ABSTRACT
La enfermedad pulmonar intersticial (EPI) es una entidad que incluye patologías raras de baja prevalencia, que involucran factores genéticos y ambientales, caracterizados por la remodelación del intersticio y los espacios aéreos pulmonares. La hiperplasia de células neuroendocrinas es una enfermedad rara del pulmón descrita por primera vez en 2005, caracterizada por retracciones de costillas, taquipnea e hipoxemia. El diagnóstico es clínico junto con la imagen de la tomografía computarizada de alta resolución (TACAR), siendo excepcional la necesidad de biopsia pulmonar. El tratamiento es sintomático, con pronóstico incierto, mejorando los síntomas con el tiempo, aunque en ciertas ocasiones pueden persistir durante años
Interstitial lung disease (ILD) is an entity that includes rare pathologies of low prevalence, involving genetic and environmental factors, characterized by the remodeling of the interstitium and lung air spaces. Neuroendocrine cell hyperplasia is a rare disease of the lung described for the first time in 2005, characterized by rib retractions, tachypnea and hypoxemia. The diagnosis is clinical along with the image of the high resolution computerized tomography (HRCT), the need for lung biopsy being exceptional. The treatment is symptomatic, with uncertain prognosis, improving the symptoms over time, although in certain occasions they may persist for years
Subject(s)
Humans , Female , Infant , Hyperplasia/diagnostic imaging , Lung Diseases, Interstitial/diagnostic imaging , Hyperplasia/therapy , Lung Diseases, Interstitial/therapy , Hyperplasia/genetics , Lung Diseases, Interstitial/genetics , Neuroendocrine Cells , Tomography, X-Ray Computed , Radiography, Thoracic , Rare Diseases/diagnosis , Rare Diseases/therapyABSTRACT
Xanthogranulomatous pyelonephritis is a rare inflammatory disease, characterized by replacement of renal parenchyma with granulomatous tissue. Initial clinical presentation includes abdominal pain and constitutional symptoms related to recurrent urinary infections. The microorganisms most commonly involved are Escherichia coli and Proteus mirabilis. Final diagnosis is made by histopathology, and the only curative treatment is total or partial nephrectomy. A recently diagnosed case in our unit is presented, as well as an update on the knowledge of this disease.
