the primary immunodeficiency (pi) disorders are abnormalities in development and maturation of the immune system. individuals with pi disease may experience frequent infections, which limit their abilities to exhibit physical and psychological well-being secondary to their illness. in this survey we compared health-related quality of life of primary immune deficient patients with healthy childr...

common variable immunodeficiency (cvid) is the most common symptomatic primary immunodeficiency disease, predisposing the patients to various tissue involvement and organ damage. here a 16-year-old boy is presented who was referred to our center with cough, dyspnea,cyanosis, and history of recurrent pneumonia. the diagnosis of cvid was made according to reduction all serum immunoglobulin levels...

Introduction: In tuberculosis (TB) endemic regions BCG vaccine is administered at birth in an effort to protect against neonatal tuberculous meningitis. However, this live vaccine facilitates overwhelming systemic infections by otherwise innocuous organisms in infants with cellular primary immunodeficiencies . Case Report: Our case is a seven month old infant who developed abscess at BCG vaccin...

This review, based on a talk given at the RCPE Respiratory Medicine Symposium 2014, outlines the clinical spectrum of immune deficiency - antibody (B cell) deficiency, T cell defects and innate/phagocytic disorders - and discusses the relevant clinical presentations, investigations and treatments, focusing particularly on the management of adults with recurrent respiratory infections. It descri...

Middle East and North Africa region (MENA) populations are of different ethnic origins. Consanguineous marriages are common practice with an overall incidence ranging between 20 and 50%. Primary immunodeficiency diseases (PIDs) are a group of heterogeneous genetic disorders caused by defects in the immune system that predisposes patients to recurrent infections, autoimmune diseases, and maligna...

Primary immunodeficiency disorder (PID) refers to a heterogeneous group of over 130 disorders that result from defects in immune system development and/or function. PIDs are broadly classified as disorders of adaptive immunity (i.e., T-cell, B-cell or combined immunodeficiencies) or of innate immunity (e.g., phagocyte and complement disorders). Although the clinical manifestations of PIDs are h...

Adaptive immune mechanisms recognize and neutralize foreign molecules or microorganisms in a specific manner. Lymphocytes, B and T cells, can respond specifically to thousands of nonself materials. Adaptation is further acquired with memory of previous infections. Immunodeficiencies impair the functioning of the immune system. Deficiencies are highly variable with regard to symptoms, phenotype,...

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...

background if skeletal system does not have a correct form in childhood, certainly person will face many problems in the later stages of life. this study aimed to evaluate the prevalence of skeletal disorders in primary school students in abadan, 2015. materials and methods  this cross-sectional study was conducted on 383 primary school students in abadan, iran, which were selected by cluster s...