GHD) in adults is associated with impaired cardiac function, contributing to increased mortality due to cardiovascular disease. Research has shown that adults with GHD have high levels of brain natriuretic peptide as a measure of cardiac function. GH replacement therapy can improve cardiac function and lower BNP levels. The scarce research carried out in children appears to show that GHD is linked to decreased left-ventricle size and reduced left-v...

hrp0097p2-168 | Adrenals and HPA Axis | ESPE2023

Cushing’s Disease: an Example of Drug Shortage’s Impact in Pediatric Endocrinology.

Del Medico Giulia , Chiti Nicolo' , Stagi Stefano

Cushing’s disease (CD) is defined as hypercortisolism secondary to an adrenocorticotropin (ACTH) secreting pituitary adenoma. It is rare in the pediatric age and early diagnosis and treatment are important to prevent long-term complications. In the diagnostic workup, bilateral inferior petrosal sinus sampling (BIPSS) of ACTH after corticotropin-releasing hormone (CRH) stimulation is the gold standard in the differential diagnosis of ACTH-dependent hypercortisolism. We re...

hrp0095p1-482 | Fat, Metabolism and Obesity | ESPE2022

Liraglutide SC in the treatment of severe obesity in Pediatrics: a missed therapeutic opportunity?

Diez-Lopez Ignacio , Sarasua-Miranda Ainhoa , Jose Espina-Diez Mari

Liraglutide 3.0 mg has been shown to improve body mass index (BMI) and weight in obese adolescents. And it has done so in a randomized, double-blind, phase 3 clinical trial that has investigated the effect of liraglutide 3mg. compared with placebo for weight control in 251 obese minors, and as a complement to a healthy lifestyle. Adverse events associated with the use of liraglutide 3.0 in pediatric patients are not very different from those observed in adults. There may be ca...