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By rafat mosalli
HEMOSTASIS: The ability of the body to control the flow of blood following vascular injury is paramount to continued survival. The process of blood clotting and then the subsequent dissolution of the clot, following repair of the injured tissue. Is sum total of specialized function within the circulating blood & its vessels designed to stop hemorrhage.
HEMOSTASIS: is composed of 4 major events that occur in a set order following the loss of vascular integrity: Vascular constriction -limits the flow of blood to the area of injury. Platelet aggregation Blood platelets clump when binding to collagen that becomes exposed following rupture of the endothelial lining of vessels. -Blood platelets become activated and aggregate at the site of injury (thrombin and fibrinogen-mediated effects). Upon activation, platelets release ADP and TXA2 (which activate additional platelets).
Clot formation -to insure stability of the initially loose platelet plug, a fibrin mesh (also called the clot) forms and entraps the plug. Fibrinolysis -the clot must be dissolved in order for normal blood flow to resume following tissue repair. The dissolution of the clot occurs through the action of plasmin.
Vascular Phase
Vasoconstriction Exposure to tissues activate Tissue factor and initiate coagulation
Tissue Factor
Platelet phase
Non-nucleated - arise from magakaryocytes blood vessel wall (endothelial cells) prevent platelet adhesion and aggregation platelets contain receptors for fibrinogen and von Willebrand factor after vessel injury Platelets adhere and aggregate. Release permeability increasing factors (e.g. vascular permeability factor, VPF) Loose their membrane and form a viscous plug
Coagulation Phase
Two major pathways Intrinsic pathway Extrinsic pathway Both converge at a common point 13 soluble factors are involved in clotting Biosynthesis of these factors are dependent Vitamin K1 and K2 Most of these factors are proteases Normally inactive and sequentially activated Hereditary lack of clotting factors lead to hemophilia -A
on
Intrinsic cascade: initiated when contact is made between blood and exposed endothelial cell surfaces. Extrinsic pathway: initiated upon vascular injury which leads to exposure of tissue factor (TF), a sub endothelial cell-surface glycoprotein that binds phospholipids.
Intrinsic Pathway All clotting factors are within the blood vessels Clotting slower Activated partial thromboplastin test (aPTT)
Extrinsic Pathway Initiating factor is outside the blood vessels tissue factor Clotting - faster in Seconds Prothrombin test (PT)
Bleeding time
It is the primary ,oldest test for the primary hemostasis( vascular &platelets phase). Measure interval time required for hemostasis following standard superficial incision 1-2mm deep & up to 5mm length in the skin of forearm with venous pressure maintained at 40mmHg. Gives information immediately Ideally done with help of template and related to age usually in children 4-7 minutes.
When performed with the standard methods I t depend on the following: platelet no., vascular factors, temperature& hormones. Could be affected when Aspirin and other drugs ingested within 7d. Prolongation doesnot correlate with bleeding risk.
NB : the bleeding disorders might not be associated with any abnormalities in the screening tests: Mild factors deficiency Factors 13 deficiency. HSP. Ehler danlos syndrome . Scurvy. Hereditary hemorrhagic telengectasia.
Intrinsic Pathway
Blood Vessel Injury XII XI IX X Factors affected By Heparin XIIa XIa
Extrinsic Pathway
Tissue Injury Tissue Factor Thromboplastin
IXa
Xa
VIIa X
VII
Prothrombin
Fibrinogen
Thrombin
Fribrin monomer Fibrin polymer
XIII
Activation
XIIa
Inactive XI
Active XIa
Thrombosis
Arterial Thrombosis :
Adherence of platelets to arterial walls White in color - Often associated with MI, stroke and ischemia
Venous Thrombosis :
Develops in areas of stagnated blood flow (deep vein thrombosis), Red in color- Associated with Congestive Heart Failure, Cancer, Surgery.
Fibrinolysis
Enhance degradation of clots Activation of endogenous protease Plasminogen (inactive form) is converted to Plasmin (active form) Plasmin breaks down fibrin clots
Diagnostic approach
Phase one: -Thorough history& physical examination as well as standard screening laboratory test. Phase two: If the initial screening test is negative then test for VWD, platelets dysfunction, factor 13 and or dysfibrongenemia. Phase three: Interprets the abnormal result&&try to define the specific disorders.
History:
Spontaneous bleeding? Bleeding in unusual site without significant trauma? Bruising and bleeding disproportionate to injury? large or palpable bruising? Poorly controlled epistaxis?is it unilateral Excessive bleeding with tooth extraction? Abnormal bleeding at or after circumcision or surgical procedure?
Physical examination:
General stability, vitals signs, evidence of chronic disease, evidence of malignancy. Skin stigmata : petechiae purpura ecchymosis hematoma
Laboratory aids:
Phase one; Initial laboratory screening . platelet count,PT,PTT, bleeding time. Phase two; special confirmatory tests - If qualitative platelets defect suspected platelet aggregation studies with restocetin, collagen, thrombin,and ADP. - VWF analysis for VWF disease . - Thrombin time or fibrinogen for dys fibronegenimia..
Prolonged PT inhibitor screen factors 7,10,5,2,1. Prolonged PTT, PT: Test for DIC, liver disease, sever vitamin K deficiency.factor 10, 2.
summary
When you face a child with bleeding problem what should I do? Careful history including past and family history. Detailed clinical examination. Few screening test then appropriate specialized tests Appropriate referral This will help in proper diagnosis and hence better management of bleeding child.