RESUMO
Hereditary (HAE) and acquired (AAE) angioedema are vascular reactions involving the sub mucosal tissues, representing localized edema caused by dilatation and increased permeability of the capillaries. HAE and AAE are clinical disorders characterized by angioedema that require prompt differentiation from other causes of angioedema in order to receive the most pertinent and effective therapeutic interventions. The aim of this report is to describe the clinical characteristics of patients with both HAE and AAE identified and followed at the Immunology Clinic of the University Hospital at the Puerto Rico Medical Center, their response and side effects to danazol therapy and their comparison with other series of similar patients reported in the literature. Overall, the patients in this sample presented a similar clinical profile compared to other reported series in the literature.
Assuntos
Angioedema , Adolescente , Adulto , Idoso , Angioedema/diagnóstico , Angioedema/tratamento farmacológico , Angioedema/genética , Angioedema/imunologia , Criança , Complemento C1/análise , Complemento C1q/análise , Complemento C4/análise , Proteínas Inativadoras do Complemento/análise , Danazol/administração & dosagem , Danazol/efeitos adversos , Danazol/uso terapêutico , Interpretação Estatística de Dados , Diagnóstico Diferencial , Ensaio de Imunoadsorção Enzimática , Antagonistas de Estrogênios/administração & dosagem , Antagonistas de Estrogênios/efeitos adversos , Antagonistas de Estrogênios/uso terapêutico , Feminino , Humanos , Imunodifusão , Masculino , Pessoa de Meia-Idade , Fatores de TempoRESUMO
Sheep hydatid cyst fluid (SHCF) was fractionated on Sephacryl HR S-200 and the anticomplementary activity (alpha-C activity) determined in the fractions obtained; 67% of total alpha-C activity of SHCF was recovered in the void volume fraction (SHCF-I) which contained 61% of SHCF carbohydrates. The bulk of the activity of SHCF-I was eluted by FPLC chromatofocusing on Mono P HR at pH 5.9. After heating at 100 degrees C for 15 min, SHCF and SHCF-I conserved 74 and 54%, respectively of their alpha-C activity. In addition, 37 and 11% of SHCF and SHCF-I alpha-C activity, respectively bound to Protein A. Components not bound to Protein A (SHCFPA and SHCF-IPA) were fractionated on Con A-Sepharose; 71 and 65%, respectively of their total alpha-C activity was retained by this lectin indicating the presence of alpha-D mannoside and alpha-D glucoside residues in the active molecules. Our results suggest that SHCF could contain two classes of alpha-C components: immune complexes and thermoresistant molecules with high carbohydrate content.
Assuntos
Proteínas Inativadoras do Complemento/análise , Proteínas do Sistema Complemento/imunologia , Equinococose/veterinária , Doenças dos Ovinos/imunologia , Animais , Proteínas Inativadoras do Complemento/química , Equinococose/imunologia , OvinosRESUMO
A 7-year-old boy with mild renal failure and signs and symptoms of acute poststreptococcal glomerulonephritis including severe hypocomplementemia had, by renal biopsy, numerous crescents but no deposits in the glomerular capillary loops. Instead, deposits identical in location and composition to those described for children with idiopathic rapidly progressive glomerulonephritis were present. The severe hypocomplementemia was found to be due to high levels of C3 nephritic factor; niether nephritic factor nor hypocomplementemia has been reported in rapidly progressive glomerulonephritis of the idiopathic type. Following prompt therapy with methylprednisolone intravenously, serologic abnormalities disappeared and renal function greatly improved, but a later biopsy showed 50% of the glomeruli obliterated by scarring. The case is of importance not only in indicating that severe hypocomplementemia does not rule out idiopathic rapidly progressive glomerulonephritis but also in adding to the list of diseases in which nephritic factor can be found.