Pediatric Antiphospholipid Syndrome
Introduction
Introduction to anti-phospholipid syndrome in children Antiphospholipid syndrome (APS) is a group of clinical signs caused by antiphospholipid antibodies (APL antibodies), mainly characterized by thrombosis, habitual abortion and thrombocytopenia. basic knowledge The proportion of illness: 0.0025% Susceptible people: children Mode of infection: non-infectious Complications: avascular necrosis myocardial infarction cerebral thrombosis cerebral hemorrhage epilepsy
Cause
Pediatric antiphospholipid syndrome etiology
the reason
The cause is still unclear and is an immune disorder.
(two) pathogenesis
The exact pathogenesis of this disease is still unclear.
Antiphospholipid antibodies have an effect on vascular endothelial cells and platelet function. When antiphospholipid antibodies bind to phospholipids of vascular endothelial cells, the function of endothelial cells is impaired, resulting in decreased synthesis and release of prostacyclin. Prostacyclin has Important anti-platelet aggregation and strong vasodilator action. Anti-phospholipid antibodies bind platelet phospholipids to activate platelets, releasing thromboxane A2 (thromboxane, TXA2), a vasoconstrictor and pre-aggregation substance. When APL is positive, the patient's serum has a significant increase in thromboxane and a decrease in prostacyclin levels. Due to the imbalance of the two, the blood vessels contract, the blood flow is slow, and the anti-platelet aggregation function is weakened.
In addition, antiphospholipid antibodies can affect the function of some endothelial cell proteins, such as decreased levels of antithrombin III (ATIII), and the interaction of antiphospholipid antibodies with thrombomodulin on endothelial cells leads to hypercoagulability In the state, antiphospholipid antibodies can also bind to placental anticoagulant protein 1, PAP1, which reduces the local anticoagulant ability of the placenta, leading to placental thrombosis and spontaneous abortion.
Prevention
Pediatric antiphospholipid syndrome prevention
The etiology and pathogenesis of this disease are not clear enough, so there is no precise preventive measure. Such a disease can be properly conditioned by traditional Chinese medicine, and qi and blood to raise the spleen and stomach to adjust sleep, rationality to enhance body resistance. You can use six gentlemen, Guipi Tang, Yangxintang and other drugs, pay attention to the daily maintenance of combined vitamin foods, actively prevent the disease and prevent children's antiphospholipid syndrome.
Prognosis
Patients who are treated with oral anticoagulants are at a very low risk of recurrence of thrombosis. There are currently no validated treatment options for patients with severe APS, and such patients should be closely observed.
The patient's 4-year mortality rate is 15%. The prognosis of patients with complications is not good, renal failure, cerebrovascular accident, myocardial infarction can be the cause of death. Children are more likely to have cerebral arterial thrombosis.
Complication
Pediatric antiphospholipid syndrome complications Complications ischemic necrosis myocardial infarction cerebral thrombosis cerebral hemorrhage
Arterial thrombosis can occur, ischemic necrosis, such as myocardial infarction, intestinal infarction, can lead to renal failure, etc.; nervous system damage can be complicated by cerebral ischemia, cerebral thrombosis, cerebral hemorrhage, mental behavior abnormalities, epilepsy, chorea and spinal cord Lesions, such as paraplegia, can lead to thrombocytopenia; spontaneous abortion and stillbirth, gangrene of the extremities, chronic ulcers of the limbs, heart valve damage and endocarditis.
Symptom
Symptoms of antiphospholipid syndrome in children Common symptoms Venous thrombosis Reticular leukoplakia renal failure Abortion Infarction Lower extremity edema Cerebral ischemia Myocardial infarction Thrombocytopenia
Thrombosis
Thrombosis can occur in the arteries or in the veins. The clinical manifestations depend on the type, location and size of the affected vessels. They can be manifested as single vessels or multiple vessels. The most common site is deep vein thrombosis of the lower extremities. Leg edema and pain, in addition, the brain, kidney, retinal arteries and veins, sagittal sinus and cavernous sinus can be thrombosis, although thrombosis can occur in various movements, veins and their branches, but the greater threat to the patient is the artery Thrombosis, arterial circulatory obstruction, avascular necrosis, such as myocardial infarction, intestinal infarction, etc., renal artery and smaller blood vessels obstruction can lead to renal failure, Beijing Children's Hospital observed 2 anti-phospholipid antibody-positive lupus The visual acuity was reduced in children with occlusion of the retinal artery. In one patient with lupus, the subclavian vein and the iliac vein were embolized, resulting in upper extremity edema and pain.
2. Thrombosis
Thrombocytopenia is one of the manifestations of antiphospholipid syndrome. It has been reported that 30% of patients with idiopathic thrombocytopenic purpura are positive for antiphospholipid antibodies, and about 50% of patients with systemic lupus erythematosus may have thrombocytopenia throughout the disease. It may be that the antiphospholipid antibody binds to the platelet membrane phospholipid, activates the platelets, accelerates the agglutination, and causes thrombocytopenia.
3. Neuropsychiatric system damage
Neuropsychiatric symptoms are mainly characterized by transient ischemic attacks, cerebrovascular accidents, including cerebral thrombosis, cerebral hemorrhage, abnormal mental behavior, epilepsy, chorea and spinal cord lesions such as paraplegia.
4. Spontaneous abortion and stillbirth
Women with antiphospholipid antibodies often have multiple spontaneous abortions or a history of end-stage fetal death in the fetus. Pathological studies have found that placental vascular thrombosis and obstruction in the placenta of anti-phospholipid antibody-positive habitual abortion As a result, the vasospasm segment is separated from the placenta, resulting in poor blood perfusion of the placenta, which is the main cause of abortion caused by reduced placenta and fetal blood supply.
5. Other performance
Skin reticular bluish, hemolytic anemia and gangrene due to arterial thrombosis, and venous thrombosis can cause thrombophlebitis or chronic ulcers of the limbs. In addition, see also related to heart valve injury and endocarditis. Report.
Examine
Pediatric antiphospholipid syndrome test
1. The positive rate of syphilis serum false positive test (BFP-STS) and VDRL test in lupus or other connective tissue diseases is 5% to 19%, so the sensitivity of these two methods for thrombosis of this disease Sex and specificity are not high.
2. lupus anticoagulant (LA) LA is an IgG or IgM immunoglobulin that interferes with and prolongs various phospholipid-dependent coagulation tests in vitro. The most sensitive method for screening LA is In the kaolin agglutination test (KCT), LA does not only occur in lupus, it is associated with thrombosis.
3. Anti-phospholipid antibody, anti-lipinoid antibody Anti-phospholipid antibody is a group of antibodies that can react with various antigenic substances containing phospholipid structure. Anticardiolipin antibody (ACL) is one of them, ACL and clinical Some of the above are associated with thrombosis, habitual abortion, thrombocytopenia and neuropsychiatric diseases, and the specificity is very strong, so many scholars often combine the above clinical manifestations with anti-phospholipid syndrome of such antibodies, also known as For anti-cardiac syndrome.
Routine imaging examination, you can choose B-ultrasound, echocardiography, selective angiography, brain CT and other examinations.
Diagnosis
Diagnostic identification of anti-phospholipid syndrome in children
diagnosis
Antiphospholipid syndrome is not a specific disease, but a comprehensive clinical manifestation. In 1999, the international standard for the diagnosis of antiphospholipid syndrome was simplified:
Clinical standard
(1) vascular thrombosis.
(2) One or more arterial, venous or small vessel thrombosis in any tissue or organ clinically
Pregnancy complications.
(3) One or more unexplained deaths of a normal morphological fetus at 10 weeks or 10 weeks after gestation; or unexplained death of a morphologically normal newborn one or more times before or at 34 weeks of gestation Or a spontaneous spontaneous abortion of unknown cause for more than 3 times before 10 weeks of gestation.
2. Laboratory standards
(1) Anti-cardiolipin antibodies are increased.
(2) Medium or high titer anti-cardiolipin antibodies IgG or IgM were detected in the blood for more than 2 times, and the two tests were at least 6 weeks apart.
(3) Lupus anti-agglutination antibody.
(4) According to the guidelines of the thrombosis and hemostasis association: lupus anti-agglutination antibodies are detected in the blood more than 2 times, and the two tests are at least 6 weeks apart.
Differential diagnosis
Primary and secondary antiphospholipid syndromes must be identified. It is difficult to diagnose PAPS from clinical manifestations or laboratory tests. A patient with moderate to high titer aCL or LA is positive, and PAPS may be considered in the following cases: 1 Interpretation of arterial or venous thrombosis; 2 thrombosis in uncommon areas (such as kidney or adrenal gland); 3 thrombosis in young people; 4 recurrent thrombosis; 5 recurrent thrombocytopenia; 6 miscarriage occurring in the second trimester of pregnancy .
Venous thrombosis requires protein C, protein S and antithrombin III deficiency, thrombotic thrombocytopenic purpura, fibrinolysis abnormalities, nephrotic syndrome, paroxysmal nocturnal hemoglobinuria, Behcet's disease and associated with oral contraceptives Identification of diseases such as thrombosis.
Arterial thrombosis needs to be differentiated from hyperlipidemia, diabetic vascular disease, thromboangiitis obliterans, vasculitis, hypertension and other diseases.
It should be noted that the presence of aPL does not necessarily cause thrombosis. About 12% of normal people may have IgG or IgM aCL antibody positive, syphilis and AIDS, Lyme disease, infectious mononucleosis, tuberculosis and other diseases respectively. 93%, 39%, 20%, 20% positive rate of antiphospholipid antibodies, some drugs such as phenothiazine, procainamide, chlorpromazine, hydralazine, phenytoin, quinine, phenalol And oral contraceptives can also induce aPL; in addition, some malignant tumors such as melanoma, nephroblastoma, lung cancer, lymphoma and leukemia can also be aCL or antibody positive.
