MindMap Gallery hemophilia
The internal medicine nursing hematologic system summarizes the definition of hemophilia, etiology and selection rules, clinical manifestations, laboratory and other examinations, treatment points, nursing diagnosis and measures, etc.
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hemophilia
definition
It is a bleeding disorder caused by a group of genetic coagulation factors
Classification
Hemophilia A
The most common hereditary hemorrhagic disease in clinical practice accounts for 0.85 of hemophilia
Hemophilia B
Positive family history, juvenile onset, continuous bleeding after spontaneous or mild trauma, hematoma formation, joint cavity bleeding is clinically characterized
Causes and genetic rules
Hemophilia A and B are typical sex chromosome (X)-linked invisible hereditary
Gene mutations
Clinical manifestations
Depend on the severity of the corresponding coagulation factor deficiency, mainly manifested as compression symptoms and signs caused by bleeding and local hematoma formation
Bleeding: The most important clinical manifestation of hemophilia patients
Spontaneous bleeding
Continuous bleeding after minor trauma
In severe cases, intracranial hemorrhage can occur and life-threatening
Joint bleeding
Repeated bleeding causes joint deformity Dysfunction
Common in the knee, ankle, elbow joint
Muscle bleeding
Pain Swelling Limited movement
Bleeding in other areas
Epiphany, bleeding gums, hematuria, bleeding stool, etc.
Hematoma compression manifestations
Laboratory and other inspections
Laboratory inspection
Coagulation function determination
Coagulation time (CT) prolongs
Activated partial thromboplastin time (APTT) prolongs
Prothrombin time (PT) is normal
Coagulation factor determination
Hemophilia A: Lower activity of coagulation factor VIII
Hemophilia B: reduced activity of coagulation factor IX
Genetic testing
Defining the type of gene mutation assisted diagnosis
Key points of treatment
Alternative Therapy
Supplementary coagulation factor (FVIII FIX)
Preventive infusion reduces bleeding risk
Drug treatment
Use antifibrinolytic drugs (such as aminocaproic acid)
Use recombinant coagulation factors
Symptom-based treatment
Treatment of joint hemorrhage Muscle hematoma
Physical therapy Rehabilitation training
Nursing diagnosis and measures
Risk of bleeding: related to lack of certain coagulation factors
Prevent bleeding
Pay attention in life
Avoid or reduce unnecessary puncture and injection. After removing the needle, press locally for more than 5 minutes until bleeding stops
Use of intravenous trocar is prohibited
Avoid surgery and supplement sufficient coagulation factors if necessary
Avoid the use of aspirin and other drugs to inhibit coagulation
Cooperation of local bleeding treatment
Hemorrhage on the surface of the skin
Compression and hemostatic method
Nasal mucosa bleeding
Use batreczyme, thrombin, isopressurization or tamponization to stop bleeding as prescribed by doctors
Bleeding after tooth extraction
Cover the wound with a sticker containing the relevant coagulation factors
Patients with local deep tissue hematoma/articular cavity hemorrhage
Rest cold compress and raising the affected limb
Throat bleeding
Head tilts to one side. Use a suction device to suck it out if necessary. Prepare for tracheal intubation
Correctly infused various coagulation factor products
Thaw at 37℃ in advance and quickly enter
Medication care
Fast static push and vasopressin can cause headaches, heart rate and other symptoms
Risk of autism syndrome: related to repeated joint cavity bleeding
Assessing joint cavity bleeding and lesions
Joint rehabilitation training
Anxiety: related to lifelong bleeding tendency and worry about losing capacity
Fear: It is related to fear of bleeding more than life-threatening
Pain: It is related to deep tissue hematoma or joint cavity bleeding