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发表于 2002-11-23 11:38
5. Liver Involvement in Extrahepatic Disorders. The liver may be affected by numerous conditions, particularly autoimmune disorders, in which the immune system attacks the body's own normal tissues. Some examples include rheumatic diseases (such as systemic lupus erythematosus and rheumatoid arthritis) and inflammatory bowel diseases (such as ulcerative colitis and Crohn's disease).
5.牵连的肝脏肝外异常。肝脏会受很多情况影响,最常见的是自身免疫异常,免疫系统攻
击身体的正常组织。例子有风湿病(如系统性红斑狼疮,类风湿关节炎)和爆发性肠病(如
溃疡性肠炎和克罗恩氏病(节段性回肠炎)。
Systemic infections, such as tuberculosis, candidiasis, and toxoplasmosis, may spread to the liver. In addition, heart failure can lead to liver congestion, scarring, and ascites, because blood cannot drain from the liver properly when the heart is not pumping effectively.
系统性感染,如肺结核,念珠菌病,弓形体病,会扩散到肝脏。另外,心脏衰竭会使肝脏充
血,起疤痕,腹水,因为当心脏泵力不够时,血不能正常从肝脏排出,
Clinical Manifestations of Liver Dysfunction
肝功能不良的临床表现
A variety of insults can damage the liver, but their signs and symptoms are similar and may be due to hepatic dysfunction and/or obstruction to blood or bile flow. These manifestations include symptoms and signs of hepatitis, jaundice, cirrhosis, hepatocellular carcinoma, and hepatic failure.
很多侵害会损伤肝,但他们的征候和症状相似,可能由肝功不良造成,也可能由血流或胆汁
受阻造成。这些表现包括肝炎的症状,黄疸,硬化,肝癌,和肝衰竭。
1. Natural History of Hepatic Disease. Hepatocyte injury, such as occurs in acute viral hepatitis infection, stimulates an inflammatory response designed to try to eliminate the cause of the injury. Hepatocytes are frequently damaged and destroyed during the course of the inflammation; the death of these cells is termed hepatocellular necrosis. This type of injury may be initially well tolerated due to a large reserve of functioning hepatic tissue (compensated liver disease), and the injury may be repaired by regeneration with minimal loss.
1.肝病的自然进程。肝细胞损伤,如发生急性病毒性感染时,刺激强烈的反应,目的是清
除侵害原。肝细胞经常在急性的发炎过程中受伤害和破坏;这些死去的细胞称作肝细胞坏疽。
该类型的损伤一般会由肝组织强大的储备能力承受,损伤会被再生能力修复到最小损失。
However, with severe, continuing, or repeated damage, such as in chronic hepatitis, compensatory regeneration eventually fails to repair the whole injury (it may be too large and/or the framework may have been destroyed), and the destroyed hepatocytes are replaced by scar tissue (fibrosis), leading to cirrhosis. The liver is a resilient organ and can tolerate a certain amount of cell loss, but eventually a threshold is reached and liver function declines markedly. Such decompensation may be manifested by the following:
然而,严重,持续,反复的伤害,例如慢性肝炎,补偿性的再生最终不能修复全部损伤(可
能因为太大或框架被破坏),受损的肝细胞被疤痕组织代替(纤维化),导致硬化。肝脏是
有回复力的器官,可以承受一定量的细胞损失,但最终到达极限,肝功能显著下降。这样的
失代偿有如下表现:
?synthetic defect - fewer normally functioning liver cells reduce the amounts of serum albumin and coagulation factors (a marked abnormality in blood clotting is associated with a poor prognosis)
甲:合成能力缺陷——缺乏正常功能的肝细胞减少了血清白蛋白和凝血因子的数量(血液凝
结明显异常,预后不良)
?abnormal clearance - decreased hepatic clearance of gut-absorbed proteins and ammonia can produce hepatic encephalopathy, a poisoning of the brain with symptoms ranging from confusion to coma
乙:反常清除——对肠吸收的蛋白质清除减少,氨可以造成肝性脑病(脑中毒,症状从混乱
到昏迷)
?abnormal excretion - accumulation of serum bilirubin, which is normally taken up by the liver and excreted into bile, resulting in jaundice
丙:异常排出——血清胆红素聚积,形成黄疸。正常时应由肝吸收并分泌到胆汁。
?ascites - increased sinusoidal pressure, as with severe inflammation or scarring of the liver, leads to fluid accumulation in the abdomen that becomes more difficult to control with progressive decompensation
丁:腹水——窦状小管压力升高,伴随肝脏严重发炎或结疤,导致体液积聚到腹部,使更难
控制进一步的失代偿。
?portal hypertension - scarred liver tissue acts as a barrier to blood flow and causes increased portal blood pressure; a major risk is the rupture of esophageal varices, resulting in massive bleeding that may be fatal
戊:门脉高压——斑痕的肝组织成为血流的障碍,造成门脉血压升高;主要危险是食道血管
曲张破裂,后果是大出血,甚至致命。
2. Inflammation/Hepatitis. As described previously, hepatitis involves inflammation and damage of normal liver cells and is most commonly associated with viral and toxic insults. In general, the diagnosis is often made by excluding various viral, toxic, and metabolic etiologies until only one remains. For hepatitis B and hepatitis C, however, tests are available that may definitively detect the presence of virus in the blood.
2. 发炎/肝炎。如上所述,肝炎涉及发炎和正常的肝细胞受损,经常伴随病毒或毒物侵害。
一般,作诊断要考虑排除各种病毒,毒素,免疫原因等,直到剩下唯一的原因。对于乙肝和
丙肝,有很好的检测方法,可以精确探测血液中存在的病毒。
a. Acute hepatitis. Clinically, patients with hepatitis may be completely asymptomatic and without jaundice. More commonly, they complain of such symptoms as anorexia (loss of appetite), nausea, weakness, headache, muscle aches, altered small or taste, aversion to foods or tobacco, fever, abdominal pain, and jaundice. Hepatitis is generally considered acute if it resolves without sequelae (long-term changes) within six months. Serological tests for hepatitis A IgM should be positive if the person has acute hepatitis A. If the person has acute hepatitis B, the serological markers that may be positive include hepatitis B surface antigen, hepatitis B e antigen or hepatitis B IgM core antibody.
a. 急性肝炎。临床上,肝炎患者可以是完全无症状、无黄疸的。更普遍的,他们会抱怨诸
如厌食,恶心,无力,头痛,肌肉痛,味觉改变,厌恶食物或烟草,发烧,腹痛,黄疸等症
状。肝炎若愈后6个月内无后遗症(长期变化),一般认为是急性的。有急性甲肝的人血
清学检测甲肝IgM阳性。如果人有急性乙肝,血清学标志有乙肝表面抗原,核心抗原或乙
肝IgM核心抗体。
b. Fulminant hepatitis. Rarely, a severe hepatitis results in acute, massive destruction of large portions of the liver or the entire organ. The most common causes are viruses and drug reactions. Decompensation rapidly occurs, manifested by encephalopathy, fever, marked jaundice, and either an enlarged tender liver or a shrunken liver, with a severe decline in liver function. The risk of death is high, and survival depends on the ability of the liver to regenerate. If patients recover, they do so fully. Hepatitis B is most likely to cause fulminant hepatitis; hepatitis C is a less frequent cause, while A causes fulminant hepatitis only rarely.
b. 爆发性肝炎。偶尔的,严重的肝炎导致大部分或全部肝器官急性、大量被破坏。最常见
的原因是病毒或药物反应。迅速发生失代偿,表现为肝性脑病,发烧,显著黄疸,肝扩大或
缩小,肝功能严重下降。死亡的危险高,存活依赖于肝的再生能力。如果患者复原,他们可
以完全恢复。乙肝最常引起爆发性肝炎;丙肝其次,甲肝引起爆发性肝炎很罕见。
c. Chronic hepatitis. Chronic hepatitis implies ongoing liver inflammation that has existed for more than six months. Chronic hepatitis is most likely caused by hepatitis B or C, if there is an infectious etiology. The anti-HCV test will be positive in the case of the latter, and HBsAg test positive in the former. The presence of HBEAG is indicative of ongoing hepatitis B infection. Symptoms vary greatly and may include fatigue, abdominal pain, and jaundice. A symptomatic or mildly symptomatic patients may escape diagnosis until a routine checkup reveals abnormal liver chemistries or an enlarged liver. Importantly, such persistent inflammation may lead to progressive liver scarring and cirrhosis.
c. 慢性肝炎。慢性肝炎意味着肝脏持续发炎超过6个月。慢性肝炎最常由乙肝或丙肝引起,
如果有感染源的话。丙肝会检测到anti-HCV阳性,乙肝会检测到HbsAg阳性。出现HBEAG
阳性表示正在进行的乙肝感染。症状有很大不同,包括:疲倦,腹痛,黄疸。单一症状或轻
微症状的患者可能不会就珍,直到例行体检发现肝脏化学指标异常或肝脏扩大。重要的是,
这种持续的发炎可以导致进一步的肝脏疤痕和硬化。
Historically, histologic patterns such as chronic persistent hepatitis (CPH) and chronic active hepatitis (CAH) were used to predict disease progression and prognosis. These classifications were originally developed to describe types of idiopathic autoimmune chronic active hepatitis (IACAH). It has since been recognized that in chronic viral hepatitis, histological activity is not directly related to prognosis. Instead, the presence and replicative activity of the virus are most important in determining disease progression. Although histologic examination is still important in clinical assessment, it has been proposed that chronic hepatitis be classified primarily by etiology see Figure 10., without using histology to imply prognosis.
历史上,象慢性稳定型肝炎(CPH)和慢性活动型肝炎(CAH)这样的组织学方式用预测病程和
预后情况。这些分类法是来源于描述先天性自体免疫型慢性活动型肝炎(IACAH)的发展进
程。现已广泛认为,在病毒性肝炎中,组织学活性和预后情况不直接关联。替代为,病毒的
存在活性和复制活性对决定预后情况更重要。虽然在临床评估中组织学检测还很重要,现已
提议慢性肝炎应按病因分类(见Figure 10),而不用组织学方法来判断预后。
Firgure(10)
d. Carrier state. Carriers are completely asymptomatic individuals who harbor the virus without evidence of liver inflammation or damage. Liver chemistries and tissue biopsies are normal. Lifespan is considered normal, barring additional independent insults to the liver. The carrier state most commonly occurs with hepatitis B infection, in about 5% of those infected as adults.
d. 携带状态。携带者是完全无症状的个体,载有病毒,但没有肝脏发炎或受损的证据。肝
脏化学指标和组织活检正常。预期寿命正常,除非有额外的对肝脏的侵害。携带者最常见于
乙肝感染者,占成年感染者中的5% 。
3. Jaundice. A patient with jaundice has a yellow appearance, due to increased serum bilirubin (hyperbilirubinemia) , with deposition of bile pigment in the skin, mucous membranes, and sclera (whites of the eyes).
3.黄疸。有黄疸的患者有黄色外貌,由血液中胆红素升高引起,胆红素沉积在皮肤,黏膜
和巩膜(眼白)。
Jaundice does not occur until the serum bilirubin exceeds 2 to 2.5 mg/dL and is generally asymptomatic. However, a jaundiced person may complain of pruritus , or itching of the skin; this is more common with chronic cholestasis. In addition, stools may be "clay-colored" (due to decreased biliary pigment excreted into the gut) and urine may be dark (due to increased bilirubin excreted in the urine).
黄疸在血液胆红素超过2~2.5 mg/dL时才发生,通常无症状。然而,有黄疸的人会抱怨
皮肤瘙痒;这常伴有慢性胆汁淤积。另外,大便会呈“黏土色”(由于胆红素排泄到肠减少)
尿会发黑(由于尿中胆红素增加)。
4. Cirrhosis. Cirrhosis is the common endpoint of many chronic liver diseases, since inflammation and cell death eventually yield to fibrosis, or scar formation. Cirrhosis involves irreversible damage to the lobular architecture, with diffuse fibrous bands of scar tissue surrounding nodules of regenerating hepatocytes. A pathologic diagnosis based on liver biopsy, cirrhosis is described as micronodular if the nodule diameter is less than 3 mm and macronodular if it is more than 3 mm.
4.硬化。硬化通常是很多慢性肝病的终点,因为发炎和细胞死亡最后产生纤维化,形成疤
痕。硬化包括不可逆的小叶结构损伤,伴随扩散的纤维化的带状疤痕组织,围绕着新生细胞
形成的结节。病理诊断依据肝活检;如果结节直径小于3mm,硬化可以描述为微结节,如
果超过3mm,可描述为大结节。
Patients with cirrhosis have significantly shortened life spans and are at risk for decompensation and hepatic failure, as well as the development of hepatocellular carcinoma.
肝硬化患者会明显缩短寿命,并有肝失代偿和肝衰竭的危险,并有发展成肝癌的可能。
Chronic viral hepatitis, chronic autoimmune hepatitis, and alcoholic liver disease are the predominant causes of cirrhosis in the United States. Viral infection can be investigated by checking specific blood tests, such as serum antigens or antibodies (discussed in Section III). Autoimmune liver disease may be detected by discovering certain autoimmune antibodies in the blood. Alcoholic cirrhosis can usually be inferred from a history of chronic alcohol consumption. A liver biopsy may be helpful in distinguishing causes, but in some patients, an exact etiology cannot be determined (cryptogenic cirrhosis). Cirrhosis may also be caused by metabolic diseases (Wilson's disease, hemochromatosis, and alpha 1 - antitrypsin deficiency, all of which can be diagnosed by laboratory tests and/or liver biopsy) or toxins (diagnosed by positive history for drugs, environmental or industrial toxins, and confirmatory lab investigation).
在美国,慢性病毒性肝炎,慢性自体免疫性肝炎,酒精肝是造成肝硬化的主要原因。病毒感
染可以由专门的血液检查来判断,如血清抗原或抗体(在第3节讨论)。自体免疫肝病可
以通过检查血中特定自免疫抗体来发现。酒精肝可以由长期酗酒史推断。肝活检有助于辨别
病因,但有些患者,不能准确判断病因(隐发性硬化)。
硬化也可由代谢疾病引起(威尔逊氏病,血色沉着病,α-1型胰岛素缺乏症,以上均可在
实验室检验或经肝活检确诊)或由毒素引起(可以经用药史,接触环境或工业毒素史,或实
验室检查判断)。
Cirrhosis may be clinically silent for many years except for progressive weight loss, fatigue, and chronic jaundice. Eventually, liver failure and portal hypertension develop, with deepening jaundice, bleeding from esophageal varices, intractable ascites, and encephalopathy. Death usually occurs as a result of bleeding, hepatic coma, infections (such as spontaneous bacterial peritonitis, or infection of ascitic fluid), or kidney failure.
肝硬化可能很多年都没有临床表现,除非出现持续的体重减轻,疲劳,慢性黄疸,肝衰竭和
门脉高压等症状,伴随深度黄疸,食道曲张、出血,难治型腹水,及肝性脑病。通常以下原
因导致死亡:出血,肝昏迷,感染(自发的细菌性腹膜炎,腹水引起的感染),或肾衰竭等。
5. Hepatocellular Carcinoma. Primary malignant cancer of the liver, or hepatocellular carcinoma (HCC), is associated with a dismal prognosis since it is usually diagnosed at a late stage. Cirrhosis often progresses to the development of this tumor; in fact, about 75% to 95% of patients who develop HCC have cirrhosis. This tumor is also associated with certain hepatitis viruses (B and C), as indicated by its prevalence in certain areas of the world where the incidence of viral hepatitis is high.
5.肝癌。大部分恶性肝癌(HCC)都预后很糟,因为通常他们都在后期发现。硬化经常发
展成肿瘤;实际上,有75%到95%的肝癌患者有肝硬化。肿瘤还与特定的病毒有关(HBV,
HCV),世界上病毒流行地区发病率也高。
a. Etiology/epidemiology. HCC is most common in Africa, the Far East (Taiwan, southeast China, Japan), and southern Europe, probably due to the high incidence of chronic viral hepatitis in these areas.
a.病因学/流行病学。肝癌最常见于非洲,远东(台湾,中国南部,日本),和南欧。可能
因为这些地区慢性病毒性肝炎发病率高。
In the United States, approximately 3 people per 100,000 develop HCC each year. HCC is more common in men, particularly in their 50s and 60s. It generally occurs in those with long-standing cirrhosis associated with alcoholism, post-viral hepatitis B and C, hemochromatosis, or alpha 1 - antitrypsin deficiency. HCC rarely develops in patients with cirrhosis due to Wilson's disease or primary biliary cirrhosis.
在美国,每年大约十万分之三的人发生肝癌。肝癌常见于男性,特别是50到60岁间。它
通常发生于因酒精中毒,感染HBV、HCV,血色沉着病,α-1型胰岛素缺乏症等引起长期
肝硬化者身上。肝癌极少发生于威尔逊氏症引起的硬化或早期胆汁型硬化者。
b. Pathogenesis. Chronic inflammatory diseases of the liver, as in other tissues, may increase the risk of developing cancer. Nodular regeneration in cirrhotic livers may lead to cellular dysplasia (alteration in shape, size, and organization of cells), since errors are more likely to be made in more actively dividing cells. This is most likely an intermediate step in the progression to carcinoma, the proliferation of mutated cells. Additionally, excessive cell turnover probably increases the susceptibility to carcinogens (agents that may induce cancer) and may promote the expression of oncogenes ("cancer geners"). Hepatitis B genomic material (fragments of hepatitis B virus DNA) has been found integrated in cancer cells, but the significance of this finding is not clear.
b. 发病机理。慢性肝炎,如同在其他组织的炎症,会增加发展成癌症的风险。肝硬化生成
的小结节会使肝细胞分裂异常(形状,大小,细胞组织结构变化),因为分裂旺盛的细胞更
易产生错误。变异的细胞分裂,很可能是癌变的中间过程。另外,大量的细胞更新可能增加
接受致癌物质的机会,促进致癌基因的表达。乙肝遗传物质(HBV病毒DNA片段)被发
现整合在癌细胞中,但该发现的意义还不清楚。
c. Clinical features and prognosis. Hepatocellular carcinoma is usually well advanced by the time patients present with symptoms, which include right-upper quadrant pain (often a "dull ache"), weight loss, anorexia, nausea, fever, or sudden worsening of jaundice or ascites. Severe pain may be associated with bleeding into the liver or abdomen. On physical exam, the liver may be stony hard. Unfortunately, the prognosis is dismal, primarily because the disease is at an advanced stage at diagnosis (extensive hepatic enlargement and/or metastatic spread to the lungs). Complete surgical removal of the affected tissue offers the only potential cure. Chemotherapy is ineffective, and most patients die within 3-6 months of presentation (primarily from liver failure or bleeding).
c.临床特征和预后。患者表现出症状时,肝癌往往已是晚期。这些症状包括:右上第四象限
区疼痛(常是“钝痛”),体重减轻,厌食,恶心,发烧,或突然恶化为黄疸或腹水。严重
的疼痛可能和血流入肝或腹部有关。身体检查,肝会象石一样硬。不幸的是,预后很糟,主
要因为该病确诊时都在晚期(肝部扩大,或癌扩散到肺)。全部靠手术切除受影响肝组织只
有可能的疗效。化疗无效,多数患者在发现后3到6个月死亡(一般从肝衰竭或出血算起)。
6. Hepatic Failure. Hepatic failure involves the systemic complications associated with severe liver injury and dysfunction. It may occur in a patient without pre-existing liver disease or may be superimposed on chronic liver injury. The diagnosis of acute liver failure requires the presence of symptoms, including jaundice and encephalopathy. Mortality exceeds 50%, even in the best circumstances. Management involves general supportive measures until the liver can regenerate and resume function. In acute liver failure without pre-existing disease, liver transplant may be life-saving.
6.肝衰竭。肝衰竭包括与严重的肝损伤和功能失调相关的全身性并发症。它可在没有既往
肝病史的患者上发生,或在过去慢性肝损伤基础上发生。急性肝衰竭的诊断要依据症状表现,
包括黄疸和肝性脑病。死亡率超过50%,甚至在医疗条件最好的情况下。护理包括一般的
生命支持相关测量直到肝能再生和恢复功能。无既往肝病史的急性肝衰竭患者,肝移植可以
挽救生命。
a. Underlying conditions. About 2000 patients a year develop fulminant hepatic failure in the United States. The most common cause is viral hepatitis, followed by hepatotoxic drugs.
a. 基本情况。在美国,每年大约有2000患者发生爆发性肝衰竭。最常见的原因史病毒性
肝炎,其次是药物性肝炎。
b. Clinical features. Fulminant hepatic failure impairs all liver functions, causing decreased bilirubin metabolism (jaundice), decreased clearance of ammonia and gut-derived proteins (encephalopathy), and decreased clotting factor production (coagulopathy ). It may also cause kidney failure (such as hepatorenal syndrome respiratory failure (due to infection, water accumulation, or inflammation of the lungs), shock (severe fall in blood pressure), and sepsis (systemic infection, probably a result of decreased clearance of portal bacteria by the liver).
b. 临床特征。爆发性肝衰竭会削弱所有的肝功能,造成胆红素代谢减少(黄疸),氨和肠
吸收的蛋白质清除减少(肝性脑病),凝血因子合成减少(凝血困难)。它还可造成肾衰竭
(例如肝肾综合症)呼吸衰竭(例如感染,积水,或肺炎),休克(严重低血压),和脓血
症(全身性感染,可能是肝脏对门脉血中细菌清除减少的原故)。
Without a liver transplant, more than 50% of patients will die, usually from a combination of the above conditions. However, if patients survive without transplantation, their livers may regenerate to normal functional capacity, especially if the liver was healthy before failure began.
没有肝移植,超过50%的患者死亡,经常是死于以上的合并症。然而,如果患者不经肝移
植而存活,他们的肝会恢复到正常的功能容量,尤其是衰竭开始前肝是健康的情况。
Summary
概要
Liver disease may have a variety of origins. Inflammation, or hepatitis, may be caused by infection (especially viral infection), exposure to toxins (including alcohol and drugs), or autoimmune reactions. Portal hypertension and metabolic diseases, such as Wilson's disease and hemochromatosis, can also cause significant liver problems. In addition to benign primary tumors and malignant metastases, the liver may develop hepatocellular carcinoma, which carries a dismal prognosis. The liver may also become involved in a number of systemic conditions, including autoimmune disorders, infections, and heart failure.
肝病有很多原因。肝炎,可以由感染(特别是病毒感染),毒素(包括酒精和药物),或自
体免疫反应造成。门脉高压和代谢性疾病,象威尔逊氏病和血色沉着病,能导致严重的肝脏
问题。除良性的原发肿瘤和恶性转移外,肝会发生肝细胞癌,预后不好。肝还会被很多全身
性疾病影响,包括自体免疫失调,感染,和心脏衰竭。
Regardless of the specific cause, liver disease frequently presents similar clinical manifestations. Initially, symptoms may be mild, since this resilient organ can compensate for a certain amount of damage. However, eventually decompensation occurs, characterized by a marked decline in liver functions and the development of ascites and portal hypertension.
不管什么原因,肝病经常有类似的临床表现。基本上,症状是轻微的,因为这样有恢复力的
器官能代偿很大量的伤害。然而,最终发生失代偿,特征是显著的肝功下降,发生腹水和门
脉高压。
Hepatitis may be acute (with recovery within six months), fulminant (acute critical illness with high mortality rate), or chronic (persistent disease for more than six months). Manifestations of hepatic desease include jaundice (hyperbilirubinemia), cirrhosis (irreversible fibrosis and scarring), hepatocellular carcinoma (primary malignant cancer of the liver), and hepatic failure (severe decline in all liver functions).
肝炎可以是急性的(在6个月内痊愈),爆发性的(突然急性爆发,高死亡率),或慢性
的(持续超过6个月)。肝病的表现包括黄疸(高胆红素),硬化(不可恢复的纤维化和
疤痕),肝癌(肝原发性癌症),和肝衰竭(全部肝功能严重下降)。
[ This page was updated by liver411 on 2001-12-26.06:09:41. ]
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