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diagnosis hematology amyloid amyloidosis common cvid hemophagocytic history hlh hypogammaglobulinemia immunodeficiency immunology lymphohistiocytosis macrophage mas pediatrics peds primarycare rheumatology treatment
Helpful Clinical Features in Evaluating Bleeding Disorders Age of onset • Neonate - in 20% of haemophilias,
Helpful Clinical ... relatives (if all ... Marfan syndrome, ... osteogenesis imperfecta ... #Bleeding #Disorders
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
Macrophage Activation ... the secondary or acquired ... Clinical Presentation ... purpura to mucosal bleeding ... #Management #Hematology
Common Variable Immunodeficiency (CVID) What? CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
Immunodeficiency disorder ... to impaired B-cell ... to 8 years Clinical ... severe community-acquired ... : • Nephrotic syndrome
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
: Overwhelming clinical ... syndrome associated ... excessive macrophage activation ... Low or absent NK cell ... Lymphohistiocytosis #diagnosis #management
Amyloidosis - Summary Group of disorders associated with extracellular deposition of fibrils formed from low-molecular-weight proteins in
Summary Group of disorders ... Clinical suspicion ... : • Nephrotic syndrome ... failure • GI bleeding ... Inflammation → Macrophage activation
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