Epstein-Barr virus positive (EBV+) smooth muscle tumors (SMTs) constitute a very rare oncological entity. They usually develop in the context of secondary immunodeficiency caused by human immunodeficiency virus infection or immunosuppressive treatment after solid organ transplantation. However, in a small fraction of predominantly pediatric patients, EBV+ SMTs may occur in patients with primary...

epstein-barr virus (ebv) is a ubiquitous human -herpesvirus that infects about 95% of the adult population. the majority of primary infections occurs in early childhood and is generally subclinical; it can cause infectious mononucleosis (im), which is usually a self-limiting lymphoproliferative disorder. however, infection of ebv occasionally results in severe, often lethal diseases, which inc...

‘Swarnaprashana’ as a lehana chikitsa and several Ayurveda Rasayana drugs can boost the immune system in specific or nonspecific manner including both innate adaptive arms of response enhance body’s overall natural resistance to disease-causing agent rather than directly neutralize itself children. Aim: To critically review analyze role management childhood immunodeficiency disorders. Method: V...

objective: the aim of this study was to evaluate the type, severity and progression of psychiatric pathologies in a sample of 372 outpatients (age range 18–65 years) referred by their primary general practitioners (gps) to an urgent referral team (urt) based in a psychiatric hospital in aberdeen, scotland. this team offers immediate appointments (1- to 7-day delays) for rapid assessments and ea...

Lymphocyte proliferation assays (LPAs) are widely used to assess T-lymphocyte function of patients with human immunodeficiency virus infection and other primary and secondary immunodeficiency disorders. Since these assays require expertise not readily available at all clinical sites, specimens may be shipped to central labs for testing. We conducted a large multicenter study to evaluate the eff...

The marketing approval of genetically engineered hematopoietic stem cells (HSCs) as the first-line therapy for the treatment of severe combined immunodeficiency due to adenosine deaminase deficiency (ADA-SCID) is a tribute to the substantial progress that has been made regarding HSC engineering in the past decade. Reproducible manufacturing of high-quality, clinical-grade, genetically engineere...

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...

The underlying cause of the enigmatic coexistence of immunodeficiency and autoimmune disorders in patients with primary immunodeficiency such as Omenn syndrome is largely due to the inefficient negative selection within thymus where T cells develop. Recent advances in molecular biology and animal models answered one of the key questions on the relationship between the partially impaired T cell ...

Primary immunodeficiency diseases (PID) are congenital disorders secondary to an impaired immune response. Infections, autoimmune disorders, atopy, and lymphoproliferative syndromes are commonly associated with this disorder. OBJECTIVE To present and discuss 3 infants diagnosed with PID. CLINICAL CASES The cases are presented of three patients with PID diagnosed during their first admission...

Recurrent, unusual and complicated infections are the hallmark of immunodeficiency. Primary Immunodeficiency (PID) is caused by a genetic defect impairing the immune response. Common Variable Immunodeficiency (CVID) is classified as a primary immunodeficiency with prevalent antibody defect, manifesting with respiratory and gastrointestinal infections. However, CVID is characterized by an increa...