RESUMO
Hereditary angioedema is a congenital disorder with recurrent attacks of localized swelling of submucosal and subcutaneous tissue, or both caused by a deficiency of the plasma protein C1 inhibitor. It is caused by heterozygous defects in the C1 inhibitor gene located on chromosome 11q, and it has an autosomal dominant inheritance pattern. This disease afflicts 1 in 10,000 to 1 in 150,000 persons. Hereditary angioedema has been reported in all races, and no sex predominance has been found. Skin and visceral organs may be involved by the typically massive local edema. The most commonly involved viscera are the respiratory and gastrointestinal systems, and it can affect the upper airways resulting in severe life-threatening symptoms, including the risk of asphyxiation. There are three types of hereditary angioedema, which difference lies in the inheritance pattern and in the C1 esterase inhibitor and C4 concentrations. The treatment is complicated and it should be treated with intravenous purified C1 inhibitor concentrate; corticosteroids, antihistamines and epinephrine can be useful adjuncts but they are not effective. We report a patient with hereditary angioedema type 1 and make a review of the medical literature.
Assuntos
Angioedema/genética , Proteínas Inativadoras do Complemento 1/deficiência , Serpinas/deficiência , Adulto , Angioedema/classificação , Angioedema/tratamento farmacológico , Angioedema/epidemiologia , Angioedema/fisiopatologia , Angioedema/terapia , Bradicinina/fisiologia , Terapia Combinada , Proteínas Inativadoras do Complemento 1/genética , Proteínas Inativadoras do Complemento 1/uso terapêutico , Proteína Inibidora do Complemento C1 , Complemento C4/deficiência , Danazol/uso terapêutico , Quimioterapia Combinada , Feminino , Genes Dominantes , Humanos , Incidência , Serpinas/genética , Serpinas/uso terapêuticoRESUMO
BACKGROUND: Atopic dermatitis is a skin inflammatory disease which has been associated to high levels of IgE, eosinophiles and change of T lymphocytes. The transfer factor is an immunomodulator active substance and decreases the number of inflammatory cells and the severity of the symptoms of atopic dermatitis. OBJECTIVE: To determine the efficacy of the transfer factor as treatment of moderate and severe atopic dermatitis. MATERIAL AND METHODS: Articles related to treatment with transfer factor in the atopic dermatitis were looked up in Medline and EMBASE, and the ones referring to controlled studies in patients with moderate and severe atopic dermatitis in accord to SCORAD. RESULTS: We found seven articles with 121 patients and 88 controls demonstrating significant decrease in the symptoms of the SCORAD index, decreased IgE, and eosinophils in patients treated with transfer factor. CONCLUSIONS: The transfer factor is a choice treatment for moderate and severe atopic dermatitis.
Assuntos
Dermatite Atópica/tratamento farmacológico , Fator de Transferência/uso terapêutico , Dermatite Atópica/imunologia , Humanos , Índice de Gravidade de DoençaRESUMO
BACKGROUND: First reports on sublingual immunotherapy were published in 1980. OBJECTIVE: To compare safety and effectiveness of sublingual immunotherapy, as compared with placebo, in asthmatic patients. MATERIALS: In a blinded randomized controlled trial asthmatic patients with positive skin prick tests to Dermatophagoides pteronyssinus, and with serum IgE at least 200 UI were included. According to GINA, asthma severity was mild persistent and moderate. All patients improved their baseline FEV1 at least by 14% after inhaled albuterol. Spirometry was performed again after three and six months after initiating treatment. Patients were randomized to receive for six months either sublingual immunotherapy with Der p 1 standardized allergens (IPI-ASAC, México) at a total dose of 10,469 UBE or identically looking and tasting placebo. Both groups received conventional pharmacological therapy. RESULTS: Sixty four patients enter the study; four were excluded because of systemic oral steroid therapy. Sixty patients underwent randomization. Both groups (30 patients in each one) were similar in their baseline characteristics. After six months, patients that received sublingual immunotherapy had less exacerbations than those in the control group (61 vs 123, T 2.61, p<0.001, IC 1.8-7.2), better FEV1 as compared with baseline values (25% vs 9%, Z=0.66, p=0.03), and less need of albuterol (50% of initial dose, vs 21% (Z=1.4, p=0.03, IC 1.39-1.49). CONCLUSION: Sublingual immunotherapy improves patient symptoms and pulmonary functional tests, makes exacerbations less frequent, and reduces albuterol needs. It may improve asthma related quality of life.