ABSTRACT
Much emphasis has been given to the deafness of Ludwig van Beethoven and its potential causes. However, when analyzing several symptoms reported by himself throughout his life in many letters and his final illness, a common etiology emerges. This article reports the medical history of this artist, based on authoritative scientific sources.
Subject(s)
Humans , History, 18th Century , History, 19th Century , Inflammatory Bowel Diseases/history , Deafness/history , Famous Persons , Immune System Diseases/history , Music/history , Inflammatory Bowel Diseases/complications , Deafness/etiology , Germany , Immune System Diseases/etiology , Liver Cirrhosis/historyABSTRACT
Miocardites são um grupo de doenças heterogêneas que podem ser compreendidas com uma fisiopatologia imunológica. Os autores discutem aspectos históricos e a imunologia dessas doenças, correlacionando o trabalho de cientistas como Carlos Chagas, Noel Rose e Nelson Vaz. Levam em consideração a evolução histórica do conhecimento científico e a mudança de paradigma em andamento na imunologia. Também analisam as principais manifestações clínicas e estratégias para o diagnóstico e abordam novas propostas para investigação clínica dessas síndromes.
Subject(s)
Myocarditis/diagnosis , Myocarditis/physiopathology , Myocarditis/therapy , Immune System/pathology , Immune System Diseases/complications , Immune System Diseases/etiologyABSTRACT
Introducción: El Síndrome de Activación Macrofágico (SAM) es una entidad poco frecuente en la práctica pediátrica que se caracteriza por una excesiva activación del sistema macrofágico y por una liberación exagerada de citoquinas por parte de los linfocitos T, y que clínicamente se manifiesta como un síndrome semejante a una falla orgánica múltiple. Existe actualmente disparidad en la nomenclatura de este síndrome, y es así como a nivel de la reumatología pediátrica se mantiene el término de SAM, mientras que para los hemato-oncólogos esta enfermedad está incluida dentro de las diferentes variedades de histiocitosis. Objetivo: Actualizar el conocimiento respecto de la etiología, clínica y tratamiento del SAM, enfermedad de baja frecuencia y alta mortalidad, en relación al análisis de 4 casos clínicos. Casos clínicos: 2 pacientes eran portadores de una enfermedad reumatológica, Artritis Idiopática Juvenil, el tercer paciente presentaba elementos de Inmunodeficiencia, y el último niño de linfohistiocitosis familiar congénita. Los factores desencadenantes del SAM fueron fármacos en dos pacientes y una posible causa infecciosa en los dos restantes. Los cuatro pacientes sobrevivieron al proceso inicial después de recibir una terapia agresiva inmunosupresora con esteroides en altas dosis y ciclosporina intravenosa. Conclusión: La importancia de dar a conocer este síndrome, radica en que un diagnóstico precoz y una terapia agresiva, preferentemente con ciclosporina y pulsos de esteroides en altas dosis, puede mejorar significativamente el pronóstico de esta enfermedad.
Subject(s)
Humans , Adolescent , Female , Infant , Child , Arthritis, Juvenile/complications , Arthritis, Juvenile/immunology , Immune System Diseases/diagnosis , Immune System Diseases/etiology , Immune System Diseases/drug therapy , Macrophage Activation , Immunosuppressive Agents/therapeutic use , Histiocytosis , Streptococcal Infections/complications , Methylprednisolone/therapeutic use , Methotrexate/adverse effects , Sulfasalazine , Syndrome , Treatment OutcomeABSTRACT
Primary immunodeficiencies [PID] seem to have two particularities in the Maghreb countries: a higher frequency than in the occidental countries and a higher proportion of deficiency of HLA class II antigens. Patients and method: a four-year study reports 73 PID cases between 1997 March and 2001 February in the Clinical Immunology Unit of the Casablanca Children Hospital. These 73 cases were diagnosed, according to the WHO criteria, among 358 children with recurrent and/or severe infections. They had an immunological assessment: tuberculin IDR, blood cells, serum immunoglobulin A G and M, lymphocytes classes. we found 18 combined immunodeficiency including 5 deficiencies of HLA class II antigens, 18 antibody defects, 17 complex immunodeficiencies including 11 ataxia-telangiectasia syndromes, 13 PID associated to an other disorder including 4 hyper IgM syndrome, 6 phagocyte disorders and one complement defect. Because of their high cost, the intravenous immunoglobulins were used in only 29 children and the bone marrow transplantation is not available in our country. Twenty six children died in this study. Comments: as in Tunisia, our study confirms the high frequency of combined and complex PID in comparison to the occidental studies, and we have to develop the PID diagnostic and treatment resources in our country
Subject(s)
Humans , Male , Female , Immune System Diseases/etiology , Immune System Diseases/classification , Consanguinity , ChildABSTRACT
Blood components are indicated in a wide variety of disease states. Although most transfusion therapies are administered uneventfully, there are a number of potential adverse transfusion reactions, some of which can assume serious dimensions. These reactions could occur during or even days after a transfusion. A brief description of the adverse effects of transfusion therapy has been outlined in this review. The etiopathogenesis, recognition and treatment of the adverse transfusion reactions have been highlighted. It is imperative that each transfusion of blood components has components be monitored carefully. Prompt recognition of an adverse event and early institution of remedial measures would help in decreasing transfusion related morbidity and mortality.
Subject(s)
Blood Group Incompatibility/diagnosis , Blood Transfusion/adverse effects , Hemolysis , Humans , Immune System Diseases/etiology , Risk FactorsABSTRACT
Immunologic disturbance of T and B lymphocytes was reported in uremic patients. An approach to the etiology of such immunocompromization was made by determination of plasma adenosine deaminase [ADA], 5'-nucleotidase, IgG, zinc and vitamin B6 together with erythrocyte adenosine-5-phosphate deaminase [AMP], adenosine deaminase [ADA] and 5'-nucleotidase in 30 uremic patients before and after dialysis. The results were compared with their counterparts in 30 healthy controls which were age matched to patients. The results clarified that the immune disturbances in patients with uremia were multifactorial resulting from decreased ADA together with nutritional deficiency of zinc and vitamin B6. Therefore, supplement of these patients with deficient nutrients should be considered
Subject(s)
Immune System Diseases/etiology , Kidney Failure, Chronic/physiopathology , Renal Dialysis , Renal InsufficiencyABSTRACT
A relacao entre a incidencia de infeccoes e o estado nutricional de ferro e bastante controvertida, sendo relatados tanto maioir susceptibilidade como maior resistencia a infeccoes na anemia ferropriva. O ferro e essencial para o cresciemnto de mocroorganismos e a sua carencia ao lado de limitar a proliferacao dos mesmos determina alteracoes imunologicas no hospedeiro. Estas anormalidades caracterizam uma imunodeficiencia multifatorial, porem parcial. Assim, a susceptibilidade a infeccoes nos pacientes com anemia ferropriva seria determinada pelo balanco entre as alteracoes imunologicas e a limitacao do crescimento dos microorganismos condicionada pela carencia de ferro. Variacoes do equilibrio deste binomio poderiam explicar os resultadosdiscrepantes assinaladosna literatura