cirrhosis of the liver in children
Introduction
Introduction to pediatric cirrhosis Hepatic cirrhosis (hepaticsclerosis) is a chronic diffuse progressive liver disease. It has many causes, which can be caused by diseases of the liver itself, or it can be a part of systemic diseases. The clinical symptoms vary greatly, showing different degrees of liver dysfunction and Portal hypertension. Pathological changes are mainly fibrosis of the liver and formation of pseudolobules. basic knowledge Sickness ratio: 0.0001% Susceptible people: children Mode of infection: non-infectious Complications: upper gastrointestinal bleeding, blood in the stool, hepatic encephalopathy, hepatorenal syndrome
Cause
Causes of cirrhosis in children
(1) Causes of the disease
Infection is the main cause of cirrhosis in children. Congenital biliary obstruction and congenital metabolic defects and genetic diseases are also common causes of cirrhosis in children:
Infection
Viral hepatitis (hepatitis B, hepatitis C, hepatitis D, hepatitis G, cytomegalovirus hepatitis), rubella virus, herpes simplex virus, varicella virus; congenital syphilis; parasitic disease (Schistosoma japonicum, liver fluke , toxoplasmosis) and so on.
2. Congenital genetic abnormalities
Wilsons disease, hepatic glycogen accumulation (type IV), galactosemia, alpha 1 antitrypsin deficiency, congenital tyrosinosis, hereditary fructose intolerance Disease, Fanconi syndrome, mucoviscidosis, -lipoproteinemia, hereditary hemorrhagic telangiectasia (Osler-Weber disease), hemochromatosis (hemochromatosis) [Idiopathic Hemochromatosis (IHC), neonatal hemochromatosis (NHC), secondary hemochromatosis], peroxisomal disorders (PD) [brain-liver-renal syndrome] (also known as Zellwegar syndrome), bile duct dysplasia with biliary hypoplasia with trihydroxy coprostanic acidemia (THCA).
3. Acquired metabolic abnormalities
Alcoholism, nutritional, toxic (neonatal infections, toxins, drugs).
4. Immune abnormalities
Home immune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) in children.
5. Liver and biliary cystic diseases
(cystic diseases of the biliary tract and liver) congenital hepatic fibrosis (CHF), congenital intrahepatic bile duct dilatation (Caroli disease), congenital choledochal cyst (congenital choledochal cyst).
6. Congestive cirrhosis
Budd-Chiari syndrome, chronic constrictive pericarditis.
7. Hidden cirrhosis
Indian childhood cirrhosis (ICC), familial cirrhosis (also known as Alper disease).
(two) pathogenesis
Pathogenesis
(1) Infection: After infection with the liver due to viruses, bacteria, fungi and parasites, it gradually progresses to cirrhosis. In children, it is common for hepatitis B or C (Table 1); after severe acute hepatitis, necrotic liver Hardening; neonatal due to imperfect immune function, sub-clinical process after infection with hepatitis, easy to develop into chronic hepatitis or cirrhosis; cytomegalovirus, rubella virus, herpes simplex virus intrauterine infection, congenital syphilis are easy to invade the liver, develop into Chronic hepatitis, cirrhosis; advanced schistosomiasis of parasitic diseases, clonorchiasis, toxoplasmosis and malaria; bacterial toxic hepatitis rarely develops into cirrhosis, but staphylococcus aureus sepsis or infants under 1 year old Malnutrition or viral hepatitis can easily lead to cirrhosis.
(2) Hereditary or congenital metabolic defects: mainly due to defects in certain enzymes, esters, amino acids, fats or trace elements of metals such as copper, iron, etc., which accumulate liver, cause liver cirrhosis, and often have hepatolenticular degeneration, 1 resistance Trypsin deficiency, tyrosinemia, galactosemia, glycogen accumulation disease type IV, hereditary fructose intolerance, Fanconi syndrome, Gaucher disease, etc.; hemosiderin and hemochromatosis Late progression of (hemachromatosis) is cirrhosis, but rare; Zellweger syndrome (brain, liver and kidney syndrome) is a rare autosomal recessive disorder with peroxisome developmental defects or peroxidase The loss of an enzyme in the body is caused by cirrhosis, renal cyst, muscle weakness, convulsions, special face and glaucoma, cataract and other congenital eye diseases, accompanied by abnormal iron metabolism.
(3) Toxic and drug poisoning: such as arsenic, phosphorus, strontium, chloroform, isoniazid, cinchon, poisonous phlegm and other poisoning.
(4) Immune abnormalities: French scholar Vajro et al. conducted a prospective study of 92 cases of chronic hepatitis in children, including 46 cases of hepatitis B and autoimmune hepatitis. The former developed 32% of cirrhosis, while the latter was 89%. In the post-hepatitic cirrhosis of children in France, autoimmune hepatitis accounts for the first cause of etiology.
(5) Liver and biliary cystic disease: a group of congenital liver fibrosis and biliary cystic disease, congenital liver fibrosis (CHF) is an autosomal recessive genetic disease, the cause is unknown, about 40% are siblings The 302 Hospital of the People's Liberation Army admitted to the brothers and sisters as CHF. The diagnosis was mainly based on liver biopsy. The genetic pattern of Caroli disease was the same as that of CHF. The diagnosis was confirmed by B-ultrasound, CT. If necessary, endoscopic retrograde biliary, pancreatic ductography (ERCP), congenital choledochal The cyst is detailed in the biliary tract disease.
(6) Congestive cirrhosis: due to hepatic venous malformation, thrombosis, chronic congestive heart failure, chronic constrictive pericarditis caused by long-term liver congestion leading to cirrhosis.
(7) Other reasons: It is not uncommon to find the cause. The cirrhosis of Indian children mainly occurs in India and its neighboring areas. It is also reported in West Africa and Central America. It is more than 1 to 3 years old. The hepatomegaly is the initial. Performance, other fever, anorexia and jaundice, most of them rapidly progress to cirrhosis, liver failure, the cause of this disease is unclear.
2. Pathological changes Cirrhosis is characterized by connective tissue hyperplasia, hepatocyte degeneration of more hepatocytes or hepatic lobules, after necrosis, fibrous tissue hyperplasia replaces normal liver tissue, if the lesion involves several small leaflets, the lobular structure is disordered, and the stent collapses. Due to the contraction of fibrous tissue and the extrusion of regenerative nodules, the intrahepatic blood flow disorder can cause portal hypertension, and the lesions continue to form collateral circulation, which is generally divided into portal vein, post-necrotic and biliary liver. Hardened type III, often mixed in the late stage.
(1) portal cirrhosis (portal cirrhosis): children are less than adults, due to chronic hepatitis or liver congestion, early liver enlargement, later shrinkage, hard, irregular surface granules or nodules, histology visible Degeneration or necrosis of hepatocytes, normal structural destruction of hepatic lobule, regenerative nodules of varying sizes, hyperplasia of fibrous tissue around the nodules and portal area, widespread distribution, varying degrees of inflammatory cell infiltration and bile ducts between fibrous tissues Hyperplasia, the hepatic vascular network is compressed and displaced.
(2) Post-necrotic cirrhosis: more than a few months after acute severe hepatitis or toxic hepatitis, the liver shrinks, the quality becomes hard, and the liver parenchyma is mostly massive necrosis, involving one or several leaflets. The stent collapses, the connective tissue proliferates, and the fibrous bundle is wide. The hepatocytes adjacent to the necrotic area regenerate to form nodules, which vary in size. Normal hepatic lobules often exist in larger nodules, and inflammation in fibrous tissue Cell infiltration and bile duct hyperplasia were more obvious.
(3) biliary cirrhosis (biliary cirrhosis): in children mostly secondary, the pathological changes are mainly obvious hepatocytes and small bile duct cholestasis, histology see connective tissue hyperplasia in the portal area, which may have new bile duct Inflammatory cell infiltration, intrahepatic small bile duct dilatation, cholestasis and bile thrombosis, hyperplastic connective tissue grows between the interlobular and lobular, no obvious regeneration of hepatocytes.
Prevention
Pediatric cirrhosis prevention
Infection is the main cause of cirrhosis in children, strengthen pregnancy care, prevent various infectious diseases during pregnancy, especially hepatitis B virus, cytomegalovirus, rubella virus, herpes simplex virus, congenital syphilis and other infections, so as not to cause liver damage in children. In the occurrence of this disease, there are hundreds of drugs and poisons that can cause damage to liver cells, avoid long-term, large-scale contact, in order to prevent the occurrence of this disease, and should be properly fed, balanced diet, and various vaccination work for children after birth. , develop good hygiene habits, prevent and cure various chronic inflammatory bowel diseases and so on.
Complication
Pediatric cirrhosis complications Complications upper gastrointestinal bleeding, hepatic encephalopathy, hepatorenal syndrome
1. Upper gastrointestinal bleeding due to portal hypertension with varicose veins and abnormal blood coagulation mechanism, children with hematemesis, blood in the stool, gastrointestinal bleeding is one of the common causes of death, easy to induce hepatic encephalopathy.
2. Hepatic encephalopathy.
3. Secondary infections can mainly occur as bacterial infections or fungal infections, especially in the biliary system.
4. Portal vein thrombosis is rare in children, thrombosis is slowly formed, clinical symptoms are not obvious, if it happens suddenly, there is severe abdominal pain, blood in the stool, and even shock.
5. Hepatorenal syndrome occurs in advanced cirrhosis, liver failure, renal blood flow reduction, and hypokalemia, secondary renal failure, that is, hepatorenal syndrome, children with oliguria or no urine, Systemic edema, azotemia.
Symptom
Symptoms of cirrhosis in children Common symptoms: Abdominal ascites, hepatosplenomegaly, abdominal distension, spiders, liver, appetite, nausea, vomiting, weight loss, diarrhea
The clinical symptoms of cirrhosis are disparate. The compensation period may only have poor appetite, nausea, vomiting, abdominal distension, diarrhea and other gastrointestinal symptoms. The main signs are hepatomegaly, the spleen may also increase, and the clinical manifestation of decompensation is gradually obvious. Gradual weight loss, fatigue, portal hypertension, ascites, hypersplenism, bleeding tendency, liver palm, spider mites, but less infancy, children with dull complexion, often accompanied by anemia, late collateral The circulation is formed, and there is a varicose vein at the lower end of the esophagus and the fundus, which is the result of portal hypertension.
Portal cirrhosis
The onset process is slow and can be lurking for several years.
2. Post-necrotic cirrhosis
Once this type of cirrhosis occurs, it develops more rapidly and is not easy to compensate. It often causes liver failure due to secondary infection in a short period of time. If the progress is slower, the symptoms and signs of cirrhosis may gradually appear, but most of the sick children often Liver failure as the earliest manifestation, jaundice, gastrointestinal symptoms, easy to be misdiagnosed as acute hepatitis, Beijing Children's Hospital pathology reported 14 cases of post-necrotic cirrhosis, from symptoms to death mostly for 2 to 3 months, the shortest 11 days The longest 2 cases are half a year and 2 years and 3 months respectively. The speed of cirrhosis development is directly related to the age of children. The development of hepatitis in neonates and infants is faster. There are pathological data to prove that neonatal hepatitis develops into cirrhosis. More than one to six months of death.
3. Biliary cirrhosis
Can be divided into 2 types:
(1) Primary: There are special clinical manifestations, most of which have fever, fatigue, loss of appetite and upper abdominal discomfort, due to capillary cholangitis, early manifestation of obstructive jaundice, itchy skin, dark urine, diarrhea, stool becomes white Or light color, liver significantly increased, jaundice may continue to prolong bleeding may occur and skin jaundice, laboratory tests are mainly obstructive jaundice, the course of the disease is benign, up to several years, eventually due to cirrhosis, liver failure and Gastrointestinal hemorrhage worsens, the relationship between this disease and hepatitis virus is not clear, and may also be related to autoimmunity.
(2) secondary: in childhood, mainly for extrahepatic biliary obstruction secondary to cirrhosis, the clinical manifestations of obstructive jaundice, similar to the primary case, and the prognosis and treatment are completely different, so must be identified And avoid the wrong surgery.
Examine
Examination of cirrhosis in children
Decreased whole blood count will confirm the presence and extent of hypersplenism. Serum transaminase levels are an important basis for liver disease (although it may be normal in cirrhosis-compensated periods), cirrhosis-compensated serum bilirubin, ALT, coagulation The zymogen time can be normal, the slight decrease of serum albumin level is an early indication of liver function disorder; serum alkaline phosphatase, cholesterol and phospholipids increase, ALT moderate or slight increase; while albumin decreases, globulin increases, Inverted white/globulin ratio is a laboratory abnormality of active lesions in cirrhosis. Prolonged prothrombin time with cholestasis suggests advanced liver disease. At this time, appropriate examination should be performed to confirm the cause of chronic liver disease. Liver biopsy.
Ultrasound examination
Although B-mode ultrasound often cannot accurately measure the size of the liver or spleen, it can not diagnose the hardness of the liver, but it can evaluate the diameter of the portal vein and its degree of distortion, and help detect a small amount of ascites and abnormal kidney structure. Doppler ultrasonography, the formation of collateral vessels around the portal vein is a sign of extrahepatic portal vein obstruction. Under normal circumstances, it can be seen that breathing has a regulating effect on portal blood flow. If this sign disappears, it indicates portal hypertension, but this occurs. The sign can not absolutely rule out portal hypertension.
2. Esophageal X-ray barium angiography
Esophageal X-ray barium angiography can show the presence or absence of esophageal varices and its extent. The varicose veins are located 2/3 of the lower esophagus, indicating the shadow of the filling defect of the tincture, such as when the varicose veins are severe and wide. The degree of varicose veins, the degree of varicose veins has nothing to do with the cause or the age of the child. In general, thicker sputum is better than thin sputum, but sometimes it is necessary to use thick and thin sputum to make delicate esophagus Mucosal lines are more clearly shown. In adults, the sensitivity of esophageal varices detected by barium angiography is about 70%. In recent years, due to the wide application of endoscopy, this technique has rarely been used for the examination of esophageal varices. Moreover, the detection value of active gastrointestinal bleeding is extremely limited.
3. Gastroscopic examination
Gastroscopic examination can directly observe the presence or absence of esophageal varices and its extent. At present, endoscopic examination is feasible and safe for all pediatric age groups, and it is the most accurate and sensitive method for confirming the existence of esophageal varices. For children with upper gastrointestinal bleeding, once the condition is stable, a gastroscopy should be performed immediately to determine the location of the bleeding as soon as possible, the nature of the lesion and rapid hemostasis treatment. For patients with asymptomatic or stable period, gastroscopy can also be performed. In order to predict the risk of variceal bleeding and the timing of the choice of preventive sclerotherapy.
4. Selective superior mesenteric artery angiography
The femoral artery is inserted into the catheter and the superior mesenteric artery angiography is performed. Under normal circumstances, the portal vein can be developed within 10 to 20 s. When the portal vein thrombosis occurs, the portal vein cannot be filled. In patients with cavernous hemangioma-like variation, There are many small collateral vessels around the occluded portal vein. Possible vascular images include: reverse flow of portal vein blood flow, esophageal varices, and other parts of the portal.
Diagnosis
Diagnosis and diagnosis of cirrhosis in children
Advanced cirrhosis is easier to diagnose. Early cirrhosis should be based on medical history, clinical symptoms and signs, plus laboratory and other auxiliary examinations. Initial cirrhosis can be considered, and further gastroscopy, ultrasound, liver biopsy, etc. According to the biopsy of liver biopsy, in addition to the cause of the disease diagnosis, biliary cirrhosis should be differentiated whether it is primary or secondary.
Patients with ascites should be identified with tuberculous peritonitis, constrictive pericarditis, dystrophic edema, as far as possible for the diagnosis of the cause, biliary cirrhosis should be distinguished by intrahepatic or extrahepatic obstruction, in addition to congenital hepatic fibrosis (congenital hepatic fibrosis) with fibrous tissue as the main pathological changes in the portal area, while the structure of the lobular leaves remained normal, with hepatosplenomegaly, portal hypertension, hematemesis, etc., can be treated by portal vein shunt.
