Neonatal hepatosplenomegaly

　　1. Causes of disease

　　There are many reasons for neonatal liver and spleen enlargement. According to the frequency of clinical occurrence, the order is as follows.

　　1. Infections

　　Such as sepsis caused by various bacterial infections, neonatal hepatitis caused by intrauterine or perinatal infection, toxoplasmosis caused by protozoan infection, etc. The viruses that cause neonatal hepatitis are more common, such as hepatitis B virus, cytomegalovirus, rubella virus, and herpes zoster virus, etc.

　　2. Hematological diseases

　　Such as neonatal母婴 blood type incompatibility hemolytic disease, hereditary spherocytosis, thalassemia, etc.

　　3. Cardiac diseases

　　Liver enlargement caused by congestive heart failure can be seen in hypoxic ischemic myocardial damage after asphyxia, as well as in various congenital heart diseases such as large ventricular septal defects, great vessel transposition, left ventricular hypoplasia, and aortic stenosis, etc.

　　4. Biliary diseases

　　Primarily congenital biliary atresia.

　　5. Genetic metabolic diseases

　　Such as glycogen storage disease, galactosemia, hyperlipidemia, tyrosinemia, and lipoidosis, etc.

　　6. Cell proliferation and tumors

　　Such as congenital leukemia, malignant histiocytosis, lymphoreticular cell sarcoma, liver cysts, and liver tumors, etc.

　　The causes of neonatal liver enlargement can be divided into two major categories according to whether jaundice is present. Those with jaundice include neonatal hepatitis, neonatal hemolytic disease, sepsis, extrahepatic biliary atresia, choledochal cyst, hereditary metabolic diseases, etc.; those without jaundice include heart failure, immunological and non-immunological fetal edema, glycogen storage disease, lysosomal disease, and liver cysts, etc.

　　Patients with moderate to severe liver enlargement should consider infections caused by various pathogens, congestive heart failure, congenital biliary atresia, glycogen storage disease, mucopolysaccharidosis, lipidosis, and galactosemia, etc.

　　The most common causes of splenomegaly in newborns are infection and hemolysis. Neonatal sepsis and neonatal hepatitis can cause both liver and spleen to swell. Neonatal hemolytic disease caused by incompatibility of blood types is the most common hemolytic disease in the neonatal period, followed by G-6-PD deficiency, hereditary spherocytosis, thalassemia, and sickle cell anemia. Other diseases that can cause splenomegaly, such as marble bone disease, Gaucher's disease, mucopolysaccharidosis, etc., are rare.

　　2. Pathogenesis

　　The liver and spleen are two important organs in the abdominal cavity, which have both unique features and common points in terms of physiological function. They are interrelated in blood circulation, and therefore, they are often closely related in clinical pathology as well.

　　The liver and spleen of normal newborns are relatively large. The weight of the liver is 120～130g, accounting for 4% of the body weight, while in adults it is 2%. The weight of the spleen is about 10g, which is 1/30 of an adult's spleen.

　　1, Liver enlargement

　　Neonates are prone to liver enlargement, which is related to the pathophysiological characteristics:

　　(1) Easy to become engorged and swollen: The liver cells and lobules of neonates are not fully differentiated, with rich blood vessels, which are prone to become engorged and swollen. The blood circulation of the liver is very rich, and in cases of severe heart failure, especially in cases of heart failure and obstruction of inferior vena cava return, engorged liver enlargement can occur.

　　(2) Extramedullary hematopoiesis: In the embryonic period, the liver is the main hematopoietic organ. After birth, if there is anemia, the liver will participate in extramedullary hematopoiesis again, causing liver enlargement.

　　(3) Metabolic and detoxification function: The liver is the largest metabolic organ in the human body, responsible for the metabolism of protein, fat, sugar, and other substances and the detoxification of toxins. Therefore, some congenital metabolic diseases and toxins can cause liver enlargement.

　　(4) Bile pigment metabolism and excretion function: The liver is the organ of metabolism and excretion of bilirubin. Disorders of bilirubin metabolism and congenital malformations of the bile duct can cause severe liver enlargement.

　　(5) Reticuloendothelial system: The liver is an organ rich in reticuloendothelial system, with defensive functions. When the tissue itself is affected by acute or chronic infections, it can cause liver enlargement or reticuloendothelial system hyperplasia and swelling.

　　2, Splenomegaly

　　The pathophysiological factors of neonatal splenomegaly include:

　　(1) Reticuloendothelial system: The spleen is one of the important reticuloendothelial system organs, responsible for the body's defense function. Infections by bacteria, viruses, and parasites can cause splenomegaly, but it is extremely rare for the spleen itself to be infected.

　　In diseases of the reticuloendothelial system and metabolic diseases, the reticuloendothelial cells phagocytize a large amount of abnormal metabolic substances, which can cause splenomegaly. In addition, the spleen is also the place for the destruction of aged, damaged, and abnormal blood cells in the blood circulation. When the destruction of blood cells increases (such as congenital hemolytic anemia, congenital malaria, etc.), it can cause significant splenomegaly.

　　(2) Hematopoietic organs: The spleen, like the liver, is also one of the hematopoietic organs in the fetal period. In the postnatal period, when the hematopoietic compensatory function is hyperactive due to factors such as infection, it can cause splenomegaly.

　　(3) Blood storage organs: The spleen is a blood storage organ, and the splenic vein flows into the portal vein. When the portal vein is obstructed, it causes congestion in the spleen, resulting in congestive splenomegaly.

　　(4) Lymphoid organs: The spleen is also a lymphoid organ, so the malignant tumors of the spleen (mainly lymphoma and various types of non-lymphoid leukemia) can have splenic infiltration and lead to splenomegaly.

　　The complications of splenomegaly vary depending on the primary disease. For example, splenomegaly diseases caused by neonatal infectious diseases can be accompanied by neonatal sepsis, purulent meningitis, pneumonia, lung abscess, metastatic foci (such as cellulitis, osteomyelitis, pyelonephritis, etc.), and multi-organ dysfunction syndrome.

　　1, Neonatal sepsis (septicemia of newborn):It refers to the systemic infection caused by pathogenic bacteria entering the neonatal blood circulation through various pathways, growing and reproducing in it, and producing toxins.

　　2. Meningitis:It is a disease in which a delicate meninges or cerebrospinal meninges (a layer of membrane between the skull and the brain) is infected. This disease is usually accompanied by complications of bacterial or viral infection in any part of the body, such as ear, sinus, or upper respiratory tract infection.

　　3. Pneumonia:It refers to the inflammation of the terminal airways, alveoli, and interstitium of the lung, which can be caused by pathogenic microorganisms, physical and chemical factors, immune injury, allergy, and drugs. Bacterial pneumonia is the most common pneumonia and one of the most common infectious diseases.

　　4. Lung abscess (lung abscess):It is a suppurative lesion of lung tissue caused by various etiologies. In the early stage, it is a suppurative inflammation, followed by necrosis and the formation of an abscess.

　　5. Osteomyelitis:It is an infection and destruction of bone caused by aerobic or anaerobic bacteria, mycobacteria, and fungi. Osteomyelitis often occurs in the vertebrae; in diabetic patients, the foot or in the case of penetrating bone injury caused by trauma or surgery, the most common site in children is the long bones with good blood supply, such as the diaphysis or femur. Osteomyelitis can be acute or chronic according to the development of the disease.

　　6. Pyelonephritis (pyelonephritis):It refers to the inflammation of the renal pelvis, mostly caused by bacterial infection, usually accompanied by lower urinary tract inflammation. It is difficult to strictly differentiate in clinical practice. According to the clinical course and disease, pyelonephritis can be divided into acute and chronic stages. Chronic pyelonephritis is an important cause of chronic renal insufficiency.

　　1. Antenatal history

　　If liver and spleen enlargement is caused by hereditary metabolic diseases, family members may suffer from the same disease. Liver and spleen enlargement caused by neonatal hemolytic disease may have a history of jaundice in previous pregnancies, maternal infection history during pregnancy, such as Toxoplasmosis infection (i.e., maternal infection with plasmodium, rubella, cytomegalovirus, and herpesvirus, etc.), which can cause congenital abnormalities and liver and spleen enlargement in newborns. If the mother has hepatitis B virus infection before childbirth, it is helpful for the diagnosis of neonatal hepatitis syndrome. If the mother has a history of malaria before childbirth, it can cause neonatal malaria.

　　2. Birth history

　　Maternal infection during childbirth can cause neonatal sepsis leading to liver and spleen enlargement.

　　3. Symptoms

　　The causes of liver and spleen enlargement vary, and the clinical symptoms of newborns are different. Symptoms such as fever or hypothermia, refusal to breastfeed, no weight gain, abdominal distension, and jaundice are often related to infection, such as neonatal sepsis, neonatal liver abscess, and neonatal hepatitis syndrome. Galactosemia patients may have hypoglycemic seizures, vomiting, jaundice, cataracts, obstructive jaundice, neonatal hepatitis syndrome, severe jaundice, grayish-white stools, and liver and spleen enlargement. Neonatal hemolytic disease, malaria, and others may have anemia, jaundice with liver and spleen enlargement, a tendency to bleed and purpura, which are more common in neonatal thrombocytopenic purpura and leukemia.

　　4. Physical Examination

　　Firstly, determine the presence and extent of liver and spleen enlargement, and it is best to perform the physical examination when the newborn is calm. The palpation should be gentle, and when palpating liver and spleen enlargement, attention should be paid to the degree and texture of the enlargement, as well as the characteristics of the liver and spleen themselves. The degree of liver and spleen enlargement can serve as a reference for diagnosis, treatment effects, and prognosis judgment.

　　One, the key to preventing neonatal liver and spleen enlargement should be the treatment of the underlying cause. If there is an infection, appropriate anti-infection treatment should be carried out. If there is a blood disease, active treatment and the removal of precipitating factors should be carried out. Correcting heart failure, etc.

　　Prenatal examination, early diagnosis after delivery, and timely treatment should be effective preventive measures.

　　One, blood examination

　　1, blood picture

　　White blood cell count and cell morphology observation have diagnostic value for infectious diseases and leukemia. Hemoglobin, anemia, and increased reticulocytes suggest hemolytic anemia.

　　2, quantitative and qualitative examination of blood bilirubin

　　Liver function tests are indispensable for diagnosing jaundice with liver and spleen enlargement. The determination of serum bilirubin concentration is the most common laboratory test for neonatal liver enlargement. Neonatal blood diseases are one of the most common causes of neonatal jaundice. Many liver enlargement diseases with jaundice need to be differentiated, especially within the first week of life. If the serum bilirubin level continues to increase for more than 2 weeks after birth, and primarily due to direct bilirubin, it should be considered as a liver disease.

　　3, liver function examination

　　Liver function tests, hepatitis B surface antigen (HBsAg), hepatitis B core antigen, and E antigen tests are important for diagnosing whether neonatal hepatitis syndrome is caused by the hepatitis B virus. In neonates, the brain phospholipid flocculation test and zinc sulfate turbidity test in liver function tests often do not show positive reactions. Alanine aminotransferase and aspartate aminotransferase are also abundant in cardiac and muscle tissues, and these enzymes can be released in large quantities into the blood after asphyxia and hypoxia. Lactate dehydrogenase increases during hepatitis but not during obstructive jaundice, indicating that enzymes with cholestasis include alkaline phosphatase, leucine aminopeptidase, and gamma-glutamyl transpeptidase, and serum 5'-nucleotidase also significantly increases in bile duct atresia.

　　4, etiologic examination

　　Blood bacterial culture, virus isolation, and detection of specific antibodies can help determine the types of bacteria and viruses causing infection.

　　5, other tests

　　If there is a suspicion of abnormal glucose metabolism, blood glucose and glucose tolerance tests should be measured. To confirm blood type incompatibility hemolytic disease, antihuman globulin direct test, free antibody determination, and antibody release test should be performed. Serum protein electrophoresis, alpha-fetoprotein, immunoglobulins, and other tests can be selectively performed as needed.

　　Two, bone marrow examination

　　Bone marrow biopsy should be performed when there is a suspicion of blood disease or malignant cell proliferation, which is very valuable for diagnosing leukemia, thrombocytopenic purpura, malaria, and so on.

　　Three, liver biopsy

　　Consider liver and spleen biopsy after liver and spleen puncture for diagnosis of unexplained liver and spleen enlargement or suspected tumors. This is very helpful in determining the nature of neonatal liver enlargement, such as primary liver tumors or secondary tumors, distinguishing neonatal hepatitis syndrome from intrahepatic bile duct atresia, and so on.

　　1. B-ultrasound Examination

　　Ultrasound examination can help determine the size of the liver and spleen, especially necessary when the abdominal distension of children is serious and the liver and spleen are unclear. The application of ultrasound scanning can observe the position, shape, size of the liver, check the movement of the diaphragm, show the relationship between the liver and adjacent organs, and ultrasound examination can also provide etiological information, such as neonatal hepatitis when the basic wave pattern is dense microwave and dense micro wave; neonatal liver cancer is丛状波, dull wave and outwave attenuation; liver abscess can be seen liquid level segment, etc., B-ultrasound is very useful for distinguishing liver cysts, liver abscesses, and liver tumors and other liver space-occupying lesions, liver cirrhosis, fatty liver, and congestive liver can also be distinguished under ultrasound images, and gallbladder ultrasound examination can detect the existence of choledochal cyst.

　　Ultrasound examination can observe the position, shape, and size of the spleen, and the neonatal degree of cooperation, abdominal muscle tension, and ascites, etc., have little impact on it. Ultrasound examination is more sensitive and accurate than palpation in judging splenomegaly, and can also show internal structure, and can distinguish from congestive splenomegaly, lymph granuloma, primary spleen tumor, and subcapsular hematoma of the spleen, etc.

　　2. Radionuclide Imaging

　　Radionuclide imaging can also be used for the diagnosis of liver and spleen enlargement. Colloid 99mTc is used to understand the position, shape, size of the liver, and to detect whether there are space-occupying lesions in the liver. The spleen can be imaged simultaneously with the liver. When the spleen function is normal, the spleen shadow is lighter than that of the right lobe of the liver. When the spleen function is hyperactive, the spleen shadow can be denser than that of the liver shadow. It is also very useful for the diagnosis of space-occupying lesions and infiltrative lesions in the spleen.

　　1. Pay attention to light food in diet, and it is better to eat easily digestible foods such as vegetable porridge and noodles soup.
　　2. You can eat more fresh fruits and vegetables to ensure the intake of vitamins.
　　3. Provide liquid or semi-liquid foods such as various porridge, rice soup, etc.

　　1. Treatment

　　The treatment of neonatal liver and spleen enlargement varies depending on the primary disease, see the relevant content of each disease.

　　2. Prognosis

　　The enlargement of the neonatal liver and spleen is often reduced with the improvement of the primary disease and returns to normal with the cure of the primary disease. The size of the liver and spleen is often one of the indicators for observing the course of the disease. If the liver and spleen continue to enlarge and become harder, it often reflects a poor prognosis.