background: primary immunodeficiency disorders (pid) are a group of hereditary disorders characterized by an increased susceptibility to severe and recurrent infections, autoimmunity, lymphoproliferative disorders, and malignancy. objective: to evaluate the demographic and clinical data of pid patients diagnosed in a referral pediatric hospital. method: all pid cases with a confirmed diagnosis,...

epidemiological studies have shown wide geographical and racial variation in the prevalence and patterns of immunodeficiency disorders. to determine the frequency of primary immunodeficiencies (pid) in iran, the iranian primary immunodeficiencies registry (ipidr) was organized in 1999. the diagnosis of immunodeficiency in our patients was based on standard criteria. the patient’s data were extr...

objective: primary immunodeficiency diseases (pid) are rare and heterogeneous congenital diseases leading to increased unusual susceptibility to developing infections and causingsome malignancies and autoimmune diseases. this study was conducted to evaluate the epidemiological characteristics of primary these diseases in patients attending the clinic of immunodeficiency diseases in kerman.subje...

Major histocompatibility complex (MHC) class II deficiency is a primary immunodeficiency disease characterized by abnormality of MHC class II molecules surface expression on peripheral blood lymphocytes and monocytes. Clinical manifestations include extreme susceptibility to viral, bacterial, and fungal infections but the immunodeficiency is not as severe as SCID (severe combined immunodeficien...

primary immunodeficiency diseases (pids) consist of a group of genetic disorders that predispose the patients to immune-mediated complications. the aim of this study was to assess the knowledge of iranian general practitioners and pediatricians about pids. a questionnaire consisting 52 closed questions on clinical symptoms, laboratory data,associated syndromes and management of pids patients wa...

primary immunodeficiency disorders are a heterogeneous group of genetic disorders, with different modes of inheritance, consisting of more than 100 different types. we constructed the dna banking of primary immunodeficiency disorders for the first time in iran. the dna of 31 immunodeficient patients and their families (total of 92 samples) were collected, as the first step for construction of d...

Pyoderma vegetans (PV) is a rare inflammatory disorder characterized by vegetating pustules and plaques affecting the skin and mucosal membranes. It is believed that this entity is mostly associated with inflammatory bowel disease (IBD), chronic malnutrition, human immunodeficiency virus (HIV), malignancies, and other immunocompromised states. Pyoderma vegetans occurs more commonly in young and...

primary immunodeficiencies constitute a group of highly complex congenital disorders most of which are characterized by a very poor prognosis. allogeneic hematopoietic stem cell transplantation (hsct) has become an established curative treatment approach in many of these disorders, which may be permanently corrected. in this presentation basic and practical aspects of hsct are presented, with a...

conclusions subcutaneous nodules in the absence of a local reaction at the site of bcg vaccination may be the sole manifestation of disseminated bcg disease. case presentation we report on an 11-months-old boy with prolonged fever and diffuse hyperpigmented subcutaneous nodules. due to lymphopenia, oral thrush and severe adverse reaction to bcg vaccination, the possibility of primary immunodefi...