Anesthetic Considerations in Patients with Inherited Coagulation Defects
Radwa khairy Abd El Mohsen;
Abstract
Hemostasis is defined as the arrest of bleeding. It comes from Greek, where, haeme means blood and stasis means to stop. When a vessel is injured or ruptured, hemostasis occurs by several mechanisms:- (1) vascular constriction, (2) formation of a platelet plug, (3) formation of a blood clot as a result of blood coagulation, and (4) the growth of fibrous tissue into the blood clot which closes the hole in the vessel permanently.
Hereditary bleeding disorders are diverse group of diseases that include abnormalities of primary and secondary hemostasis. The most common congenital bleeding disorders include:- von Willebrand disease, Hemophilia A (factor VIII deficiency), Hemophilia B (factor IX deficiency) Less common congenital bleeding disorders include:- Factor I (fibrinogen) deficiency, Factor II (prothrombin) deficiency, Factor V deficiency, Factor VII deficiency, Factor X deficiency, Factor XI deficiency, Factor XIII deficiency, Platelet disorders.
Primary hemostasis involves formation of the platelet plug which involves platelets, the blood vessel wall and von Willebrand factor; abnormalities can include problems in platelet number, adhesion or aggregation. Secondary hemostasis involves the formation of fibrin through the humoral coagulation cascade; abnormalities include deficiencies of coagulation factors or contact factors, deficiencies or abnormalities of fibrinogen or connective tissue diseases. Mutations can be inherited in an autosomal dominant, recessive or X-linked pattern.
Preoperative assessment ideally must be done by a team of hematologist, surgeon, and anesthetist, so that a tailored individual plan is formulated and discussed with the patient.Detailed history about the type of hemophilia and VWD (von willibrand disease) and its severity must be obtained. Prior informationabout response to DDAVP(desmopressin),use of recombinant factors VIII and IX, and previoustransfusion of blood will be useful. A complete blood count, coagulation profileand fibrinogen level, and specific factor assays must be done if indicated.
There are case series and reports of central neuraxial blocks with catheters in hemophiliac patients undergoing lower limb orthopedic surgeries after correction of factor levels, but there are norandomized controlled trials.Therefore, the risk–benefit ratio must be assessed individually on a case-to-case basis.
Hereditary bleeding disorders are diverse group of diseases that include abnormalities of primary and secondary hemostasis. The most common congenital bleeding disorders include:- von Willebrand disease, Hemophilia A (factor VIII deficiency), Hemophilia B (factor IX deficiency) Less common congenital bleeding disorders include:- Factor I (fibrinogen) deficiency, Factor II (prothrombin) deficiency, Factor V deficiency, Factor VII deficiency, Factor X deficiency, Factor XI deficiency, Factor XIII deficiency, Platelet disorders.
Primary hemostasis involves formation of the platelet plug which involves platelets, the blood vessel wall and von Willebrand factor; abnormalities can include problems in platelet number, adhesion or aggregation. Secondary hemostasis involves the formation of fibrin through the humoral coagulation cascade; abnormalities include deficiencies of coagulation factors or contact factors, deficiencies or abnormalities of fibrinogen or connective tissue diseases. Mutations can be inherited in an autosomal dominant, recessive or X-linked pattern.
Preoperative assessment ideally must be done by a team of hematologist, surgeon, and anesthetist, so that a tailored individual plan is formulated and discussed with the patient.Detailed history about the type of hemophilia and VWD (von willibrand disease) and its severity must be obtained. Prior informationabout response to DDAVP(desmopressin),use of recombinant factors VIII and IX, and previoustransfusion of blood will be useful. A complete blood count, coagulation profileand fibrinogen level, and specific factor assays must be done if indicated.
There are case series and reports of central neuraxial blocks with catheters in hemophiliac patients undergoing lower limb orthopedic surgeries after correction of factor levels, but there are norandomized controlled trials.Therefore, the risk–benefit ratio must be assessed individually on a case-to-case basis.
Other data
| Title | Anesthetic Considerations in Patients with Inherited Coagulation Defects | Other Titles | اعتبارات التخدير في المرضى الذين يعانون من عيوب التخثر الموروثة | Authors | Radwa khairy Abd El Mohsen | Issue Date | 2017 |
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