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Macrophage activation syndrome is a severe, potentially life-threatening, complication of several chronic rheumatic diseases of childhood. It occurs most commonly with systemic-onset juvenile idiopathic arthritis (SoJIA).
Reduced life expectancy Maroteaux–Lamy syndrome , or Mucopolysaccharidosis Type VI (MPS-VI) , is an inherited disease caused by a deficiency in the enzyme arylsulfatase B (ARSB). [ 3 ] ASRB is responsible for the breakdown of large sugar molecules called glycosaminoglycans (GAGs, also known as mucopolysaccharides).
It is a life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines. It is classified as one of the cytokine storm syndromes. There are inherited and non-inherited (acquired) causes of HLH.
While the disease is fatal, the age of onset is a key factor, as infants have a typical life expectancy of 2–8 years, while adults typically live more than a decade after onset. Treatment options are limited, although hematopoietic stem cell transplantations using bone marrow or cord blood seem to help in certain leukodystrophy types, while ...
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 ...
MIP-1γ is another macrophage inflammatory protein and according to the new nomenclature is named CCL9. [3] It is produced mainly by follicle-associated epithelial cells and is responsible for chemotaxis of dendritic cells and macrophages into Peyer's patches in gut through binding of CCR1. [11] MIP-1δ or MIP-5 (CCL15) binds also CCR1 and CCR3 ...
Pyroptosis is a highly inflammatory form of lytic programmed cell death that occurs most frequently upon infection with intracellular pathogens and is likely to form part of the antimicrobial response. This process promotes the rapid clearance of various bacterial, viral, fungal and protozoan infections by removing intracellular replication ...
Systemic or local inflammatory responses may occur with improvement in immune function. While this inflammatory reaction is usually self-limited, there is risk of long-term symptoms and death, particularly when the central nervous system is involved. [3] [4] Management generally involves symptom control and treatment of the underlying infection.