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,...

Immune reconstitution inflammatory syndrome (IRIS) presents as an exaggerated immune reaction that occurs during dysregulated restoration in immunocompromised patients late-stage human immunodeficiency virus (HIV) infection who have commenced antiretroviral treatments (ART). Virtually any opportunistic pathogen can provoke this type of disorder. In review, we focus on recent developments the id...

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,...

Four related male infants presented with neonatal diabetes mellitus, immune dysregulation with extremely high concentrations of immunoglobulin E, and intractable diarrhoea. They were all from one family, and all of them died. As far as is known this X-linked recessive disorder has not been described before. It is suggested that this is a new immunodeficiency in which type 2 T helper responses p...

Stevens-Johnson syndrome (SJS) is an acute mucocutaneous disorder that can be associated with considerable morbidity. Several previous reports, all involving either adults with acquired immunodeficiency syndrome or children, suggest that intravenous immunoglobulin may be an effective treatment for SJS. We report a case of SJS in an immunocompetent adult whose condition improved dramatically aft...

Reactivation of Epstein-Barr virus infection and polyclonal expansion of lymphocytes is well-documented in solid organ and hematopoietic stem cell transplant recipients and is considered a potential precursor to lymphoma. We report an analogous case of posttransplantation polyclonal lymphoproliferative disorder in a patient with human immunodeficiency virus infection who was successfully treate...

A case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman syndrome) in a 42-year-old man is described. The disease involved the respiratory tract and skeletal apparatus, led to considerable general impairment and was unusually responsive to glucocorticoids. Molecular and immunophenotype analysis seem to confirm the reactive nature of a disorder that shows profound T-cell immunod...

Griscelli syndrome is a rare autosomal recessive disorder characterized by partial albinism with variable immunodeficiency. Silvery gray hair with large, clumped melanosomes on microscopy of hair shafts are diagnostic. The commonest complication leading to mortality includes lymphohistiocytic proliferation in various organs, including the brain. We present a child with classic clinical features...

Wiskott-Aldrich syndrome (WAS) is an X-linked disorder characterized by thrombocytopenia, eczema and immunodeficiency. WASP, the gene responsible for WAS, has been identified by positional cloning, contains a PH domain, a GBD domain, a proline rich region, and a verprolin/cofilin homology domain. Subsequent studies suggest that WASP is involved in signal transduction and in the regulation of th...

The Human Immunodeficiency Virus (HIV) is a neurotropic virus that associated with cognitive, behavioral and motor deficits known as HIV Associated Neurocognitive Disorder (HAND). This was prospective study aimed at determining the prevalence of cognitive impairment well comparing performance positive patients on screening test, International Dementia Scale (IHDS), subtests drawn from neuropsyc...