Presentación
CONGENITAL MALFORMATIONS
AND TRANSPLANTATION

MalformacionesCongenitas_trasplante@idipaz.es
Composition
Name
Position
Institution
Francisco Hernández Oliveros 
Facultativo Especialista de Área en Cirugía Pediátrica
Hospital Universitario La Paz
Maria Virginia Amesty Morello
Médico Adjunto en Cirugía Pediátrica
Hospital Universitario La Paz
Ane Miren Andrés Moreno
Facultativo Especialista de Área en Cirugía Pediátrica
Pediátrica Profesor Asociado
Hospital Universitario La Paz
Universidad Autónoma de Madrid
Sara Cabezuelo Ballestero
Facultativo Especialista de Área en Anestesiología
Hospital Universitario La Paz
Carlos de la Torre Ramos
Médico Adjunto en Cirugía Pediátrica
Hospital Universitario La Paz
Carlos Delgado Miguel
Facultativo Especialista de Área en Cirugía Pediátrica
FIBHULP
José Luis Encinas Hernández
Facultativo Especialista de Área en Cirugía Pediátrica
Hospital Universitario Infantil La Paz
Álvaro González Rocafort
Facultativo Especialista de Cirugía Pediátrica
Hospital Universitario Infantil La Paz
Juan Carlos López Gutiérrez
Jefe de Servicio de Cirugía Pediátrica 
Hospital Universitario Infantil La Paz
María Nieves Moreno
Facultativo Especialista de Área en Oftalmología
Hospital Universitario La Paz
Susana Noval Martín
Facultativo Especialista de Área en Oftalmología
Hospital Universitario La Paz
Jesús Peralta Calvo
Facultativo Especialista de Área en Oftalmología Infantil
Hospital Universitario La Paz
Esther Ramos Boluda
Jefe de Sección. Unidad de Rehabilitación y Trasplante Intestinal
Hospital Universitario La Paz
Fernando Reinoso Barbero
Jefe de Servicio de Anestesiología y Reanimación Infantil
Hospital Universitario La Paz
Alba Sánchez Galán
Facultativo Especialista de Cirugía Pediátrica
Hospital Universitario La Paz
Javier Serradilla Rodríguez
Facultativo Especialista de Área en Cirugía Pediátrica
Hospital Universitario La Paz
Paloma Triana Junco
Facultativo Especialista de Área en Cirugía Pediátrica
Hospital Universitario La Paz
María Teresa Vallejo Cremades
Jefe de Laboratorio Inmunohisto-química (Contrato de Estabilización de Técnicos de Apoyo)
FIBHULP
Alejandra Vilanova Sánchez
Médico Interno Residente en Cirugía Pediátrica
Hospital Universitario La Paz
General objectives for the next 5 years
Anesthesia, pain management and perioperative intensive care: To develop new procedures for improving safety and efficacy in anesthesic procedures, and to investigate the multiple problems of pain management and anaesthesia support.
Vascular anomalies: To investigate genetic and molecular mechanisms involved in the pathogenesis of vascular anomalies, and to evaluate new treatments.
Cardiovascular surgery: To assess different organ perfusion devices and to develop new strategies for graft perfusion.
Congenital malformations: To gain knowledge on the cellular and molecular mechanisms of malformations using animal models, organ and cell cultures and eventually genetically engineered animals.
Oftalmologic pathology: To investigate genetic and molecular mechanisms involved in the pathogenesis of ocular malformations and ocular rare diseases.
Transplantation: To investigate new strategies for graft tolerance/avoidance of rejection and to analyze the impact of donation after circulatory death with normothermic reperfusion in pediatric transplantation. Additionally, the management of the conditions leading to organ failure would be included in our topics, like intestinal rehabilitation (intestinal transplantation), liver tumours (liver transplantation) and severe uropathies (renal transplantation).
Specific objectives for next 5 years
Scientific Objectives
Planned Research Projects
Anesthesia: We shall center our interest on safe hemoderivatives transfusion and metabolic monitoring during complex surgical procedures. Also quality initiatives will be included in our scope.
Vascular anomalies: We will continue with our recent project focused on the long-term safety and efficacy of alpelisib in patients with PIK3CA-Related Overgrowth Spectrum (PROS); and we will improve our contribution to our lymphatic malformation program.
Cardiovascular surgery: We plan to initiate our research on mitochondrial transplantation to improve recovery after infarction. We will also continue our hemodynamic assessment of ex vivo perfusion devices. In the clinic, we will prospectively analyze the recently developed program of heart transplantation from DCD.
Congenital malformations: We will deepen our study of fetal alcohol syndrome. More specifically, we aim at further investigating the participation of antioxidant agents. We will also try to gain further insight into instrumental prenatal manipulation in Spina Bifida in collaboration with the research group of Cincinnati Children Hospital.
Ocular pathology: We will continue our open research line of genetic study on congenital non syndromic bilateral cataracts and myopia with or without nistagmus.
Transplantation: Finally, as the only group involved in pediatric small bowel transplantation in Spain, we shall also continue our efforts for improving the graft viability, to deepen our understanding of rejection and GVHD phenomenons and for searching tolerance by different cell therapies.. All this work in animal models will be complemented with human studies on liver and small bowel material .
Research Lines
Paediatric surgical specialities are extremely complex due to the wide spectrum of diseases included in their scope. Many of the problems are related to rare diseases and their treatment frequently requires complex procedures.
Clinical care of paediatric surgical patients is a long-term task that requires a wide knowledge of the pathology and multidisciplinary committees. Surgical corrections usually consist of multi-step procedures, some of them in the neonatal period while others might be required in young adults.
This line is therefore characterized by an unusually large spectrum since it encompasses the study of the cellular and molecular mechanisms leading to malformations, the biology of the malformed fetus and embryo, the prenatal chances for intervention on the malformations, and the postnatal operative and perioperative surgical and anesthesic management, including complex procedures as transplantation.

Facultativo Especialista de Área en Cirugía Pediátrica

Jefe de Sección. Unidad de Rehabilitación y Trasplante Intestinal