肝硬化(Hepatic Cirrhosis.英文).ppt
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Hepatic Cirrhosis
Cirrhosis---definition
chronic, progressed, diffusehepatocellular injuryfibrosis
nodular regeneration
Incidence: 17/100000/y
Age: 20-50 yr.
Etiology of cirrhosis(I)
Etiology of cirrhosis(II)
6. Hepatic venous outflow obstruction(肝血液循环障碍)
veno-occlusive disease, Budd-Chiari syndrome, constrictive pericarditis
7. Metabolic disorders (遗传代谢性疾病)
hemochromatosis(血色病); Wilson's disease(肝豆状核变性);
8. Autoimmune hepatitis (AIH)(自身免疫性肝炎)
9. Schistosomiasis (血吸虫病)
10. Cryptogenic (隐原性)
11. Mixed: alcohol+virus ,HBV+HCV,HBV+schistosomiasis
Pathogenesis: chronic, progressed, diffuse
? Hepatocyte injury leading to necrosis.
? Chronic inflammation - (hepatitis).
? Capillarization (肝窦毛细血管化) of the space of Disse is a key event.
? Bridging fibrosis.
? Regeneration of remaining hepatocytes proliferate as round nodules surrounded by fibrous septa.
? Loss of vascular arrangement results in regenerating hepatocytes ineffective.
? Cirrhosis may lead to liver failure, portal hypertension, or development of hepatocellular carcinoma
Histopathologic classification
? micronodular
uniformly small nodules (< 3 mm in diameter) and regular bands of connective tissue
? macronodular
nodules that vary in size (3 mm to 5 cm in diameter)
? mixed macro and micronodular
(incomplete septal cirrhosis) combines elements of micronodular and macronodular cirrhosis.
Consequences ofportal hypertension[I]
1.Splenomegaly (脾肿大)
2.Formation and open of portal-systemic collateral's (门体侧支循环开放)--Esophageal/gastric varices (食管/胃静脉曲张) (short gastric/coronary veins)--Rectal collateral's(痔静脉丛) (Suphemorrhoidal/middle & inf. hemorrhoidal)--Caput medusae(水母头)( umbilical/epigastric)--abdominal wall varices (腹壁静脉曲张)--Portal system and left renal
Consequences ofportal hypertension[II]
3. Ascites (腹水)
Theories of ascites formation
?Underfilling theory (灌注不足假说)
?Overflow theory (泛溢假说)
?Arterial vasodilation theory (动脉扩张假说)
Ascites
? Sodium retention---Renin angiotension aldosterone system(RAAS)?---sympathetic nerve system ?,norepinephrine?--- Intrarenal factors: Kallikrein-kinin system, Adenosine .
? Water retention---Antidiuretic hormone(ADH)?---Impaired renal synthesis of PGs (PGE2?)
? Renal vasoconstriction--- RAAS, AngiotensionII?---SNS---ADH?---ET?
Endocrine system
(gynecomastia(男性乳房发育),telangiectases (毛细血管扩张症),spider nevi(蜘蛛痣),palmar erythema(肝掌)
testicular atrophy(睾丸萎缩)
menstrual irregularities (月经失调)
Pulmonary manifestations
?Hepatic hydrothorax (肝性胸水)
?Hepatopulmonary syndrome
(HPS, 肝肺综合征)
HRS is characterized clinically by the triad of pulmonary vascular dilatation causing arterial hypoxemia in the setting of advanced liver disease.
HRS(Hepatorenal syndrome, 肝肾综合征)
( Occurred in the setting of:---chronic liver disease---advanced hepatic failure---portal hypertension
( characterized by:---impaired renal function---marked abnormalities in arterial circulation---activation of endogenous vasoactive system
( Classified into 2 different types:---Type I: Rapidly progressive---Type II: Not rapidly progressive. Often results in mild renal insufficiency causing diuretic resistant ascites
Clinical features[I]
Compensated cirrhosis (代偿期)
Many people experience few symptoms at the onset of cirrhosis,symptoms are typically vague and nonspecific.---Fatigue and loss of energy.---Loss of appetite and nausea.---Spider angiomas---liver function is normal
Decompensated cirrhosis (失代偿)
( Symptoms caused by loss of functioning liver cells--- System：fatigue, weakness, weight loss, malnutrition--- Digestive System:Loss of appetite, nausea, diarrhea.
Clinical features[II]
Clinical features[III]
Portal hypertension (门静脉高压)
1. Splenomegaly: anemia, leukopenia,thrombocytopenia due to hypersplenism
2. development and open of collateral vessels in portalhypertension
a. Esophageal varices
b. Rectal collateral's
c. Caput medusae
d. Abdominal wall varices
e. Portal system and left renal
3. Ascites、hepatic hydrothorax
Clinical features[IV]
( Palpation ofliver
firm, hard,irregular,enlargement
rounded or sharp edge
below the right lower ribs.
( The spleen is often palpable, and may be very large.
Complications[I]
? Upper gastrointestinal bleeding(上消化道出血):
Hematemesis(呕血)/melena(黑粪). Esophageal/ gastric variceal bleeding (食管/胃静脉出血);
portal hypertensive gastropathy (门脉高压性胃病);
peptic ulcer(消化性溃疡)
Infections:
spontaneous bacterial peritonitis(自发性细菌性腹膜炎) (4-8%)：Fever, worsening jaundice or renal dysfunction, abdominal pain (occurring only in 50% of patients), and encephalopathy are the most common clinical findings in SBP.
However, the patient is frequently asymptomatic. Because culture of ascites fluid is negative in a large number of patients with SBP, diagnosis should be based on the presence of >250 neutrophils/mm3.
Complications[III]
? Hepatorenal syndrome(HRS):
Oliguria(少尿), azotemia(氮质血
症), hypotension(低血压), dilutional
hyponatremia(稀释性低钠血症), low
urinary sodium(低钠尿)
Complications[IV]
Electrolyte and acid-base imbalance(电介质酸硷平衡失调)
hyponatremia, hypokalemia
And hypochloremic alkalosis
Immunology
? Cellular immune?, hormonal
? immune?autoimmune liver disease:
IgG?,globulin? ANA(+), SMA(+)
? PBC: IgM?, AMA(+)
? Marker of virus
? AFP
Laboratory findings[III]
( Radionuclide:99m TC-MIBI,H/L
( liver biopsy: to confirm the diagnosis
( Laparoscopy
( HVPG (hepatic vein pressure gradient)(肝静脉压力梯度) (wedged - free )hepatic venous pressure
Normal: 5-6mmHg,>10mmHg: varices;
>12mmHg:rupture......（后略) ......
Hepatic Cirrhosis
Cirrhosis---definition
chronic, progressed, diffusehepatocellular injuryfibrosis
nodular regeneration
Incidence: 17/100000/y
Age: 20-50 yr.
Etiology of cirrhosis(I)
Etiology of cirrhosis(II)
6. Hepatic venous outflow obstruction(肝血液循环障碍)
veno-occlusive disease, Budd-Chiari syndrome, constrictive pericarditis
7. Metabolic disorders (遗传代谢性疾病)
hemochromatosis(血色病); Wilson's disease(肝豆状核变性);
8. Autoimmune hepatitis (AIH)(自身免疫性肝炎)
9. Schistosomiasis (血吸虫病)
10. Cryptogenic (隐原性)
11. Mixed: alcohol+virus ,HBV+HCV,HBV+schistosomiasis
Pathogenesis: chronic, progressed, diffuse
? Hepatocyte injury leading to necrosis.
? Chronic inflammation - (hepatitis).
? Capillarization (肝窦毛细血管化) of the space of Disse is a key event.
? Bridging fibrosis.
? Regeneration of remaining hepatocytes proliferate as round nodules surrounded by fibrous septa.
? Loss of vascular arrangement results in regenerating hepatocytes ineffective.
? Cirrhosis may lead to liver failure, portal hypertension, or development of hepatocellular carcinoma
Histopathologic classification
? micronodular
uniformly small nodules (< 3 mm in diameter) and regular bands of connective tissue
? macronodular
nodules that vary in size (3 mm to 5 cm in diameter)
? mixed macro and micronodular
(incomplete septal cirrhosis) combines elements of micronodular and macronodular cirrhosis.
Consequences ofportal hypertension[I]
1.Splenomegaly (脾肿大)
2.Formation and open of portal-systemic collateral's (门体侧支循环开放)--Esophageal/gastric varices (食管/胃静脉曲张) (short gastric/coronary veins)--Rectal collateral's(痔静脉丛) (Suphemorrhoidal/middle & inf. hemorrhoidal)--Caput medusae(水母头)( umbilical/epigastric)--abdominal wall varices (腹壁静脉曲张)--Portal system and left renal
Consequences ofportal hypertension[II]
3. Ascites (腹水)
Theories of ascites formation
?Underfilling theory (灌注不足假说)
?Overflow theory (泛溢假说)
?Arterial vasodilation theory (动脉扩张假说)
Ascites
? Sodium retention---Renin angiotension aldosterone system(RAAS)?---sympathetic nerve system ?,norepinephrine?--- Intrarenal factors: Kallikrein-kinin system, Adenosine .
? Water retention---Antidiuretic hormone(ADH)?---Impaired renal synthesis of PGs (PGE2?)
? Renal vasoconstriction--- RAAS, AngiotensionII?---SNS---ADH?---ET?
Endocrine system
(gynecomastia(男性乳房发育),telangiectases (毛细血管扩张症),spider nevi(蜘蛛痣),palmar erythema(肝掌)
testicular atrophy(睾丸萎缩)
menstrual irregularities (月经失调)
Pulmonary manifestations
?Hepatic hydrothorax (肝性胸水)
?Hepatopulmonary syndrome
(HPS, 肝肺综合征)
HRS is characterized clinically by the triad of pulmonary vascular dilatation causing arterial hypoxemia in the setting of advanced liver disease.
HRS(Hepatorenal syndrome, 肝肾综合征)
( Occurred in the setting of:---chronic liver disease---advanced hepatic failure---portal hypertension
( characterized by:---impaired renal function---marked abnormalities in arterial circulation---activation of endogenous vasoactive system
( Classified into 2 different types:---Type I: Rapidly progressive---Type II: Not rapidly progressive. Often results in mild renal insufficiency causing diuretic resistant ascites
Clinical features[I]
Compensated cirrhosis (代偿期)
Many people experience few symptoms at the onset of cirrhosis,symptoms are typically vague and nonspecific.---Fatigue and loss of energy.---Loss of appetite and nausea.---Spider angiomas---liver function is normal
Decompensated cirrhosis (失代偿)
( Symptoms caused by loss of functioning liver cells--- System：fatigue, weakness, weight loss, malnutrition--- Digestive System:Loss of appetite, nausea, diarrhea.
Clinical features[II]
Clinical features[III]
Portal hypertension (门静脉高压)
1. Splenomegaly: anemia, leukopenia,thrombocytopenia due to hypersplenism
2. development and open of collateral vessels in portalhypertension
a. Esophageal varices
b. Rectal collateral's
c. Caput medusae
d. Abdominal wall varices
e. Portal system and left renal
3. Ascites、hepatic hydrothorax
Clinical features[IV]
( Palpation ofliver
firm, hard,irregular,enlargement
rounded or sharp edge
below the right lower ribs.
( The spleen is often palpable, and may be very large.
Complications[I]
? Upper gastrointestinal bleeding(上消化道出血):
Hematemesis(呕血)/melena(黑粪). Esophageal/ gastric variceal bleeding (食管/胃静脉出血);
portal hypertensive gastropathy (门脉高压性胃病);
peptic ulcer(消化性溃疡)
Infections:
spontaneous bacterial peritonitis(自发性细菌性腹膜炎) (4-8%)：Fever, worsening jaundice or renal dysfunction, abdominal pain (occurring only in 50% of patients), and encephalopathy are the most common clinical findings in SBP.
However, the patient is frequently asymptomatic. Because culture of ascites fluid is negative in a large number of patients with SBP, diagnosis should be based on the presence of >250 neutrophils/mm3.
Complications[III]
? Hepatorenal syndrome(HRS):
Oliguria(少尿), azotemia(氮质血
症), hypotension(低血压), dilutional
hyponatremia(稀释性低钠血症), low
urinary sodium(低钠尿)
Complications[IV]
Electrolyte and acid-base imbalance(电介质酸硷平衡失调)
hyponatremia, hypokalemia
And hypochloremic alkalosis
Immunology
? Cellular immune?, hormonal
? immune?autoimmune liver disease:
IgG?,globulin? ANA(+), SMA(+)
? PBC: IgM?, AMA(+)
? Marker of virus
? AFP
Laboratory findings[III]
( Radionuclide:99m TC-MIBI,H/L
( liver biopsy: to confirm the diagnosis
( Laparoscopy
( HVPG (hepatic vein pressure gradient)(肝静脉压力梯度) (wedged - free )hepatic venous pressure
Normal: 5-6mmHg,>10mmHg: varices;
>12mmHg:rupture......（后略) ......
相关资料1：
- 2010-EASL.临床实践指南;肝硬化腹水、自发性腹膜炎、肝肾综合征处理.pdf
- 2009-AASLD原发性胆汁性肝硬化处理指南（中文翻译）.pdf
- 当代名医临证精华__肝炎肝硬化专辑.pdf
- 肝硬化腹水病人的处理_美国肝病学会实践指南相关问答.pdf
- 肝硬化腹水诊治指南评价.pdf
- 实用临床诊疗规范——消化系统疾病(三)——第八节 肝硬化.pdf
- 2004美国肝病学会关于肝硬化腹水的治疗要点.pdf
- 2009-年美国肝病研究学会成人肝硬化腹水处理指南（全文）.pdf
- 2008-肝硬化门静脉高压食管胃静脉曲张出血的防治共识（杭州）.pdf
- 肝硬化门静脉高压食管胃静脉曲张出血的防治共识（2008，杭州）.PDF
- 肝硬化中医辨证施治浅识.pdf
- 肝硬化腹水病人的处理_美国肝病学会实践指南相关问答.pdf
- 原发性胆汁性肝硬化治疗进展.pdf
- Mayo评分与Child分级评估肝硬化患者预后.pdf
- 肝硬化患者肝移植术中血流动力学与机体氧代谢的变化.pdf
- 18.当代名医临证精华 - 肝炎肝硬化专辑.pdf