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...

1Unité Développement Normal et Pathologique du Système Immunitaire, INSERM U 429 and 2Laboratoire d’Anatomie Pathologique, Hôpital Necker–Enfants Malades, Paris, France. 3Service de Cytogénétique and 4Laboratoire d’Immunologie et d’Hématologie, Hôpitaux Universitaires de Strasbourg, Strasbourg, France. 5Unité d’Immunologie et d’Hématologie Pédiatrique and 6Centre d’Étude des Déficits Immunitair...

Two patients with severe combined immunodeficiency and enterovirus infections were successfully treated with pleconaril. There were no adverse affects.

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...

Background: The aim of the present study was to examine factors that may affect the outcome of testis tissue xenografting. Recipient factors were examined by grafting small fragments of testis tissue from newborn piglets under the back skin of immunodeficient mice of different strains [severe combined immunodeficiency (SCID) vs. nude), sex (male vs. female) and gonadal status (intact vs. gonade...

Despite the first application of gene therapy in 1990, gene therapy has until recently failed to meet the huge expectations set forth by researchers, clinicians, and patients, thus dampening enthusiasm for an imminent cure for many life-threatening genetic diseases. Nonetheless, in recent years we have witnessed a strong comeback for gene therapy, with clinical successes in young and adult subj...

Interleukin (IL)-2, IL-4, IL-7, IL-9, IL-15, and IL-21 form a family of cytokines based on the sharing of a receptor component, the common cytokine receptor γ chain, γ c, which is encoded by the gene mutated in humans with X-linked severe combined immunodeficiency (XSCID). Together, these cytokines play critical roles in lymphoid development, differentiation, growth, and survival as well as med...

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...