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Three types of Griscelli syndrome are recognised: type 1 has neurologic symptoms and mutations in MYO5A. Prognosis depends on the severity of neurologic manifestations. Type 2 has mutations in RAB27A and haemophagocytic syndrome, with abnormal T-cell and macrophage activation. This type has a grave prognosis if untreated.
Despite marked systemic inflammation, the erythrocyte sedimentation rate (ESR) is paradoxically depressed, caused by low fibrinogen levels. The low ESR helps to distinguish the disorder from a flare of the underlying rheumatic disorder, in which case the ESR is usually elevated. A bone marrow biopsy or aspirate usually shows hemophagocytosis.
Autoimmune lymphoproliferative syndrome (ALPS) is a form of lymphoproliferative disorder (LPDs). It affects lymphocyte apoptosis. [2]It is a rare genetic disorder of abnormal lymphocyte survival caused by defective Fas mediated apoptosis. [3]
Sudden death syndrome SHF Systolic heart failure: SIDS Sudden infant death syndrome: SIRS Systemic inflammatory response syndrome: SIS Shaken infant syndrome: SLE Systemic lupus erythematosus: SM Selective Mutism: SJS Stevens–Johnson syndrome: SMA Spinal muscular atrophy: SMD Sensory modulation disorder SMEI Severe myoclonic epilepsy of ...
These inflammatory cytokines cannot be cleared and inflammatory mediators cause fever, cytopenias, coagulopathy, and central nervous system inflammation, which can progress to sepsis-like pathophysiology, shock, and death. The progression of macrophage activation in the context of rheumatic diseases is historically called MAS, and in the ...
The primary cause triggers an uncontrolled inflammatory response. [citation needed] Sepsis is the most common cause of multiple organ dysfunction syndrome and may result in septic shock. In the absence of infection, a sepsis-like disorder is termed systemic inflammatory response syndrome (SIRS). Both SIRS and sepsis could ultimately progress to ...
Inflammation is an important and growing area of biomedical research and health care because inflammation mediates and is the primary driver of many medical disorders and autoimmune diseases, including ankylosing spondylitis, psoriasis, psoriatic arthritis, Behçet's disease, rheumatoid arthritis, inflammatory bowel disease (IBD), and allergy ...
Macrophages have been classified as M1 or M2 depending on the adaptive immune response that elicited the phenotype: Th1 or Th2 respectively. [2] [4] [5] The phrase 'alternatively activated macrophage' is used to refer to M2 macrophages. [2] Regulatory macrophages do not fit into the M1/M2 classification system, and they display different ...