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COAGULATION  DISORDERS AND  ANAESTHESIA PRESENTERS:  DR UNNIKRISHNAN P DR SUNEESH THILAK CO-ORDINATOR DR C MADHUSOODHANAN PILLAI MODERATORS: DR GEETHA N K DR ASHA K S
What is normal hemostasis? ,[object Object],[object Object]
Components of hemostasis Interactive
Components: vascular Intact endothelium: Non-thrombogenic (-) (-)
Components: vascular Endothelial damage: (+) (+) Stress hormones Trauma Surgery Plaque rupture Inflammation… ,[object Object],[object Object]
The first event….. ,[object Object],[object Object],[object Object],[object Object]
 
So the well equipped guy comes ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Components: platelets   Adhesion Activation Aggregation Secretion Procoagulant activity
Endothelial damage: Platelet plug formation ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
No one can hide the insults from them…… ,[object Object],[object Object],[object Object],[object Object]
 
Leads to….   PRIMARY HEMOSTASIS ,[object Object]
The  balancing act ,[object Object],[object Object],[object Object],[object Object]
In need of…. FIBRIN ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Prompt………. But finely controlled ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
For example… ,[object Object],xii------>xii a Ca
 
Components: coagulation pathways Extrinsic (TF) Intrinsic  Initiation Amplification Thrombin Pivotal point of coagulation
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Thrombin generation to fibrin-platelet clot formation
Cascade vs. cell-based model ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Tissue factor bearing cells 1. Initiation Platelets Activated platelets 2. Amplification 3. Propagation IIa IIa
Cellular components ,[object Object],[object Object],[object Object],[object Object]
Molecular components ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Current model of hemostasis
Normal Hemostasis Hoffman et al.  Blood Coagul Fibrinolysis  1998;9(suppl 1):S61 . X II II X IX TF-Bearing Cell Activated Platelet Platelet TF VIIIa Va VIIIa Va Va VIIa TF VIIa Xa IIa IX V Va II VIII /vWF VIIIa IXa X IXa IXa VIIa Xa IIa IIa Xa
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Endothelial damage: Initiation of thrombin generation ,[object Object],Exposure to tissue factor Initiation of extrinsic pathway Initiate thrombin generation Activate FXI (intrinsic pathway) Amplify thrombin generation
Soldiers….. I  FIBRINOGEN II  PROTHROMBIN III  THROMBOPLASTIN/TISSUE FACTOR IV  CALCIUM V  PROACCELERIN/LABILE FACTOR VII  PROCONVERTIN/STABLE FACTOR VIII  ANTIHAEMOPHILIC FACTOR A IX  ANTIHAEMOPHILIC FACTOR  B X  STUARTPROWER FACTOR XI  ANTIHAEMOPHILIC FACTOR C / PTA XII  HAEGEMAN FACTOR / GLASS FACTOR XIII  FIBRIN STABILIZING FACTOR PREKALLIKREIN / FLETCHER FACTOR KALLIEKREIN PLATELET PHOSPHOLIPID … They work in concert to form a beautiful definitive clot!
 
Clot: The end product of hemostasis
The rebels…. ,[object Object],[object Object],[object Object]
Antithrombin  iii ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Protein C & Protein S ,[object Object],[object Object]
TFPW-inhibitor ,[object Object]
Two more… ,[object Object],[object Object],[object Object],[object Object],[object Object]
Fibrinolysis ,[object Object]
 
Serine Proteases ,[object Object],[object Object],[object Object],[object Object],[object Object]
Cofactors ,[object Object],[object Object],[object Object],[object Object],[object Object]
VITAMIN-K dependent Factors ,[object Object]
Question hour in AAC INFANCY SURGERIES FAMILY HISTORY DRUGS  HORMONAL REPLACEMENT / OCP HISTORY OF BLEEDING IN THE PAST
What to look for…? ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Surgery induces an increase in.. ,[object Object],[object Object],[object Object],[object Object],[object Object]
These factors arise concern about the hemostasis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
MONITORING HEMOSTASIS ,[object Object]
Feel.. There is no plan to stop ,[object Object]
Monitoring hemostasis Cascade vs. cell-based model ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
BLEEDING TIME ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Platelet count ,[object Object],[object Object],[object Object],[object Object]
Prothrombin Time ,[object Object],[object Object],[object Object]
Prothrombin Time Intrinsic Pathway Extrinsic Pathway Common Pathway CLOT PT
What is INR? ,[object Object],[object Object],[object Object],[object Object]
Prolonged??? Think of…. ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
aPTT ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Activated Partial Thromboplastin Time Intrinsic Pathway Extrinsic Pathway Common Pathway CLOT APTT
Thrombin Time ,[object Object],[object Object],[object Object],[object Object],[object Object]
Thrombin Time Intrinsic Pathway Extrinsic Pathway Common Pathway CLOT TT
CLOTTABLE FIBRINOGEN CONCENTRATION ,[object Object],[object Object]
Activated clotting time ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Activated Clotting Time Intrinsic Pathway Extrinsic Pathway Common Pathway CLOT ACT
Thromboelastography ,[object Object],[object Object]
The TEG® System ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
.
 
.
..
PLOT  ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Application of TEG analysis ,[object Object]
TEG analysis and clinical outcomes ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
???? ,[object Object],[object Object],[object Object],[object Object],Next
Platelet function analyzers ,[object Object],[object Object]
Still not over…?$# ,[object Object]
DISORDERS OF COAGULATION ,[object Object]
DISEASE OF KINGS ,[object Object]
What is Hemophilia? ,[object Object]
Degrees of Severity of Hemophilia ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
CLINICAL FEATURES
Types of Bleeds ,[object Object],[object Object],[object Object],[object Object],[object Object]
Life-Threatening Bleeding ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Characteristics
Age of presentation….
Do we bother about carriers?
Investigations… ,[object Object],[object Object]
Our weapons….
8 8 8 8 8 8 8 8 8 8 8 8 8 8 8 8 8  ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Infusion rate
In another way…
Perioperative needs..
Recommendations  SOFT TISUE BLEED- 15 TO 20 % HEMARTHROSIS/RETROPERITONEAL-25-50% x72h MAJOR Sx/ LIFE THREATENING BLEED- 50% x2 wk
B4 Sx …..
INHIBITORS
Prophylaxis
Specialty posting!
These should be kept in mind..
Iron deficiency anemia ???? ,[object Object],[object Object]
Precautions
Cryoprecipitate / FFP
Desmopressin
TA & EACA
Anesthetic Implications  ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
SURGERY/ MINOR PROCEDURE
Good news….
Hemophilia B ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
=….HEMOPHILIA B ,[object Object]
Rx
F IX/FFP/others
PROTHROMBIN COMPLEX
Any factor concentrate for exhausted audience..???
Who am I  ? ,[object Object],[object Object],[object Object]
vWD 1/100-500 10mg/L AUTOSOMAL DOMINANT Affect PLATELET adhesion
Missing you… vWF
Lab report..
Treatment ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
And….
A FEW STRANGERS
… .
Hereditary Haemorrhagic Telengiectasia  ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Hereditary thrombocytopenia
Can our routine tests detect a fibrinolytic defect? ,[object Object],[object Object],[object Object],[object Object]
HYPERCOAGULABLE STATES ,[object Object],[object Object],[object Object]
What’s it? ,[object Object],[object Object],[object Object],[object Object],[object Object]
Protein C Deficiency ,[object Object],[object Object],[object Object],[object Object],[object Object]
Antiphospholipid antibody syndrome
Strategy ?? ,[object Object],[object Object],[object Object]
THANK YOU
No thanks …………..?%#
References ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
… ,[object Object],[object Object],[object Object],[object Object],[object Object]

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