Neonatal persistent cholestatic jaundice
Introduction
Introduction to neonatal persistent cholestasis jaundice Infant cholestasis refers to a persistent reduction in bile excretion in infants due to hepatocyte damage and/or intrahepatic and external bile duct obstruction. The main clinical manifestations are high-binding bilirubinemia [ie, blood direct bilirubin 25.5micro; mol/L (1.5mg/dl), or direct and total bile ratio 20%], hepatosplenomegaly, abnormal liver function And fat malabsorption, some people call this disease baby hepatitis syndrome, because the cases included, do not all have pathological changes in hepatitis, so this name deserves further discussion. basic knowledge The proportion of illness: 0.005% Susceptible people: infants and young children Mode of infection: non-infectious Complications: cirrhosis, hypersplenism, pneumonia, sepsis
Cause
Neonatal prolonged cholestasis jaundice
Viral hepatitis (30%):
So far, there is no report of intrauterine infection caused by hepatitis A virus (HAV) in the placenta. China is a high incidence area of hepatitis. Most of the pregnant mothers have hepatitis A IgG antibody, which makes the baby passively immunized, so the chance of developing hepatitis A in infants under 3 months is very small. .
Hepatitis B (HBV) and HBsAg carriers can be transmitted by intrauterine and postpartum mother-to-child transmission, with the main route of infection, maternal and infant infection rate of 20% to 50%, mother HBeAg positive, infection rate Higher, but infected infants more than 3 months after birth, HBsAg began to turn positive, a small number of ALT with mild increase, HBV intrauterine infection rate is generally reported as 2.5% ~ 7.7%, but in recent years through the cord blood lymph Cell and/or serum HBV DNA assay confirmed that the intrauterine infection rate can be as high as 22%. HBV intrauterine infection, except for a few cases that have been reported to cause outbreaks of hepatitis, generally manifest as persistent or transient HBsAg, rarely causing bile The symptoms of siltation, HBV postpartum infection, the incidence of more than 3 months, so the incidence of cholestasis within 3 months, caused by HBV, is actually rare, especially female HBsAg and other negative.
In recent years, it has been confirmed that hepatitis C virus can be transmitted through mother-to-child transmission, and infants often develop from 3 to 12 weeks after birth.
Giant cell inclusion body virus (CMV) (25%):
CMV in China is the most important cause of cholestasis. It is generally reported to be about 25%. In recent years, polymerase-linked reaction (PCR) technology has been used to detect CMV DNA in urine of infant hepatitis patients. The positive rate is as high as 67% to 78.3%. The healthy children's control was only 14.7% to 36.8% (P<0.01).
The positive rate of CMV IgG antibody in pregnant women in China is 94.6%, but the incidence of cholestasis in infants is far less than this. This is because the mother CMV IgG antibody can pass through the placenta, so that the baby can be protected, and the baby is born with intrauterine infection after CMV. More than 90% are asymptomatic, some have symptoms of cholestasis, and the prognosis is good. CMV postpartum infections cause respiratory symptoms and rarely cause cholestasis.
Toxoplasmosis (25%):
The infection rate of the domestic population varies greatly, ranging from 1.4% to 38.6%, generally <8%. Rural areas are significantly higher than cities. Congenital Toxoplasma infection can cause miscarriage, premature birth and stillbirth. Survival cases can be recessive infections, and symptoms can also occur. The latter mainly manifests as central nervous system and ocular lesions. Some children can cause cholestasis. Some people have detected serum Toxoplasma antibodies in 75 cases of cholestasis. 9.3%, while the normal control group was 2.5%, indicating that Toxoplasma gondii is one of the pathogens of infant hepatitis. Because of the effective treatment of this disease, timely diagnosis is very important. The diagnosis of congenital infection can be positive according to the serum Toxoplasma gondii IgM antibody (such as indirect fluorescent antibody). Tests) or Toxoplasma gondii (including antigen or DNA positive) were detected in body fluids, and the disease was effective against sulfa, pyrimethamine, spiramycin and clindamycin.
High intravenous nutrition (10%):
Neonates use intravenous nutrition for more than 2 weeks, 20% to 35% of children can have cholestasis, premature infants can reach 30% to 50%, has been confirmed mainly related to amino acids, discontinued intravenous nutrition for 1 to 4 months, liver function and Liver pathological changes can generally be restored.
5.1-antitrypsin (1-AT) deficiency
1-AT is a glycoprotein synthesized by the liver and has a strong protease inhibitory effect. The exact mechanism of liver damage caused by deficiency is unknown.
The disease belongs to autosomal codominant inheritance. According to gene gel electrophoresis, there are at least 24 protein inhibitor (Pi) alleles in the population. Normal people are PiMM. The cholestasis caused by 1-AT deficiency is PiZZ. Type, Westerners account for about 1/1600 to 2,000 live births of PiZZ, of which only 11% to 20% have cholestasis, 7% have only abnormal liver function, and the rest are asymptomatic. In European and American literature, cholestasis is caused by 1AT deficiency. It accounts for 5% to 18%. There are a few cases in Japan. In recent years, more than 99% of people in China have been PiMM-type, and PiZZ has not been found.
Zellweger syndrome (5%)
Also known as brain-liver-renal syndrome, it is characterized by low intelligence, special face (prominent forehead, large forehead, long eye distance, internal hemorrhoids, severe muscle tension, multiple skeletal deformities, such as cartilage calcification, femoral condyle Detachment, renal cortical cysts are asymptomatic, this disease is caused by abnormal bile acid metabolism, and the sick children die within 6 months.
Prevention
Neonatal prolonged cholestatic jaundice prevention
HBV-induced neonatal hepatitis is diverse. If no measures are taken (such as using HBIG or vaccine) to prevent infection, 70% to 90% of HBsAg-positive mothers will receive HBV infection at birth, and most infected infants will become HBV. Carriers, usually for the rest of their lives, in order to prevent perinatal transmission, all HBsAg-positive mothers (regardless of their HBeAg status) should receive hepatitis B immunoglobulin and hepatitis B vaccination within the first 24 hours after birth. Vaccination was repeated at 1 month and 6 months.
Complication
Neonatal persistent cholestasis jaundice complications Complications, cirrhosis, spleen hyperfunction, pneumonia
Common complications include cirrhosis, liver failure, neonatal hemorrhage, multiple organ dysfunction, portal hypertension syndrome, itching, hypersplenism, pneumonia, sepsis, etc.
Symptom
Neonatal persistent cholestatic jaundice symptoms Common symptoms Astragalus fetal growth retardation Loss of appetite, edema, bloating, infant feeding difficulties, no bile, fecal papules, leukocytosis, drowsiness
General symptoms
Clinical features of signs of cholestasis with signs of intrauterine growth disorders, premature delivery, feeding difficulties, vomiting, slow growth and partial or intermittent biliary deficiencies (white stool), jaundice can be seen in the neonatal period, but often delayed to 2 ~3 weeks, urine color is dark, and diapers will be dyed. The stool is often pale yellow, light brownish yellow, gray or white. The exudation of bilirubin products around the intestinal mucosa often makes the stool slightly yellow, and the liver is very common. It has a different degree of hard feeling; in the future, splenomegaly may occur, and younger children may have itching, clubbing (toe), yellow tumor and rickets, and can hear heart murmur in the entire anterior or posterior region. Increased cardiovascular output or bronchial artery shunt, by 2 to 6 months, the growth curve reflects little weight gain, may be the result of fat malabsorption and increased oxygen consumption, late complications such as ascites and hemorrhage, generally In 2 weeks after birth, jaundice can gradually increase, but it can be as late as 2 to 3 months. The appetite is often low, the sucking reflex is weakened, lethargy, vomiting, and sputum, papules or sputum may occur. Embodiment, slow growth may only symptom, even visible liver failure, thrombocytopenia, edema (edema non-hemolytic) and neonatal bleeding disorders.
2. Neonatal hepatitis B virus infection
HBV-induced neonatal hepatitis is diverse. If no measures are taken (such as using HBIG or vaccine) to prevent infection, 70% to 90% of HBsAg-positive mothers will receive HBV infection at birth, and most infected infants will become HBV. Carriers, usually lasting for life, are rarely reported for subacute severe hepatitis, especially those infected with infected blood at the time of delivery or postpartum, but can also occur in cases of viral infections transmitted by mothers. Such cases are mainly clinically manifested as Progressive jaundice, coma, liver enlargement and abnormal blood coagulation, followed by respiration, circulatory and renal failure, histologically, large liver hepatic necrosis, reticular destruction, microinflammation, occasional pseudolobular formation, According to reports, a small number of survivors have reconstructed the liver structure and are close to normal.
In a few severe cases, focal hepatocyte necrosis with mild portal inflammatory response is seen, cholestasis is intracellular and tubular, and chronic persistent and chronic active hepatitis can persist for several years with persistence Sexual antigenemia (HBsAg) and transaminase are slightly elevated, and chronic active hepatitis can develop into cirrhosis within 1 to 2 years.
3. Neonatal bacterial hepatitis
Most of the neonatal liver bacterial infections are caused by the mother's birth canal or cervical infection causing amnion, which is invaded by the placenta. The onset is urgent, usually 40 to 72 hours after birth, and there is sepsis, and common shock, insufficient In 25% of cases, jaundice can occur, but early appearance and mixed jaundice, rapid liver enlargement, histological changes to extensive hepatitis, with or without small or huge abscesses, the most common pathogen is Escherichia coli Listeria monocytogenes and group B streptococci, rare tuberculin tuberculosis, isolated liver abscess caused by Escherichia coli and Staphylococcus aureus are often associated with umbilical inflammation and umbilical vein cannulation, bacterial hepatitis and newborns Liver abscess requires high-dose specific antibiotics. In a few cases, surgical drainage is required, often with death, but survivors do not have the sequela of long-term liver disease.
4. Giant cell inclusion body virus (CMV)
CMV infection in China is quite common, mainly causing respiratory symptoms and causing cholestasis. The diagnosis is based on the detection of CMV (including CMV antigen or DNA) from the urine or secretion of the sick child, or positive for serum CMV IgM, positive for CMV IgG antibody. Can not be diagnosed as CMV infection, because it can come from the mother, unless the double serum titer is increased by 4 times, or the titer is higher than the mother at 2 months.
5.1-antitrypsin (1-AT) deficiency
The disease is autosomal dominant, can occur cholestasis, more than 3 months after birth, liver function abnormalities, jaundice can naturally disappear in about 8 months, but most of them develop cirrhosis after 5 years old.
6. Neonatal jaundice with urinary tract infection
Infected babies are usually male, usually jaundice in the second to fourth week after birth, manifested as lethargy, fever, loss of appetite, jaundice and liver, except for mixed hyperbilirubinemia, other liver function changes are not obvious There may be leukocytosis, bacterial culture can confirm the original infection, the mechanism of liver function damage is unclear, it was thought to be related to the toxic effects and inflammatory reaction of bacterial products (endotoxin), and the treatment of infection can make cholestasis disappear quickly and there is no The sequelae of the liver, metabolic liver disease can exist at the same time as the urinary tract infection of Gram-negative bacteria, and should be noted.
7. Intrahepatic bile duct dysplasia
It is characterized by persistent jaundice, which can occur without bile feces, blood bile acid, cholesterol is significantly increased, the latter can be as high as 14.3 ~ 26.0mmol / L (550 ~ 1000mg / dl), skin itching, yellow tumor, ALT mild Elevated, alkaline phosphatase increased significantly, liver histological changes to the interlobular bile duct is significantly rare, the disease can be divided into two types:
(1) Alagille-Watson syndrome: also known as arterio-hepatic dysplasia, which is characterized by cholestasis, and has a special face (forelogy width, chin tip, eye depression, wide eye distance), youth with eyes The corneal arch, the vertebrae often have deformities, such as the vertebral vertebrae, the semi-vertebrae and the anterior arch are not fused. The most common pulmonary abnormalities are pulmonary artery stenosis. Occasionally, the aorta is narrowed. This type has a good prognosis and rarely develops into a liver. hardening.
(2) Asymptomatic type: no such performance, clinical difficulty and biliary atresia and special infant hepatitis distinguish, poor prognosis, progressive development of cirrhosis, diagnosis mainly depends on liver biopsy.
8. Bile sticky syndrome (inspissated bilesyndrome)
The bile duct is blocked by sticky mucus or bile, which usually occurs after severe hemolysis in neonates. Symptoms are difficult to distinguish from biliary atresia. Some children can be relieved naturally, or relieved after treatment with phenobarbital. Some require surgical lavage. treatment.
9. Congenital biliary atresia
The child is generally good at birth, normal weight, meconium is normal, persistent jaundice appears 1 to 2 weeks after birth, stool color is pale, even gray, urine dark, bilirubin positive, the disease more women than men, May be associated with multiple splenic syndrome, abdominal visceral translocation, poor bowel rotation, right heart and intra-abdominal vascular malformation, when the jaundice is heavier, the feces of the sick child can be pale yellow, but if the stool color is very yellow or green, then Except for this disease, the liver progressively increases, and often involves the left and right sides of the liver; after a few weeks, most of the sick children gradually increase spleen, blood alkaline phosphatase, 5-nucleotidase and low-density lipoprotein (lipoprotein X, LP-X) increased significantly, B-ultrasound can be found in the absence of gallbladder or dysplasia.
10. Other extrahepatic bile duct diseases
Common bile duct cysts can sometimes cause swelling and mass in the right upper abdomen. Smaller babies can cause complete obstruction of the common bile duct. Clinical manifestations like biliary atresia, B-ultrasound is easy to identify, cholelithiasis is rare, long-term intravenous high-nutrition infants, and newborns Children with hemolytic disease, the incidence of increased, ultrasound examination is helpful for diagnosis, cholestasis with progressive bloating, umbilical and groin skin yellow staining, should think of spontaneous perforation of the common bile duct, ascites with bile support diagnosis.
Children with hereditary metabolic diseases are often accompanied by various deformities. Vertebral arch defects include vertebral or anterior vertebral arch fusion (butterfly deformation) and reduced distance between the pedicles of the thoracolumbar spine. Eye deformities (congenital corneal circumference) Turbidity and kidney malformations (kidney dysplasia, tubular dilatation, single kidney and hematuria), growth retardation, IQ (IQ) usually low, hypogonadal hypoplasia, small penis, weak and sharp, may have nervous system abnormalities (reflex disappearance, movement disorder, ophthalmoplegia), accompanied by multiple splenic syndrome, abdominal visceral translocation, poor bowel rotation, right heart and intra-abdominal vascular malformation.
Neonatal lupus erythematosus can be associated with cytomegalovirus; thrombocytopenia, rash or congenital heart block is a common occurrence.
Examine
Examination of neonatal persistent cholestasis jaundice
1. Blood examination: Blood cell count shows neutropenia and thrombocytopenia.
2. Blood biochemical examination : high binding bilirubinemia (34.2 ~ 256.5mol / L), serum alkaline phosphatase, -glutamate transferase and cholesterol are significantly increased, transaminase can be Increased, other liver proteins are usually normal, may have mild hemolysis, prolonged clotting time, mild acidosis and increased cord blood IgM suggesting congenital infection, intrahepatic bile duct dysplasia, bile acid, cholesterol is significantly increased, the latter It can be as high as 14.3 to 26.0 mmol/L (550 to 1000 mg/dl), alanine aminotransferase (ALT) is slightly elevated, and alkaline phosphatase is highly marked.
Dynamic observation of blood bilirubin also helps to identify, such as gradual decline, should be considered for hepatitis, blood alpha-fetoprotein is significantly increased in infant hepatitis, and blood alkaline phosphatase, 5-nucleotidase and low density in biliary atresia Lipoprotein X (LP-X) increased significantly, but there was a small overlap between the two.
3. Neonatal hepatitis B virus infection : In addition to blood, hepatitis B virus can be present in most body fluids, including milk, but does not appear in the stool, chronic HBsAg carrying mother if:
(1) HBeAg positive and HBeAb negative.
(2) Serum-specific hepatitis B virus DNA polymerase with detectable levels.
(3) There is a higher level of serum HBcAb, and the fetus and infant are at the highest risk of infection, and a few survivors have a liver structure reconstruction that is close to normal.
4. Etiology : Nasopharyngeal lavage, urine, stool and cerebrospinal fluid can be used to culture the virus, and special serological tests can help diagnose (TORCH titer).
5.1-antitrypsin (1-AT) deficiency : laboratory test serum protein electrophoresis 1 globulin decreased significantly, trypsin inhibition capacity decreased, immunodiffusion assay for serum 1-AT concentration was reduced by more than 50%, liver pathology Sliced hepatocytes have amylase-resistant PAS-positive bodies, but the most reliable is the Pi genotype assay.
6. Liver histology : liver histological examination obtained by percutaneous liver puncture can distinguish intrahepatic or extrahepatic cholestasis, but can not distinguish the specific infectious agents in liver tissue, except cytomegalovirus can be in hepatocyte bile duct epithelium The formation of inclusion bodies in the cytoplasm of the cells, as well as the intranuclear eosinophilic inclusion bodies of herpesviruses, are commonly characterized by focal necrosis of lobular structural disorders, formation of multinucleated giant cells, vacuolation of hepatocytes, disappearance of cord-like structures, and hepatocytes Internal and tubular cholestasis can be persistent, portal changes are not obvious, but new nodular hyperplasia and slight fibrosis can occur.
The hepatic histological changes of intrahepatic biliary dysplasia are markedly rare in the interlobular bile duct. The pathological features of biliary atresia are: bile duct hyperplasia, bilirubin deposition in early hepatocytes, bile thrombosis in bile ducts, and inflammatory reaction in portal vein. There is fibrosis between the lobules, the structure of the hepatic lobule is basically intact, and the structure of the hepatic lobule of the infant hepatitis is deformed, the inflammatory cell infiltration is obvious, and the focal hepatocyte necrosis is present, and the bile duct lesion is relatively mild.
Liver biopsies can distinguish between cholestasis and bile duct atresia in more than 90% of cases.
7. Genetic testing : Some genetic hereditary congenital metabolic defects, parents should have a genetic test as soon as possible.
8. B-ultrasound : Ultrasound examination of the bile duct system can be used to determine the presence of choledochal cysts, such as the absence of gallbladder or dysplasia, should be considered for biliary atresia, often accompanied by multiple splenic syndrome, abdominal internal organs translocation, intestinal rotation Poor, right heart and intra-abdominal vascular malformations, cardiovascular abnormalities include: peripheral and valvular pulmonary vascular stenosis (most common), atrial septal defect, aortic coarctation and tetralogy of Fallot.
9. X-ray examination : biliary atresia routine X-ray chest X-ray can show the deformity of multiple splenic syndrome. If necessary, long bone X-ray examination can be found in the tibia, femur, and metaphysis of the humerus.
10. CT and MRI examination : intracranial calcification can be found by CT and MRI if necessary.
11. Determination of duodenal drainage fluid : After duodenal intubation, the duodenal juice is collected once every 2 hours until bile is found or reaches 24 hours. 10-15 ml of 25% magnesium sulfate can be injected during the drainage process to promote Bile is fully excreted. If bile is found, biliary atresia can be ruled out. There is no bile discharge at 24h, and biliary atresia may be very large. Recently, duodenal fluid bilirubin quantification and bile acid identification have been performed. Results: 21 cases in hepatitis group, 20 In the case of duodenal fluid bilirubin > 17.1 mol / L (1 mg / dl), cholic acid was positive. In 17 cases of biliary atresia, bilirubin was <17.1mol/L (1mg/dl), and cholic acid was negative.
12. Nuclide examination : The hepatobiliary scan revealed a decrease in the function of the liver to remove circulating nuclides from the blood and excreted into the intestinal lumen.
(1) 131I rose bengal excretion test: After intravenous administration, the feces collected for 48 hours are collected. If the radionuclide in the feces is less than 10% of the injected amount, the biliary atresia may be large. The disadvantage is that urine should be collected when collecting feces. Completely separate, there are often practical difficulties.
(2) 99mTc-labeled iminodiacetic acid derivative excretion test: 99mTc can be taken up by liver cells when biliary atresia is absent, but no nuclides are discharged to the intestine, and special hepatitis liver uptake nuclides are delayed. Finally, it can be discharged to the biliary tract and the intestine. Although the excretion is less than normal, the advantage is that the radiation dose is small, and the resolution of gamma imaging is high. The disadvantage is that the half-life is short, and the image cannot be formed after 24 hours, which can delay the emission of a small number of discharged nuclide. Infant hepatitis, a false negative.
In order to improve the correct rate of radionuclide examination, phenobarbital (5mg/kg per day) can be taken for 3 to 5 days before the examination. If the biliary tract is smooth, the nuclides can be excreted from the biliary tract to reduce false negative results.
Diagnosis
Diagnosis and differential diagnosis of persistent cholestasis of jaundice in neonates
According to clinical manifestations and laboratory tests, the diagnosis can be confirmed, but the cause diagnosis should be confirmed at the same time. The medical history should be consulted, including the mother's pregnancy history, production history, feeding history, etc. Detailed medical examination, laboratory examination and auxiliary examination should be selected, and the cause diagnosis should be confirmed as early as possible. .
Differential diagnosis
1. Perinatal and neonatal hepatitis caused by infection
Intrahepatic infectious cholestasis and hereditary, metabolic causes (congenital abnormalities) must be carefully distinguished because their clinical manifestations are very similar, and galactosemia, congenital fructose intolerance and tyrosinemia should be promptly performed. Check, because special diet therapy can be implemented, should also consider 1-antitrypsin deficiency, cystic fibrosis and neonatal iron storage abnormalities, when considering Alagille or Zellweger syndrome, special physical characteristics are helpful for diagnosis Unless the bile duct is spontaneously perforated, the children with extrahepatic cholestasis generally perform well; the stool is usually completely white, the liver is enlarged and hard, and the histology is shown in Table 1. Premature infants, children below the gestational age are considered as infants. hepatitis.
2. Intrahepatic or extrahepatic cholestasis.
3. "Bile Concentration Syndrome"
This condition is due to the fact that some neonates with hemolytic disease (Rh, ABO) and some infants who receive total venous nutrition have their bile accumulated in the bile duct or medium-sized bile duct. The same mechanism can cause the obstruction of the common bile duct. Hypoxic-reperfusion injury in the absence of Rh blood group can also cause cholestasis. In severe hemolysis, cholestasis can be complete with white stool, and bilirubin levels can be as high as 40 mg/dl (684 mol/L). Direct reaction, if bile concentration occurs in the extrahepatic bile duct, it is more difficult to distinguish from bile duct atresia, feasible choleretic (cholestamide, phenobarbital, ursolic acid deoxycholic acid) test treatment, once the stool color turns For normal or 99mTc-DIDA scans to see bile excretion into the duodenum, it can be determined that the extrahepatic bile duct is open, and during the transition of stool color to normal, sometimes parents complain that small bile-colored plugs are found in the feces of the child. Although most cases need to recover slowly within 2 to 6 months, further examination (ultrasound, DIDA scan, liver biopsy) is required for complete cholestasis lasting more than 2 weeks. Laparotomy extrahepatic bile duct, common bile duct in a concentrated rinse bile obstruction substance removal if necessary.
When suspected to be idiopathic neonatal hepatitis (no infection, metabolic and poisoning causes), bile ducts should be confirmed rather than extrahepatic "surgical" disease, which may be helpful in DIDA scans and ultrasound examinations. Some people used a gut test during DIDA scan to prove that there is no occlusion in the biliary tract. The liver biopsy often has diagnostic significance, especially for infants older than 6-8 weeks. However, biopsy may be less than 4 weeks. The baby is misleading. If the opening of the biliary tree is not detected, the liver biopsy does not have a typical diagnosis of the disease, or persistent total cholestasis (white stool), suggesting that an experienced surgeon is needed. Small laparotomy and intraoperative cholangiography, occasionally showing a small but unclosed extrahepatic bile duct tree (hypopy), it is likely to be the result of reduced bile flow, not a cause, no need to rebuild hypoplasia Bile duct.
4. Extrahepatic bile duct atresia
The clinical distinction between biliary atresia and infantile hepatitis is sometimes difficult, but it is very important. Infant hepatitis mistakenly believes that biliary atresia and anesthesia, surgery will undoubtedly cause harm to the sick child; and biliary atresia within 2 months after surgery The success rate can reach 80%. After 3 months, the majority of the patients have failed. So far, no laboratory examination can completely separate the biliary atresia from the infant hepatitis. The diagnosis depends on the medical history, physical examination and laboratory examination. Comprehensive analysis, there must be dynamic observation of fashion, for example, the disease is more women than men, hepatitis is the opposite, accompanied by multiple malformations, the disease is very likely, premature infants, lower than the gestational age children are considered to be infant hepatitis, If the color of the stool is very yellow or green, the disease may be excluded, hepatitis and metabolic abnormalities, hepatosplenomegaly in the early neonatal period, and the liver is lighter in the early stage of the disease, and then progressively increases, and often involves the left and right sides of the liver. After a few weeks, the spleen of most sick children gradually increased. Dynamic observation of blood bilirubin also helped the identification. If it gradually decreased, it should be considered as hepatitis. The blood alpha-fetoprotein was obviously increased in infant hepatitis. And biliary atresia, blood alkaline phosphatase, 5-nucleotidase and low-density lipoprotein (lipoprotein X, LP-X) increased significantly, although there is a small overlap between the two, B-ultrasound found if the gallbladder is absent or Dysplasia should be considered as the disease. When the two are difficult to distinguish, duodenal drainage fluid, radionuclide examination, liver biopsy, etc. can be used, and the correct rate can reach about 95%.
In the early stage of the disease, treatment with phenobarbital [3 ~ 5mg / (kg · d), 5 ~ 7 days] DIDA secretion studies can distinguish between cholestasis is intrahepatic or extrahepatic, although persistent bile duct atresia Increased levels of serum gamma-glutamate transpeptidase or alkaline phosphatase, as well as prolonged prothrombin time, but these changes are also severe in neonatal hepatitis, alpha 1-antitrypsin deficiency and bile duct deficiency It has been reported that, in addition, these tests can not distinguish the obstruction site of the extrahepatic system. Generally, transaminase can only be slightly increased when the bile duct is atresia, serum protein and coagulation factors will not be affected at the beginning of the disease, and conventional X-ray chest X-ray can show more Malformations of the spleen syndrome, ultrasound examination of the biliary system can be used to determine the presence of a choledochal cyst.
The main problem with diagnosis is that the disease is difficult to distinguish from neonatal hepatitis, bile duct deficiency, common bile duct cyst or original biliary obstruction (calculus, bile thrombus), although spontaneous extrahepatic bile duct perforation leads to jaundice and white stool. Infants are often severely ill due to chemical peritonitis caused by biliary ascites, but no liver is found.
If bile duct atresia cannot be ruled out 60 days before birth, a surgical examination is required. The laparotomy should include a biopsy of the liver. If the gallbladder is present, a cholangiography is also performed. Yellow bile is present in the gallbladder, indicating that the extrahepatic bile duct system is near. No closure at the end, radiography to see the contrast agent in the duodenum, can rule out the distal extrahepatic bile duct obstruction.
