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Idiopathic CD4+ lymphocytopenia (ICL) is a rare medical syndrome in which the body has too few CD4 + T lymphocytes, which are a kind of white blood cell. [2] ICL is sometimes characterized as "HIV-negative AIDS", though, in fact, its clinical presentation differs somewhat from that seen with HIV/AIDS. [ 3 ]
A congenital disorder of glycosylation (previously called carbohydrate-deficient glycoprotein syndrome) is one of several rare inborn errors of metabolism in which glycosylation of a variety of tissue proteins and/or lipids is deficient or defective. Congenital disorders of glycosylation are sometimes known as CDG syndromes.
Image of CD4 co-receptor binding to MHC (Major Histocompatibility Complex) non-polymorphic region. In molecular biology, CD4 (cluster of differentiation 4) is a glycoprotein that serves as a co-receptor for the T-cell receptor (TCR). CD4 is found on the surface of immune cells such as helper T cells, monocytes, macrophages, and dendritic cells.
The depletion of CD4 T cells and the development of chronic inflammation are signature processes in HIV pathogenesis that propel progression to acquired immune deficiency syndrome (AIDS). CD4 T cell depleted to the cell count of less than 200cell/μL in blood during AIDS allows various pathogens to escape T cell recognition, thus allowing ...
Carbohydrate-deficient Glycoprotein Syndrome (CDGS) Type Ia, Congenital Disorder of Glycosylation (CDG) Type Ia,Phosphomannomutase Deficiency [1],Jaeken Syndrome, PMM2-CDG , CDG1a PMM2 protein PMM2 deficiency or PMM2-CDG , previously CDG-Ia , is a very rare genetic disorder caused by mutations in PMM2 .
ITCLD-GT occurs more commonly in males of middle age (median age 48.4, range 15–77 years in one study). [2] Individuals with the disease present with GI tract symptoms which often are serious and/or debilitating [9] and may mimic those occurring in malignant lymphoproliferative, inflammatory, or autoimmune bowel diseases. [4]
Affected individuals typically present with low frequencies of CD4 +, a T-cell marker, and decreased circulation of regulatory T cells and iNKT cells. Notably, approximately 10% of people display CD4 + T cell counts lower than 200 cells/mm 3 ; this particular phenotype of CVID has been named LOCID (Late Onset Combined Immunodeficiency) and has ...
The goal serum trough is 5–15 ng/ml with Sirolimus and PCP prophylaxis is usually not needed. Sirolimus is a mTOR inhibitor active against lymphomas, especially EBV+ lymphomas. However, a risk with any immunosuppressive agent in a pre-cancerous syndrome is decreased tumor immunosurvellence.