Тема - темы
Humans , Amyloidosis/diagnosis , Amyloidosis/therapy , Cardiomyopathies/diagnosis , Cardiomyopathies/therapyРеферат
Amyloidosis is a disease caused by extracellular deposition of insoluble protein fibrils, that results in changes in tissue architecture and consequently modification of the organ structure. Cardiac involvement is common in amyloidosis. Two major types of systemic amyloidosis affect the myocardium immunoglobulin light chain and transthyretin amyloidosis each leading to different prognosis. Early detection and diagnosis of cardiac amyloidosis are the main objectives in the assessment of the disease. New techniques of magnetic resonance imaging have minimized the need for biopsies for the diagnosis. Late gadolinium enhancement technique, and more recently T1 mapping, have allowed a simplified evaluation of amyloid deposits and extracellular volume. The aim of this review was to describe basic concepts and updates of the use of magnetic resonance imaging for the diagnosis amyloidosis and evaluation of its severity
Тема - темы
Humans , Male , Female , Magnetic Resonance Imaging/methods , Amyloidosis/diagnosis , Amyloidosis/therapy , Prognosis , Diagnostic Imaging/methods , Echocardiography/methods , Biomarkers , Immunoglobulin Light Chains , Contrast Media , Plaque, Amyloid/diagnostic imaging , Electrocardiography/methods , Gadolinium , Heart Ventricles , Myocarditis/pathologyТема - темы
Humans , Male , Adolescent , Amyloidosis/diagnosis , Amyloidosis/therapy , Diagnostic Imaging/methods , Echocardiography/methods , Hemangiosarcoma/surgery , Hemangiosarcoma/therapy , Coronary Vessels/surgery , Drug Therapy , Early Diagnosis , Heart Failure/complications , Heart Failure/diagnosis , Heart Ventricles , Neoplasms/diagnosisРеферат
La amiloidosis cutánea es un tipo de amiloidosis localizada que se diferencia de la sistémica en que el depósito de la sustancia amiloide se halla exclusivamente en la piel. Dentro de las amiloidosis cutáneas localizadas se encuentra la variante maculosa, que afecta a mujeres adultas y se caracteriza por la presencia de máculas hiperpigmentadas pruriginosas ubicadas en espalda. Es frecuente de observaren algunas áreas geográficas (Asia, América del Sur y América Central).Presentamos cinco pacientes (cuatro mujeres y un varón) con amiloidosis macular, todas de igual localización cutánea...
Тема - темы
Humans , Amyloidosis/classification , Amyloidosis/diagnosis , Amyloidosis/pathology , Amyloidosis/therapy , Skin Diseases/pathology , Plaque, AmyloidРеферат
Amyloidosis is a rare infiltrative disease characterized by multiple clinical features. Various organs are involved and the cardiovascular system is a common target of amyloidosis. Cardiac involvement may occur with or without clinical manifestations and is considered as a major prognostic factor. To analyze the clinical features of cardiac involvement, to review actual knowledgement concerning echocardiographic diagnostic and to evaluate recent advances in treatment of the disease. An electronic search of the relevant literature was carried out using Medline and Pubmed. Keys words used for the final search were amyloidosis, cardiopathy and echocardiography. We considered for analysis reviews, studies and articles between 1990 and 2007. Amyloidosis represents 5 to 10% of non ischemic cardiomyopathies. Cardiac involvement is the first cause of restrictive cardiomyopathy which must be evoked. In front of every unexplained cardiopathy after the age of forty. The amyloid nature of cardiopathy is suggested if some manifestations were associated as a peripheral neuropathy, a carpal tunnel sydrome and proteinuria > 3g/day. Echocardiography shows dilated atria, a granular sparkling appearance of myocardium, diastolic dysfunction and thickened left ventricle contrasting with a low electric voltage. The proof of amyloidosis is brought by an extra-cardiac biopsy, the indications of endomyocardial biopsy are very limited. The identification of the amyloid nature of cardiopathy has a direct therapeutic implication: it indicates the use of digitalis, calcium channel blockers and beta-blockers. Today the treatment of amyloidosis remains very unsatisfactory especially in the cardiac involvement. An early diagnosis before the cardiac damage may facilitate therapy and improve prognosis
Тема - темы
Humans , Heart Diseases , Amyloidosis/physiopathology , Amyloidosis/diagnosis , Amyloidosis/therapy , Echocardiography , ElectrocardiographyРеферат
La amiloidosis es una enfermedad por depósito de proteínas de configuración anormal que puede tener diversas etiologías y manifestaciones clínicas. Existen formas primarias, secundarias y hereditarias, asociándose cada una a un tipo especial de proteína precursora que originará a la fibrilla amiloídea. Dependiendo del órgano afectado y de la magnitud de la infiltración será la expresión clínica y pronóstico a futuro. Existen varios métodos diagnósticos, siendo hasta ahora la tinción de Rojo Congo y la visualización de una birrefrigencia verde-manzana patognomónicas de esta enfermedad. Los tratamientos disponibles en la actualidad tienen el inconveniente de tener alta toxicidad a nivel medular, siendo potencialmente carcinogénicos; por esta razón se han realizado importantes avances en crear agentes que intervienen en cada proceso de formación de la fibrilla. La inmunoterapia dirigida contra epítopes específicos ha demostrado ser altamente efectiva en modelos experimentales, estando próximos a ser aplicados en ensayos clínicos en humanos.
Amyloidosis is a disease setting depot of abnormal proteins that can have different etiologies and clinical manifestations. There are primary, secondary and hereditary forms, each one associated with a special kind of precursor protein fibrils that lead to amyloidosis. Depending on the organ affected and the extent of the infiltration will be the expression and clinical prognosis for the future. There are several diagnostic methods, being so far Congo Red stain and the display of a apple-green birefringence pathognomonic of the disease. The treatments available today have the disadvantage of having high toxicity level and potentially carcinogenic, that is why there has been significant progress in creating agents involved in every process of the formation of fibrils. Immunotherapy directed against specific epitopes has proved highly effective in experimental models, staying close to be applied in clinical trials in humans.
Тема - темы
Humans , Amyloidosis/diagnosis , Amyloidosis/therapy , Amyloidosis/classification , Immunotherapy , PrognosisРеферат
The treatment of AL amyloidosis was not successful until the advent of myeloablative chemotherapy consisting of high-dose intravenous melphalan followed by autologous peripheral blood stem cell transplantation. This new treatment has achieved better survival rates and, remarkably, it has obtained complete remission. Among patients with renal involvement, achievement of a complete hematological response was associated with a 50% reduction in proteinuria and stable creatinine clearance in more than 2/3 of patients. Despite of these excellent results, this new therapy is associated with significant toxicity, including the development of acute renal failure due to white blood cell lysis syndrome. We report a 59 year-old female with a nephrotic syndrome due to primary amyloidosis successfully treated autologous stem cell transplantation who developed acute renal failure caused by white blood cell lysis syndrome. The patient required treatment with granulocytic colony stimulating factor and intermittent hemofiltration and was discharged 23 days after melphalan administration with a satisfactory renal function and white blood cell count. After one year of follow up, she maintains a good glomerular filtration rate, a proteinuria of less than, 1 g/day and normal hematological values.
Тема - темы
Female , Humans , Middle Aged , Amyloidosis/complications , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Melphalan/adverse effects , Myeloablative Agonists/adverse effects , Peripheral Blood Stem Cell Transplantation/adverse effects , Tumor Lysis Syndrome/etiology , Acute Kidney Injury , Amyloidosis/blood , Amyloidosis/therapy , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Melphalan/blood , Melphalan/therapeutic use , Myeloablative Agonists/therapeutic use , Transplantation, Autologous , Tumor Lysis Syndrome/blood , Tumor Lysis Syndrome/drug therapyРеферат
La amiloidosis forma parte de las enfermedades cutáneas de depósito. Puede afectar múltiples órganos (amiloidosis sistémica) o estar localizada en un solo órgano. La amiloidosis sistémica es una entidad poco frecuente, pero de alta mortalidad. Se clasifica según su patogenia y el tipo de amiloide que se deposita. En algunos tipos de amiloidosis sistémica, las manifestaciones cutáneas son frecuentes, las que pueden ser la forma de presentación de la enfermedad. El diagnóstico definitivo se realiza mediante el estudio histopatológico de los tejidos comprometidos. Actualmente no existe un tratamiento curativo para la amiloidosis sistémica. La quimioterapia es el tratamiento más usado, mejorando sustancialmente el pronóstico global. En este trabajo revisaremos la clasificación, epidemiología, patogenia, manifestaciones clínicas, estudio histopatológico, diagnóstico y opciones terapéuticas de la amiloidosis sistémica.
Тема - темы
Humans , Amyloidosis/classification , Amyloidosis/diagnosis , Amyloidosis/therapy , Amyloidosis/epidemiology , Prognosis , SurvivalРеферат
Las amiloidosis corresponden a un grupo de enfermedades de depósito, en la cual se acumula una sustancia denominada amiloide en diversos órganos, alterando la estructura y la función de los tejidos. Las amiloidosis se dividen en formas sistémicas y formas localizadas; estas últimas pueden ser exclusivamente cutáneas. La amiloidosis cutánea localizada primaria comprende la amiloidosis liquenoide, la amiloidosis macular y la amiloidosis nodular. La importancia de esta última deriva en que se ha descrito su progresión a amiloidosis sistémica. En este artículo revisaremos la clasificación, patogenia, manifestaciones clínicas, histopatología y tratamiento de las amiloidosis cutáneas primarias.
Тема - темы
Humans , Amyloidosis/pathology , Skin Diseases/pathology , Amyloidosis/classification , Amyloidosis/therapy , Skin Diseases/therapyРеферат
The authors report one case of amyloidosis associated with muscular pseudohypertrophy in a 46-year-old woman, who developed weakness, macroglossia and muscle hypertrophy associated with primary systemic amyloidosis. Electromyography showed a myopathic pattern and bilateral carpal tunnel syndrome. The muscle biopsy presented with a type I and II fiber hypertrophy and infiltration of amyloid material in the interstitious space and artery walls. She underwent bone marrow transplantation with stabilization and subjective improvement of the clinical picture
Тема - темы
Humans , Female , Middle Aged , Amyloidosis/complications , Muscular Diseases/complications , Amyloidosis/diagnosis , Amyloidosis/therapy , Bone Marrow Transplantation/methods , Hypertrophy/complications , Hypertrophy/diagnosis , Hypertrophy/therapy , Muscles/pathology , Muscular Diseases/diagnosis , Muscular Diseases/therapyРеферат
Presentamos el caso de un paciente de 62 años con amiloidosis sistémica. El diagnóstico definitivo se confirmó por biopsia hepática. Se hace una revisión de las características más importantes, diagnóstico, tratamiento y pronóstico de esta enfermedad, resaltando la importancia de la sospecha clínica para llegar al diagnóstico.
Тема - темы
Humans , Male , Adult , Amyloidosis/diagnosis , Amyloidosis/therapy , PrognosisРеферат
O termo Amiloidose refere-se a um grupo de condiçöes clínicas caracterizadas pelo depósito de material proteico anormal no espaço extrecelular dos órgäos e tecidos. Os amiloidomas säo incomuns e näo estäo associados à amiloidose sistêmica. Define-se a amiloidose pulmonar localizada como o depósito amilóide restrito ao trato respiratório, sem deposisçäo sistêmica...
Тема - темы
Humans , Male , Aged , Amyloidosis/physiopathology , Amyloidosis/diagnosis , Amyloidosis/therapy , Diagnosis, DifferentialРеферат
Realizamos uma revisäo sobre as principais doenças granulomatosas que podem acometer a laringe, devido à grande ocorrência dessas afecçöes no Brasil e em outros países de clima tropical e subtropical. Ressaltamos os aspectos histopatológicos para o seu diagnóstico, assim como o seu tratamento.
Тема - темы
Humans , Actinomycosis/physiopathology , Amyloidosis/physiopathology , Blastomycosis/physiopathology , Pharyngeal Diseases/physiopathology , Granulomatosis with Polyangiitis/physiopathology , Leprosy/physiopathology , Histoplasmosis/physiopathology , Leishmaniasis/physiopathology , Rhinoscleroma/physiopathology , Sarcoidosis/physiopathology , Syphilis/physiopathology , Tuberculosis/physiopathology , Actinomycosis/diagnosis , Actinomycosis/drug therapy , Amyloidosis/diagnosis , Amyloidosis/therapy , Blastomycosis/diagnosis , Blastomycosis/drug therapy , Pharyngeal Diseases/diagnosis , Pharyngeal Diseases/drug therapy , Pharyngeal Diseases/therapy , Granulomatosis with Polyangiitis/diagnosis , Granulomatosis with Polyangiitis/drug therapy , Leprosy/diagnosis , Leprosy/drug therapy , Histoplasmosis/diagnosis , Histoplasmosis/drug therapy , Leishmaniasis/diagnosis , Leishmaniasis/drug therapy , Rhinoscleroma/diagnosis , Rhinoscleroma/drug therapy , Sarcoidosis/diagnosis , Sarcoidosis/drug therapy , Syphilis/diagnosis , Syphilis/drug therapy , Tuberculosis/diagnosis , Tuberculosis/drug therapyРеферат
Alzheimer's Disease. The untold story. After considering the potential relationship between amyloid deposits and myxedematous infiltrations, the hypothesis is formulated that Alzheimer's disease may be due to functional hypothyroidism and may thus respond to thyroid therapy
Тема - темы
Humans , Amyloidosis/complications , Alzheimer Disease/etiology , Brain Diseases/complications , Hypothyroidism/complications , Amyloidosis/therapy , Alzheimer Disease/therapy , Brain Diseases/therapy , Hypothyroidism/therapyРеферат
Describimos el caso de una paciente con amiloidosis sistémica adquirida, quien presentó compromiso de la columna lumbar con lesiones líticas y fracturas de cuerpos vertebrales por invasión amiloide, polineuropatía documentada por electromiografía y hepatomegalia evidenciada por gamagrafía y examen físico. El compromiso directo de hueso se corroboró por biopsia de L4. La paciente se ha manejado con colchicina fundamentalmente y en la actualidad tiene supervivencia de 12 años y se encuentra en buen estado general. Es el primer caso en la literatura médica confirmado de affeción de columna lumbar por amiloidosis.