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Abstract Hemophilia is a hemorrhagic disorder with a sex-linked inherited pattern, characterized by an inability to amplify coagulation due to a deficiency in coagulation factor VIII (hemophilia A or classic) or factor IX (hemophilia B). Sequencing of the genes involved in hemophilia has provided a description and record of the main mutations, as well as a correlation with the various degrees of severity. Hemorrhagic manifestations are related to levels of circulating factor, mainly affecting the musculoskeletal system and specifically the large joints (knees, ankles and elbows). This document is a review and consensus of the main genetic aspects of hemophilia, from the inheritance pattern to the concept of women carriers, physiopathology and classification of the disorder, the basic and confirmation studies when hemophilia is suspected, the various treatment regimens based on infusion of the deficient coagulation factor as well as innovative factor-free therapies and recommendations for the management of complications associated with treatment (development of inhibitors and/or transfusion transmitted infections) or secondary to articular hemorrhagic events (hemophilic arthropathy). Finally, relevant reviews of clinical and treatment aspects of hemorrhagic pathology charachterized by acquired deficiency of FVIII secondary to neutralized antibodies named acquired hemophilia.
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BACKGROUND: Chronic idiopathic urticaria is a clinic entity that is manifested by wheals of more than six weeks of evolution, without identification of the causing agent, and sometimes resistant to conventional treatment. There are improvements with leukotriene receptor antagonists. OBJECTIVE: To evaluate the clinical efficacy of montelukast and desloratadine individually and combined, compared with hydroxicine. PATIENTS AND METHODS: The trial included 40 subjects with chronic idiopathic urticaria referred from the outpatient allergy service. They were randomly divided into four groups to receive: hydoxicine, montelukast, montelukast plus desloratadine, and desloratadine alone during six weeks. The assessments compared the first and sixth weeks. RESULTS: All therapeutic options are effective, with statistical significance, highlighting that the new therapeutic modes are safe and have better resolution of lesions and symptoms. CONCLUSIONS: All the alternatives are viable, considering they are adjusted to each patient, adverse symptoms, socio-economic status and clinical severity.
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Acetatos/uso terapéutico , Angioedema/tratamiento farmacológico , Antialérgicos/uso terapéutico , Antagonistas de los Receptores Histamínicos H1 no Sedantes/uso terapéutico , Antagonistas de los Receptores Histamínicos H1/uso terapéutico , Hidroxizina/uso terapéutico , Antagonistas de Leucotrieno/uso terapéutico , Loratadina/análogos & derivados , Quinolinas/uso terapéutico , Urticaria/tratamiento farmacológico , Acetatos/administración & dosificación , Antialérgicos/administración & dosificación , Antipruriginosos/administración & dosificación , Antipruriginosos/uso terapéutico , Enfermedad Crónica , Ciclopropanos , Esquema de Medicación , Quimioterapia Combinada , Antagonistas de los Receptores Histamínicos H1/administración & dosificación , Antagonistas de los Receptores Histamínicos H1 no Sedantes/administración & dosificación , Humanos , Antagonistas de Leucotrieno/administración & dosificación , Loratadina/administración & dosificación , Loratadina/uso terapéutico , Estudios Prospectivos , Quinolinas/administración & dosificación , Sulfuros , Resultado del TratamientoRESUMEN
OBJECTIVE: To compare the efficacy of nasal budesonide plus oral zafirlukast against nasal budesonide plus oral loratadine/pseudoephedrine combination in the control of symptoms of rhinitis and asthma. PATIENTS AND METHODS: A controlled, clinical, randomized, double blind and crossover study was made in 36 patients with allergic rhinitis and asthma following one of the next treatment regimes: group a) nasal budesonide plus oral zafirlukast twice a day or group b) nasal budesonide plus oral loratadine/pseudoephedrine twice a day, both of them during six weeks, and two weeks of washing and crossover of the treatments during six more weeks. Changes in the rhinitis and asthma symptoms, blood eosinophils, pulmonary function testing, and nasal cytology were evaluated before and after the treatment. RESULTS: 19 patients were assigned to group a, whereas 17 patients to group b. The age ranged between 16 to 45 years, and it predominated the female group, 70 and 89%, respectively (statistically no significant). During the first six weeks of the treatment, V0 to V3, both groups of patients got better nasal symptoms but group a was superior to group b. However, in bronchial symptoms, cough, wheezing and breathlessness, group a showed efficacy in comparison with group b, where no significant improvement was shown. Once the crossover was made, from V5 to V7, there was no difference between both groups. The other evaluated indicators, such as eosinophilia, VEF1 and nasal eosinophils, had a significant improvement before and at the end of the study. CONCLUSIONS: The association of a nasal steroid with a leukotriene modifier (zafirlukast) was more effective for controlling nasal symptoms and especially bronchial symptoms than the association of a nasal steroid with antihistamines (loratadine) with pseudoephedrine. Other inflammation indicators, such as eosinophilia and nasal eosinophilia, were diminished; the VEF1 increased significantly in both treatment groups. All the above may be due to the nasal steroid use associated to a leukotriene modifier.
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Adrenérgicos/uso terapéutico , Antialérgicos/uso terapéutico , Antiasmáticos/uso terapéutico , Budesonida/uso terapéutico , Efedrina/uso terapéutico , Antagonistas de los Receptores Histamínicos H1 no Sedantes/uso terapéutico , Antagonistas de Leucotrieno/uso terapéutico , Loratadina/uso terapéutico , Hipersensibilidad Respiratoria/tratamiento farmacológico , Compuestos de Tosilo/uso terapéutico , Administración Intranasal , Administración Oral , Adolescente , Adrenérgicos/administración & dosificación , Adulto , Antialérgicos/administración & dosificación , Antiasmáticos/administración & dosificación , Asma/tratamiento farmacológico , Budesonida/administración & dosificación , Estudios Cruzados , Método Doble Ciego , Quimioterapia Combinada , Efedrina/administración & dosificación , Femenino , Antagonistas de los Receptores Histamínicos H1 no Sedantes/administración & dosificación , Humanos , Indoles , Antagonistas de Leucotrieno/administración & dosificación , Loratadina/administración & dosificación , Masculino , Persona de Mediana Edad , Fenilcarbamatos , Rinitis Alérgica Perenne/tratamiento farmacológico , Rinitis Alérgica Estacional/tratamiento farmacológico , Sulfonamidas , Compuestos de Tosilo/administración & dosificación , Resultado del TratamientoRESUMEN
INTRODUCTION: In 1980 AIDS appeared in the USA in the homosexual community. CHARACTERISTICS OF THE VIRUS: A particle of HIV is formed by two identical RNA chains that are packed within a center of viral proteins, and surrounded by a double layer of fosfolipids derived from the cellular membrane of the host. CLINICAL SYMPTOMS: It starts with a severe infection, evolving in a progressive chronic infection reaching a final phase with a T CD4+ cellular count under 200 cells per mm3, with incident infections, neoplasias, consumption syndrome, renal dysfunction, and degeneration of the central system. CONSEQUENCES IN ALLERGIC REACTION: Reactions to the use of drugs are the most frequent form of hypersensitivity caused by HIV compared to the general population. In this condition, a profile of TH2 cytocines are produced and they induce the synthesis of IgE against the drugs. IgE levels have been related to the progression of this condition. CONCLUSION: The increase of IgE is a risk factor to allergic reactions against these medications, as well as a severity index.
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Infecciones por VIH/complicaciones , Hipersensibilidad/etiología , Humanos , Hipersensibilidad/epidemiología , Hipersensibilidad/inmunologíaRESUMEN
BACKGROUND: It has been established that 27-50% of patients with idiopathic chronic urticaria have antibodies directed against the alpha chain of the high-affinity IgE receptor, which are indirectly detected by cutaneous tests with autoserum. Thus, an autoimmune urticaria diagnosis can be settled. OBJECTIVE: To prove methotrexate's efficiency in patients with autoimmune urticaria. MATERIAL AND METHODS: Seven patients took part in the study. A series of tests was performed in order to rule out any possible infectious, metabolic, or physical etiology. Initial treatment with methotrexate with doses of 2.5 mg every 12 hours, two days a week was provided. In case there were no toxicity data, doses would increase to three days a week for a 6-week period. RESULTS: Statistically significant improvement was observed in the itching, as well as the presence of spots, repercussion on daily activities, sleep disorders. There was no statistical difference regarding the extension of the lesions and the presence of angioedema. Adverse effects were not significant. CONCLUSION: We conclude that methotrexate is effective in the treatment of autoimmune urticaria.