
Hepatology
An Evidence-Based Clinical Compendium
- 1st Edition - November 5, 2024
- Imprint: Academic Press
- Editors: Sanjaya K. Satapathy, Mamun- Al-Mahtab, Shivaram Prasad Singh, Sheikh Mohammad Fazle Akbar, Aftab Ala, Thomas D Schiano
- Language: English
- Hardback ISBN:9 7 8 - 0 - 4 4 3 - 3 0 0 5 2 - 3
- eBook ISBN:9 7 8 - 0 - 4 4 3 - 3 0 0 5 3 - 0
Hepatology: An Evidence-Based Clinical Compendium provides current and comprehensive insights into the field of hepatology, covering various aspects such as diagnosis, manage… Read more

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Request a sales quoteMoreover, the book goes beyond the discussion of epidemiology, pathogenesis, and pathophysiology, and presents updated treatment and management guidelines for liver diseases, making it a go-to reference for Clinicians treating liver diseases.
- Presents current and comprehensive insights into the diagnosis, management, control, techniques, procedures, and technology in hepatology
- Facilitates evidence-based decision-making for better patient care, enabling practitioners to effectively incorporate research findings into their treatment approaches
- Guides the selection of optimal treatment and management options, equipping readers with valuable resources to overcome challenges encountered in clinical practice
- Enhances knowledge and skills for clinical practice, offering valuable support to healthcare professionals seeking to improve their expertise
- Hepatology
- Cover image
- Title page
- Table of Contents
- Front Matter for Volume 1
- Front Matter for Volume 2
- Copyright
- Copyright
- Dedication
- Contributors for Volume 1
- Contributors for Volume 2
- About the editors
- Foreword
- Preface
- Volume 1
- Chapter 1 Anatomy and histopathology of the liver⊛
- Abstract
- Keywords
- Introduction
- Macroanatomy of the liver
- Microscopic architecture
- Portal tracts
- The biliary system
- Hepatic mesenchyme
- Lobular and acinar functional units
- Cellular microanatomy
- Hepatocytes
- Cholangiocytes
- The hepatic sinusoid
- Innervation
- Heterogeneity
- Summary
- References
- Chapter 2 The liver and immunology⊛
- Abstract
- Keywords
- Introduction
- Cellular populations of the liver: Unique, diverse, special
- Hepatocytes, the functional unit of the liver
- The non-parenchymal cells of the liver
- Hepatic vascular system: both arterial and venous blood feed the liver
- Functions of the liver
- The liver and immunology
- The fundamentals of the hepatic immune system
- Innate immunity
- Acquired or adaptive immunity
- Immunological cascade
- Immunology of hepatocyte, the parenchymal cells of the liver
- Immune-related functions of non-parenchymal cells (NPCs) of the liver
- Liver sinusoidal endothelial cells (LSECs)
- DC
- MDSC
- Granulocytes
- NK cells and NKT cells
- T and B lymphocytes
- Hematopoietic progenitor cell populations in the liver
- Stellate cells
- Kupffer cells (KC)
- The tolerogenic liver
- Liver regeneration and immunity
- The immunological mechanism underlying liver diseases
- Hepatotropic virus-induced liver damage
- HAV and HEV
- HBV-induced liver damages
- HCV-induce liver damages
- Nonalcoholic fatty liver disease (NAFLD): An intrinsic pathological entity resulting from hepatic microenvironment or mishandling of metabolites
- Drug-induced liver injury (DILI)
- Alcohol
- Immune intervention of liver diseases for management
- Fundamental issues of drug development for immune-related liver diseases
- Immune therapy for CHB
- Immune therapy for HCC
- Conclusion
- Summary
- References
- Chapter 3 Bilirubin metabolism and its disorders⊛
- Abstract
- Key words
- Acknowledgments
- Introduction
- Bilirubin formation
- Photochemistry of bilirubin
- Physiological function and health benefits of biliverdin and bilirubin
- Bilirubin toxicity
- Bilirubin throughput
- The role of albumin binding
- Bilirubin uptake by hepatocytes
- Bilirubin conjugation
- Secretion of bilirubin glucuronides into bile
- Reverse transport of bilirubin glucuronides into sinusoidal blood and reuptake by hepatocytes
- Additional transporters in the hepatocyte canalicular membranes
- Coordinated expression of genes involved in bilirubin throughput in the liver
- Bilirubin degradation in the gastrointestinal tract
- Alternative pathways of bilirubin excretion
- Quantification of serum bilirubin
- Quantification by diazo reaction
- Chromatographic analysis
- Transcutaneous bilirubinometry
- Measurement of non-protein bound (free) bilirubin
- Bilirubin in various body fluids
- Bilirubin in plasma
- Bilirubin in bile
- Mechanisms of hyperbilirubinemia
- Neonatal hyperbilirubinemia
- High bilirubin load
- Low hepatic bilirubin uptake
- Immaturity of bilirubin glucuronidation
- Effect of maternal milk
- Maternal serum jaundice
- Low canalicular bilirubin excretory capacity
- Increased intestinal reabsorption
- Hyperbilirubinemia due to bilirubin overproduction
- Inherited disorders of bilirubin glucuronidation
- Crigler-Najjar syndrome: Types
- Crigler-Najjar syndrome type 1 (CN1)
- Investigational therapies
- Crigler-Najjar syndrome type 2 (CN2)
- Gilbert syndrome
- Inherited disorders associated with predominantly conjugated hyperbilirubinemia
- Dubin-Johnson syndrome (DJS)
- Rotor syndrome
- Hyperbilirubinemia resulting from monogenic cholestasis syndromes
- Progressive familial intrahepatic cholestasis type 1 (PFIC-1)
- Progressive familial intrahepatic cholestasis type 2 (PFIC-2)
- Progressive familial intrahepatic cholestasis type 3 (PFIC-3)
- Benign recurrent intrahepatic cholestasis (BRIC)
- Progressive familial intrahepatic cholestasis type 4 (PFIC-4)
- Progressive familial intrahepatic cholestasis type 5 (PFIC-5)
- Progressive familial intrahepatic cholestasis type 6 (PFIC-6)
- Mitochondrial scaffold defects
- Cholangiopathy of North American Indian childhood cirrhosis
- Gracile syndrome
- Structural disorders of known genetic basis
- Alagille syndrome
- Abnormal villin expression
- Summary
- References
- Chapter 4 Non-invasive diagnosis of metabolic dysfunction associated steatotic liver disease (MASLD)⊛
- Abstract
- Keywords
- Introduction
- Clinical association between histology and outcomes
- Limitation of liver biopsy
- Steatosis
- Clinical prediction model
- Ultrasound
- Transient elastography
- Magnetic resonance imaging
- Serum-based: OMICs approach
- Fibrosis
- Non-invasive fibrosis tests
- Imaging
- Serum-based fibrosis biomarkers
- MASH
- Serum based
- Imaging
- Summary
- References
- Chapter 5 Hematologic disorders and the liver⊛
- Abstract
- Keywords
- Introduction
- Disordered hematopoiesis in liver disease
- Brief overview of the coagulopathy of liver disease
- Normal hemostasis
- The coagulopathy of liver disease
- Factor VIII
- Fibrinogen
- Viscoelastic tests
- Procedural bleeding assessment and management
- Anemia in liver disease
- Macrocytic anemia
- Iron deficiency anemia
- Hemolytic anemia
- Aplastic anemia
- Hepatic vein thrombosis (Budd-Chiari syndrome)
- Portal vein thrombosis
- PVT in cirrhosis (without underlying hepatocellular cancer)
- Non-cirrhotic PVT
- Management of bleeding in liver disease
- Variceal bleeding
- Non-variceal bleeding (intractable mucosal and puncture wound oozing)
- Elective procedures in patients with liver disease
- Other hematologic conditions in liver disease
- Coagulopathy in acute liver failure (fulminant hepatic failure)
- Hemophagocytic lymphohistiocytosis
- Summary
- References
- Chapter 6 Acute liver failure (ALF)⊛
- Abstract
- Keywords
- Introduction
- Definition
- Etiology
- Pathogenesis
- Role of ammonia [18,19]
- Loss of cerebral auto regulation [7,20]
- Complications of ALF [7,9,11,13]
- Gender, pregnancy and acute liver failure [7,9,11,13,23]
- Outcome
- Causes of death in ALF
- Management [9,27–30]
- Medical therapy directed to control encephalopathy and cerebral edema and raised intracranial pressure (ICP)
- Therapies directed towards management of complications
- Plasma exchange (PLEX) and bioartificial liver support systems [27,29,31–33]
- Therapy directed at etiology of ALF [27,29]
- Liver transplant and prognostic models [7,9,13,19,27,33–38]
- Summary
- References
- Chapter 7 Acute-on-chronic liver failure⊛
- Abstract
- Keywords
- Introduction
- Development and evolution of the concept of ACLF
- Definition of ACLF
- Epidemiology of ACLF
- Onset and clinical course of ACLF
- Pathophysiology of ACLF
- Gut dysbiosis and ACLF
- Principles of treatment
- Treatment of precipitating event
- Supportive therapy
- Liver transplantation in ACLF
- Prevention of ACLF
- Palliative care in ACLF
- Forecasting trends and unmet needs in ACLF
- Conclusion
- Summary
- References
- Chapter 8 Hepatic fibrogenesis⊛
- Abstract
- Keywords
- Introduction
- Natural history of hepatic fibrosis
- Cellular and molecular features of hepatic fibrosis
- Cellular anatomy of sinusoids (Fig. 3)
- Sinusoidal cellular and extracellular matrix changes in response to injury (Fig. 4)
- Cellular sources of extracellular matrix in hepatic injury and fibrogenesis
- Stellate cell activation: A central feature of hepatic fibrosis
- Local interactions influencing fibrogenesis
- Matrix production (fibrogenesis) and degradation (fibrinolysis)
- Clinical aspects of hepatic fibrosis
- Methods to monitor fibrosis progression or regression
- Invasive methods of diagnosis
- Non-invasive methods of diagnosis
- Emerging antifibrotic targets and strategies
- Summary
- References
- Chapter 9 Cirrhosis, portal hypertension and pathophysiology⊛
- Abstract
- Keywords
- Cirrhosis
- Introduction
- Definition, etiologies, and pathophysiology of cirrhosis
- Diagnosis of cirrhosis
- Portal hypertension
- Hepatic portal system
- Hemodynamics of portal hypertension
- Causes of portal hypertension
- Cirrhosis
- Schistosomiasis
- Portal vein thrombosis
- Idiopathic
- Cardiac-related
- Uncommon causes of portal hypertension
- Portal pressure measurement
- Prognosis of cirrhosis
- Management of cirrhosis
- Summary
- References
- Chapter 10 Ascites and hepatorenal syndrome⊛
- Abstract
- Keywords
- Introduction
- Ascites
- Pathophysiology
- Assessment of ascites
- Prognosis of ascites
- Uncomplicated ascites (Fig. 3)
- Complicated ascites
- Hepatic hydrothorax
- Diagnosis and clinical presentation of hepatic hydrothorax
- Hepatorenal syndrome
- Pathophysiology (Fig. 2)
- Definition, classification, and diagnosis
- Management
- Summary
- References
- Chapter 11 Management of esophageal and gastric varices⊛
- Abstract
- Keywords
- Introduction
- Diagnosis
- Endoscopic diagnosis of gastroesophageal varices
- Non-invasive tests for gastroesophageal varices
- Approach to patients without prior variceal hemorrhage
- Pre-primary prophylaxis of gastroesophageal varices
- Primary prophylaxis of variceal hemorrhage
- Management of acute esophageal variceal hemorrhage
- Blood products
- Antibiotic prophylaxis
- Vasoactive agents
- Endoscopic variceal ligation
- Early transjugular intrahepatic portosystemic shunt
- Secondary prophylaxis of esophageal variceal hemorrhage
- Rescue therapies
- Balloon tamponade
- Esophageal stents
- Transjugular intrahepatic portosystemic shunt
- Overview of gastric varices
- Management of gastric variceal hemorrhage
- Management of gastric varices according to vascular anatomy
- Endoscopic cyanoacrylate injection
- Endoscopic thrombin injection
- Endoscopic ultrasound-guided therapies
- Balloon-occluded retrograde transvenous obliteration
- Transjugular intrahepatic portosystemic shunt
- Gastroesophageal varices and non-cirrhotic portal hypertension
- Summary
- References
- Chapter 12 Hepatic encephalopathy⊛
- Abstract
- Keywords
- Introduction
- Historical perspectives
- Definition and nomenclature
- Epidemiology of HE
- Precipitants and predictors of HE
- Pathophysiology of HE
- Effect of indigenous toxin
- Effect of inhibitory neurotransmission
- Effect of systemic inflammation
- Effect of gut microbiota
- Effect of sarcopenia
- Effect of alteration in amino acid metabolism
- Clinical features
- Ammonia measurement and implications in HE
- Diagnosis of HE
- Diagnosis of covert HE
- Diagnosis of overt HE
- Treatment of HE
- Treatment of MHE
- Treatment of episode of overt HE
- Prevention of overt HE
- Emerging therapies in HE
- Nutrition and HE
- Areas of controversy and future research
- Summary
- References
- Chapter 13 Jaundice/cholestasis⊛
- Abstract
- Keywords
- Introduction
- Jaundice
- Physiology of bilirubin metabolism
- Cholestasis
- Classification of jaundice
- Pre-hepatic jaundice
- Hepatic jaundice
- Post-hepatic jaundice
- Unconjugated hyperbilirubinemia
- Increased bilirubin production
- Decreased hepatic bilirubin uptake
- Impaired hepatic conjugation
- Conjugated hyperbilirubinemia
- Intrahepatic cholestasis
- Inherited disorders of cholestasis
- Autoimmune disorders of cholestasis
- Primary biliary cholangitis (PBC)
- Primary sclerosing cholangitis (PSC)
- Overlap syndrome
- Ig-G4 disease
- Miscellaneous causes of cholestasis
- Total parenteral nutrition
- Vanishing bile duct syndrome
- Sickle cell intrahepatic cholestasis
- Drug-induced cholestasis
- Extrahepatic biliary obstruction
- AIDs cholangiopathy
- History
- Associated symptoms
- Risk factors
- Drug history
- Physical examination
- Demographics
- General examination
- Mental status
- Skin examination
- Abdominal examination
- Treatments
- Pruritus
- Vitamin supplements (ADEK)
- Summary
- References
- Chapter 14 Gallstones and gallbladder disorders⊛
- Abstract
- Keywords
- Introduction
- Biliary tract: Anatomy and physiology
- Bile secretion and the enterohepatic circulation
- Bile acid synthesis and metabolism
- The enterohepatic circulation
- Diseases of the gallbladder
- Developmental anomalies
- Gallstone disease
- Tumors of gallbladder
- Summary
- References
- Chapter 15 Benign biliary stricture: Causes and management⊛
- Abstract
- Keywords
- Introduction
- Clinical manifestation
- Diagnosis
- Post cholecystectomy strictures
- Post liver transplantation
- Primary sclerosing cholangitis
- Infections
- Portal cavernoma cholangiopathy
- IgG4-related sclerosing cholangitis
- Chronic pancreatitis and choledocholithiasis
- Role of good clinical assessment and antibiotics
- Endoscopic methods
- Percutaneous intervention for BBS
- Surgical management of BBS
- Innovative techniques
- Role of endoscopic ultrasound
- Other novel techniques
- Summary
- References
- Chapter 16 Cystic diseases of the liver⊛
- Abstract
- Keywords
- Summary
- Evaluation of liver cysts
- Simple cysts
- Polycystic liver disease
- Autosomal recessive polycystic kidney disease
- Von Meyenburg Complexes
- Caroli's disease
- Cystadenomas
- Echinococcosis
- Malignant cysts
- Future therapeutic options
- Conclusion
- References
- Chapter 17 Congenital cholestatic liver disease⊛
- Abstract
- Keywords
- Article I. Biliary atresia
- Clinical presentation
- Diagnosis
- Management
- Complications
- Liver transplantation
- Article II. Alagille syndrome
- Clinical presentation
- Diagnosis
- Management
- Liver transplantation
- Article III. Progressive familial intrahepatic cholestasis and benign recurrent intrahepatic cholestasis
- PFIC1
- PFIC2
- PFIC3
- PFIC4
- PFIC5
- PFIC6
- Diagnosis
- Medical management
- Surgical management
- Article IV. Bile acid synthesis disorders
- Secondary bile acid synthesis disorders
- Article V. Choledochal cyst
- Classification
- Clinical presentation
- Diagnosis
- Management
- Summary
- References
- Chapter 18 Primary biliary cholangitis⊛
- Abstract
- Keywords
- Introduction
- Epidemiology
- Etiology and pathogenesis
- Natural history
- Prognosis
- Clinical features
- Physical examination
- Diagnostic approach
- Laboratory tests
- Imaging
- Liver biopsy
- Ludwig and Scheuer histologic classification of PBC (Table 3) [92,93]
- Diagnosis
- Clinical characteristics of PBC variants
- AMA negative PBC
- Overlap PBC/AIH
- Management principles
- Pharmacological therapy
- First-line therapy: Ursodeoxycholic acid
- Second line therapy: Obeticholic acid (OCA)
- Off label therapy: Fibrates
- Combination therapy
- New drugs under investigation
- Management of PBC-AIH overlap syndrome
- Monitoring and follow-up
- Symptomatic treatment
- Pruritus
- Fatigue
- Sicca syndrome
- Treatment of metabolic complications
- Osteopenia/osteoporosis
- Hyperlipidemia
- Fat-soluble vitamins
- Liver transplantation
- Challenges and controversies
- Summary
- References
- Recommended reading
- Chapter 19 Primary sclerosing cholangitis⊛
- Abstract
- Keywords
- Introduction
- Epidemiology and pathogenesis
- Diagnosis, natural history, prognosis
- Malignancy in PSC
- Management
- Medical treatment
- Modulation of bile composition
- Modulators of inflammation
- Anti-fibrotics
- Optimizing the gut microbiome
- Endoscopic treatment
- Surgical
- Recurrent PSC and cancer risk
- Summary
- Author contributions
- Financial support
- Conflicts of interest
- References
- Chapter 20 Enterically transmitted viral hepatitis-hepatitis A and E⊛
- Abstract
- Keywords
- Hepatitis A
- Introduction
- Virology
- Epidemiology and transmission
- Pathogenesis
- Clinical features
- Acute hepatitis
- Diagnosis
- Control and prevention of hepatitis a
- Hepatitis E
- Introduction
- Virology
- Epidemiology and transmission
- Pathogenesis
- Clinical features
- Diagnosis
- Treatment
- Control and prevention of Hepatitis E
- Vaccination
- HEV in pregnancy
- References
- Recommended reading
- Chapter 21 Hepatitis B⊛
- Abstract
- Keywords
- Introduction
- Epidemiology
- Screening guidelines
- Molecular biology
- Pathophysiology
- Clinical features
- Acute HBV infection
- Chronic HBV infection
- Acute flare of chronic HBV infection
- HBV and hepatitis D infection
- Diagnostic approaches
- Serological testing
- Molecular testing
- Management
- Acute HBV infection
- Chronic HBV infection
- When to start treatment
- Treatment options
- When to change antiviral medications
- Duration of therapy
- Management in special populations
- Coinfection with HIV
- Coinfection with hepatitis C
- Decompensated cirrhosis/liver transplantation recipients
- Pregnancy
- Renal and bone dysfunction
- Immunosuppression therapy/chemotherapy
- Newer approaches to treatment
- Therapeutic HBV vaccines
- Referral for liver transplantation
- Occult HBV infection
- Prognosis
- Prevention
- Pre-exposure vaccination
- Post-exposure prophylaxis
- Elimination
- Summary
- References
- Chapter 22 Hepatitis C⊛
- Abstract
- Keywords
- Introduction
- Hepatitis C virology
- Natural history of hepatitis C
- Acute hepatitis C
- Chronic hepatitis C
- Transmission of hepatitis C
- Hepatitis C presentation
- Extrahepatic manifestations
- Hematological disorders
- Renal disorders
- Dermatological disorders
- Endocrine disorders
- Other extrahepatic manifestations
- Hepatitis C diagnosis
- HCV RNA assays
- Hepatitis C genotype
- Assessment of fibrosis
- Non-invasive serum modalities to assess fibrosis
- Non-invasive imaging modalities to assess fibrosis
- Liver biopsy
- Treatment
- History of treatment
- Currently available therapies
- Special populations
- Pregnancy
- Pediatrics
- Acute HCV
- Decompensated cirrhosis
- Organ transplant
- DAA adverse effects and drug-drug interactions
- Post-treatment monitoring
- Summary
- References
- Chapter 23 The worldwide medical impact of hepatitis D virus infection: Focus to Central Asia⊛
- Abstract
- Keywords
- Introduction
- Hepatitis D virology and serology
- The complex epidemiology of HDV
- The decline of HDV among local-born populations of high-income countries
- Epidemiology of HDV in the rest of the world
- The changing clinical scenario of hepatitis D
- Focus to Central Asia
- Turkey
- Pakistan
- Epidemiology of HDV infection in former USSR countries
- Treatment
- Pegylated interferon alpha 2a and Nucleos(t)ide Analog Combination therapy
- HDV entry inhibitor: Bulevirtide
- The HDAg prenylation inhibitor: Lonafarnib
- HBsAg secretion inhibitor: REP 2139
- Conclusion and the way forward
- Summary
- Issues to be resolved
- References
- Chapter 24 Hepatitis through non-A–E viruses⊛
- Abstract
- Keywords
- Introduction
- Herpes simplex virus (HSV)
- Varicella zoster virus
- Cytomegalovirus (CMV)
- Epstein Barr virus (EBV)
- Human herpes virus 6 (HHV-6)
- Human herpes virus 7 and 8 (HHV-7, HHV-8)
- Human parvovirus B 19
- Dengue hepatitis
- Coronavirus
- Summary
- References
- Volume 2
- Chapter 25 HIV related liver diseases⊛
- Abstract
- Keywords
- Introduction
- Hepatitis C co-infection
- Hepatitis B co-infection
- NAFLD/NASH
- Medication induced liver disease
- Infiltrative/systemic diseases (non-liver specific)
- AIDS cholangiopathy
- Acalculous cholecystitis
- Summary
- References
- Chapter 26 Autoimmune hepatitis⊛
- Abstract
- Keywords
- Introduction
- Epidemiology
- Incidence, prevalence, gender differences and mortality
- Genetics, race, and ethnicity
- Definition
- Pathophysiology
- Classification
- Other variants
- Diagnosis
- Clinical presentation
- Laboratory markers
- Further testing
- Complications
- Advanced liver disease and cirrhosis
- COVID-19 infection
- Management
- First line
- Second line
- Off-label therapies—Beyond second line
- Liver transplant
- Treatment withdrawal and relapse
- Prognosis
- Treatments in the pipeline
- Summary
- References
- Chapter 27 Drug induced liver injury⊛
- Abstract
- Keywords
- Introduction
- Hepatic drug metabolism
- Acetaminophen hepatotoxicity (APAP-DILI)
- Epidemiology
- Clinical features
- Diagnostic approaches
- Therapeutic approaches
- Challenges and controversies involved in management
- Outcomes of treatment
- Complications of treatment
- Idiosyncratic drug induced liver injury (iDILI)
- Epidemiology
- Drugs associated with iDILI
- Clinical features
- Management principles
- Diagnostic approaches
- Therapeutic approaches
- Challenges and controversies involved in management
- Outcomes of treatment
- Complications of treatment
- Herbal and dietary supplement induced liver injury (HILI)
- Epidemiology
- HDS associated with HILI
- Clinical features
- Management principles
- Diagnostic approaches
- Therapeutic approaches
- Challenges and controversies involved in management
- Outcomes of treatment
- Complications of treatment
- Summary
- References
- Chapter 28 Alcohol-associated liver disease⊛
- Abstract
- Keywords
- Epidemiology of ALD
- Disease modifiers
- Pathogenesis of alcoholic liver disease
- Diagnosis of ALD
- Screening for alcohol use
- Screening for liver disease
- Diagnosis of alcoholic hepatitis
- Treatment of alcohol-associated liver disease
- Treatment of alcohol use disorder
- Medical treatment of liver disease
- Liver transplantation for ALD
- Emerging pharmacological therapies
- Prognosis in ALD
- Emerging biomarkers
- Prevention of alcohol-associated liver disease
- Conclusion and future prospects
- Summary
- References
- Chapter 29 Iron overload and the liver⊛
- Abstract
- Keywords
- Acknowledgments
- Introduction and history of developments in hemochromatosis
- HFE related hemochromatosis and epidemiology
- Mechanisms of iron homeostasis and absorption (Fig. 2)
- Ceruloplasmin and iron
- Hepcidin and its expression (Fig. 3)
- Fibrosis and iron
- Biochemical variations in iron parameters
- Classification of hereditary hemochromatosis iron overload syndromes
- Type 1 HH (OMIM235200)
- Type 2 HH (OMIM 602390, 613313)
- Type 3 HH (OMIM604250)
- Type 4 HH (OMIM606069)
- Type 5 HH (OMIM615517)
- Clinical features of HH
- Diagnosis and work-up (Figs. 4A and 4B)
- Genetic testing, clinical and biochemical penetrance
- Histological evaluation of iron overload
- Screening—Family and population
- Other important iron over load states (Table 3)
- Treatment
- Secondary iron overload and treatment
- Developing future therapies in HH
- Iron homeostasis
- Treatment
- Summary
- References
- Chapter 30 Wilson disease⊛
- Abstract
- Keywords
- Introduction
- Copper metabolism/ATP7B
- Clinical presentation
- Diagnosis
- Management of Wilson disease
- Liver transplantation
- Future potential therapies
- Hepatocyte transplant
- Gene therapy
- Gene repair with CRISPR-Cas9 (clustered regularly interspaced palindromic repeats)
- Summary
- References
- Chapter 31 Metabolic-dysfunction associated steatotic liver disease (MASLD)⊛
- Abstract
- Keywords
- Introduction
- Historical perspective and etymology
- Epidemiology
- Pathogenesis
- Ethnicity and genetic modulation of risk of MASLD
- Lipid trafficking in the liver and the central role of insulin resistance
- Lipotoxicity, oxidative stress, mitochondrial dysfunction and activation of inflammatory pathways
- Adiposopathy
- Gut dysbiosis
- Hepatic parenchymal iron overload
- Cross talk between hepatic steatosis, inflammation and fibrosis
- Pathogenesis in lean MASLD
- Natural history (Fig. 1)
- Dynamic model of MASLD (Fig. 2)
- Clinical features
- Lean MASLD
- Cirrhosis and HCC
- Extra-hepatic associations
- Diagnostic evaluation of MASLD
- Diagnosing fatty liver using non-invasive techniques
- Non-invasive differentiation of MASLD and MASH
- Non-invasive evaluation of fibrosis
- Liver biopsy
- Management
- Holistic approach to managing metabolic co-morbidities
- Weight loss
- MASH specific pharmacotherapy (Table 8)
- Management of MASH cirrhosis and HCC
- Future therapeutic approaches in MASH
- Summary
- Disclosures
- Funding
- Conflict of interest
- Suggested readings
- Chapter 32 Sarcopenia, frailty, and its implications in cirrhosis⊛
- Abstract
- Keywords
- Acknowledgment
- Sarcopenia in cirrhosis
- Causes and mechanisms of sarcopenia in cirrhosis
- Diagnostic criteria and assessment tools for sarcopenia in cirrhosis
- Biomarkers and laboratory assessments
- Frailty in cirrhosis
- Definition and characteristics of frailty
- Prevalence and risk factors for frailty in cirrhosis
- Clinical implications and impact of frailty on cirrhosis outcomes
- Shared pathophysiological mechanisms between sarcopenia and frailty
- Overlapping mechanisms between sarcopenia and frailty in cirrhosis
- Role of gut dysbiosis and systemic inflammation in the development of sarcopenia and frailty
- Clinical implications
- Prognostic significance of sarcopenia and frailty in cirrhosis
- Management strategies for sarcopenia and frailty in cirrhosis
- Implications for cirrhosis screening and surveillance programs
- Summary
- Summary of key findings and implications
- Future research directions and areas for further investigation
- Importance of addressing sarcopenia and frailty in the management of cirrhosis
- References
- Chapter 33 Liver disease in infants and children⊛
- Abstract
- Keywords
- Neonatal cholestasis
- Genetics of pediatric cholestasis
- Hepatitis B in children
- Hepatitis C in pediatrics
- Acute liver failure
- Autoimmune hepatitis and overlap syndrome
- Type 1 AIH, type 2 AIH, and seronegative AIH
- Overlap syndromes
- Post-transplant manifestations of AIH
- Treatment
- Liver tumors
- Frailty and nutrition
- Liver transplantation
- Summary
- References
- Chapter 34 Liver diseases in pregnancy: A comprehensive review⊛
- Abstract
- Keywords
- Introduction
- Physiological changes in pregnancy
- When to suspect liver disease in pregnancy
- Pregnancy-specific liver diseases
- Hyperemesis gravidarum
- Various defining criteria for HG
- Pathophysiology
- Clinical and laboratory parameters
- Treatment and prognosis
- Intrahepatic cholestasis of pregnancy
- Pathophysiology
- Clinical and laboratory parameters
- Treatment and prognosis
- Hypertension-related liver disease and pregnancy
- Pathophysiology
- Clinical presentation
- Management
- HELLP syndrome
- Pathophysiology
- Clinical and laboratory parameters
- Management
- Acute fatty liver of pregnancy
- Pathophysiology
- Clinical and laboratory parameters
- Management
- Conclusion
- Summary
- References
- Chapter 35 Viral hepatitis in pregnancy⊛
- Abstract
- Keywords
- Introduction
- Hepatitis A
- Transmission
- Symptoms
- Diagnosis
- Clinical aspects of HAV in pregnancy
- Treatment
- Prevention
- Hepatitis B
- Transmission
- Symptoms
- Diagnosis
- Clinical aspects of HBV in pregnancy
- Prevention of perinatal transmission of HBV
- Preferred antiviral therapy during pregnancy
- Management of HBV infection
- Hepatitis C
- Transmission
- Symptoms
- Diagnosis
- Clinical aspects of HCV in pregnancy
- Treatment
- Prevention or management of HCV infection
- Hepatitis D
- Transmission
- Diagnosis
- Clinical aspects and management of HDV in pregnancy
- Prevention of HDV infection
- Hepatitis E
- Transmission
- Symptoms
- Diagnosis
- Clinical aspects of HEV in pregnancy
- Treatment
- Management and prevention of HEV infection during pregnancy
- Maternal-to-child transmission
- Pregnancy with advanced cirrhosis
- Summary
- References
- Chapter 36 Liver in systemic diseases—Part 1⊛
- Abstract
- Keywords
- Introduction
- Critically ill patients
- Hypoxic liver injury
- Cholestasis and icterus
- Sclerosing cholangiopathy of critically ill patients
- Drug induced liver injury in critically ill patients
- Cardiovascular diseases
- Acute cardiogenic liver injury
- Chronic venous congestion (syn. congestive hepatopathy)
- Cardiac cirrhosis
- Renal disease
- Renal cell carcinoma and paraneoplastic liver involvement
- Endocrinopathies and liver
- Thyroid diseases and liver
- Hypothyroidism
- Hyperthyroidism
- Cushing syndrome
- Adrenal insufficiency
- Diabetes mellitus
- Gastrointestinal diseases
- Celiac disease
- Inflammatory bowel disease and liver
- Primary sclerosing cholangitis
- Other liver diseases in IBD
- Gallstone disease and IBD
- Nonalcoholic fatty liver disease and IBD
- Portal vein and hepatic vein thrombosis
- Liver abscess and IBD
- Secondary amyloidosis
- Drug induced liver injury
- Intestinal failure associated liver disease
- Connective tissue disorders
- Joint pain and liver
- Systemic lupus erythematosus
- Rheumatoid arthritis
- Sjogren syndrome
- Scleroderma
- Adult Still’s disease
- Polyarteritis nodosa
- Anticardiolipin syndrome
- Polymyalgia rheumatica
- Hematological disorders and liver
- Lymphoproliferative disorders
- Hodgkin's disease and liver
- Non-Hodgkin's lymphoma
- Primary hepatic lymphoma
- Myeloproliferative disorders
- Polycythemia vera
- Primary myelofibrosis
- Myeloproliferative neoplasms and visceral thrombosis
- Chronic lymphocytic leukemia
- Hairy cell leukemia
- Multiple myeloma
- Acute leukemia
- Sickle cell disease and liver
- Thalassemia and liver involvement
- Summary
- References
- Chapter 37 Liver in systemic diseases—Part 2⊛
- Abstract
- Keywords
- Liver in sepsis
- Specific infections and liver
- Enteric fever and liver
- Scrub typhus and liver
- Leptospirosis
- Tuberculosis
- Brucellosis
- Uncommon bacterial infections involving liver
- Q fever
- Syphilis
- Lyme disease
- Protozoal infections affecting liver
- Malaria
- Viral infections affecting liver
- Dengue
- Cytomegalovirus
- Epstein-Barr virus
- Herpes simplex virus
- HIV
- COVID-19
- Fungal infections
- Hepatic candidiasis
- Histoplasmosis
- Coccidioidomycosis
- Miscellaneous conditions
- Amyloidosis
- Cystic fibrosis and liver
- Heat stroke and liver disease
- Obstructive sleep apnea and liver disease
- Sarcoidosis and liver
- Summary
- References
- Chapter 38 Bacterial, fungal and parasitic infections and the liver⊛
- Abstract
- Keywords
- Introduction
- Common hepatic and biliary disease entities
- Pyogenic liver abscess
- Cholangitis
- Cholecystitis
- Etiologies of hepatic and biliary infections
- Bacterial infections
- Fungal infections
- Parasitic infections
- Summary
- References
- Chapter 39 Parasitic diseases of the liver and biliary tract⊛
- Abstract
- Keywords
- Introduction
- Amebic liver abscess
- Hepatobiliary and pancreatic ascariasis
- Echinococcosis
- Cystic echinococcosis
- Alveolar echinococcosis
- Polycystic echinococcosis
- Hepatosplenic schistosomiasis
- Fascioliasis
- Clonorchiasis and Opisthorchiasis
- Summary
- References
- Chapter 40 Benign liver tumors⊛
- Abstract
- Keywords
- Introduction
- General approach to a solid liver lesion
- Focal nodular hyperplasia
- Epidemiology
- Pathophysiology
- Histopathology
- Clinical features
- Management
- Prognosis
- Hepatocellular adenoma
- Classification
- Clinical features
- Radiology
- Management
- Macroregenerative nodule
- Nodular regenerative hyperplasia (NRH)
- Focal hepatic steatosis and focal fatty sparing
- Bile duct adenoma
- Hepatic hemangioma
- Pathophysiology
- Clinical features
- Radiology
- Management
- Surgical management
- Radiological management
- Mesenchymal hamartoma
- Hepatic angiomyolipoma
- Inflammatory pseudotumor of the liver (IPTL)
- Hepatic peliosis
- Summary
- References
- Chapter 41 Malignant liver tumors⊛
- Abstract
- Keywords
- Introduction
- Hepatocellular carcinoma
- Risk factors
- Hepatotropic viruses
- Metabolic risk factors
- Other risk factors
- Gut microbiota and HCC
- Clinical manifestations
- Pathogenesis
- Gross pathology
- Histology
- Immunohistochemistry
- Diagnosis of HCC
- Staging
- Treatment
- Hepatic resection
- Liver transplantation
- Local therapies
- Trans-arterial chemoembolization
- Trans-arterial radioembolization: Table 4
- Radiation therapy in HCC
- Systemic therapy
- Fibrolamellar hepatocellular carcinoma
- Prevention of HCC
- Intrahepatic cholangiocarcinoma
- Introduction
- Classification and nomenclature
- Risk factors
- Clinical presentation
- Diagnostic work up
- Staging
- Treatment
- Surgery
- Chemotherapy
- Liver secondaries
- Introduction
- Epidemiology and etiology
- Molecular pathogenesis of liver metastasis
- Approach to diagnosis
- Management
- Postoperative follow up
- Ablative therapy
- Rare types
- Epidemiology and risk factors
- Diagnosis
- Management
- Epidemiology
- Presentation and diagnosis
- Management
- Conclusion
- Summary
- Disclosures
- Conflicts of interest
- Author contributions
- References
- Suggested reads
- Chapter 42 Liver targeted therapies for benign and malignant tumors of the liver⊛
- Abstract
- Keywords
- Introduction
- Benign tumors of the liver
- Malignant tumors of the liver
- Thermal ablation
- Trans-arterial radioembolization
- Trans-arterial chemoembolization
- External body radiation therapy
- Comparison of locoregional therapies
- Conclusions
- Summary
- References
- Chapter 43 Liver transplantation⊛
- Abstract
- Keywords
- Introduction
- Indications for liver transplantation and recipient evaluation
- Indications for liver transplantation
- Recipient evaluation
- Donor evaluation
- Living donor evaluation
- Deceased donor evaluation
- Extended donor criteria and outcomes
- Donor-recipient matching
- Liver allocation system
- Combined liver transplantation with other organs
- Post-liver transplant complications
- Post-liver transplant infections
- Acute and chronic allograft rejection
- Post-liver transplant malignancy
- Post-liver transplant renal complications
- Post-liver transplant metabolic complications
- Adverse effects of immunosuppression
- Recurrent disease after liver transplantation
- Hepatitis B virus infection
- Hepatitis C virus infection
- Autoimmune liver diseases
- Hepatocellular carcinoma
- Re-transplantation
- Gender and racial disparities in LT
- Summary
- References
- Chapter 44 Liver transplantation in combination with other organs⊛
- Abstract
- Keywords
- Liver transplantation: basics
- Indications for liver transplantation
- Combined liver transplant with other organs
- Simultaneous liver-kidney transplant (SLK)
- Evaluating renal function and considerations for SLKT in end-stage liver disease patients
- Role and limitations of kidney biopsy in simultaneous liver-kidney transplantation
- Assessment of SLKT candidacy in individuals with end-stage renal disease (ESRD)
- Comparing simultaneous liver and kidney transplantation outcomes to liver transplant alone
- Complexities of simultaneous liver-kidney transplantation (SLKT)
- Metabolic dysfunction-associated steatohepatitis (MASH) cirrhosis: Evaluating the appropriate approach—Simultaneous liver-kidney transplantation or liver transplantation alone?
- Conclusion
- Combined liver-lung transplant (CLLT)
- Combined liver-lung allocation system
- Outcomes of combined liver-lung transplant in USA
- Conclusion
- Combined liver-heart transplant (CLHT)
- Outcomes of CHLT
- Conclusion
- Combined liver-pancreas transplant
- Conclusion
- Combined liver-intestine transplant
- Summary
- References
- Chapter 45 Surgical aspects of liver transplantation⊛
- Abstract
- Keywords
- Donor preparation by OPO (Organ Procurement Organization)
- Deceased donor operation
- Split liver procurement for transplantation
- Living donor hepatectomy
- Left lateral segment
- Left lobe
- Right lobe
- Liver preservation/Machine perfusion
- Liver viability on machine perfusion
- Machine perfusion outcome
- Back-table preparation of liver allograft
- Brain death donor
- Living donor
- Orthotopic liver transplant
- Recipient hepatectomy
- Standard (Caval replacement) technique
- Piggyback (Caval preservation) technique
- Venous bypass
- Vascular modifications
- Bile duct reconstruction
- Common operatively-relate liver transplantation complications
- Summary
- References
- Chapter 46 COVID-19 and the liver⊛
- Abstract
- Keywords
- Introduction
- Epidemiology and transmission
- Coronaviruses and liver injury
- Pathogenesis of liver dysfunction
- Risk factors for liver injury
- Liver histology in COVID-19
- Pattern of liver injury in COVID-19
- COVID-19 in the setting of preexisting liver disease
- COVID-19 and alcohol associated liver disease
- COVID-19 and nonalcoholic fatty liver disease
- COVID-19 and hepatitis B
- COVID-19 and hepatitis C
- Autoimmune liver disease and COVID-19
- COVID-19 and hepatocellular carcinoma
- COVID-19 and liver transplantation
- Liver injury in pregnant patients with COVID-19
- COVID-19 and patient care
- Vaccination in CLD patients with COVID-19
- Telemedicine in hepatology during COVID-19
- Prevention and management of liver injury in COVID-19
- Long COVID and liver injury
- Summary
- References
- Chapter 47 Inherited and metabolic disorders of the liver⊛
- Abstract
- Keywords
- Introduction
- Techniques for genetic analysis
- Epidemiology
- Clinical features
- When should one suspect an inherited and metabolic liver disease?
- Clues to diagnosis
- Classification of metabolic liver diseases based on clinical presentation
- Inherited metabolic liver diseases
- Galactosemia
- Hereditary fructose intolerance (HFI)
- Glycogen storage diseases (GSDs)
- Congenital disorders of glycosylation (CDG)
- Tyrosinemia type I
- Urea cycle disorders (UCDs)
- Lysosomal acid lipase (LAL) deficiency
- Familial hypercholesterolemia
- Abetalipoproteinemia
- Gaucher disease (GD)
- Niemann-Pick disease type C
- Bile acid synthesis defects
- Zellweger spectrum disorders
- Wilson disease
- Hereditary hemochromatosis
- Hereditary hyperbilirubinemias [23]
- Miscellaneous
- Syndromes associated with liver disease
- Alagille syndrome
- Trichohepatoenteric syndrome (THES)
- Turner syndrome
- Infantile liver failure syndrome type 2
- Hardikar syndrome
- Aagenaes syndrome
- Ciliopathies
- Alstrom syndrome
- Liver transplantation for inherited diseases
- Recent updates and future directions
- Summary
- References
- Chapter 48 Liver and Radiology⊛
- Abstract
- Keywords
- Introduction
- Ultrasound (US)
- Computed tomography (CT)
- Magnetic resonance imaging (MRI)
- Digital subtraction angiography (DSA)
- Diffuse liver disease
- Hepatic steatosis
- Liver cirrhosis
- Viral hepatitis
- Liver iron overload
- Malignant diffuse liver disease
- Benign focal liver lesions
- Cystic lesions
- Solid lesions
- Malignant focal liver lesions
- Hepatocellular carcinoma
- Fibrolamellar HCC
- Lymphoma
- Angiosarcoma
- Metastases
- Vascular diseases
- Budd-Chiari syndrome
- Portal hypertension
- Portal vein thrombosis
- Benign biliary pathology
- Choledocholithiasis
- Hepatolithiasis
- Benign biliary strictures
- Primary sclerosing cholangitis
- Postoperative strictures
- Mirizzi’s syndrome
- IgG4-related sclerosing cholangitis
- AIDS cholangiopathy
- Acute bacterial cholangitis
- Recurrent pyogenic cholangitis
- Parasitic infections
- Ascaris lumbricoides
- Hydatid disease
- Biliary cystic disease
- Biliary tumors
- Cholangiocarcinoma
- Biliary cystadenoma and carcinoma
- Intraductal papillary neoplasm of the bile duct (IPNB)
- Conclusion
- Summary
- References
- Chapter 49 Gall bladder cancer⊛
- Abstract
- Keywords
- Introduction
- Epidemiology
- Etiology and risk factors
- Age and gender
- Gallstones
- Gallbladder polyps
- Primary sclerosing cholangitis (PSC)
- Anomalous pancreaticobiliary ductal junction (APBDJ)
- Chronic infection
- Toxins and medicines
- Family history and genetic factors
- Lifestyle factors
- Pathogenesis
- Genomics
- Pathology
- Gross pathology
- Diagnosis
- Magnetic resonance imaging (MRI)
- Staging of GBC
- Treatment
- Surgery
- Summary
- References
- Index for Volumes 1 and 2
- Edition: 1
- Published: November 5, 2024
- Imprint: Academic Press
- No. of pages: 1612
- Language: English
- Hardback ISBN: 9780443300523
- eBook ISBN: 9780443300530
SS
Sanjaya K. Satapathy
MA
Mamun- Al-Mahtab
Mamun Al Mahtab is a notable hepatologist, medical scientist, author, and columnist from Bangladesh. With an impressive portfolio of 290 publications in national and international peer-reviewed journals, Dr. Mahtab currently serves as the Head of the Division of Interventional Hepatology at Bangabandhu Sheikh Mujib Medical University (BSMMU). Dr. Mahtab plays a crucial role in medical research and development. He leads the clinical study of the Bangladesh-developed SARS-CoV-2 mRNA candidate, GBPD060. Additionally, he serves as the lead researcher for NASVAC's Phase I/II and III clinical studies. Notably, his discovery of NASVAC earned him the prestigious 'Premio Nacional' award from the Cuban Academy of Sciences in 2019. Apart from his research endeavors, Dr. Mahtab actively organizes awareness programs on liver diseases throughout Bangladesh. He is also credited with inventing the NASVAC drug, which aims to provide an affordable and effective solution for Hepatitis B to the masses in Bangladesh.
SS
Shivaram Prasad Singh
Dr. Shivaram Prasad Singh is a highly accomplished medical professional. He graduated from Utkal University with degrees in MBBS and MD in Internal Medicine. He obtained his DM from C.M.C., Vellore at Madras University, India. Dr. Singh is a Fellow of the Society for Gastrointestinal Endoscopy of India, the American College of Gastroenterology, and the American Gastroenterological Association.
His remarkable contributions include initiating World Hepatitis B Eradication Day in India, endorsed by WHO, and serving on the World Hepatitis Alliance Public Health Panel. Dr. Singh held leadership positions in renowned organizations such as INASL and ISG. He has received prestigious awards, including the ISG S.R. Naik Memorial Award for Research Publications and the Indian Society of Gastroenterology Zydus Oration.
With over 160 publications and numerous national guideline papers, Dr. Singh is an acclaimed authority in gastrointestinal and liver diseases. He currently serves as the President-Elect of INASL.
SA
Sheikh Mohammad Fazle Akbar
Dr. Akbar is an esteemed researcher in epidemiology, clinical studies, and immunology, focusing on chronic diseases like cancers. His molecular analysis has provided valuable insights into HBV, HCV, and HEV in Bangladesh, Nepal, Indonesia, and Japan. He developed a groundbreaking immune therapeutic drug for chronic hepatitis B and cancers, completing successful phase I/II and II clinical trials, with approvals in select countries. As a core member of the US-Japan Cooperative Medical Science Program (USJ CMSP), he contributes to global health research. Dr. Akbar has authored 150+ articles, compiled books, and holds editorial roles.
AA
Aftab Ala
Professor Aftab Ala, MBBS, MD, PhD, FRCP, is Professor of Hepatology and Consultant Hepatologist at the Institute of Liver Studies at King’s College Hospital in London, UK, and the University of Surrey, Guildford, UK, and Chair of the Rare Liver Disease Special Interest Group at the British Association for the Study of the Liver (BASL). He obtained his medical qualification from Charing Cross and Westminster (Imperial College Medical School) in London in 1992. Professor Ala pursued specialized training in Gastroenterology and Hepatology at King’s and Royal Free Hospitals in London and Advanced Hepatology Fellowship at Mount Sinai Hospital in New York, USA, from 2003 to 2004 where he focused on rare liver and inherited metabolic liver diseases and liver transplantation. He subsequently won an International Sheila Sherlock Royal College of Physicians Fellowship at the Center of Excellence for Wilson Disease, Yale University, USA.
Professor Ala earned a PhD from University College, London, in 2005 and received a Wellcome Trust Research Training fellowship successfully focusing on immune and inflammatory mechanisms that connect liver and gut diseases.
Professor Ala has made significant contributions to the field of medicine, research, and education. His ongoing research and clinical trials programs (phases 1–3) in complex liver disease and health inequality are supported by international industry partners, the UK National Institute for Health and Care Research (NIHR) and the UKRI/MRC
TS
Thomas D Schiano
Dr. Thomas D. Schiano is a highly skilled medical professional with expertise in hepatology, gastroenterology, and clinical nutrition. He specializes in the management of cirrhosis, liver diseases, and pre- and post-liver transplantation care. Dr. Schiano's clinical research interests encompass various areas, including alcohol-related liver disease, nutritional aspects of liver disease, recurrence of disease post-transplantation, living donor liver transplantation, and complications of cirrhosis. He is actively involved in the care of patients undergoing intestinal transplantation and is a member of the liver thrombosis program, an international referral center for complex blood clotting disorders affecting the liver and intestines.
As a UNOS-certified Liver Transplant physician, Dr. Schiano holds board certifications in clinical nutrition, gastroenterology, and transplant hepatology. Currently, Dr. Schiano serves as a Professor of Medicine, Medical Director of Adult Liver Transplantation, and Director of Clinical Hepatology at the Recanati/Miller Transplantation Institute. He is also the Medical Director of the Intestinal Transplant program.