chronic granulomatous disease is an infrequent primary immunodeficiency characterized by defective intracellular killing of ingested microorganisms thereby making patients highly susceptible to recurrent lite threatening bacterial and fungal infections. in this study, we review the medical course of an 8 yr old girl with ar-cgd. she suffered from recurrent dermal and deep abscesses, retractable...

Background: Job’s syndrome or hyper-immunoglobulin E (IgE) (HIES) is an extremely rare primary immunodeficiency disease with approximate annual incidence of less than 1/1000000. It characterized by recurrent cold staphylococcal infections, unusual eczematous dermatitis, severe lung and extensively high concentrations the serum antibody IgE. Case Presentation: A typical case a family history Kaw...

Common variable immunodeficiency (CVID) is a primary immunological disease characterized predominantly by hypogammaglobulinemia. The main clinical manifestations are severe recurrent infections that often lead to structural damage of affected organs. The early start of adequate intravenous immunoglobulin therapy has significantly improved the prognosis of this serious disorder. Patients with CV...

PURPOSE OF REVIEW Dendritic cells are specialized antigen-presenting cells which link innate and adaptive immunity, through recognition and presentation of antigen to T cells. Although the importance of dendritic cells has been demonstrated in many animal models, their contribution to human immunity remains relatively unexplored in vivo.Given their central role in infection, autoimmunity, and m...

E osinophilia, defined as greater than 450 eosinophils/ L, can occur in various disease processes, including infectious, allergic, neoplastic, and primary hematologic disorders. Marked eosinophilia ([1500 eosinophils/ L) can be a diagnostic dilemma because blood eosinophilia rarely exceeds 1500/ L in atopic/allergic diseases. Hyperimmunoglobulinemia E is seen in allergic, parasitic, and primary...

Background Common variable immunodeficiency (CVID) is one of the most common primary immunodeficiency in adults and is characterized by defective antibody production, low levels of serum immunoglobulins and increased susceptibility to infection. About 20% of developed different gastrointestinal pathology. But also the pathology of the gastrointestinal tract in patients with CVID showed a wide s...

Background Primary ciliary dyskinesia (PCD) is a rare disorder (prevalence 1/20000), caused by congenital dysmotility of the respiratory cilia. Humoral immunodeficiency (HID) often presents in a similar way with recurrent ear, nose and sinopulmonary infections, not seldom evolving to chronic lung disease. Although isolated IgG subclass deficiencies and IgA deficiency are common conditions, Comm...

Feline immunodeficiency virus (FIV) induces a disease similar to acquired immunodeficiency syndrome (AIDS) in cats, yet in contrast to human immunodeficiency virus (HIV), CD4 is not the viral receptor. We identified a primary receptor for FIV as CD134 (OX40), a T cell activation antigen and costimulatory molecule. CD134 expression promotes viral binding and renders cells permissive for viral en...

We describe a child with primary defect of polymorph bacterial killing associated with systemic lungs erythematosus. We suggest that her autoimmune disease results from chronic bacterial antigen stimulation and propose a hypothetical model linking immunodeficiency with autoimmunity.