Pediatric Bleeding Disorders

Introduction to children with bleeding disorders Hemorrhagic disease is a general term for diseases in which hemorrhage is the main manifestation of normal hemostatic function. Clinically, hemorrhage after spontaneous bleeding or minor injury is not only characteristic. basic knowledge The proportion of illness: 0.030% Susceptible people: children Mode of infection: non-infectious Complications: anemia, heart failure, shock, gastrointestinal bleeding

Causes of pediatric bleeding disorders

(1) Causes of the disease

There are many types of hemorrhagic diseases. According to the pathogenesis, they can be divided into five categories: 1 hemorrhagic disease caused by vascular factors; 2 hemorrhagic diseases caused by platelet factors; 3 decreased blood coagulation factors, hemorrhagic caused by lack or abnormality Disease; 4 hemorrhagic disease caused by excessive anticoagulation; 5 hemorrhagic disease caused by compound factors.

1. Hemorrhagic disease caused by vascular factors

(1) hereditary: hereditary telangiectasia, vascular pseudohemophilia, familial simple purpura, pulmonary hemosiderosis.

(2) Metabolic: Diabetes, uremia.

(3) Infectivity: viruses (such as epidemic hemorrhagic fever), bacteria (such as epidemic meningitis, bacterial sepsis, etc.), spirochetes (such as leptospirosis).

(4) Toxicity: animal toxins (such as bee venom, snake venom).

(5) drug properties: sulfonamide, barbital.

(6) Allergic: allergic purpura, self-DNA allergy, autologous hemoglobin allergy.

(7) Vitamin deficiency: vitamin C deficiency, vitamin P deficiency.

(8) Connective tissue disease: systemic lupus erythematosus, nodular polyarteritis, Ehlers-Danlos syndrome.

(9) abnormal proteinemia: hyperglobulinemia purpura, primary 2-macroglobulinemia, cryoglobulinemia purpura, high protein C disease. High protein S disease.

(10) Aster caused by amyloidosis.

(11) Simpleness: simple purpura, mechanical purpura.

2. Hemorrhagic disease caused by platelet factors

(1) abnormal platelet count: 1 thrombocytopenia: idiopathic (immune), secondary (immune, non-immune), congenital (immune, non-immune), 2 thrombocytosis: primary, Secondary.

(2) platelet dysfunction: 1 hereditary: thrombocytopenia, mild thrombocytopenia, platelet factor deficiency, 2 acquired: uremia, liver disease, drugs (aspirin, dipyridamole, etc.), myeloproliferative diseases, malignant Tumor.

3. Reduced clotting factors, hemorrhagic diseases caused by lack or abnormality

(1) thromboplastin production disorder: coagulation factor VII deficiency (hereditary, acquired), coagulation factor IX deficiency (hereditary, acquired), coagulation factor XI deficiency (hereditary, acquired), coagulation factor XII deficiency (hereditary, acquired).

(2) thrombin generation disorder: prothrombin deficiency (hereditary, acquired), coagulation factor V deficiency (hereditary, acquired), coagulation factor XII deficiency (hereditary, acquired), coagulation factor X Deficiency (hereditary, acquired).

(3) Fibrin formation disorders: fibrinogen deficiency (hereditary, acquired), abnormal fibrinogenemia (hereditary, acquired), coagulation factor XIII deficiency (hereditary, acquired).

4. Hemorrhagic disease caused by excessive anticoagulation

(1) Anticoagulant substances in the blood circulation: 1 anti-fibrinogen; 2 anti-V factor; 3 anti-factor VIII; 4 anti-factor IX; 5 anti-factor XI; 6 anti-factor XII; 7 anti-factor XIII; Live enzyme; 9 anti-thrombin-III (AT-III); 10 heparin and heparin-like anticoagulant,

(2) fibrinolytic hemorrhagic disease: 1 primary: seen in liver disease, etc., 2 secondary: seen in disseminated intravascular coagulation, after prostate surgery.

5. Hemorrhagic diseases caused by complex factors diffuse intravascular coagulation, liver disease, hereditary combined coagulation factor deficiency.

(two) pathogenesis

Under physiological conditions, the three elements involved in hemostasis are blood vessel wall, platelets and blood coagulation factors, hemorrhagic diseases caused by vascular factors, including abnormal blood vessels and abnormalities caused by extravascular factors, hemorrhagic diseases, allergic purpura, vitamin C deficiency, and heredity. Capillary telangiectasia is caused by abnormal blood vessels itself; hypercystemia and other abnormalities caused by extravascular abnormalities, changes in platelet count and adhesion, aggregation, release response and other dysfunction can cause bleeding, idiopathic Thrombocytopenic purpura, drug-induced thrombocytopenia, and thrombocytopenia are all hemorrhagic diseases caused by abnormal platelet counts, thrombocytopenia, giant platelet disease, etc., and hemorrhagic diseases caused by platelet dysfunction. Reduced, lack of or abnormalities caused by bleeding disorders, including congenital coagulation factors and acquired acquired coagulation factor abnormalities, such as hemophilia A (lack of factor VIII) and hemophilia B (lack of factor IX) Chromosomal recessive hereditary hemorrhagic disease, vitamin K deficiency, and hemorrhage caused by liver disease are mostly acquired Abnormally caused by clotting factors, excessive anticoagulation can also cause bleeding disorders, diffuse intravascular coagulation, liver disease, hereditary combined coagulation factor deficiency, is a hemorrhagic disease caused by a combination of factors.

Pediatric bleeding prevention

1. Pay attention to the prevention of genetic diseases.

2. Actively prevent and treat various infectious diseases and do a good job in vaccination. Guide self-protection methods, such as not taking contact with infected patients during medication, wearing masks in public places, dressing moderately, avoiding infection as much as possible to avoid aggravation or recurrence.

3. Pay attention to your diet. In food selection, it is advisable to eat more fruits and vegetables containing more vitamin C, and pay attention to cooking methods to avoid coarse foods such as fish and bones, so as to avoid accidentally piercing the mucous membranes of the digestive tract and causing bleeding.

4. Prevent bleeding disorders caused by X-rays, drugs and various poisons.

5. Guide the prevention of trauma, such as not using a hard toothbrush, not digging the nostrils, fasting hard and prickly food. The bed rails are wrapped with soft plastic products. Avoid playing sharp toys and restricting intense activities to avoid bleeding caused by bruises, stab wounds and falls. Keep the stool smooth, so as not to cause intracranial hemorrhage caused by increased abdominal pressure caused by defecation.

6. Protect the skin and mucous membranes. Patients should reduce irritation to the skin and mucous membranes. When brushing your teeth, use a soft toothbrush or a cotton ball to avoid damage to the gums and cause bleeding. The clothes should be slightly wider. Avoid using sharp tools during activities. Try to avoid collisions between the limbs and external objects, prevent skin damage and subcutaneous bleeding.

7. Minimize intramuscular injection to avoid deep hematoma.

8. Eliminate the fear and fear caused by the operation of bleeding and hemostasis in children, and strive for cooperation with children.

9. If traumatic bleeding occurs at home, try to stop bleeding and limit joint activity at the bleeding site. The method of hemostasis has pressure dressing to stop bleeding, and is covered with a dry towel and a handkerchief in the wound; the finger pressure hemostasis method is used to press the end of the hemorrhage artery close to the heart with a finger, palm or fist, which can cut off the blood flow and achieve the purpose of temporarily stopping bleeding. If you can't reach complete hemostasis in a short time, you should go to the hospital urgently for treatment.

10. Should guide the method of oppression and hemostasis; guide parents to identify signs of bleeding, such as sputum, sputum, once the bleeding is found, return to hospital for review and treatment.

11. Care should be taken to avoid trauma and prevent infections, especially respiratory infections. Do not take drugs that inhibit platelet function, such as aspirin. For patients with severe bleeding or long-term treatment, the following special treatments should be performed.

12. Drugs that are frequently exposed to life, such as aspirin, dipyridamole, indomethacin, phenylbutazone, and dextran, are unsafe drugs for patients with bleeding disorders because they have the effect of inhibiting platelet aggregation and dilating blood vessels. Can increase bleeding.

13. Always keep ice packs in the patient's home. Patients with blood diseases have low body resistance and often have a fever. When you have a fever, your family should use the physical cooling method. You can put ice packs under the head and under the arm to achieve the purpose of cooling. You can also use cold water or alcohol to clean the bath to avoid cooling.

14. Prevent cross-infection at home. Cross-infection is a common cause of secondary infection of blood diseases. When relatives and friends suffer from respiratory infections or other infectious diseases, avoid contact with patients and keep the indoor environment clean.

Complications of pediatric bleeding disorders Complications anemia heart failure shock gastrointestinal bleeding

Long-term repeated bleeding can be complicated by anemia, severe bleeding can occur heart failure, acute massive hemorrhage can occur hemorrhagic shock, can cause joint deformity, dyskinesia, retinal hemorrhage can cause blindness, urinary tract bleeding, intracranial hemorrhage, gastrointestinal bleeding can cause death .

Symptoms of children with hemorrhagic disease Common symptoms Mantle coagulopathy Lymph node enlargement Upper gastrointestinal bleeding Retinal hemorrhage Intracranial hemorrhage Neonatal hematemesis and blood in the stool Blood vessels Thrombocytopenia Joint deformity

Hemorrhagic disease has various manifestations in clinical bleeding:

1. , freckle capillary, thrombocytopenia or platelet abnormalities, often manifested as sputum, small, scattered ecchymosis, bleeding caused by vascular factors, also seen in thrombocytopenia and thrombocytopenia, large The flaky ecchymosis is seen in coagulative bleeding, and widespread and large flaky ecchymoses are common in disseminated intravascular coagulation and fibrinolysis.

2. Nose bleeding, oral mucosal bleeding, nose bleeding, blood stasis, oral mucosal blood blister, tongue mucous blood blisters, seen in blood vessels and platelet factors caused by bleeding.

3. Joints, muscle bleeding usually occurs in the knees, ankles, hips, elbows, wrists and other large joints that are under stress or vulnerable to trauma, more common in severe hereditary coagulation factor deficiency, especially hemophilia A and B, joints Bleeding can often cause joint deformities, excessive anticoagulant substances and hemophilia A and B can cause muscle bleeding.

4. Retinal bleeding

More common in thrombocytopenic purpura.

5. Intracranial bleeding

Found in thrombocytopenic purpura and hemophilia.

6. Digestive tract and urinary tract bleeding

It can be seen in any factor caused by bleeding.

7. Post-traumatic bleeding

Postoperative bleeding is a common manifestation of various hemorrhagic diseases. If fibrinolysis or disseminated intravascular coagulation occurs during and after surgery, the wound will not ooze, local compression will be ineffective, and no blood clots will form on the surgical wound. Cuts cause bleeding, more common in hemorrhagic diseases caused by blood vessels and platelets.

Examination of pediatric bleeding disorders

The diagnosis of hemorrhagic diseases is highly dependent on laboratory tests. The specimen collection process has a great influence on the results of blood coagulation tests. The following points should be noted during the specimen collection process: venous puncture should be "one shot at a stroke", blood samples should not be used Mixed with air bubbles or tissue fluid; blood should not be taken at the indwelling needle because heparin or other drugs may be mixed; the ratio of anticoagulant (3.2% or 3.8% sodium citrate) to blood should be 1:10, if blood cells If the specific volume is too high (such as neonatal or cyanotic heart disease), the amount of tannic acid must be adjusted, so that the accuracy of the test results can be guaranteed. The specimen placed at room temperature must be measured within 2 hours after the blood is taken, if not Immediately, the plasma should be frozen immediately; the frozen plasma should be quickly thawed at 37 °C before testing, and immediately tested. After careful medical history and detailed physical examination, the following laboratory tests must be performed to confirm the diagnosis.

Screening test

Includes capillary fragility test, platelet count, bleeding time (BT), clotting time (CT), partially activated thromboplastin time (APTT), prothrombin time (PT), prothrombin consumption test (PCT), coagulation Enzyme time (TT), etc., PT is a screening test for exogenous coagulation pathways, but generally only when the level of coagulation factor II, V, VII or X is less than 30% or the level of fibrinogen is less than 1.0/L, PT is prolonged, APTT is a screening test for endogenous coagulation pathways, fibrinogen, factor II, V, VIII, IX, X, XI, XII, prokinin releasing enzyme or high molecular weight kininogen A lack of factors can lead to prolongation of APTT. The lack of the last three coagulation factors can significantly prolong APTT without clinically significant bleeding. APTT can also be used to screen circulating anticoagulant substances. In the presence of heparin, APTT is also prolonged, fibrin When the original content and function are reduced, TT can be prolonged. When TT is significantly prolonged, it often indicates the presence of heparin-like substances. It must be noted that the sensitivity and repeatability of the APTT test depend on the reagents selected, for the vast majority. Reagents It is said that APTT can only be prolonged when the level of factor VIII is lower than 35%. Generally speaking, the normal range of APTT in children and adults is 26-35 s, and that in term infants is longer (30-54 s), premature infants. It is longer, bleeding time and clotting time are too difficult to be standardized in clinical practice due to too many influencing factors, often can not provide useful information, and have now been eliminated, of course, if the bleeding time is determined strictly according to the formal operation For the primary hospitals to identify bleeding disorders still have a certain significance.

2. Confirmation test

After the above preliminary screening test is completed, it is generally determined that the condition of the child may be caused by abnormal platelets, abnormal blood coagulation or abnormal blood vessels. At this time, it is necessary to further examine the special coagulation-related indicators to confirm the diagnosis. Abnormal, specific clotting factor activity should be determined: 1 coagulation first stage: antigen and activity determination of factor VII and TF, thromboplastin production and corrective test; 2 coagulation second stage: prothrombin antigen and activity, prothrombin Fragment 1 2 (F1 2) determination; 3 coagulation third stage: fibrinogen, abnormal fibrinogen, blood (urine) fibrin peptide A (FPA) determination, factor XII antigen and activity determination, etc., if suspected anticoagulant system Abnormal, the following tests can be performed: 1AT-III antigen and activity or thrombin-antithrombin complex (TAT) determination; 2 protein C and related factor determination; 3 lupus anticoagulant determination, etc., if fibrinolytic abnormality is suspected, The following tests can be performed: 1 protamine secondary coagulation (3P) test; 2 blood, urinary fibrin degradation product (FDP) determination; 3D-dimer; 4 plasminogen determination; 5t-PA determination, if blood vessels are suspected Anomaly can enter Capillary angioscopy and vWF determination, if suspected platelet dysfunction, can check platelet adhesion and aggregation test, etc., it must be noted that the body's coagulation index is largely affected by age, many procoagulant factors, Anticoagulant factors and proteins involved in fibrinolysis are affected by pregnancy. The neonatal hemostatic system gradually matures in the first few weeks after birth, and is close to the level of normal adults at 6 months after birth. Hemorrhage or thromboembolic complications must also take into account the difference between the normal values of neonatal coagulation-related indicators and adults. If defined according to the normal values of adults, some normal newborns may be misdiagnosed as a certain clotting factor deficiency. disease.

In summary, for the evaluation of hemorrhagic diseases, the medical history must be carefully inquired. This is crucial for a clear diagnosis. Only on the basis of obtaining a complete and accurate medical history, it is possible to carry out the necessary coagulation screening and confirmatory tests. Finally, it should be pointed out that in the clinical and laboratory evaluation of children, non-hematological diseases must be considered to avoid delays in diagnosis. Children with flu, meningitis or liver disease may have DIC, malnutrition There may be thrombocytopenia or vitamin K-dependent clotting factor deficiency. Children with congenital heart disease may have thrombocytopenia, abnormal platelet function and even decreased levels of clotting factors.

According to the clinical manifestations and the cause of the disease, various imaging examinations, such as X-ray examination, B-ultrasound examination, CT examination, electrocardiogram examination, etc., are selected.

Diagnosis and diagnosis of hemorrhagic diseases in children

First, medical history

It is important to understand the history of bleeding in patients. Pay attention to the following aspects:

(1) Type of bleeding

The main points of the skin and mucous membranes, ecchymosis, more suggestive platelet or vascular bleeding, such as plaque bulge, more suggestive of vascular. If the deep tissue (muscle and joint cavity) bleeding is the main, it indicates the lack of coagulation factors. In addition, the first two tend to bleed immediately after trauma and have a short duration; the latter occurs slowly and lasts for a long time.

(2) Causes of bleeding

There is a history of drug exposure, more suggestive of platelet; such as bleeding after minor injuries, mostly clotting factor disorders.

(3) Family history

Because of hereditary bleeding diseases, there are often certain genetic methods. Parents, parents, siblings, grandparents, and uncles should be asked for similar medical history and bleeding history.

Second, physical examination

Observe the shape and distribution of the bleeding, whether it is symmetrical, flat or higher than the skin. Whether there is muscle bleeding or bleeding in the joint cavity, with or without systemic disease.

Third, laboratory inspection

According to the medical history and physical examination may provide some diagnostic clues, first use some simple tests to screen, find out at that point, and then do more complex diagnostic tests.

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