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diagnosis syndrome activation antinxp2 antinxp2dm behcet clarksons dermatology dermatomyositis hematology hemophagocytic leak lymphohistiocytosis macrophage mas signs summary symptoms timeline
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
+, Adult-onset Still ... disease, Infection ... Lymphohistiocytosis #diagnosis #management ... #treatment #summary ... #rheumatology
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
lymphohistiocytosis (HLH ... • Adult-onset Still ... Treatment: • Corticosteroids ... Syndrome #Diagnosis #Management ... #Hematology #Rheumatology
Clarkson’s Disease - Capillary leak syndrome Epidemiology: Roughly 150 published cases, Median age 50 years, No sex
- Capillary leak ... tissues: Myalgia, paresthesia ... #Capillary #leak ... syndrome #diagnosis #management ... #rheumatology
Behçet's Syndrome Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy Epidemiology: • Young
common in Asia • HLA-B51 ... association • ... Arthritis, AS Treatment ... Syndrome #diagnosis #management ... signs #symptoms #rheumatology
Anti-NXP-2 Dermatomyositis What? DM with generalized subcutaneous edema Pts have more myalgias, more severe weakness, and an increased prevalence
But can still have ... had more severe disease ... positive patients Treatment ... Dermatomyositis #diagnosis #management ... #rheumatology #
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