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Cirrhosis is a late stage of hepatic parenchymal necrosis and scarring (fibrosis) most commonly due to hepatitis C infection and alcoholic liver disease. Patients may present with jaundice, ascites, and hepatosplenomegaly. Complications of cirrhosis include hepatic encephalopathy, portal hypertension and ascites, and hepatorenal syndrome. Diagnosis is based on clinical, laboratory, and imaging findings. Management requires treating the underlying cause and complications; liver transplantation may be required in some cases.

Wilson Disease (Clinical)

Wilson disease (hepatolenticular degeneration) is an autosomal recessive disorder caused by various mutations in the ATP7B gene, which regulates copper transport within hepatocytes. Dysfunction of this transport mechanism leads to abnormal copper accumulations in the liver, brain, eyes, and other organs, with consequent major and variably expressed hepatic, neurologic, and psychiatric disturbances. Liver involvement may manifest as hepatitis, liver failure, or cirrhosis, while basal ganglia involvement causes the extrapyramidal signs. Most patients are diagnosed between the ages of 5 and 35 years (mean: 13 years). Diagnosis is established if the patient has low plasma ceruloplasmin, corneal deposits of copper (Kayser-Fleischer rings), and elevated copper levels in the urine. However, other tests are often needed since not all patients will have all these findings. The prognosis is good for patients without advanced liver disease and for those who are treated with the chelating agents penicillamine or trientine. Untreated Wilson disease is ultimately fatal, with patients dying from cirrhosis, acute liver failure, or complications due to progressive neurologic disease.

Hepatic Encephalopathy (Clinical)

Hepatic encephalopathy is a reversible condition in which elevated ammonia levels cause impaired brain function in patients with advanced liver disease. Hepatic encephalopathy can be precipitated by conditions that affect the normal absorption, metabolism, or clearance of ammonia, including dehydration, renal failure, infections, and gastrointestinal bleeding. Patients present with a progression of symptoms, from minimal confusion and asterixis to stupor and coma. Diagnosis is clinical and requires the exclusion of alternative diagnoses. Management involves addressing the causative factor and decreasing the systemic absorption of ammonia with lactulose or rifaximin.

Hepatorenal Syndrome (Clinical)

Hepatorenal syndrome (HRS) is a “functional” type of acute kidney injury (AKI) in patients who have portal hypertension, usually due to cirrhosis. HRS may also be caused by other liver diseases which are associated with portal hypertension, which causes decreased systemic arterial blood volume and activation of neurohormonal mechanisms, including the renin-angiotensin-aldosterone system. The resulting vasoconstriction and reduced renal perfusion trigger the release of vasodilatory substances like nitric oxide, which induces splanchnic arterial vasodilatation, leading to a decrease in systemic vascular resistance and effective circulating volume, thereby exacerbating renal hypoperfusion. HRS is a diagnosis of exclusion, and is associated with a poor prognosis. Without treatment, most patients die within weeks of the onset of AKI. Patients typically present with a progressive rise in serum creatinine, a benign urine sediment, no (or minimal) proteinuria, and a low urine sodium concentration. Ascites is often present, and severe cases are associated with oliguria. Treatment is aimed at improving liver function, if possible. Medical therapy includes increasing the systemic blood pressure with norepinephrine and albumin infusions, and administering agents that cause splanchnic vasoconstriction (e.g., terlipressin). Transjugular intrahepatic portosystemic shunt (TIPS) may be successful, and dialysis is a temporizing solution while waiting for liver transplant. The prognosis of patients with HRS is highly dependent upon reversal of the hepatic failure, whether spontaneous, following medical therapy, or following transplantation.

Hereditary Hemochromatosis (Clinical)

Hereditary hemochromatosis (HH) is an autosomal recessive disorder most often associated with HFE gene mutations. Patients have increased iron intestinal absorption and iron deposition in several organs, such as the liver, heart, skin, and pancreas. The clinical presentation is usually with nonspecific symptoms and signs such as fatigue and joint pain in patients over 40, abnormal liver function tests, and hypogonadism in men. Diagnosis is made by laboratory testing showing high transferrin saturation or ferritin indicative of iron overload. Other testing includes genetic testing for HFE variants and MRI to quantify liver or heart iron overload. If untreated, HH can lead to cirrhosis, type 2 diabetes, and bronze-colored skin. Other findings depend on the organ(s) involved. Genetic screening is recommended for family members. Management requires phlebotomy (or iron chelation therapy in some cases) to prevent disease progression. The prognosis depends on the severity of iron overload and organ injury at the time of diagnosis and the extent of iron removal. The presence of hepatic fibrosis is a poor prognostic factor.

Primary Sclerosing Cholangitis (Clinical)

Primary sclerosing cholangitis (PSC) is an inflammatory disease that causes fibrosis and strictures of the intrahepatic and extrahepatic bile ducts. The exact etiology is unknown, but there is a strong association with inflammatory bowel disease (IBD). Patients typically present with an insidious onset of fatigue, pruritus, and jaundice, which can progress to cirrhosis and complications related to biliary obstruction. The diagnosis is established with magnetic resonance cholangiopancreatography (MRCP) or endoscopic retrograde cholangiopancreatography (ERCP). Liver transplantation is the only definitive treatment and is indicated in patients with advanced liver disease.

Primary Biliary Cholangitis (Clinical)

Primary biliary cholangitis (PBC) is a chronic disease resulting in autoimmune destruction of the intrahepatic bile ducts. The typical presentation is that of a middle-aged woman with pruritus, fatigue, and right upper quadrant abdominal pain. Elevated liver enzymes and antimitochondrial antibodies (AMAs) establish the diagnosis. Medical management includes ursodeoxycholic acid as the 1st-line treatment and for symptom management. Definitive treatment is liver transplantation, which is performed in late stages (cirrhosis).

Nonalcoholic Fatty Liver Disease (Clinical)

Nonalcoholic fatty liver disease is a spectrum of liver pathology that arises due to accumulation of triglycerides in hepatocytes. Risk factors include diabetes mellitus, insulin resistance, obesity, hypertension, and medications, among others. Nonalcoholic fatty liver disease ranges from fatty liver or hepatic steatosis but can lead to nonalcoholic steatohepatitis (NASH), which features fatty deposits and inflammation. Progressive liver injury and fibrosis can irreversibly develop into cirrhosis and, possibly, primary liver cancer. Patients are usually asymptomatic, but may present with hepatomegaly and right upper quadrant discomfort. Although liver biopsy is the diagnostic gold standard, the diagnosis can also be established by clinical history, imaging, and laboratory tests. The mainstay of management is lifestyle modifications (weight loss and exercise) with control of associated comorbidities.

Drug-Induced Liver Injury (Clinical)

Drug-induced liver injury (DILI) is the most common cause of acute liver failure (ALF). Hepatotoxic drugs can cause dose-dependent, direct liver injury or idiosyncratic reactions mediated by immune or metabolic processes. This injury can result in hepatitis, cholestasis, steatosis, or overlapping changes. The presentation can be acute or chronic, with severe toxicity manifesting as fulminant liver failure. The diagnosis of DILI requires a thorough history, liver function tests, and drug levels, if available. Management consists of discontinuing the drug, supportive therapy, and monitoring for complications. Acetaminophen is the most common cause of DILI and is treated with a specific therapy, N-acetylcysteine (NAC).

Liver and Biliary Tract Imaging

The hepatobiliary system is composed of the liver, gallbladder, and bile ducts (within the liver and external to the liver). The liver produces bile, which is a fluid made of cholesterol, phospholipids, conjugated bilirubin, bile salts, electrolytes, and water. Bile, which assists in digestion and helps eliminate waste products, is stored in the gallbladder. The hepatobiliary system can be affected by infections, cysts, solid masses, ischemia, and mechanical flow obstruction, which mandate the presence of reliable imaging tests to determine the etiology. The methods that evaluate structural changes in the liver and biliary tract include ultrasonography, CT scan, and MRI (including magnetic resonance cholangiopancreatography). Additionally, cholescintigraphy, a functional imaging study, helps identify gallbladder pathology by tracking the biliary pathway.

Liver Function Tests

Liver function tests, also known as hepatic function panels, are one of the most commonly performed screening blood tests. Such tests are also used to detect, evaluate, and monitor acute and chronic liver diseases. Liver function tests assess the levels of various hepatic proteins and enzymes to determine the state of liver metabolic activity, homeostasis, bile metabolism, and protein synthesis capacity. The standard hepatic panel includes the levels of total protein, bilirubin, albumin, ALT, AST, AST/ALT ratio, and alkaline phosphatase (ALP).

Organ Transplantation

Transplantation is a procedure that involves the removal of an organ or living tissue and placing it into a different part of the body or into a different person. Organ transplantations have become the therapeutic option of choice for many individuals with end-stage organ failure. Transplantation can offer the individual a definitive treatment for a given disease entity. Over the past 50 years, organ transplantation has become established worldwide, with ever-improving results, conferring an immense benefit to hundreds of thousands of individuals. Both solid organs and bone marrowâ€“derived hematopoietic cells can be successfully transplanted for a number of different indications. Tolerance of the transplanted organ by the immune system of the host is achieved through the use of immunosuppressive and immunomodulating strategies. The main complications of transplantation are organ rejection or graft failure; however, chronic immunosuppression also carries the risk of serious complications, including potentially life-threatening infections.

Immunosuppressants

Immunosuppressants are a class of drugs widely used in the management of autoimmune conditions and organ transplant rejection. The general effect is dampening of the immune response. There are multiple targets in the immune system, as well as varied mechanisms in inhibiting inappropriate immune activity. Biologic agents are medications derived from living organisms that target particular components of the immune system. The targets can be tumor necrosis factor (TNF), interleukins (ILs), or B- or T-cell activity. Calcineurin inhibitors halt the activity of calcineurin, a phosphatase involved in T-cell activation. Corticosteroids interfere with the cell cycle of inflammatory cells and modify the activity of other immune components. mTOR inhibitors are proliferation signal inhibitors, reducing immune-cell proliferation. Some immunosuppressants, such as cytotoxic agents, have antineoplastic activity; these are used in rheumatoid arthritis, as prophylaxis for transplant rejection, and for malignant diseases.

Antivirals for Herpes Virus

Antiviral agents against human herpesviruses (HHVs) include acyclovir, cidofovir, and foscarnet. Human herpesviruses are DNA viruses in the Herpesviridae family. Herpes simplex virus (HSV), varicella-zoster virus (VZV), cytomegalovirus (CMV), Epstein-Barr virus (EBV), and HHV-8 belong to the Herpesviridae family. Antivirals against the group generally act via inhibition of DNA polymerase. Acyclovir (the prototypical nucleoside analog) requires viral kinase for phosphorylation to become a triphosphate, which is incorporated in viral DNA. Cidofovir requires phosphorylation by host cellular kinase, which allows cidofovir to have activity against mutated viruses and become deficient in viral kinase. Foscarnet (a pyrophosphate analog) does not require phosphorylation. Nephrotoxicity is a shared adverse effect in the agents. Acyclovir can also cause obstructive crystalline nephropathy and foscarnet carries a risk of electrolyte abnormalities and seizures. The nephrotoxic effect of cidofovir can be reduced with IV saline and probenecid.

Antivirals for Hepatitis B

Antivirals for hepatitis B include the nucleoside/nucleotide analogs, also known as nucleoside/nucleotide reverse transcriptase inhibitors (NRTIs). Because of their similar chemical structure to nucleosides and nucleotides, NRTIs are able to integrate into viral DNA during the replication process. This process inhibits the function of viral RNA-dependent DNA polymerase, resulting in chain termination. All of these medications are administered orally and are excreted by the kidneys. Indications include chronic hepatitis B infection, and some (such as lamivudine) are also used for HIV. Adverse effects include GI symptoms, evidence of mitochondrial toxicity (such as lactic acidosis), and rebound infection upon discontinuation.

Portal Hypertension

Portal hypertension is increased pressure in the portal venous system. This increased pressure can lead to splanchnic vasodilation, collateral blood flow through portosystemic anastomoses, and increased hydrostatic pressure. There are a number of etiologies, including cirrhosis, right-sided congestive heart failure, schistosomiasis, portal vein thrombosis, hepatitis, and Budd-Chiari syndrome. Most individuals are asymptomatic until complications arise, including esophageal varices, portal hypertensive gastropathy, ascites, and hypersplenism. The diagnosis is clinical, but it can be supported by ultrasound findings (and hepatic venous pressure gradient measurement in unclear cases). Management requires treating the underlying etiology and managing the complications. This can include nonselective beta blockers to prevent bleeding from varices, diuretics and sodium restriction for ascites, and transjugular intrahepatic portosystemic shunt for refractory complications.

Hepatitis C Virus

Hepatitis C is an infection of the liver caused by the hepatitis C virus (HCV). Hepatitis C virus is an RNA virus and a member of the genus Hepacivirus and the family Flaviviridae. The infection can be transmitted through infectious blood or body fluids and may be transmitted during childbirth or through IV drug use or sexual intercourse. Hepatitis C virus can cause both acute and chronic hepatitis, ranging from a mild to a serious, lifelong illness including liver cirrhosis and hepatocellular carcinoma (HCC). Hepatitis C infection is diagnosed by testing for the presence of HCV antibodies and HCV RNA. Management is supportive but includes direct antiviral agents (DAAs) if infection does not resolve spontaneously.

Hepatitis B Virus

Hepatitis B virus (HBV) is a partially double-stranded DNA virus, which belongs to the Orthohepadnavirus genus and the Hepadnaviridae family. Hepatitis B virus is transmitted by exposure to infectious blood or body fluids. Examples of types of exposure include sexual intercourse, IV drug use, and childbirth. The virus can cause potentially life-threatening liver disease. Most individuals with acute HBV infection are asymptomatic or have mild, self-limiting symptoms. Chronic infection can be asymptomatic or create hepatic inflammation, leading to liver cirrhosis and hepatocellular carcinoma (HCC). Management of acute hepatitis is typically supportive. Administration of antivirals or liver transplantation may be necessary in fulminant and chronic cases.

Cirrhosis

Cirrhosis is a late stage of hepatic parenchymal necrosis and scarring (fibrosis) most commonly due to hepatitis C infection and alcoholic liver disease. Patients may present with jaundice, ascites, and hepatosplenomegaly. Cirrhosis can also cause complications such as hepatic encephalopathy, portal hypertension, portal vein thrombosis, and hepatorenal syndrome. Diagnosis is clinical. Management requires treating the underlying disease, managing complications, and, if required, liver transplantation.

Ascites

Ascites is the pathologic accumulation of fluid within the peritoneal cavity that occurs due to an osmotic and/or hydrostatic pressure imbalance secondary to portal hypertension (cirrhosis, heart failure) or non-portal hypertension (hypoalbuminemia, malignancy, infection). Patients often present with progressive abdominal distention and weight gain. Abdominal exam may reveal shifting dullness and a positive fluid wave. Diagnosis is established with an ultrasound, and etiologies can be distinguished by ascitic fluid analysis from paracentesis. Treatment involves dietary sodium restriction, diuretics, and treatment of the underlying cause.

Nonalcoholic Fatty Liver Disease

Nonalcoholic fatty liver disease is a spectrum of liver pathology that arises due to accumulation of triglycerides in hepatocytes. Risk factors include diabetes mellitus, insulin resistance, obesity, and hypertension, among others. Nonalcoholic fatty liver disease ranges from fatty liver or hepatic steatosis but can lead to nonalcoholic steatohepatitis (NASH), which features fatty deposits and inflammation. Progressive liver injury and fibrosis irreversibly develop into cirrhosis and, possibly, primary liver cancer. Patients are usually asymptomatic but may present with hepatomegaly and right upper quadrant discomfort. Although liver biopsy is the diagnostic gold standard, the diagnosis can also be established by clinical history, imaging, and laboratory tests. The mainstay of management is lifestyle modifications (weight loss and exercise) with control of associated comorbidities.

Budd-Chiari Syndrome

Budd-Chiari syndrome is a condition resulting from the interruption of the normal outflow of blood from the liver. The primary type arises from a venous process (affecting the hepatic veins or inferior vena cava) such as thrombosis, but can also be from a lesion compressing or invading the veins (secondary type). The patient typically presents with hepatomegaly, ascites, and abdominal discomfort. Onset is often subacute or chronic. Diagnosis is confirmed by Doppler ultrasound. Treatment involves addressing the underlying condition that caused the venous occlusion. Further management involves prevention of further clotting (anticoagulation), restoration of blood flow, and decompressing the liver. Liver transplantation is considered if initial treatment fails and/or the patient has decompensated liver cirrhosis.

Primary Biliary Cholangitis

Primary biliary cholangitis (PBC) is a chronic disease resulting in autoimmune destruction of the intrahepatic bile ducts. The typical presentation is that of a middle-aged woman with pruritus, fatigue, and right upper quadrant abdominal pain. Elevated liver enzymes and antimitochondrial antibodies (AMAs) establish the diagnosis. Medical management is limited to using ursodeoxycholic acid, a disease-modifying agent. Definitive treatment is liver transplantation, which is performed in late stages (cirrhosis).

Benign Liver Tumors

The most common benign liver tumors include hepatic hemangiomas, focal nodular hyperplasia, and hepatic adenomas. These tumors are mostly asymptomatic and/or found incidentally on abdominal imaging. While these tumors are benign, large lesions can cause symptoms such as upper abdominal pain, or produce complications such as bleeding. Malignant potential is a concern for hepatic adenoma, depending on risk factors. The diagnosis is based on imaging studies, with characteristic findings defining the tumor. Biopsy generally is reserved for equivocal cases. Management is observation for most small, asymptomatic, and non-growing tumors. However, high-risk factors, symptoms, increasing tumor size, and complications dictate the need for surgical intervention.

Pyogenic Liver Abscess

A pyogenic liver abscess is a polymicrobial infection arising from contiguous or hematogenous spread. Pyogenic liver abscess is the most common type of visceral abscess. Patients may present with a triad of fever, malaise, and RUQ pain. Laboratory analysis can be informative with elevated WBC and abnormal liver function tests, and imaging may reveal solitary or multiple lesions on ultrasound or CT scan. On contrast imaging, the lesions generally appear well defined with rim enhancement. Diagnosis requires aspiration with Gram stain and culture and, in some cases, a drainage catheter may be placed. A combination of drainage and IV antibiotic therapy is the primary method of treatment. Surgical drainage or resection is utilized in specific cases.

Rare Malignant Liver Tumors

Although hepatocellular carcinoma (HCC) is by far the most common malignant liver tumor, there are several rare malignant tumors that are important to keep in mind when making a differential diagnosis. These tumors include cholangiocarcinoma; hepatoblastoma and mesenchymal tumors, such as epithelioid hemangioendothelioma (EHE); and angiosarcoma. While these conditions differ in their etiology and pathology, they often present similarly, with nonspecific symptoms such as fatigue, weight loss, and abdominal discomfort. Diagnosis is by imaging, which can be supported by a biopsy. Surgical excision is the only curative approach for these tumors.

Hepatitis E Virus

The hepatitis E virus (HEV) is a small nonenveloped virus that contains linear, single-stranded, positive-sense RNA, making it similar to norovirus. Transmission of HEV is via the fecal–oral route and is clinically similar to that of hepatitis A. However, unlike hepatitis A, hepatitis E is quite severe, especially in pregnant women, and may cause fulminant hepatitis along with hepatic encephalopathy over a period of approximately 8 weeks. Management is mainly preventive and includes avoiding contaminated drinking water, good sanitation, and adequate personal hygiene.

Alpha-1 Antitrypsin (AAT) Deficiency

Alpha-1 antitrypsin deficiency is a genetic disorder caused by inherited genetic mutations of the SERPINA1 gene, causing the defective production of the protease inhibitor alpha-1 antitrypsin (AAT). These mutations can lead to deficiency of the enzyme causing lung disease, production of an abnormal form of the enzyme leading to liver dysfunction, or both. Patients commonly present with emphysema, spontaneous pneumothorax, cirrhosis, hepatitis, or hepatocellular carcinoma. There is no known cure. Management is supportive and includes infusion of AAT, treatment of comorbidities, and liver transplantation. Prognosis may vary based on the form of disease contracted and the severity of symptoms.

Hepatitis A Virus

The hepatitis A virus (HAV) is a nonenveloped virus of the Picornaviridae family with single-stranded RNA. The virus replicates in the liver, is excreted in the bile, and is found in high concentrations in the stool of acutely infected individuals. The 2 main routes of infection are consumption of contaminated food or water and direct contact with an infected person. HAV causes an acute, highly contagious hepatitis with unspecific prodromal symptoms such as fever and malaise followed by jaundice and elevated liver transaminases. Most individuals recover fully within a few months, and the immunity resulting from HAV infection is lifelong. Unlike hepatitis B and C, HAV infection does not result in chronic infection or chronic liver disease. Preventive vaccination is available for HAV and is recommended for individuals with increased risk of exposure and, in some countries such as the United States, for all children > 12 months of age.

Hepatitis D Virus

Hepatitis D virus (HDV) is a small enveloped, single-stranded RNA virus. Hepatitis D virus is considered a satellite virus, as it requires the presence of hepatitis B virus (HBV) for assembly and secretion. Therefore, in order for an individual to contract hepatitis D, coinfection or superinfection with HBV is required. Like HBV, HDV is transmitted parenterally, through unprotected sexual intercourse, or perinatally. Clinical presentation is that of a classical viral hepatitis, including coinfection of HDV and HBV, which is considered the most serious form of hepatitis because of the high mortality rate. For acute cases, management is supportive, whereas for chronic cases, pegylated interferon alfa (PEG-IFN-α) is needed.

Alcoholic Liver Disease

Alcoholic liver disease is a spectrum of disorders ranging from fatty liver to cirrhosis secondary to chronic alcohol abuse. Excessive and prolonged consumption of alcohol results in impairment of the lipolysis pathway, causing inflammatory changes within the hepatocytes. Patients typically present during the hepatitis stage with jaundice, fever, and abdominal pain. Diagnosis is based on a history of alcohol abuse and confirmed by laboratory derangement with an AST/ALT ratio > 2. Alcoholic liver disease carries a high mortality rate if patients present with severe hepatitis. Management aims at alcohol abstinence for reversal (at certain stages) and addressing contributing factors (such as viral infections or drugs) to minimize damage to the hepatocytes. Approximately 10% of patients regress with alcohol abstinence during the hepatitis stage. Cirrhosis is frequently irreversible.

Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is an inflammatory disease that causes fibrosis and strictures of the bile ducts. The exact etiology is unknown, but there is a strong association with inflammatory bowel disease (IBD). Patients typically present with an insidious onset of fatigue, pruritus, and jaundice, which can progress to cirrhosis and complications related to biliary obstruction. The diagnosis is established with magnetic resonance cholangiopancreatography (MRCP) or endoscopic retrograde cholangiopancreatography (ERCP). Liver transplantation is the only definitive treatment and is indicated in patients with advanced liver disease.

Drug-induced Liver Injury

Drug-induced liver injury (DILI) is the most common cause of acute liver failure (ALF). Hepatotoxic drugs can cause injury to the hepatocytes directly in a predictable dose-dependent way or through idiosyncratic reactions (which may be mediated by immune or non-immune processes). The injury mechanisms can have the following effects: hepatitis, cholestasis, vascular lesions, or overlapping changes. The presentation can be acute or chronic, with severe toxicity manifesting as fulminant liver failure. The diagnosis of DILI requires a thorough history and laboratory tests including liver function tests (LFTs) and drug levels, if available. Management consists of discontinuing the drug, supportive therapy, and monitoring for complications. Acetaminophen, 1 of the most common causes of DILI, has a specific treatment, N-acetylcysteine (NAC).

Hereditary Hemochromatosis

Hereditary hemochromatosis (HH) is an autosomal recessive disorder most often associated with HFE gene mutations. Patients have increased iron intestinal absorption and iron deposition in several organs, such as the liver, heart, skin, and pancreas. The clinical presentation includes the triad of cirrhosis, diabetes, and skin bronzing. Other findings depend on the organ(s) involved. Diagnosis consists of iron studies, showing transferrin (Tf) and ferritin elevation. Genetic screening is recommended among family members. Imaging and invasive studies are performed depending on the associated complications. Management requires phlebotomy (or iron chelation therapy in some cases) to prevent disease progression. The prognosis is good for patients who are early in the disease and undergoing treatment. The presence of hepatic fibrosis is a poor prognostic factor.

Hepatic Encephalopathy

Hepatorenal Syndrome

Hepatorenal syndrome (HRS) is a potentially reversible cause of acute kidney injury that develops secondary to liver disease. The main cause of HRS is hypovolemia, often as a result of forced diuresis or drainage of ascites. This leads to renal vasoconstriction resulting in hypoperfusion of the kidneys. Patients typically present with edema, oliguria or anuria, and ascites in the setting of acute or chronic liver injury. Hepatorenal syndrome is considered a diagnosis of exclusion. Treatment is with agents that cause systemic vasoconstriction and, therefore, improve renal perfusion. This includes octreotide and midodrine. Most cases of hepatorenal syndrome have a poor prognosis.

Liver

The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism (carbohydrates, proteins, lipids, hormones), nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. The liver can be divided into 4 lobes or 8 segments. Microscopically, it is divided into hepatic lobules. Its main neurovascular bundle is found within the transverse fissure of the liver, also called the porta hepatis.

Wilson Disease

Wilson disease (hepatolenticular degeneration) is an autosomal recessive disorder caused by various mutations in the ATP7B gene, which regulates copper transport within hepatocytes. Dysfunction of this transport mechanism leads to abnormal copper accumulations in the liver, brain, eyes, and other organs, with consequent major and variably expressed hepatic, neurologic, and psychiatric disturbances. Liver involvement may manifest as hepatitis, liver failure, or cirrhosis, while basal ganglia involvement causes the extrapyramidal signs. Most patients are diagnosed between the ages of 5 and 35 years (mean: 13 years). Diagnosis is established if the patient has low plasma ceruloplasmin, corneal deposits of copper (Kayser-Fleischer rings), and elevated copper levels in the urine. However, other tests are often needed since not all patients will have all these findings. The prognosis is good for patients without advanced liver disease and who are treated with the chelating agents penicillamine or trientine. Untreated Wilson disease is ultimately fatal, with patients dying from cirrhosis, acute liver failure, or complications due to progressive neurologic disease.

Autoimmune Hepatitis

Autoimmune hepatitis (AIH) is a rare form of chronic liver disease in which the immune system attacks the liver causing inflammation. It predominantly affects women. Clinical presentation ranges from asymptomatic cases to patients that present with symptoms of acute liver failure (jaundice, right upper quadrant pain). Because AIH has a 40% mortality in 6 months without treatment, early diagnosis and treatment are imperative. Diagnosis is established by detecting anti-smooth muscle antibodies and performing a confirmatory liver biopsy. Treatment includes corticosteroids and azathioprine. If treatment is initiated early in the disease course, the prognosis is favorable.

Liver Disease: Introduction and Commonly Used Liver Test 07:40 min

Liver Disease: Introduction and Commonly Used Liver Test 5 quiz questions

Liver Disease: Introduction and Commonly Used Liver Test 3 Concept Pages
Hepatitis A 02:44 min

Hepatitis A 3 quiz questions

Hepatitis A 1 concept page
Hepatitis B 07:05 min

Hepatitis B 4 quiz questions

Hepatitis B 3 Concept Pages
Diagnosis of Hepatitis B Virus Infection (HBV) and Interpretation of HBV Serology 06:13 min

Diagnosis of Hepatitis B Virus Infection (HBV) and Interpretation of HBV Serology 6 quiz questions

Diagnosis of Hepatitis B Virus Infection (HBV) and Interpretation of HBV Serology 1 concept page
Management of Hepatitis B 01:54 min

Management of Hepatitis B 2 quiz questions

Management of Hepatitis B 1 concept page
Hepatitis C 08:35 min

Hepatitis C 7 quiz questions

Hepatitis C 1 concept page
Hepatitis D & E 02:24 min

Hepatitis D & E 4 quiz questions

Hepatitis D & E 2 Concept Pages
Viral Hepatitis: Overview 02:09 min

Viral Hepatitis: Overview 1 quiz question

Viral Hepatitis: Overview 3 Concept Pages
Other Types of Viral Hepatitis 03:11 min

Other Types of Viral Hepatitis 2 quiz questions

Other Types of Viral Hepatitis 2 Concept Pages
Autoimmune Diseases 03:41 min

Autoimmune Diseases 2 quiz questions

Autoimmune Diseases 2 Concept Pages
Autoimmune Hepatitis 01:53 min

Autoimmune Hepatitis 2 quiz questions

Autoimmune Hepatitis 2 Concept Pages
Primary Biliary Cirrhosis 03:55 min

Primary Biliary Cirrhosis 3 quiz questions

Primary Biliary Cirrhosis 3 Concept Pages
Primary Sclerosing Cholangitis 06:35 min

Primary Sclerosing Cholangitis 3 quiz questions

Primary Sclerosing Cholangitis 3 Concept Pages
Hereditary Liver Diseases 01:00 min

Hereditary Liver Diseases 1 quiz question

Hereditary Liver Diseases 3 Concept Pages
Hereditary Hemochromatosis 04:41 min

Hereditary Hemochromatosis 6 quiz questions

Hereditary Hemochromatosis 3 Concept Pages
Wilson's Disease 05:46 min

Wilson's Disease 7 quiz questions

Wilson's Disease 3 Concept Pages
Alpha-1 Antitrypsin Deficiency: Diagnosis 04:44 min

Alpha-1 Antitrypsin Deficiency: Diagnosis 4 quiz questions

Alpha-1 Antitrypsin Deficiency: Diagnosis 1 concept page
Alcoholic Liver Disease 03:33 min

Alcoholic Liver Disease 2 quiz questions

Alcoholic Liver Disease 2 Concept Pages
Alcoholic Hepatitis 06:30 min

Alcoholic Hepatitis 6 quiz questions

Alcoholic Hepatitis 2 Concept Pages
Alcoholic Cirrhosis 02:34 min

Alcoholic Cirrhosis 2 quiz questions

Alcoholic Cirrhosis 2 Concept Pages
Non-alcoholic Steatohepatitis (NASH) 04:41 min

Non-alcoholic Steatohepatitis (NASH) 3 quiz questions

Non-alcoholic Steatohepatitis (NASH) 4 Concept Pages
Drug-Induced Liver Disease & Acetaminophen Toxicity 03:48 min

Drug-Induced Liver Disease & Acetaminophen Toxicity 3 quiz questions

Drug-Induced Liver Disease & Acetaminophen Toxicity 3 Concept Pages
Fulminant Liver Failure 06:49 min

Fulminant Liver Failure 2 quiz questions

Fulminant Liver Failure 4 Concept Pages
Liver Diseases of Pregnancy 07:19 min

Liver Diseases of Pregnancy 7 quiz questions

Liver Diseases of Pregnancy 0 Concept Pages
Types of Hepatobiliary Tumors 01:58 min

Types of Hepatobiliary Tumors 4 quiz questions

Types of Hepatobiliary Tumors 2 Concept Pages
Malignant Liver Tumors 02:06 min

Malignant Liver Tumors 3 quiz questions

Malignant Liver Tumors 1 concept page
Benign Liver Tumors 01:06 min

Benign Liver Tumors 3 quiz questions

Benign Liver Tumors 1 concept page
Space-Occupying Lesions (SOLs) 01:56 min

Space-Occupying Lesions (SOLs) 3 quiz questions

Space-Occupying Lesions (SOLs) 1 concept page
End-Stage Liver Disease 15:02 min

End-Stage Liver Disease 10 quiz questions

End-Stage Liver Disease 5 Concept Pages
Portal Hypertension 04:36 min

Portal Hypertension 3 quiz questions

Portal Hypertension 1 concept page
Variceal Hemorrhage 03:18 min

Variceal Hemorrhage 2 quiz questions

Variceal Hemorrhage 1 concept page
Spontaneous Bacterial Peritonitis 02:36 min

Spontaneous Bacterial Peritonitis 2 quiz questions

Spontaneous Bacterial Peritonitis 0 Concept Pages
Hepatorenal Syndrome 02:07 min

Hepatorenal Syndrome 6 quiz questions

Hepatorenal Syndrome 2 Concept Pages
Hepatic Encephalopathy 01:58 min

Hepatic Encephalopathy 3 quiz questions

Hepatic Encephalopathy 2 Concept Pages
Liver Transplantation 01:36 min

Liver Transplantation 2 quiz questions

Liver Transplantation 2 Concept Pages

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Liver Diseases: Basic Principles with Carlo Raj

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Your Educators of course Liver Diseases: Basic Principles with Carlo Raj

Carlo Raj, MD

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