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1.
Rev. chil. enferm. respir ; 35(1): 58-62, mar. 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-1003647

ABSTRACT

Introducción: La linfangioleiomiomatosis Pulmonar (LAM) es una rara y progresiva enfermedad; caracterizada por proliferación excesiva de células musculares lisas a partir de vasos linfáticos, sanguíneos y vías aéreas. En conjunto al anormal crecimiento celular descrito, se aprecia degeneración quística difusa del parénquima pulmonar, lo que puede reflejarse desde cuadros completamente asintomáticos hasta el deterioro severo del intercambio gaseoso con insuficiencia respiratoria fulminante. Descripción del caso: Paciente femenino de 41 años de edad, con cuadro clínico consistente en tos seca ocasional, asociada a dolor leve de características pleuríticas en 'puntada de costado ' derecha. Ante la no mejoría clínica, se indica estudio imagenológico donde se demuestra neumotorax espontáneo derecho. En estudio tomográfico se aprecian además lesiones pulmonares quísticas. El estudio anátomo-patológico demuestra cambios estructurales que se reportan compatibles con LAM. Conclusión: Dada la simplicidad de los síntomas con que la LAM puede debutar, su confirmación diagnóstica se genera en fases avanzadas de la enfermedad, cuando el daño pulmonar importante conlleva a la aparición de factores clínicos con mayor repercusión sobre el estado general de los pacientes por lo que la realización de estudios imagenológicos tempranos gana vital importancia.


Introduction: Pulmonary lymphangioleiomyomatosis (LAM) is a rare and progressive disease; characterized by airway, lymphatic and blood vessels-smooth muscle cells excessive proliferation. Added to the abnormal cell growth, parenchymal cystic degeneration is present, which can be reflected initially as a asymptomatic course and can progress to severe gaseous exchange deterioration and fulminating respiratory insufficiency. Case description: A 41-year-old female patient with a clinical course consisting of occasional dry cough, associated with mild pleuritic pain on the right side of thorax. As no improvement was achieved, thoracic imaging study was performed, where a right pneumothorax was found. Tomography images showed multiple lung cystic lesions. Anatomopathological study reports structural changes compatible with LAM. Conclusion: Given the simplicity of the symptoms that LAM can debut with, its diagnostic confirmation is generated in advanced stages of the disease, when the important pulmonary damage leads to the appearance of clinical factors with greater impact on the general state of patients so early thoracic imaging studies gain vital importance.


Subject(s)
Humans , Female , Adult , Lymphangioleiomyomatosis/diagnosis , Lymphangioleiomyomatosis/therapy , Lung Neoplasms/diagnosis , Lung Neoplasms/therapy , Pneumothorax/etiology , Spirometry , Radiography, Thoracic , Tomography, X-Ray Computed , Lymphangioleiomyomatosis/complications , Sirolimus/therapeutic use , Cysts/etiology , Lung Neoplasms/complications
3.
Article in English | IMSEAR | ID: sea-157402

ABSTRACT

Lymphangioleiomyomatosis (LAM) is a progressive disease affecting women of childbearing age. It is microscopically characterized by abnormal smooth muscle proliferation in the walls of lymphatics and lymph nodes. A 52-year old female presented with pain in left leg and bilateral pedal oedema. USG and CT abdomen revealed a retroperitoneal cystic mass suspicious of malignancy. Retroperitoneal exploration was done and the mass was excised which revealed milky contents within. On gross examination of the specimen, a spongy mass was noted . Histopathological examination led to a diagnosis of lymphangioleiomyomatosis. LAM is a rare disease unfamiliar to many physicians and may pose a diagnostic dilemma to the physician, possibly resulting in delayed or missed diagnosis . Many treatment modalities including corticosteroids, cytotoxic drugs, chemotherapy, radiation and hormonal therapy are suggested. The prognosis of LAM varies with the individual, but many patients respond well to intramuscular medroxyprogesterone injections.


Subject(s)
Cell Proliferation , Diagnostic Errors , Female , Humans , Leg/pathology , Lymphangioleiomyomatosis/complications , Lymphangioleiomyomatosis/diagnosis , Lymphangioleiomyomatosis/drug therapy , Lymphangioleiomyomatosis/pathology , Lymphangioleiomyomatosis/radiotherapy , Lymphangioleiomyomatosis/therapy , Muscle, Smooth, Vascular/pathology , Retroperitoneal Space
4.
J. bras. med ; 98(4): 29-33, ago.-set. 2010. ilus, tab
Article in Portuguese | LILACS | ID: lil-566753

ABSTRACT

As doenças pulmonares intersticiais (DPIs) são conceituadas como distúrbios que acometem o parênquima pulmonar - o endotélio cailar, os alvéolos, o epitélio alveolar e os espaços entreestas estruturas, bem como os tecidos perivasculares e linfáticos - , podendo ser classificadas segundo critérios histopatológicos, distinguindo-se dois grandes grupos: 1. das associadas à inflamação e fibrose; e 2. daquelas com reação granulomatosa predominante na área intersticial u vasculas. A linfangioliomiomatose (LAM) é uma rara DPI, idiópática, e com altas taxas de morbimortalidade, sendo caracterizada por uma multiplicação acelerada de células musculares lisas imaturas em qualquer estrutura pulmonar. No presente artigo apresentar-se-á uma revisão da literatura enfocando a etiopatogenia, a epidemiologia, o quadro clínico, o diagnóstico - procedimentos, critérios e diagnóstico diferencial - o tratamento e o prognóstico da LAM.


The interstitial pulmonary diseases (IPDs) are a range of disorders that affect the pulmonary parenchyma - the capillary endothelium, alveoli, alveolar epithelium and the spaces between thesestructures, as well as the perivascular and lymphatic tissues. The IPDs may be classified according to histopathologic criteria, and are divided into two large groups: 1. those associated with inflammation and fibrosis; and 2. those associated with granulomatous reactions predominantly in the interstitial or vascular area. Lymphangioleiomyomatosis (LAM) ia a rare, idiopathic IPD with high morbimortality rates, which is characterized by an accelerated multiplication of immature smooth muscle cellsin any pulmonary structure. In this article, we present a review of the literature onthe etiopathogenesis, epidemiology, clinical picture, diagnosis and differential diagnosos, treatment, and prognosis of this condition.


Subject(s)
Humans , Female , Lung Diseases, Interstitial/classification , Lymphangioleiomyomatosis/diagnosis , Lymphangioleiomyomatosis/epidemiology , Lymphangioleiomyomatosis/etiology , Lymphangioleiomyomatosis/physiopathology , Lymphangioleiomyomatosis/therapy , Diagnosis, Differential , Tuberous Sclerosis/complications , Estrogens/adverse effects , Prognosis , Loss of Heterozygosity/genetics
6.
Rev. colomb. neumol ; 8(4): 212-5, nov. 1996. ilus, graf
Article in Spanish | LILACS | ID: lil-190578

ABSTRACT

La linfangioleiomiomatosis es una enfermedad rara con menos de 250 casos reportados en la literatura universal, que ocurre únicamente en mujeres, usualmente en edad reproductiva y que se caracteriza por la proliferación de células de musculo liso en tejido pulmonar y en los vasos linfáticos. Presentamos el caso de una mujer de 29 años, con disnea progresiva, tos con escasa espectoración y neumotórax espontáneo bilateral. El diagnóstico se realizó mediante biopsia pulmonar a cielo abierto, donde se encontró proliferación de haces de músculo liso los cuales se disponían preferentemente alrededor de las estructuras vasculares,bronquiales y septo alveolares. Comentamos los principales hallazgos clínicos, radiológicos e histopatológicos.


Subject(s)
Humans , Female , Adult , Lymphangioleiomyomatosis/classification , Lymphangioleiomyomatosis/complications , Lymphangioleiomyomatosis/diagnosis , Lymphangioleiomyomatosis/epidemiology , Lymphangioleiomyomatosis/etiology , Lymphangioleiomyomatosis/physiopathology , Lymphangioleiomyomatosis/pathology , Lymphangioleiomyomatosis/drug therapy , Lymphangioleiomyomatosis/therapy , Lymphangiomyoma , Lymphangiomyoma/classification , Lymphangiomyoma/complications , Lymphangiomyoma/diagnosis , Lymphangiomyoma/drug therapy , Lymphangiomyoma/epidemiology , Lymphangiomyoma/etiology , Lymphangiomyoma/pathology , Lymphangiomyoma/physiopathology , Lymphangiomyoma/therapy , Lung Neoplasms , Lung Neoplasms/classification , Lung Neoplasms/complications , Lung Neoplasms/diagnosis , Lung Neoplasms/drug therapy , Lung Neoplasms/epidemiology , Lung Neoplasms/etiology , Lung Neoplasms/mortality , Lung Neoplasms/pathology , Lung Neoplasms/physiopathology , Lung Neoplasms/metabolism , Lung Neoplasms/therapy
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