Consanguinity and primary immunodeficiencies.

Consanguinity in primary immunodeficiency disorders; the report from Iranian Primary Immunodeficiency Registry.

PROBLEM Primary Immunodeficiency Disorders (PiD) are a heterogeneous group of genetic disorders, with different modes of inheritance. This study was accomplished in order to determine the frequency of consanguineous marriages in the families of patients with PiD. METHOD In this study, the records 515 Iranian PiD patients were reviewed during a 25-year period. RESULTS The mean proportion of ...

Distribution of Primary Immunodeficiency Disorders Diagnosed in a Tertiary Referral Center, Tehran, Iran (2006-2013)

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,...

Cost-effectiveness analysis of subcutaneous immunoglobulin replacement therapy in Iranian patients with primary immunodeficiencies

    Background: Economic evaluation of subcutaneous immunoglobulin therapy (SCIG) is important, and it has recently been used for treatment of patients with primary immunodeficiency (PID) diseases, and can improve allocation of resources in health care systems. The present research aimed at providing an economic assessment of SCIG and IVIG (intravenous immunoglobulin therapy) adm...

Study of primary immunodeficiencies in Algeria

Primary immunodeficiencies (PIDs) are heritable disorders of immune system function. These defects are rare, but seem to be more frequent in populations with high consanguinity. The defect may affect T cells, B cells, phagocyte cells or complement proteins. Patients have increased susceptibility to recurrent infections, and may suffer from, allergy, auto-immune disorders or cancers. The assessm...

Primary immunodeficiency diseases (PIDs) is a diverse group of diseases, characterized by a defect in the immune system. These patients are susceptible to recurrent respiratory infections, gastrointestinal problems, autoimmune diseases, and malignancies. In most cases, patients with primary immunodeficiency disorders have genetic defects and are monogenic disorders that follow a simple Mendelia...

Clinical Predictors of Primary Immunodeficiency Diseases in Children

PURPOSE To promote awareness of primary immunodeficiency (PID), the "10 warning signs" of PID and an immunodeficiency-related (IDR) score were developed. However, their efficiency in identifying PID cases was not sufficiently evaluated in clinical practice. The objective of this study was to test the validity of the 10 warning signs and IDR score in identifying PID among children with recurrent...