hrp0094p2-426 | Sex differentiation, gonads and gynaecology or sex endocrinology | ESPE2021

Uterus agenesia in a patient with CATCH 22 Syndrome. Purpose of a case

Ignacio Diez-Lopez , Mesonero Saray , Maeso Sandra , Sarasua-Miranda Ainhoa

Introduction: The CATCH 22 syndrome or DiGeorge syndrome or velocardiofacial syndrome, is a frequent pathology with the worldwide prevalence estimated between 1 / 2,000 and 1 / 4,000 live births. The classic clinic is characterized by typical facial features (narrow palpebral fissures, bulbous nasal tip, small mouth and ears, malar hypoplasia) with functional insufficiency of the soft palate in about 75% of those affected, difficulties in learning, language and behavior , cong...

hrp0094p2-438 | Sex differentiation, gonads and gynaecology or sex endocrinology | ESPE2021

Congenital adrenal hyperplasia in detail: A multicentric and interdisciplinary study in minors with different sexual development

Ignacio Diez-Lopez , Nerea Gonzalez-Temprano , Villalon Flor , Larreina de la Fuente Leire , Cancela Vanesa , Nuñez Francisco Javier , Hernandez Jone Miren

Of the 67 included DSD patients, 33% were HSCs. 68% women and 32% men, with a total concordance between legal sex, current gender and chromosomal sex. Median age: 10.5 years, IQR 8-14. The most frequent clinical form was non-classical (NC) 45.5%, followed by classical with saline loss (CPS) 41%. The distribution of each clinical form according to the karyotype is significant (P = 0.013), the most frequent with XY karyotype is CPS and in NC forms all cases are XX. Med...

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...

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...

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...

hrp0095p2-50 | Diabetes and Insulin | ESPE2022

Inhaled glucagon, a new well-accepted therapeutic tool in pediatrics

Diez-Lopez Ignacio , Sarasua-Miranda Ainhoa , José Espina-Diez Maria

On 01/14/2021, the European Commission authorized the marketing of Baqsimi® 3 mg (nasal glucagon), a powder medicine, ready to be used, in the treatment of severe hypoglycemia in adults, adolescents and children over four years of age with diabetes mellitus. Baqsimi® is the first and only nasal glucagon treatment available approved in the European Union (EU), and has been designed as a rescue treatment in cases of severe hypoglycemia, being absorbed passively into the ...

hrp0095p2-87 | Diabetes and Insulin | ESPE2022

100 years with insulin. Implementation of analogs and technology in our environment

Diez-Lopez Ignacio , Sarasua-Miranda Ainhoa , José Espina-Diez Maria

Almost 100 years ago, a major turning point in the life expectancy of people with diabetes around the world was reached with the discovery of insulin. In 1936, Hagedorn had the ingenious idea of combining insulin with a protein, protamine, and zinc and his NPH was used almost mainly in pediatrics until the end of 2003, when glargine U100 made its appearance.Objective: To evaluate the clinical experience in the incorporation of different ...

hrp0092p3-316 | Late Breaking Abstracts | ESPE2019

Epidemiological and Socioeconomic CHANGES in the Child Population from Debut DM1 in this 21st Century

Diez-Lopez Ignacio , Sarasua-Miranda Ainhoa , Lorente-Blazquez Maria Isabel

Objectives: To demographic, socio-economic and social changes in the population of children who debuted in this century with DM type 1aPatients and Methods: Study patients with Type 1 Diabetes Mellitus from January 2000 to the present. Longitudinal study of global epidemiological, social, demographic and clinical variables and by five-year periods, focusing on the latte at Basque CountryRes...