pediatric hyperviscosity syndrome
Introduction
Introduction to children with high viscosity syndrome High-viscosity syndrome, also known as sticky syndrome, purpuric hyperglobulin syndrome, Reimann syndrome, is a disease caused by elevated blood viscosity. This symptom refers to a sharp blood viscosity when the hematocrit is more than 70%. Ascending, causing an increase in blood fluid dynamics resistance, making the patient's red blood cell drive difficult, a series of unique clinical manifestations. basic knowledge The proportion of sickness: 0.003%-0.005% Susceptible people: infants and young children Mode of infection: non-infectious Complications: jaundice, hypoglycemia, purpura
Cause
Pediatric hyperviscosity syndrome etiology
Tissue hypoxia or oxygen release disorders (30%):
(1) Physiological: 1 fetal period; 2 the oxygen content in the environment is not high in the plateau area.
(2) Pathological: 1 Insufficient lung ventilation: pulmonary diseases such as bronchiectasis, pulmonary heart disease, obesity (pickwickian syndrome), 2 pulmonary arteriovenous fistula, 3 cyanotic congenital heart disease, 4 abnormal hemoglobin disease: hemoglobin M Sulfur hemoglobin and methemoglobin have poor oxygen carrying capacity.
Enhanced function of bone marrow-derived red blood cells (30%):
(1) Endogenous: 1 renal: renal embryo tissue tumor, adrenal adenoma, polycystic kidney, renal artery stenosis, etc. 2 Adrenal gland: pheochromocytoma, Cushing syndrome, congenital adrenal hyperplasia, adrenal adenoma Primary aldosteronism, etc., 3 liver: hepatoma, 4 cerebellum: hemangioblastoma.
(2) Exogenous: 1 testosterone or similar drug, 2 application of growth hormone.
Newborn (30%):
(1) Transplacental transfusion: the mother transfused to the fetus, the transfusion between the twins.
(2) The umbilical cord is ligated too late.
Pathogenesis
The main cause of redness is due to tissue hypoxia, and the compensatory enhancement of bone marrow-derived red blood cells. Increasing the red blood cells in the circulating blood can increase the oxygen carrying capacity of the blood. At this time, the secretion of EPO in the kidney is generally increased, in a few cases. For example, some kidney diseases and malignant tumors can cause Ji Hong (Beijing University of Medical Sciences Women and Children Hospital Yang Xiaoyun has reported 15 cases of pediatric nephrotic syndrome with Jihong, Beijing Children's Hospital reported a case of polycystic kidney with red), this situation There is no tissue hypoxia. At this time, the increase of red blood cells and the increase of oxygen carrying capacity have no obvious need for patients, but are pathological, which is caused by increased secretion of EPO.
2. Increased blood viscosity
The viscosity of normal blood is mainly composed of the specific product of red blood cells and plasma proteins. The increase in blood viscosity is seen in the following cases:
(1) The number of red blood cells is significantly increased, such as various polycythemia.
(2) Deformation of red blood cells, such as sickle cell disease and spherical red blood cell disease.
(3) Abnormal increase of plasma protein concentration, such as macroglobulinemia, clinically refers to the first case, arterial hypoxia can stimulate the proliferation of red blood cells by bone marrow, and increased hemoglobin can increase the oxygen carrying capacity of unit blood volume. The ability, macroglobulinemia IgM> 5g, serum viscosity > 6% can occur, because the IgM molecular weight is large, but its viscosity is also large.
Prevention
Prevention of high viscosity syndrome in children
Do a good job of pregnancy care, prevent fetal period, birth and postpartum hypoxia; prevent pregnancy, especially early pregnancy virus infection; prevent various chemical and physical toxicity damage; actively prevent congenital heart disease.
Complication
Pediatric hyperviscosity syndrome complications Complications jaundice hypoglycemia
Can be complicated by jaundice, tachycardia, heart failure, difficulty breathing, hepatosplenomegaly, purpura, hypoglycemia, hypocalcemia, hemorrhage, DIC, thrombosis, anemia, fundus lesions, hearing impairment, tinnitus, skin mucosal ulcers and gangrene , kidney damage and so on.
Symptom
Symptoms of high-viscosity syndrome in children Common symptoms Jaundice, dizziness, dyspnea, tachycardia, hepatosplenomegaly, ocular tremor, dysmotility, visual disturbance, heart failure, gingival bleeding
Newborn
Can be expressed as vomiting, jaundice, trembling, tachycardia, heart failure, shortness of breath, difficulty breathing, hepatosplenomegaly, thrombocytopenic purpura, hypoglycemia, hypocalcemia and brain symptoms.
2. Bleeding
More common, often sudden nosebleeds and bleeding gums, bleeding and M protein inhibition of coagulation factors, in recent years, blood viscosity syndrome is considered to be one of the causes of diffuse intravascular coagulation (DIC), children with cyanotic congenital heart disease Coagulopathy may be related to this.
3. Thrombosis
Most of the infants who are hypoxic within 2 years of age, the hematocrit is not very high at this age, and the older children have higher blood clots than the higher ones.
Anemia
More common in the primary disease anemia, but also with increased serum viscosity, increased plasma yield and blood dilution.
5. Eye performance
Eye symptoms are abnormal vision, double vision and visual impairment. Fundus lesions are characteristic of this disease and are parallel with changes in viscosity. For example, the retinal vein filling varicose increases, showing a sausage-like appearance, and then bleeding or bleeding.
6. Neurological symptoms
There are dizziness, hearing impairment, movement disorders, nystagmus, tinnitus and other peripheral nerve damage and pyramidal bundle symptoms.
7. Cardiovascular symptoms
Symptoms of cardiac dysfunction due to increased serum viscosity, increased blood volume, and corresponding increase in cardiac load, as well as peripheral circulatory disturbances caused by increased serum viscosity and intravascular red blood cell aggregation, such as Raynaud's phenomenon, skin mucosal ulcers, and Gangrene phenomenon.
8. Renal function changes
More common in patients with myeloma, increased serum viscosity can cause a decrease in renal blood flow, leading to damage to kidney function.
Examine
Examination of children with high viscosity syndrome
Plasma protein analysis
Determination of plasma protein, fibrinogen does not increase, and globulin increased significantly. After electrophoresis on paper or agar electrophoresis, a macroglobulin band, or a significant increase in gamma globulin, or a so-called M component, can be diagnosed. For abnormal globulinemia.
2. Qualitative and quantitative determination of abnormal proteins
In severe patients, when taking venipuncture, the blood taken out quickly solidifies, unable to continue blood collection, even using a normal amount of anticoagulant, can not block blood coagulation, blood viscosity determination, immunoelectrophoresis analysis, radiation immunity Determination and determination of sedimentation coefficient, qualitative and quantitative determination of abnormal proteins, routine chest X-ray, B-ultrasound, electrocardiogram, EEG and CT examination.
Diagnosis
Diagnosis and differentiation of children with high viscosity syndrome
diagnosis
According to the clinical manifestations can provide a diagnosis basis, but the diagnosis must rely on laboratory tests, blood cell specific volume, blood viscosity measurement (normal value of 3.5 to 5 times water), protein chemistry research, including immunoelectrophoresis analysis, radioimmunoassay Measurement and determination of sedimentation coefficient can be used for qualitative and quantitative analysis of abnormal proteins, which is of great help to diagnosis.
Differential diagnosis
It should be differentiated from polycythemia vera (true red) and pseudo-erythrocytosis (false red) caused by blood concentration.
