ABSTRACT
The @ERSTalk ECM Committee present highlights from #ERSLDN16 for Early Career Members http://ow.ly/tRYH305Owmz.
ABSTRACT
En el presente trabajo se describen las características generales, objetivos y aspectos organizativos de los registros de enfermedades respiratorias minoritarias integrados en el Registro Nacional de Enfermedades Raras del Instituto de Investigación de Enfermedades Raras (IIER), con el objetivo de dar a conocer su existencia y fomentar la participación de los profesionales. Se recoge información sobre registros de las siguientes enfermedades: déficit de alfa-1 antitripsina, estenosis traqueal idiopática, histiocitosis pulmonar de células de Langerhans del adulto, linfangioleiomiomatosis, proteinosis alveolar y sarcoidosis
This report describes the general characteristics, objectives, and organizational aspects of the registries of minority respiratory diseases included in the National Registry of Rare Diseases of the Research Institute for Rare Diseases (ISCIII), in order to publicize their existence and encourage the participation of professionals. Information is collected on the following conditions: alpha-1 antitrypsin deficiency, idiopathic tracheal stenosis, adult pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, alveolar proteinosis, and sarcoidosis (AU)
Subject(s)
Humans , Male , Female , Rare Diseases/epidemiology , Respiratory Tract Diseases/complications , Respiratory Tract Diseases/epidemiology , Records , Forms and Records Control/organization & administration , Forms and Records Control/standards , Clinical Record , alpha 1-Antitrypsin/analysisABSTRACT
La extirpación quirúrgica del bocio intratorácico puede realizarse a través de un abordaje cervical en la mayoría de los pacientes. La revisión de la literatura pone de manifiesto que los cirujanos experimentados precisan un abordaje extracervical en el 2-3% de los casos. A pesar de que el tratamiento quirúrgico del bocio retroesternal está bien definido, existe poca información acerca del abordaje quirúrgico de los bocios intratorácicos que se extienden más allá del cayado aórtico hacia el mediastino posterior. Presentamos 2 casos y proponemos una combinación de incisión cervical y toracotomía lateral con preservación muscular para la resección del bocio en el mediastino posterior. En este tipo de casos descartamos el uso de la esternotomía puesto que el mediastino posterior resulta inaccesible debido a la presencia del corazón y grandes vasos por delante de la masa tiroidea, lo cual podría llevar a realizar una peligrosa disección a ciegas. Según nuestra experiencia el abordaje transcervical combinado con la toracotomía está indicado para una resección completa y segura del bocio situado en el mediastino posterior
Surgical removal of intrathoracic goiter can be performed by a cervical approach in the majority of patients. Review of literature shows that experienced surgeons need to perform an extracervical approach in 2-3% of cases. In spite of surgical management of substernal goiter is well defined, there is little available information about surgical approach of intrathoracic goiters extending beyond the aortic arch into the posterior mediastinum. We report two cases and propose combination of cervical incision and muscle-sparing lateral thoracotomy for posterior mediastinal goiter removal. In such cases, we do not favor sternotomy as posterior mediastinum is inaccessible due to the presence of heart and great vessels anterior to the thyroidal mass that would lead to perform a perilous blind dissection. Based on our experience, transcervical and thoracotomy approach is indicated for a complete and safe posterior mediastinal goiter remova
Subject(s)
Humans , Goiter, Substernal/surgery , Thoracotomy/methods , Sternotomy/methods , Thyroidectomy/methods , Mediastinum/surgery , Treatment Outcome , Postoperative Complications/epidemiologyABSTRACT
This report describes the general characteristics, objectives and organizational aspects of the registries of rare respiratory diseases included in the National Registry of Rare Diseases of the Research Institute for Rare Diseases (ISCIII), in order to publicize their existence and encourage the participation of professionals. Information is collected on the following conditions: alpha-1 antitrypsin deficiency, idiopathic tracheal stenosis, adult pulmonary Langerhans' cell histiocytosis, lymphangioleiomyomatosis, alveolar proteinosis, and sarcoidosis.
Subject(s)
Rare Diseases , Registries , Respiration Disorders , Humans , Rare Diseases/epidemiology , Respiration Disorders/epidemiology , Spain/epidemiologyABSTRACT
Surgical removal of intrathoracic goiter can be performed by a cervical approach in the majority of patients. Review of literature shows that experienced surgeons need to perform an extracervical approach in 2-3% of cases. In spite of surgical management of substernal goiter is well defined, there is little available information about surgical approach of intrathoracic goiters extending beyond the aortic arch into the posterior mediastinum. We report two cases and propose combination of cervical incision and muscle-sparing lateral thoracotomy for posterior mediastinal goiter removal. In such cases, we do not favour sternotomy as posterior mediastinum is inaccessible due to the presence of heart and great vessels anterior to the thyroidal mass that would lead to perform a perilous blind dissection. Based in our experience, transcervical and thoracotomy approach is indicated for a complete and safe posterior mediastinal goiter removal.
