ABSTRACT
La linfangioleiomiomatosis es una enfermedad rara caracterizada por quistes pulmonares con proliferación anómala del sistema linfático. Se presenta casi en exclusiva en mujeres en edad fértil debido a una influencia hormonal, por lo que resulta sumamente infrecuente en pacientes posmenopáusicas. En estos casos suele tener relación con tratamientos hormonales sustitutivos. Es conocido que esta enfermedad tiene un alto grado de asociación con otras afecciones como la esclerosis tuberosa y los angiomiolipomas renales.Presentamos el caso de una paciente posmenopáusica afecta de linfangioleiomiomatosis, sin ningún antecedente de tratamientos hormonales, en la que se detectaron, además, angiomiolipomas renales y criterios clínicos indicativos de esclerosis tuberosa en grado de probable(AU)
Lymphangioleiomyomatosis is a rare disease characterised by pulmonary cysts with abnormal proliferation in the lymphatic system. It occurs almost exclusively in women of fertile age due to a hormonal influence, for this reason it is extremely rare in post-menopausal patients. In these cases it is usually associated to hormone replacement therapies. It is known that this diseases is strongly associated with other conditions, such as tuberous sclerosis and renal angiomyolipomas.We present a case of a post-menopausal patient suffering from lymphangioleiomyomatosis, with no history of hormone therapy, in whom were also detected renal angiomyolipomas and clinical signs indicative of a probable tuberous sclerosis(AU)
Subject(s)
Humans , Female , Middle Aged , Leiomyomatosis/complications , Leiomyomatosis/diagnosis , Leiomyomatosis/therapy , Postmenopause/physiology , Lung Neoplasms/complications , Lung Neoplasms/diagnosis , Signs and Symptoms , Bronchoscopy/methods , Progestins/therapeutic use , Tamoxifen/therapeutic useABSTRACT
Lymphangioleiomyomatosis is a rare disease characterised by pulmonary cysts with abnormal proliferation in the lymphatic system. It occurs almost exclusively in women of fertile age due to a hormonal influence, for this reason it is extremely rare in post-menopausal patients. In these cases it is usually associated to hormone replacement therapies. It is known that this diseases is strongly associated with other conditions, such as tuberous sclerosis and renal angiomyolipomas. We present a case of a post-menopausal patient suffering from lymphangioleiomyomatosis, with no history of hormone therapy, in whom were also detected renal angiomyolipomas and clinical signs indicative of a probable tuberous sclerosis.
Subject(s)
Lung/physiopathology , Lymphangioleiomyomatosis/physiopathology , Postmenopause , Aged , Biopsy , Female , Forced Expiratory Volume , Humans , Lung/diagnostic imaging , Lung/pathology , Lymphangioleiomyomatosis/diagnostic imaging , Lymphangioleiomyomatosis/pathology , Tomography, X-Ray Computed , Vital CapacityABSTRACT
OBJECTIVES: Actinomycosis is quite an infrequent bacterial infection nowadays. However it can be considered in cases with a persistent cervicofacial disease. Although it is a bacterial infection, microbiologic cultures are frequently not diagnoses, therefore histopathologic studies and image studies are essential. Our interest is to explain our experience with cervicofacial actinomycosis; the clinical behaviour, evolution and treatment, always assisted by their elected diagnostic technique: the FNAC. STUDY DESIGN: In the last 16 years, 15 patients have been diagnosed with cervicofacial actinomycosis by FNAC, treated by Maxillofacial, Internal Medicine and Paediatrics units. Clinical course, evolution, anatomical space situation, antibiotic treatment, and surgical treatment have been studied. RESULTS AND CONCLUSIONS: The fine-needle aspiration cytology (FNAC) is an easy, safe and rapid method, with a high effect, that has made the final diagnosis in 15 cases in our Hospital. All the patients have had a good clinical evolution, only in one case did we need a new treatment for recidive. In all the cases treatment has been definitive. Our interest is to explain our experience in the treatment of cervicofacial actinomycosis, its clinical presentation and evolution, together with its elected method of diagnosis, FNAC.
Subject(s)
Actinomycosis, Cervicofacial/pathology , Adolescent , Adult , Aged , Aged, 80 and over , Biopsy, Fine-Needle , Child , Female , Humans , Male , Middle Aged , Retrospective StudiesABSTRACT
Objetivos: La actinomicosis es una enfermedad infecciosa muy poco frecuente en nuestros días, a pesar de lo cual, su diagnóstico debe ser tenido en cuenta por el clínico que se enfrenta a una lesión persistente a nivel cervicofacial.Aún tratándose de una enfermedad infecciosa, los cultivos microbiológicos son pocas veces diagnósticos, por lo que el estudio histopatológico y las pruebas de imagen son esenciales. Nuestro interés es exponer nuestra experiencia en el manejo de la actinomicosis de localización cervicofacial, su presentación clínica, evolución y tratamiento, siempre apoyados en la que creemos que es su técnica diagnóstica de elección, la PAAF. Diseño del estudio: A lo largo de 16 años se han diagnosticado 15 pacientes de actinomicosis cervicofacial mediante PAAF, siendo estudiados y tratados por los Servicios de Cirugía Maxilofacial, Medicina Interna y Pediatría del Hospital Universitari de Girona Doctor Josep Trueta. La clínica, la evolución, la localización de las lesiones, cultivo y el tratamiento con antibioticoterapia de duración prolongada, asociado en algunos casos a intervención quirúrgica, son datos valorados en nuestro estudio. Resultados y conclusiones: El estudio anatomopatológico mediante la punción aspiración con aguja fina (PAAF), es un método sencillo, rápido y seguro, de una alta sensibilidad diagnóstica, puesto que nos ha permitido el diagnóstico definitivo de los 15 casos seguidos en nuestro Hospital. La evolución clínica fue favorable en todos los pacientes, con un único caso de recidiva clínica, solucionado con un segundo tratamiento. El tratamiento instaurado fue curativo en todos los casos (AU)
Objectives: Actinomycosis is quite an infrequent bacterial infection nowadays. However it can be considered in cases with a persistent cervicofacial disease. Although it is a bacterial infection, microbiologic cultures are frequently not diagnoses, therefore histopathologic studies and image studies are essential. Our interest is to explain our experience with cervicofacial actinomycosis; the clinical behaviour, evolution and treatment, always assisted by their elected diagnostic technique: the FNAC. Study design: In the last 16 years, 15 patients have been diagnosed with cervicofacial actinomycosis by FNAC, treated by Maxillofacial, Internal Medicine and Paediatrics units. Clinical course, evolution, anatomical space situation, antibiotic treatment, and surgical treatment have been studied.Results and conclusions: The fine-needle aspiration cytology (FNAC) is an easy, safe and rapid method, with a high effect, that has made the final diagnosis in 15 cases in our Hospital. All the patients have had a good clinical evolution, only in one case did we need a new treatment for recidive. In all the cases treatment has been definitive. Our interest is to explain our experience in the treatment of cervicofacial actinomycosis, its clinical presentation and evolution, together with its elected method of diagnosis, FNAC (AU)