Pediatric Jakesh syndrome

Introduction to children's Jacques syndrome Von Jakschssyndrome (von Jakschssyndrome) is a von Jaksch syndrome with pathogenic factors such as nutritional deficiencies (mainly hematopoietic deficiencies), rickets and long-term chronic or recurrent infections (such as respiratory infections, urinary tract infections, skin purulent infections, etc.). It is characterized by infancy, with severe anemia, hepatosplenomegaly, increased peripheral white blood cell count and immature granulocytes and nucleated red blood cells, and the condition is chronic. The general prognosis is good, and it can be cured after removing the cause, improving nutrition, and treating anemia. Also known as infant pseudo-leukemia anemia, infant splenomegaly, infection with nutritional anemia. basic knowledge Sickness ratio: 0.0001% Susceptible people: children Mode of infection: non-infectious Complications: vitamin D deficiency

The cause of children's Jacques syndrome

(1) Causes of the disease

This symptom is a special type of anemia in infants and young children. It is not an independent disease. It is a special reaction to the anemia and infection in infants and young children. Generally, the life span of red blood cells is shortened, the destruction is accelerated, the bone marrow hematopoietic function is poor, and the iron metabolism disorder is the main. the reason.

(two) pathogenesis

The primary infection of the child lasted for more than 1 month. In chronic infection, the reticuloendothelial system proliferated and hyperfunction, and the red blood cells were destroyed prematurely. Its life span was shortened from 120 days to 80 days, and the compensation of normal bone marrow. The ability is very strong, if the red blood cell life is mild to moderately shortened, it is easy to compensate, and no anemia occurs. When infected, the bone marrow lacks the corresponding compensatory ability, which is the main cause of anemia. The erythropoietin measurement proves that erythropoiesis Insufficient production, can not adapt to the needs of infection, in addition, there are barriers to the use of iron, serum iron is significantly reduced at the time of infection, total iron binding capacity is also reduced, which may be related to the reduction of transferrin production, liver, spleen and bone marrow, etc. The amount of stored iron in the tissue increases, but the iron released into the plasma by the reticuloendothelial system is reduced, that is, the so-called reticuloendothelial system iron block, and the iron metabolism disorder can be corrected after the infection is restored.

Childhood Jacques Syndrome Prevention

1. Do a good job in various vaccination work.

2. Strengthen nutrition, enhance physical fitness and promote health.

Complications of children's Jacques syndrome Complications vitamin D deficiency

Repeated infection, liver and spleen enlargement, may be associated with vitamin D deficiency. The occurrence of vitamin D deficiency is closely related to calcium and phosphorus metabolism. The effect on the body is systemic, and its outstanding performance is rickets or osteomalacia. It is due to the lack of vitamin D in the diet or the lack of sunlight in the human body. The synthesis of vitamin D in the body is reduced, resulting in vitamin D deficiency, which leads to abnormal bone calcification.

Symptoms of children's Yakeshi syndrome Common symptoms Repeated infection, pale skin, bleeding, irregular heat, obvious small, small edema, hepatosplenomegaly

The main clinical manifestations are extra-parenchymal hematopoiesis such as nutritional anemia and hepatosplenomegaly. Repeated infection, liver and spleen enlargement, may be associated with vitamin D deficiency.

1. The incidence of infants and young children occurs mostly in infants from 6 months to 2 years old.

2. Have a history of chronic or repeated infections. Anemia is heavier and malnourished.

3. The onset is slow, the complexion is gradually pale or waxy yellow, thin, lack of spirit, and often irregular fever.

4. Physical examination shows that the liver and spleen are obviously swollen, especially the spleen is obvious, even up to the pelvis, and the texture is hard. The lymph nodes of the whole body can be slightly enlarged, sometimes with bleeding spots or edema. The clinical features such as juvenile and young red blood cells can be considered for diagnosis.

Examination of children's Jacques syndrome

1. Blood: The degree of anemia depends on the primary disease. More manifested as moderate or above nutritional mixed anemia. Hemoglobin is more than 60 ~ 90g / L (6 ~ 9g / dl), red blood cells are correspondingly reduced, the size is not equal, easy to see abnormal, multi-staining and nucleated red blood cells, this is small cell positive pigment anemia, severe anemia can be presented Small cell hypochromic anemia, reticulocyte red blood slightly increased. Leukocytosis, even up to 30 × 109 / l ~ 40 × 109 / l, classification of immature granulocytes in each period (even a few original granulocytes), but still more mature. Reticulocytes are normal or reduced, platelets are normal, and white blood cells are increased. These two points are different from iron deficiency anemia.

2. Bone marrow: bone marrow hyperplasia is acceptable, hyperplasia is active or significantly active. Cell sorting and morphology are similar to nutritional mixed anemia. A small number of cases can be proliferated, lymphocytes are relatively increased, and megakaryocytes are reduced. The proportion of granulocytes and red blood cells is normal, and the red system is not compensated for hyperplasia. The hemosiderin in the bone marrow is increased.

3. Chest X-ray examination: In the case of respiratory infection, the chest radiograph shows an inflammatory shadow in the lungs.

4. X-ray of the long bones of the epiphysis: The X-ray findings of vitamin D deficiency in the long bones of the long bones can be found. The calcification preparation line of the early X-ray long bones is blurred; the preparation of the extreme calcification disappears, the end of the epiphysis is widened, and the caudal end It has a cup or brush shape, and the bone is sparse, the bone is bent or deformed. X-ray bone age film found that bone age is backward.

5.B super check.

Diagnosis and diagnosis of children's Jacques syndrome

Mainly according to the onset of infants and young children, there are obvious nutritional deficiencies and a history of repeated or chronic infection, anemia is heavier and malnourished, hepatosplenomegaly is especially obvious with splenomegaly, and the surrounding blood picture appears young particles, young red blood cells and other clinical features Diagnosis can be considered.

1. Iron deficiency anemia When the disease is severe, small cell hypochromic anemia can be seen. At this time, it should be differentiated from iron deficiency anemia. The latter serum iron is decreased, but the total iron binding capacity of the serum is increased, and the extracellular iron of the bone marrow is reduced or even disappeared. The spleen is generally not swollen.

2. Leukemia: The peripheral blood of the disease can be seen in immature granulocytes, and clinically accompanied by hepatosplenomegaly, it still needs to be differentiated from leukemia, mainly by bone marrow examination. The condition of acute leukemia develops rapidly, and there is a tendency to hemorrhage. The naive cells in the blood picture are mainly in the primary and young stages, and the platelets are mostly reduced. The bone marrow has typical leukemia changes. The blood and bone marrow of infants with chronic myeloid leukemia are obviously changed by granulocytes, and fetal hemoglobin is often significantly increased. The above characteristics can be identified.

3. Leukemia: There is a primary infection, the splenomegaly is lighter, the blood picture has no nucleated red blood cells, the granulocytes have infection and poisoning changes, and the blood picture returns to normal after the primary disease control.

4. Chronic hemolytic anemia should be differentiated from chronic hemolytic anemia when nucleated red blood cells and reticulocytes are increased. The latter may have abnormalities in red blood cell morphology or hemoglobin abnormalities of familial hemolytic anemia or thalassemia and confirm the presence of hemolysis. Test positive results.

5. Other diseases with extramedullary hematopoiesis: such as infantile osteopetrosis, bone marrow fibrosis, etc., also manifested as anemia, splenomegaly, peripheral blood appearing immature granules, red blood cells, but bone marrow puncture is often not successful. Bone marrow biopsy or bone x-ray film can help identify.

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