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Hemolytic jaundice

Introduction
Cause
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Diagnosis

Introduction

Introduction Hemolytic jaundice: Any disease that causes hemolysis can produce hemolytic jaundice. Hemolytic jaundice. Mainly due to the intrinsic defects of red blood cells themselves or the damage of red blood cells by exogenous factors, causing a lot of damage to red blood cells, releasing a large amount of hemoglobin, resulting in increased non-lipid bilirubin content in plasma, and jaundice exceeding the processing capacity of liver cells. . The life span of human red blood cells is generally 120 days. The hemoglobin released after the death of red blood cells is decomposed to produce indirect bilirubin (I-Bil), which is converted into direct bilirubin (D-Bil) by the liver, discharged into the biliary tract along with the bile, and finally discharged through the stool. The sum of indirect bilirubin and direct bilirubin is total bilirubin (T-Bil). Any obstacles in any of the above-mentioned links can cause jaundice. If the red blood cells are destroyed too much, the indirect bilirubin produced is too much, and the liver cannot completely convert it into direct bilirubin, and hemolytic jaundice can occur. In addition, when the spleen function is hyperactive, the red blood cells are destroyed. Due to the destruction of a large number of red blood cells, a large amount of unbound bilirubin is formed, which exceeds the uptake, binding and excretion ability of the liver cells, and on the other hand, anemia due to hemolytic activity is lacking. The toxic effects of oxygen and red blood cell destruction products weaken the metabolic function of hepatocytes on bilirubin, and the retention of unbound bilirubin in the blood, which exceeds normal levels and causes jaundice.

Cause

Cause

The etiology, pathogenesis and characteristics of hemolytic jaundice:

1) Etiology and mechanism of occurrence: Hemolytic jaundice can occur in any disease that causes massive destruction of red blood cells and causes hemolysis:

1 congenital hemolytic anemia;

2 acquired hemolytic anemia.

When a large number of red blood cells are destroyed, an excessive amount of unbound bilirubin is produced, which far exceeds the limit of hepatocyte uptake, binding and excretion. At the same time, hypoxia caused by hemolytic anemia, toxic substances released by red blood cell destruction can weaken the bile of liver cells. The function of erythromycin metabolism causes unconjugated bilirubin to remain in the blood and cause jaundice.

2) Characteristics of hemolytic jaundice:

1 sclera is more common with mild yellow staining, fever in acute attacks, back pain, skin mucosa often pale;

2 skin without itching;

3 have a spleen;

4 has a strong performance of bone marrow hyperplasia;

5 serum total bilirubin increased, generally not more than 85mol / L, mainly non-integrated bilirubin increased;

6 Urine urinary biliary tract increased without bilirubin, hemoglobinuria in acute attack, soy sauce color, urinary hemosiderin increased during chronic hemolysis, and urinary biliary excretion increased in 24 hours;

7 When the hereditary spheroid cells increase, the erythrocyte fragility increases, and the fragility decreases during thalassemia.

Examine

an examination

Related inspection

Erythrocyte serum total bilirubin indirect bilirubin by skin puncture cholangiography neonatal hemolysis screening cholangiography

diagnosis

The diagnosis of hemolytic jaundice mainly depends on the following laboratory tests:

1 The content of jejunal and urobilinogen increased;

2 serum bilirubin increased, Vanden's test showed an indirect reaction;

3 increased reticulocytes in the blood;

4 serum iron content increased;

5 bone marrow red system hyperplasia. See the hemolytic anemia section for details.

Sign

1 sclera is more common with mild yellow staining, fever in acute attacks, back pain, skin mucosa often pale;

2 skin without itching;

3 have a spleen;

4 has a strong performance of bone marrow hyperplasia;

5 serum total bilirubin increased, generally not more than 85mol / L, mainly indirect bilirubin increased;

7 When the hereditary spheroid cells increase, the erythrocyte fragility increases, and the fragility decreases during thalassemia.

Diagnosis

Differential diagnosis

First, hepatic jaundice

The diagnosis and identification of hepatic jaundice, in addition to medical history and clinical examination, often rely on experimental examination. Liver biopsy has important help in the differential diagnosis of some difficult cases, but it should be strictly controlled and carefully implemented.

(1) Huang Zeng type viral hepatitis

Since the turn of the century, viral hepatitis has been considered to be only type A and type B. Since the nature of hepatitis B virus was clarified after 1965, non-A, non-B hepatitis was also proposed in 1974 after transfusion and intestinal transmission. In 1977, hepatitis D virus (factor) was discovered in 1979 for hepatitis A virus. In vitro culture was successful. At the international conference held in Tokyo in 1989, according to current research findings, the pathogen of non-A, non-B hepatitis after transfusion was named hepatitis C virus, and the pathogen of non-A, non-B hepatitis transmitted in the intestine was named . Hepatitis virus. At present, viral hepatitis should include at least A, B, C, D, E type A, B, C, D, E)

1. Acute yellow viral hepatitis

Its jaundice is from a few days to a week. The most prominent symptoms are fatigue and loss of appetite. Nausea, liver pain or discomfort, with or without fever. Some cases are mainly misdiagnosed as gastrointestinal dyspepsia due to dyspepsia and diarrhea; some mainly show upper respiratory symptoms, often misdiagnosed as acute upper respiratory tract infection; a few cases can be misdiagnosed as fever and multiple joint pain Rheumatic fever. The clinical diagnosis of jaundice in the early stage is difficult, but at this time, the serum aminotransferase activity is often significantly increased. The positive rate of 100% is the most early diagnostic value. After the appearance of jaundice, the symptoms are relieved. At this time, the main signs are jaundice, hepatomegaly or hepatosplenomegaly, and the quality of the spleen is painful. The liver area often has sputum pain, and there is no obvious hepatomegaly. Liver function test brain blisters, turbidity, zinc turbidity are mostly positive, urinary urinary biliary discharge increased with bilirubin positive, helpful for diagnosis. Astragalus is usually 2-4 weeks, sometimes longer.

2, severe viral hepatitis

(1) Acute severe hepatitis (burst hepatitis)

This type is rare. In the course of acute hepatitis, jaundice is rapidly progressive, or (and) the body temperature continues to rise, the disease should be considered first. Other signs of aura are persistent vomiting, psychotic symptoms, and liver odor. Psychiatric symptoms mainly manifest as irritability or ambiguity or personality change, pterygium during dynamism, catching clothes, lethargy, or lethargy. In some cases, diagnosis may occur, and deep coma (hepatic faint EEG often) Displaying abnormal waveforms is helpful for early diagnosis. Physical examination often finds deep yellow staining, liver turbidity and shrinkage, skin and membrane bleeding. Significantly reduced urine volume, proteinuria and casts. Liver function tests indicate severe liver Damage. If not treated early, the patient often dies within a short period of time. The pathological manifestation is acute hepatic necrosis.

(2) Subacute severe hepatitis (

Subacute hepatic necrosis is slower than fulminant hepatitis. Astragalus membranaceus is progressively deepened, serum bilirubin is often >17umol/L, prothrombin time is prolonged, and cholinesterase activity is significantly reduced. And there is a lack of energy. Digestive disorders, nausea and vomiting, physical decline, fever, hepatomegaly (or shrinkage) and tenderness. Gradually jejunal and ascites appear gradually, and hepatorenal syndrome can occur in severe cases, and even into a coma. Some patients can recover after active treatment, or develop into cirrhosis. If the infection or liver failure is severe in the course of the disease, the prognosis is more dangerous.

(3) Chronic severe hepatitis

This type is subacute hepatic necrosis of chronic hepatitis. The clinical manifestations are as subacute severe hepatitis, but there are abnormalities in history, signs and tests of chronic hepatitis or post-hepatitis cirrhosis.

3. Chronic jaundice-type viral hepatitis

The main basis of chronic jaundice hepatitis:

1 The course of disease lasts for more than half a year;

2 The main symptoms are fatigue, loss of appetite, postprandial fullness, greasy irritability, nausea and vomiting, jaundice may be optional, and often mild and fluctuating, persistent liver enlargement and increased hardness, accompanied by tenderness With sputum pain, the diagnosis of splenomegaly is of great significance. In patients with acute hepatitis, the splenomegaly does not retract during the recovery period, which means that the course of disease is prolonged, or to chronic development; 3 abnormal liver function test, the more sensitive test is the urine urinary bilirubin test. Determination of serum aminotransferase activity and serum floc and turbidity reaction. The increase in serum electrophoresis of gamma globulin is a sign of chronic hepatitis. Acute hepatitis A does not develop chronic, while acute hepatitis B and non-A, non-B hepatitis have a chronic tendency. If it develops into cirrhosis, the liver shrinks and the hardness increases, the spleen enlarges, the serum albumin continues to decrease, and the gamma globulin continues to increase.

(B) Huang Heng type infectious mononucleosis

The disease is associated with jaundice about 5%-7%. Astragalus is generally mild. l% bed showed fever, hepatosplenomegaly, and lack of appetite. Abnormal liver function tests, atypical lymphocytes in flesh and blood, quite similar to acute jaundice-type viral hepatitis. Infectious mononucleosis is often epidemic, pharyngitis is more obvious, often there are obvious lymph nodes (especially cervical lymph nodes, gastrointestinal symptoms are mild, and there are typical blood cell morphology changes and heterophilic agglutination effect The price increases. In acute viral hepatitis, the absolute value of blood-type heterotypic lymphocytes is generally less than 900 per cubic millimeter, which lasts for only a few days (slowly after the fever period L; in the case of infectious mononucleosis, atypical lymphocytes The absolute value is usually more than 1000 per cubic millimeter and often lasts for more than two weeks.

The disease mainly affects infants and young children, and there are also adult cases reported in the domestic literature. The pathogen is Cytomegalov-irus, which can infect the fetus through the placenta through asymptomatic pregnant women with viral infection. The newborn is born with a lack of energy, pertussis-like cough, hepatosplenomegaly, digestive disorder, growth within one month of birth. Stagnant, jaundice can also occur. Infants and young children mostly exhibit interstitial pneumonia or giant cell hepatitis. Brain damage can also occur. Adults can exhibit symptoms similar to infectious mononucleosis and blood, but negative heterophilic agglutination. Liver function test serum aminotransferase activity increased, some cases of turbidity reaction abnormal. Often occurs after losing a lot of fresh blood. Diagnosis should be based on a special serum complement binding test, cytomegalovirus is isolated from urine (or saliva), which is particularly diagnostic.

(4) Leptospirosis

The diagnosis of this disease needs to be based on: 1 epidemiological history, the disease is mainly infected by contact with infected water, more common in the rice harvest season in rural areas; 2 sudden fever, conjunctival hyperemia, intestinal muscle pain, bleeding tendency, jaundice, lymphadenopathy And clinical manifestations such as liver and kidney dysfunction; 3 early onset culture and animal vaccination can obtain positive results, serum agglutination dissolution test and complement fixation test can be positive 1 week after onset. The onset of severe hepatitis is not as fast as leptospirosis, often with symptoms such as toxic jejunum and ascites, and no ball conjunctival hyperemia and intestinal irritations, no abnormal X-ray signs in the lungs, and early renal damage is not obvious. The differential diagnosis of special pathogens and serological tests is more significant.

(5) Yellow values caused by other acute systemic infections

Some acute systemic infections such as lobar pneumonia, return to heat. Malaria, typhus, typhoid fever, wavy fever, acute miliary tuberculosis, etc., can be complicated by jaundice. Astragalus is generally mild, due to liver parenchymal damage or hemolysis, or both. Acutely transmitted diseases complicated by jaundice, most of the lesions are located on the right side, especially in the lower right lobe.

(6) Primary acute pregnancy fatty liver

The disease is rare in clinical practice. The cause is unknown. Whether it is secondary to endocrine or nutritional disorders remains undetermined. It is a serious complication of obstetrics and is often misdiagnosed as fulminant hepatitis. Clinically, the following characteristics are distinguished from fulminant hepatitis:

1 more common first trimester within the 36th to 40th week;

2 have varying degrees of pregnancy edema, proteinuria or high blood pressure;

3 nausea, vomiting without incentives, followed by severe bleeding tendency, such as gums, skin, vaginal bleeding, morphine-like vomit or vomiting blood;

4 serum direct bilirubin quantification 171 umol / L%),

And urinary bilirubin is negative;

5 serum brain flocculent reaction is negative;

3 coma, jaundice depth, liver progressive reduction is generally not as severe as fulminant hepatitis.

Acute renal insufficiency occurs earlier than;

1 Ultrasound or CT examination revealed typical signs of fatty liver. It is not difficult to identify the two in histopathological examination. The literature reports that a large number of intravenous tetracyclines (about 1.5 g / d) in pregnant women can also have the performance of acute fatty liver similar to pregnancy. The two can be compared with the history of drug treatment and the above characteristics.

(7) Toxic liver damage

Some drugs or poisons have hepatotoxic effects, which can cause hepatic steatosis and central necrosis of hepatic lobules. The clinical manifestations are hepatomegaly, yellow colonization and liver damage.

1. The following drugs have been reported to cause similar viral hepatitis, such as Xin Kefen, isoniazid, and Fushun, PAS. Halothane. Methyldopa and bisphenol are called to protect the pine. Alkali amines, sulfonamides. The general therapeutic dose of chlortetracycline, novobiocin, phenobarbital, thioxime, etc. / but antibiotics has few side effects unless the patient has allergies.

2, cotton seeds, Xanthium, some virulence rate contain cell virgin toxic, after taking it can cause severe liver damage and jaundice, and even hepatic coma.

3. The main chemical substances that can cause chemically toxic hepatitis are as follows:

1 metals, metalloids and their compounds such as lead. Mercury, manganese, arsenic, yellow phosphorus, chromium, antimony, key, hesitation, etc.;

2 organic compounds: amino and nitro compounds of benzene, phenols, gasoline, carbon disulfide, methane, methyl chloride, formaldehyde, ethanol, tea, carbon tetrachloride. Tetrachloroethylene, etc.

3 pesticides: organic phosphorus, organic chlorine, organic mercury, etc.

Acute chemical toxicity hepatitis has the following clinical features.

1 The incubation period is short, the fastest is 2-3d, such as yellow phosphorus, the longer one is about 1 week, such as DDT, M carbon sulfide, p-chloronitroaniline poisoning;

2 often have jaundice and hepatomegaly, but often no obvious fever, spleen is not swollen;

3 serum aminotransferase and bilirubin increased; 4 has a significant history of toxic contact.

Drugs that cause obstructive jaundice in the liver are discussed below in this chapter.

(8) Acute alcoholic hepatitis

There are many reports on foreign literature in this disease. People who have been drinking hard alcohol for many years have recently had alcoholism, and this disease must be considered when the following conditions occur:

1 new appetite loss, weakness, weight loss, nausea and vomiting, jaundice and abdominal pain;

2 hepatomegaly and tenderness, sometimes splenomegaly, accompanied by unexplained fever;

3 serum bilirubin increased, serum albumin decreased and globulin increased, serum flocculosis reaction, serum aminotransferase increased, many cases have serum alkaline phosphatase and elevated blood glucose, anemia, leukocytosis and mononuclear Increased cells; 4 liver biopsy revealed special inflammatory lesions.

(9) Cardiogenic Huang Zeng

The causes of cardiogenic jaundice are complicated, and the most important one is caused by hepatic cell congestion and hepatocyte hypoxia, resulting in poor function of hepatocytes to treat bilirubin. Mild jaundice can be seen in right heart failure caused by various causes, especially when accompanied by relative or organic tricuspid regurgitation. In cases of recurrent right heart failure, the incidence of cardiogenic jaundice is increased, but serum total bilirubin usually does not exceed 51 umol / L (3mg / L), occasionally a deep yellow value can occur, at this time the patient It can present green jaundice similar to biliary obstruction. Urinary urinary bilirubin excretion often increases in patients, and occasionally mild bilirubinuria can occur. The retention of sodium sulfonate is often increased, and the serum flocculation and turbidity tests are mostly normal, with a few positive. There was no significant increase in serum transaminase activity in simple hepatic congestion.

(10) Cirrhosis of the liver

All types of cirrhosis can be complicated by jaundice.

(11) Hyperthyroidism

Hyperthyroidism can be complicated by fatty liver and hepatocyte necrosis, and jaundice, but this is rare and occurs in heavier cases. This type of jaundice is not easily distinguished from concurrent acute viral hepatitis. If the patient has no history of hepatitis exposure, no pre-yellowing symptoms, no obvious loss of appetite, nausea, bloating and other symptoms of digestive disorders. The jaundice is likely to originate from hyperthyroidism. If there is no high serum aminotransferase activity, it is more supportive for the diagnosis of this type of jaundice.

Second, obstructive jaundice

Obstructive jaundice is due to intrahepatic capillary bile ducts. Caused by mechanical obstruction of the hepatic bile duct or common bile duct. Skin cancer itching and bradycardia are common symptoms. Hepatomegaly is a common sign. Mechanical obstruction without concomitant infection does not cause splenomegaly. The early yellow scutellaria is golden yellow, later yellow-green, late green-brown, and even near black (black jaundice, such as the gallbladder that touches the swell, suggesting that the obstruction is in the common bile duct, the origin of the tumor is more, and the origin of the stone Very few. The diagnosis of obstructive yellowness and the identification of obstructive jaundice inside and outside the liver are shown in the laboratory and device examination.

(1) Intrahepatic obstructive yellow disease

The intrahepatic obstructive yellow value is mostly manifested in intrahepatic cholestatic syndrome. Clinical and pathological has a unique content, the course of the disease can be divided into acute and chronic, the clinical is more common in acute type, a few acute cases can develop chronic, and even evolve into primary biliary cirrhosis. Intrahepatic cholestatic syndrome has the following clinical and pathological features: 1 has a history of close contact with hepatitis patients or some history of drug treatment, jaundice is more acute, skin itching, dark urine. Light color stool, hepatomegaly and other signs; 2 blood biochemical examination in line with the characteristics of obstructive jaundice, suggesting signs of liver parenchymal damage or no liver parenchymal damage; 3 the main changes in liver biopsy are capillary bile duct and small bile duct cholestasis Formed with a gallbladder, and the liver parenchyma is mild.

I. Liver cholestatic

(1) Capillary inflammatory viral hepatitis: This type of hepatitis is a rare type of viral hepatitis, also known as cholestasis-type viral hepatitis. Main clinical features:

1 The onset is more urgent, the jaundice gradually deepens, the skin is itchy, and the depth of jaundice is not commensurate with the severity of the symptoms;

2 examination showed that jaundice and hepatomegaly, or both splenomegaly;

3 blood biochemical examination is completely consistent with obstructive jaundice, most of the serum flocculent reaction did not change significantly, but often moderate serum iron and serum aminotransferase activity increased, suggesting that liver parenchymal damage is lighter. Liver biopsy showed intrahepatic cholestatic, capillary bile duct thrombosis, often accompanied by mild liver parenchymal inflammation;

4 After the diagnosis of adrenal corticosteroids, the jaundice in most cases is significantly reduced in the short term, which is helpful for the diagnosis of this disease, but bacterial infection must be ruled out before this test can be done. Some people think that it is not appropriate to give this test before the third week of the disease. Because it can interfere with the body's defense function, the jaundice is easy to re-appear after stopping the drug, or it can become chronic. If the jaundice is not obvious, it can not exclude viral hepatitis.

5 must be excluded from other causes of intrahepatic extrahepatic obstructive jaundice, especially drug. Capillary inflammatory inflammatory hepatitis, the prognosis is generally good, the course of disease is no more than 3-6 months, a few can be extended more than years, and a few cases can evolve into secondary biliary cirrhosis.

(2) drug-induced jaundice: Many drugs can cause acute intrahepatic cholestatic syndrome, which can be divided into two categories:

a, acute intrahepatic cholestatic syndrome with inflammatory response: this type of jaundice is most common with chlorpromazine. There are also new ones. Chlorosulfonium porpoise, thioxine chewing compound, tamoxifen, sulfonamides, chloranil waste, amino-salicylic acid, yaw erythromycin propionate, etc., but the incidence is very low, generally for the drug users 1% or less. The pathogenesis is considered to be the body's allergic reaction to drugs, with the following clinical features:

1 The occurrence of jaundice has nothing to do with the dose size, usually occurs within 1 to 4 weeks of treatment;

2 clinical often accompanied by fever, rash and eosinophilia;

3 Astragalus lasts for several weeks to several days, but after re-medication, jaundice will be re-issued soon;

4 pathological biopsy showed intrahepatic cholestatic, capillary bile duct thrombosis, eosinophil infiltration around the portal area, minimal changes in liver parenchyma, mainly hepatocyte balloon-like changes, glycogen disappearance, bile pigment accumulation. Foreign literature reports that this type can be chronically developed and developed into primary biliary cirrhosis.

b, acute intrahepatic cholestatic syndrome without inflammatory reaction: this type of jaundice can be found in the application of methyltestosterone and oral contraceptives and other drugs, the structure of which contains 17a-hydroxyl, currently believed that the pathogenesis is due to this class The drug interferes with the excretion of hepatocytes on bilirubin and sodium sulphate, especially in the capillary bile duct and lysosomes. Clinically has the following characteristics:

1 After taking a certain dose of this kind of medicine, almost every case may have the retention of sulphate sodium, and some cases have jaundice;

2 clinical no fever, rash and eosinophilia;

3 After the drug is stopped, the yellow is good to dissipate within a few days to several weeks, and the re-use of the drug often causes recurrence;

4 Liver biopsy only showed intrahepatic cholestatic, but no inflammatory response.

(3) Idiopathic jaundice during pregnancy: The cause of this type of jaundice is unknown, very rare, and only a few cases have been reported in China. Astragalus often occurs in the late third trimester of pregnancy. The first symptom is usually skin itching, which can occur several weeks before the onset of jaundice. There may be dark urine and white clay-like stools, which peaked around the first week after the occurrence of Astragalus. Other symptoms are mild and mild hepatomegaly. Astragalus is consistent with intrahepatic obstruction, serum flocculent reaction is often negative, aminotransferase is normal or slightly increased, and jaundice rapidly resolves within 1 to 2 weeks after delivery. Astragalus often reappears when it is re-pregnancy. The mother and child have a good prognosis.

(4) Alcohol-Liver-Syndrome This syndrome is a unique type of clinical pathology, the pathogenesis is unknown, but related to heavy drinking, can be the end stage of alcoholic cirrhosis. The main clinical manifestation is obstructive jaundice in the liver. Patients with loss of appetite, nausea, vomiting, upper abdominal pain and hepatomegaly can be misdiagnosed as gallstone colic. Fatty liver can also cause this syndrome. At this time, liver biopsy has no inflammatory lesions in the liver parenchyma except for liver steatosis and intrahepatic cholestatic. Clinical diagnosis is determined only by the history of drinking and other factors that cause obstructive jaundice in the liver. If necessary, liver biopsy is used. Because of the clinical manifestations of biliary obstruction, the original fatty liver is often ignored. This syndrome has not been reported in China. Such as alcoholic toxic fatty liver with jaundice, hemolytic anemia and hyperlipidemia, it is called Zieve syndrome. In recent years, there have been case reports in China.

(5) After jaundice after benign surgery: Astragalus appeared in cases with more difficult operation and long operation time, mostly for abdominal or chest and abdomen surgery. In recent years, many foreign literature reports have been reported. Most of the patients developed jaundice after l-2 days (individually at 11 days) and resolved after 2-3 weeks. The patient had no fever, itchy skin, no obvious hepatosplenomegaly, and bilirubinuria. Serum aminotransferase activity is normal or slightly increased. Liver biopsy proved to be central cholecystosis in the liver without substantial inflammation, similar to drug-induced jaundice caused by methyltestosterone. This type of jaundice has a good prognosis. The pathogenesis is unknown. In the determination of jaundice after benign surgery, other reasons must be caused to cause postoperative jaundice, such as cholangitis caused by surgery, obstructive jaundice caused by cholelithiasis, occasional viral hepatitis after surgery, postoperative infection and drug Huang Wei and so on.

(6) Idiopathic benign recurrent intrahepatic cholestatic: This disease is rare and has not been reported in China. The onset age is 1-37 years old. There are familial case reports. The clinical manifestations are recurrent obstructive jaundice, no abdominal pain, chills and fever, often without hepatomegaly. Serum alkaline phosphatase activity increased during the jaundice stage, aminotransferase activity increased slightly, serum cholic acid, aggrecan and p-globulin increased, and liver biopsy showed central cholecystosis without any inflammation and necrosis. Retrograde angiography of the duodenal biliary tract shows normal biliary system. The above blood biochemical changes returned to normal during the remission period of Astragalus. The recovery period of Astragalus has been reported for up to nine years. The recurrence can be seasonal. (7) Primary biliary cirrhosis: This disease is caused by long-term obstruction of intrahepatic bile ducts and biliary cirrhosis caused by cholestatic. Clinically rare, the cause is not yet clear clinical features:

1 The onset of the disease is more than 20-4O years old, the onset is insidious, the course of disease is slow, and the general condition is better for a long time;

2 The main clinical manifestations are chronic obstructive jaundice, hepatosplenomegaly, gastrointestinal symptoms, skin cancer itching and intermittent right upper abdominal pain with cold and heat attacks, similar to acute cholangitis episodes;

3 hepatomegaly is mostly moderate, but also high, hard, smooth surface, and late nodular.

4 jaundice is volatility, deepening in the onset of upper abdominal pain and cold fever. At this time, the total number of white blood cells also increased, and after the inflammation was controlled, the jaundice also decreased or subsided;

5 laboratory tests consistent with obstructive jaundice, serum anti-mitochondrial antibody (AMA) positive rate of 84% -96%, or with increased serum, liver biopsy meets the histological changes of primary biliary cirrhosis. Ascites and upper gastrointestinal bleeding can occur in the late stage of the disease.

2, intrahepatic mechanical obstruction

(1) Primary sclerosing cholangitis: This disease involves intrahepatic or (and) extrahepatic bile ducts. In recent years, there have been dozens of reports in China.

1 factors in the bile duct: such as stones, mites, worms, clogging of blood clots, etc.;

2 bile duct wall factors: such as bile duct stricture, cholangiocarcinoma, periampullary carcinoma, cholangitis, congenital biliary atresia, etc.;

3 factors outside the bile duct: such as pancreatic cancer, pancreatitis, lymph node metastasis of the hilar area. The disease often has a history of cholelithiasis, biliary tract mites or a history of biliary tract surgery, can touch the gallbladder swell, barium meal examination shows duodenal curvature or duodenal descending inner wall membrane damage, suggesting extrahepatic obstruction The possibility. Endoscopic retrograde cholangiopancreatography can be performed under conditions, and the diagnosis often requires surgical exploration.

1, acute obstructive suppurative cholangitis

Because of the stones. Aphids or stenosis caused by obstruction of the common bile duct and/or intrahepatic bile duct. Patients often have paroxysmal cramps in the right upper quadrant, chills and high fever are relaxation heat, vomiting during nausea, with varying degrees of obstructive jaundice, and can be complicated by toxic shock. The liver is mildly and moderately enlarged, and white blood cells are significantly increased with neutrophils predominating. If there is acute right upper abdominal pain, high fever, jaundice, it is called CoK triad, showing acute suppurative biliary tract infection. Such as accompanied by symptoms of central nervous system poisoning, shock is called Regnold five-link, showing acute obstructive suppurative cholangitis.

2, common bile duct stones

The clinical feature is jaundice after paroxysmal right upper quadrant cramps, which in the past may have the same history of seizures. If the infection is combined, there will be chills and fever. Astragalus was moderate (the total amount of bilirubin was less than 120 umol/L). The test showed obstructive jaundice without liver damage. The occurrence of yellow value is not only due to stone obstruction, but also due to edema of the common bile duct smooth muscle spasm or membrane inflammation; in some cases, the gallbladder can be reached in some cases. After inflammatory edema and sputum subsidence, although the common bile duct stones are not ruled out, bile can still flow out, and jaundice can be alleviated. The X-ray film can show the shadow of the X-ray. Intravenous cholecystography can show the common bile duct dilatation and X-ray calculus, but it is not appropriate to have this examination when there is jaundice. B-mode ultrasound T examination, combined with clinical often can determine the diagnosis.

3, congenital choledochal cyst

Typical manifestations are triads: jaundice, abdominal pain, and abdominal mass. 80% are women, mostly children and adolescents. B-mode ultrasound is important for the diagnosis of this disease.

4, pancreatic head cancer

Males are more common, and the incidence is mostly 40-60 years old. Cancer occurs most in the head of the pancreas, showing progressive obstructive 1 intrabiliary factors: such as stones, mites, worms, clogging of blood clots, etc.; 2 bile duct wall factors: such as bile duct stricture, cholangiocarcinoma, ampulla around Cancer, cholangitis, congenital biliary atresia, etc.; 3 extrabiliary factors: such as pancreatic cancer, pancreatitis, lymph node metastasis of the hilar area. The disease often has a history of cholelithiasis, biliary tract mites or a history of biliary tract surgery, can touch the gallbladder swell, barium meal examination shows duodenal curvature or duodenal descending inner wall membrane damage, suggesting extrahepatic obstruction The possibility. Endoscopic retrograde cholangiopancreatography can be performed under conditions, and the diagnosis often requires surgical exploration.

Other differential diagnosis:

1, acute obstructive suppurative cholangitis

2, common bile duct stones

The clinical feature is jaundice after paroxysmal right upper quadrant cramps, which in the past may have the same history of seizures. If the infection is combined, there will be chills and fever. Astragalus was moderate (the total amount of bilirubin was less than 120 eL) and the test showed obstructive jaundice without liver damage. The occurrence of yellow value is not only due to stone obstruction, but also due to edema of the common bile duct smooth muscle spasm or membrane inflammation; in some cases, the gallbladder can be reached in some cases. After inflammatory edema and sputum subsidence, although the common bile duct stones are not ruled out, bile can still flow out, and jaundice can be alleviated. The X-ray film can show the shadow of the X-ray. Intravenous cholecystography can show the common bile duct dilatation and X-ray calculus, but it is not appropriate to have this examination when there is jaundice. B-mode ultrasound T examination, combined with clinical often can determine the diagnosis.

3, congenital choledochal cyst

Typical manifestations are triads: jaundice, abdominal pain, and abdominal mass. 80% are women, mostly children and adolescents. B-mode ultrasound is important for the diagnosis of this disease. 4, men with pancreatic head cancer are more common, the incidence is mostly 40-60 years old. Cancer occurs most in the head of the pancreas and exhibits progressive obstructive jaundice. Pancreatic body and tail cancer generally do not cause jaundice, the main symptom is jumping upper abdominal pain. The main features of pancreatic head cancer are: 1 anorexia, rapid weight loss, fatigue, general condition worsening in a short period of time; 2 chronic progressive jaundice, from incomplete obstruction to complete obstruction; 3 often persistent upper abdominal pain, Often to the left back radioactivity; 4 hepatomegaly and gallbladder enlargement; 5 late can touch the abdominal mass; 3 laboratory tests: serum pancreatic amylase and pancreatic lipase increased (early due to pancreatic duct obstruction increased, late cause Pancreatic atrophy reduces the increase of human blood glucose and mild diabetes-like glucose tolerance curve. When the pancreatic duct is completely obstructed, steatorrhea and fleshy diarrhea appear. The X-ray examination shows that the duodenal curvature is increased, and the duodenal inner wall is compressed and eroded. Signs of narrowing of the lumen of the intestine and infiltration or compression of the antrum.

5, lack of ampullary pericarcinoma

The disease can also cause progressive weight loss, deep jaundice (the total amount of bilirubin often reaches 255 ~ 510 umol / L (15 ~ 30mg / dl)], hepatomegaly and gallbladder swelling and other symptoms. Astragalus can be relieved during the course of the disease, and serum bilirubin does not fall to normal. This point has a differential diagnosis significance with stone blockage. In the latter, when the stones are displaced, the jaundice can quickly subside after the blockage is lifted, and the serum bilirubin also drops to normal. Upper gastrointestinal bleeding can be a serious symptom of ampullary cancer. If the duodenal drainage fluid is blood, it is likely to be cancerous, especially ampullary cancer. The most reliable one is cancer cells found in the eleven finger drainage. X-ray diagnosis is mainly based on indirect changes of adjacent organs; venous cholecystography can have diagnostic value for early lesions. In recent years, duodenoscopy has been used to directly observe the lesions around the ampulla, and can be used for biopsy, which is helpful for early diagnosis. Timely radical surgery, the prognosis is better. The ampullary carcinoma has fewer signs than the pancreatic head cancer. Fiber duodenoscopy is more diagnostic.

6, acute and chronic pancreatitis

Domestic reports of 18.2% of acute pancreatitis with jaundice. Deep jaundice often indicates a serious condition. In chronic pancreatitis, granulation tissue hyperplasia can sometimes cause chronic obstructive jaundice, which can be touched and differentiated from pancreatic cancer. This situation is very rare. Occasionally, the history of acute pancreatitis is not obvious, especially confusing. The jaundice of this disease is intermittent and fluctuating; the jaundice of pancreatic head cancer is progressive and has no tendency to relieve. CT scan, fiber duodenoscopy, and laparotomy for frozen biopsy help to identify the two.

7, common bile duct or hepatobiliary carcinoma

In the common bile duct or hepatobiliary duct, occasionally cancer can occur, usually adenocarcinoma, clinically manifested as painless extrahepatic obstructive jaundice. The size of the cancer is usually small, but early symptoms of obstructive jaundice, such as white clay. Bilirubinuria, hepatomegaly, etc. Gallbladder often swells when the common bile duct cancer occurs. The biliary tract infection often occurs in the late stage of the two, which may be temporarily relieved due to the collapse of the cancer tissue and the biliary drainage. This condition is difficult to see in the head cancer. Middle-aged patients, especially males, have the above-mentioned situation. The degree of jaundice is significantly greater than that of liver function. Repeated examination of duodenal angiography is necessary, and the possibility of common bile duct or hepatobiliary carcinoma should be considered. If the gallbladder is touched, the former is supported. If there is an unexplained upper gastrointestinal bleeding (the occult blood reaction is repeated strongly positive), one of the two is more likely. Two types of cancer can be confirmed by endoscopic retrograde cholangiopancreatography, but it must be diagnosed by surgery.

Common bile duct adenomyoma is rare, mostly elderly, with painless progressive obstructive jaundice as a clinical feature, which must be diagnosed by surgery.

8, primary gallbladder cancer

Rarely, chronic cholecystitis is often the basis of the disease, the incidence of more than middle-aged, about 40% -50% of cases with jaundice, and gradually deepened. Pain in the gallbladder area often precedes jaundice. Diagnosis is done first with B-mode ultrasound, if necessary, with gallbladder angiography and CT scan, early surgery can get a chance to cure.

9, duodenal ulcer

The posterior portion of the duodenal bulb refers to the curved portion where the tip of the ball is connected to the descending portion. The so-called duodenal ulcer, including duodenal ulcers in the lower part of the posterior part of the ball, is more common in the posterior part of the ball, followed by the descending part. Post-balloon ulcers: 2/3 cases resemble ball ulcers, and very few can cause jaundice. Astragalus is obstructive and easily misdiagnosed as biliary ascariasis, biliary tract infection or biliary tract bleeding. Obstructive jaundice is caused by ulceration of the epilepsy leading to stricture of the common bile duct, or lack of apical papillary edema, or Oddi's sphincter reflex sputum.

Hemolytic jaundice, mainly due to the intrinsic defects of red blood cells themselves or damage of red blood cells by exogenous factors, causes red blood cells to be destroyed a lot, releasing a large amount of hemoglobin, resulting in increased non-lipid bilirubin content in plasma, exceeding the treatment of hepatocytes. The ability is jaundice. In addition, when the spleen function is hyperactive, the red blood cells are destroyed and destroyed. Due to the destruction of a large number of red blood cells, a large amount of unbound bilirubin is formed, which exceeds the uptake, binding and excretion ability of the liver cells, and on the other hand, anemia caused by hemolytic activity, The toxic effects of hypoxia and red blood cell destruction products weaken the metabolic function of hepatocytes on bilirubin, and make unbound bilirubin in the blood, which exceeds the normal level and causes jaundice.

The skin of hemolytic jaundice and the yellow color of porcine membrane are often lightly pale lemon yellow, and often accompanied by anemia accompanied by pale skin. The causes of hemolytic jaundice are:

1 red blood cells themselves inherent defects;

2 red blood cells are damaged by external factors. Damaged red blood cells can be destroyed prematurely in the reticuloendothelial system or destroyed directly in the blood vessels.