Children's Hospital is part of the UPMC family.
Macrophage activation syndrome (MAS) is a type of systemic inflammatory response syndrome distinguished by high fever, cytopenias (low cell counts), liver dysfunction, clotting problems, extremely high levels of ferritin, and often hemophagocytosis (macrophages that have consumed other blood cells). Though rheumatologists grapple with MAS as a complication of some rheumatic diseases, MAS-like physiology is seen in other contexts, such as viral and bacterial infection, cancer and transplantation.
One feature that seems to define MAS is extraordinary elevation of the cytokine Interleukin-18 (IL-18). IL-18 requires an inflammasome for its activation, but until recently it was unclear whether IL-18 was helping drive MAS or was just an epiphenomenon. We recently discovered an association between the NLRC4 inflammasome, extraordinary IL-18, and MAS. We are actively working to fill in the mechanistic gaps.
A murine splenic hemophagocyte.
Increased proliferation of intestinal epithelial cells in NLRC4 gain-of-function mice.
Children's Hospital's main campus is located in the Lawrenceville neighborhood. Our main hospital address is:
UPMC Children’s Hospital of Pittsburgh
One Children’s Hospital Way
4401 Penn Ave.
Pittsburgh, PA 15224
In addition to the main hospital, Children's has many convenient locations in other neighborhoods throughout the greater Pittsburgh region.
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Interested in giving to Children's Hospital? Visit Children's Hospital of Pittsburgh Foundation's website to make a donation online.