Hereditary angioedema (HAE) is well documented condition with inherited deficiency or dysfunction of C1 inhibitor. However, its association other immune disorders such as autoimmunity, malignancy and primary immunodeficiency not known.

OBJECTIVE To evaluate medical knowledge of primary immunodeficiency in the city of São Paulo (SP). METHODS A 14-item questionnaire about primary immunodeficiency was applied to physicians who worked at general hospitals. One of the questions presented 25 clinical situations that could be associated or not with primary immunodeficiency, and the percentage of appropriate answers generated a kno...

Common variable immunodeficiency (CV ID) is a heterogeneous primary immunodeficiency disorder, characterized by hypogammaglobulinemia and increased susceptibility to recurrent bacterial infections. To determine the spectrum of infectious complications in patients with common variable immunodeficiency (CV ID), we reviewed the hospital records of 64 CVID patients, who were diagnosed in Child...

Patients with hypogammaglobulinemia who do not fulfill all the classical diagnostic criteria for common variable immunodeficiency (reduction of two immunoglobulin isotypes and a reduced response to vaccination) constitute a diagnostic and therapeutic dilemma, because information concerning the clinical and immunological characteristics of these patients with idiopathic primary hypogammaglobulin...

primary immunodeficiency diseases (pid) consist of a group of long-term illnesses which had permanent psychiatric effects on the patients and their parents. this study was designed to find out the most important origins and aspects of stressor in parents of pid patients.to assess the  impact of  psychiatric aspects in parents  of pid  patients, a valid and reliable questionnaire was compiled ba...

primary immunodeficiencies (pid) are a group of disorders, characterized by an unusual susceptibility to infections. delay in diagnosis results in increased morbidity and mortality in affected patients. the purpose of this study was to determine the mortality rate of iranian immunodeficient patients referred to children medical center hospital affiliated to tehran university of medical sciences...