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diagnosis management hemophagocytic hlh lymphohistiocytosis stills syndrome adultonset aosd activation acute adult calcium cardiology classification cppd criteria deposition dermatology dermatosis
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
• Periodic fever ... multiorgan failure • Fever ... - Nonremitting fever ... Diagnosis #Management #Hematology ... #Rheumatology
Adult-Onset Still's Disease Clinical triad: Fever, Arthralgia/arthritis, Skin rash ± odynophagia + Biological inflammation: Increased neutrophils, Hyperferritinemia, decreased
Adult-Onset Still's Disease ... Clinical triad: Fever ... autoinflammatory diseases ... diagnosis #management #treatment ... #rheumatology
Adult-Onset Still's Disease (AOSD) Rare inflammatory disorder of unknown etiology, difficult to diagnose because of heterogenous presentation.
Adult-Onset Still's Disease ... : High spiking fever ... Systemic AOSD: high fever ... synovitis (40%) Treatment ... #AOSD #rheumatology
Adult Onset Still's Disease Systemic inflammation with urticarial rash. Rash: • Nonpruritic, evanescent (correlates with time of
correlates with time of fever ... Daily high spiking fever ... - Fever spikes ... Fever of at least ... AOSD #diagnosis #rheumatology
Kawasaki Disease (KD) - Diagnosis and Management Summary Kawasaki Disease (KD) is the most common vasculitis of
Kawasaki Disease ... Presentation: • Fever ... Diagnostic Criteria - Fever ... KD #vasculitis #rheumatology ... diagnossi #management #treatment
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
• Clinical: fever ... , ↓ Fibrinogen level ... • Autoimmune diseases ... Unknown cause Treatment ... #summary #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
age, however the disease ... following: • Fever ... • Bicytopenia Treatment ... diagnosis #management #treatment ... #hematology
Diagnostic Criteria for Sweet Syndrome (Acute Febrile Neutrophilic Dermatosis) Major criteria 1. Abrupt onset of tender
Fever, temperature ... an underlying hematologic ... inflammatory disease ... Excellent response to treatment ... Criteria #Diagnosis #Hematology
Sjogren's Syndrome Overview Epidemiology: • F > M: 9:1 • 5-6th Decades (can be any age) Autoimmune exocrinopathy multisystemic disease
vasculitis: 10%-16% Liver ... Nephrogenic DI Hematologic ... Adenocarcinoma Treatment ... lubricants • Pharmacologic ... Sjogrens #Syndrome #Rheumatology
Hodgkin's Lymphoma Overview Hodgkin's lymphoma (HL) is an uncommon hematological malignancy arising from mature B cells. It
• Dyspnea • Fever ... number of sites of disease ... Hodgkin Lymphoma Treatment ... classification #hematology
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