Thursday, July 30, 2009

Treatment of Patients with Bleeding Disorders

Department of Pediatric Dentistry
Lutheran Medical Center

Resident’s Name: Craig Elice Date: 7/31/2009
Article title: Treatment of Patients with Bleeding Disorders
Author(s): Patton LL, Ship J:.
Journal: Dental Clinics of North America
Volume (number): 38(3)
Date: July 1994
Major topic: Hemophilia A and B, Platelet disorders and von Willebrand’s disease
Type of Article: Review of Literature
Main Purpose: This review article summarizes the tests performed to properly evaluate patients with bleeding disorders and outlines techniques and treatment suggestions to deliver safe effective care.
Findings: A thorough medical history is necessary including systemic and oral diseases, medications, hospitalizations, operations and history of transfusions. A careful review of systems with a focus on symptoms of bleeding disorders like petechiae, ecchymosis, or hematomas, etc. Lab tests should be evaluated. These include platelet counts (normal 100k-400k cells/mm3). Below 100k, abnormal bleeding may occur. Prothrombin time (PT time) measures the effectiveness of the extrinsic pathway to control fibrin clot formation. It is most influenced by anticoagulation therapy like coumadin and heparin treatment as well as aspirin therapy. PT ranges from10-15 seconds. At 1.5x or greater, the etiology could be due to decreases in fibrinogen, prothrombin, factors V, VII or X, liver damage, or coumadin therapy. Activated partial thromboplastin time tests for intrinsic and common pathways. Normal is 25-35 seconds depending on the hospital controls with an increase being related to many of the factor deficiencies or heparin therapy. Bleeding time measures vascular and platelet phases of coagulation with increased times indicating thrombocytopenia, von Willebrands, and disorders of platelet function. Thrombin time measures clotting times after the addition of thrombin and measures fibrinogen deficiency, systemic heparin, and intravascular coagulation. Lastly a physician consult should be obtained
Dental management: Preventive procedures like good oral hygiene, diets low in sugar, fluoride etc. are essential. Keep restorative treatment as supragingival as possible. Orthodontic treatment using safe technique minimizes any bleeding. Periodontal patients requiring subgingival scaling and root planning in areas of inflammation may require medical management. In terms of endodontics, pulpotomies, pulpectomies, and root canal therapy can be accomplished without bleeding complications. In patients with severe disease, especially Hemophilia A have 11% hematoma formation after inferior alveolar block therefore block anesthesia is contraindicated until correction of hemostatic defect is achieved. Local infiltration of anesthesia in firm tissue bound to bone followed by direct pressure for 3-4 minutes is a better choice as well as periodontal injections. The authors recommend 2% Lidocaine with 1x 100,000 epinephrine as drug of choice. Acetominophen and narcotics are best for postoperative pain while aspirin and NSAID’s are contraindicated. During oral surgical procedures, minimizing trauma to the surgical site, removal of osseous fragments and granulation tissue and reapproximating tissues aids in healing. Loose primary teeth can usually be removed without medical management. Aids to clotting include use of Avitene, Oxycel, or Surgicel as well as Gelfoam. Antibiotics are often prescribed when clotting aids are used to prevent infection. Lastly hemostatic sponges like INSTAT help in early hemostasis. In severe cases, diet shold be limited to cool liquids and lead to soft pureed foods. AMICAR at 50mg/kg every 6-7 hours for 10 dayscan prevent post surgical bleeding.
Platelet disorders: Thrombocytopenia occurs when the platelet count is less than 100k cells/mm3. No clinical bleeding or surgical hemorrhage is expected at dgreater than 50k cells/mm3. Spontaneous bleeding rarely occurs until counts are at 10k- 20k cells/mm3. Chemotherapy, gold, salts, etc. may indue reversible causes of decreased platelets and usually recovers 2 weeks after removal of causal agent. Irreversible causes include leukemia, lymphoma, HIV, etc. 1 Unit of platelets contains 6000 cells/mm3 and patients usually receive 6 units per transfusion. Thrombocytopathy is a qualitative abnormality in platelet activity. Aspirin and NSAID’s as well as some rare diseases may cause this. Roving the agents and waiting 7 days for recovery is necessary. In patients with end stage renal disease, procedures can be planned the day after hemodialysis.
Inherited Coagulopathies Hemophilia A is an X-linked recessive disorder with a deficiency of Factor VIII. Severe hemophilacs withfactor at less than 1% bleed spontaneously into joints and muscles. Moderate at 1-5% bleed withmoderate trauma and mild at 6-30% of normal levels have reare spontaneous bleeding and hemorrhage with severe trauma or surgery. Patients have a prolonged aPTT and normal PTT, TT, and BT. Systemic therapy should maintain levels at 30-50% every 12 hours. In mild to moderate Hemophilia, DDAVP can be used to raise levels to hemostatic levels by causing the release of von Willebrand’s factor from storage sites in endothelial cells. Given IV, peak levels are reached in 30-60 minutes and may raise factor VIII levels 2 to 3 times. In cases of moderate to severe Hemophilia, Factor VIII concentrate is necessary using recombinant DNA or monoclonal antibody products 1 unit/kg of Factor VIII raises levels by 2% Therapeutic levels after IV push occurs within minutes with a half life of 8-12 hours. In cases with high inhibitor levels, Factor IX complex or Prothrombin complex concentrates are effective in 50-75% of the cases. Hemophilia B (Christmas disease) is X linked recessive resulting in Factor IX deficiency. It accounts for 10-15% of all hemophiliac. The desired levels of Factor for dental surgery are between 30-50%. Von Willebrand’s disease is often autosomal dominant affecting both sexes. vWF . a protein that affects adhesion of platelets to the vessel wall is deficient. Type I accounts for 70-80% and involves reduced levels of vWF and responds to DDAVP. Type II affects high molecular weight vWF, and Type III is the severe form. Factor VIII concentrates are the treatment of choice for Type II and III VW disease. Cryoprecipitate contains more vWF although it has the dangers of viral contamination.
Anticoagulants: Coumarin derease vitamin K factors (VII, IX, and X and prothrombin)) is frequently prescribed in thromboembolic diseases. It has a half life of 44 ours. Heparin is given IV and has a shorter half life. Management includes either maintaining doses andusing local hemostatic agents or decrease doses with a short-term change t Heparin while under continuous hospital care. Severe Liver Disease: Vit K deficiency can also affect the systhesis of Factors.
Key points/Summary : The dentist should get a hematology consult to determine (1) the severity of the disease, (2) the bleeding risk of the prescribed dental procedures, (3) the patients history as it relates to the response to previous surgeries, trauma, and (4) historical response to previous medical therapies.
Assessment of article: Good review article with lots of board questions. We will see Hemophilia A patients and von Willebrand’s patients in our practice in RI.

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