Pediatric asplenia syndrome

　　The etiology of pediatric asplenia syndrome has not been clearly defined. During the 4th to 5th week of embryogenesis, due to the action of certain teratogenic factors, the embryonic development stops and forms various malformations. At 24 to 27 weeks of embryonic development, it is the time when the pulmonary veins develop and the stomach and duodenum are finally fixed. Certain pathogenic factors at this stage may cause this disease.

　　In addition to its clinical manifestations, pediatric asplenia syndrome can also cause other diseases. This disease often complicates with serious infections, nutritional disorders, developmental disorders, and should be highly regarded by clinical doctors and parents.

　　The clinical manifestations of pediatric asplenia syndrome mainly include the following:

　　1, Persistent cyanosis

　　Patients have persistent cyanosis, difficulty breathing, feeding difficulties, delayed growth and development, tachycardia, and oxygen deficiency symptoms from birth, resembling cyanotic congenital heart disease.

　　2, Symptoms and signs of cardiovascular abnormalities

　　Older individuals may have clubbed fingers (toes), prominence in the anterior chest area. About half of the cases have a diffuse systolic murmur along the left margin of the sternum.

　　3, Aplastic spleen

　　Abdominal ultrasound shows that the spleen is extremely small or even absent.

　　4. Poor immune function

　　Patients have low resistance and often suffer from various infections.

　　5. Multiple malformations

　　In addition to asplenia and cardiovascular malformations, there are often abnormalities such as gastrointestinal malposition.

　　The etiology of pediatric asplenia is unknown, and it can be referred to the prevention methods of congenital diseases. Preventive measures should be carried out from pre-pregnancy to pre-natal. Pre-marital physical examination plays a positive role in preventing birth defects. Pregnant women should try to avoid harmful factors, including staying away from smoke, alcohol, drugs, radiation, pesticides, noise, volatile harmful gases, and toxic and harmful heavy metals. During the process of prenatal health care during pregnancy, systematic screening for birth defects should be carried out, including regular ultrasound examination, serological screening, and chromosome examination when necessary. Once abnormal results appear, it is necessary to determine whether to terminate the pregnancy and take practical and feasible diagnostic and treatment measures.

　　In the diagnosis of pediatric asplenia, in addition to relying on its clinical manifestations, it is also necessary to rely on chemical tests. The main examination methods are as follows:

　　1. Electrocardiogram

　　The examination shows that the electrocardiogram is abnormal, and there is variability in the pacemaker site.

　　2. Cardiac angiography

　　Cardiac angiography can prove the type of cardiovascular malformation. The malformations mainly include double-chamber heart, incomplete pulmonary artery development, triple-chamber heart, vascular malposition, atrial septal defect, and persistent truncus arteriosus. In addition, it is characterized by abnormal superior vena cava reflux.

　　3. X-ray gastrointestinal examination or ultrasound examination

　　Examinations can find absent or extremely small spleen, malposition of gastrointestinal tract, and other abnormalities.

　　Patients with pediatric asplenia need to supplement vitamins and proteins. The diet should be light and nutritious, and attention should be paid to dietary balance. Spicy and stimulating foods should be avoided to prevent recurrence of the disease. Eat more fresh vegetables and fruits, and eat more foods that enhance immunity, such as yam, turtle, mushroom, kiwi, fig, apple, sardine, honey, milk, and pork liver, to enhance the body's ability to resist diseases.

　　There is no reliable treatment for pediatric asplenia. The therapeutic effects of cardiac surgery are not ideal. The main treatment measures include active prevention and treatment of infection, correction of cardiorespiratory failure, and prevention of complications.