Pathologie (Fach) / Leber (Lektion)

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• Hypoxämische Lebernekrosen 1. Läppchenzentrale Nekrosen: Eosinrote, kernlose nekrotische Hepatozyten 2. Normales Lebergewebe: In der Umgebung der Portalfelder sind die Leberzellen regelrecht 3. Intermediäre Verfettung: Zwischen Läppchenperipherie und Läppchenzentrum finden sich die Zeichen einer feintropfigen Leberzellverfettung (optisch leere Vakuolen). 
• Chronische Blutstauung der Leber Ätiologie:- Chronische Insuffizienz der rechten Herzkammerwand- Budd-Chiari-Syndrom (Hindernis zwischen Leber und rechter Vorhof)- Pericarditis constrictiva 1. Läppchenzentrale Erythrozytenvermehrung: Erweiterung zentraler Sinusoide 2. Ausbildung von Stauungsstrassen 3. Verfettung, Atrophie und Untergang des Leberparenchyms im Bereich der Stauung: Bei länger bestehender Stauung entwickelt sich - durch Sauerstoffmangel - eine Leberzellverfettung. 4. Formalinpigment
• Non-alcoholic steatohepatitis Spectrum: Steatosis ↔ Steatohepatitis (NASH) → Fibrose → Zirrhose Etiology:- Individuals with metabolic syndrome (3 of the following 5: obesity, hypertension, diabetes, hypertriglyceridemia, hyperlipidemia)- Medication (amiodarone, glucocorticoids, estrogen, antiretroviral drugs) Pathophysiology:↑ Insulin resistance- ↑ Peripheral lipolysis- ↑ Triglyceride synthesis- ↑ Hepatic uptake of fatty acids Histopathology:- Steatosis: White vacuoles- Steatohepatitis: Mallory-denk bodies (tangles of intermediate filaments), neutrophils- Chronic steatohepatitis: Stellate cells lay down fibrotic tissue → fibrosis & cirrhosis Macroscopic:- Steatosis: Large, soft, yellow, greasy Diagnosis:- ↑ Transaminases (AST/ALT ratio < 1)- Imaging studies: US, CT, MRI- Biopsy: Liver with >5% fat content is abnormal
• Alcoholic liver disease Leading cause of liver disease in Western nations.Increase in NADH/NAD+ ratio causes ↑ fat production.ROS react with proteins and DNA → cell damage. Alcoholic fatty liver (reversible):- Mostly asymptomatic- A sensation of pressure in the upper abdominal area occurs in some patients.- Hepatomegaly: soft in consistencyAlcoholic hepatitis (reversible in mild cases):- Nonspecific symptoms: nausea, loss of appetite, weight loss, low-grade fever with tachycardia- Hepatomegaly with hepatic tenderness- Jaundice- Symptoms of withdrawal in alcohol-dependent individuals- Splenomegaly and ascites may be present.- Chronic and persistent course leads to alcohol-related cirrhosis.Alcohol-related cirrhosis (irreversible) Histology:- Alcoholic fatty liver: White vacuoles of fat- Alcoholic hepatitis: Hydropic swelling and ballooning degeneration of hepatocytes; Mallory bodies (damaged intermediate filaments as a signal of hepatocyte necrosis), neutrophilic leukocytosis; formation of fibrous collagenous connective tissue in perivenous zones- Cirrhosis: Infiltration of lymphocytes; massive accumulation of fat in hepatocytes; portal-portal and portal venous fibrous septa with increasingly cirrhotic changes Diagnosis:- AST > ALT, ↑ GGT, ↑ serum ferritin, macrocytic anemia↑ CDT (carbohydrate-deficient transferrin)- Thrombocytopenia, hypoglycemia- Ultrasound: ↑ Liver echogenicity- CT: ↓ liver attenuation
• Leberverfettung Ätiologie:- Stoffwechselstörung (metabolisches Syndrom)- Länger bestehender Sauerstoffmangel- Alkohol 1. Optisch leere Vakuolen 2. Verteilungsmuster der Leberverfettung:- Zentrale Verfettung → hypoxämisch (stauungsbedingt)- Periphere Verfettung → Intoxikation- Intermediäre Verfettung → chronische Blutstauung oder chronischer Sauerstoffmangel- Unregelmässig verteilte Verfettung → Hepatitisformen 3. Schweregrad< 10% = geringe Verfettung10-30% = mittelgradige Verfettung31-50% = schwere Verfettung> 50% = Fettleber
• Fettleberhepatitis (Steatohepatitis) 1. Grosstropfige Leberzellverfettung: Zytoplasmavakuolen, die den Kern zur Peripherie verdrängen 2. Hydropische Schwellung der Hepatozyten: Vorwiegend die läppchenzentralen Hepatozyten sind geschwollen und aufgelockert 3. Mallory-Bodies: Intrazytoplasmatische eosinrote Verdichtungen sind Zeichen einer Zytoplasmateilnekrose ('alkoholisches Hyalin') 4. Entzündliche Reaktion um Zellnekrosen 5. Körnige Schwellung der Hepatozyten 6. Periportalfibrose: Bindegewebsvermehrung in den Portalfeldern 7. Zentrilobuläre Maschendrahtfibrose: Entsteht durch läppchenzentrale Leberzellnekrosen 8. Cholestase 9. Siderose
• Portale Leberzirrhose 1. Pseudolobuli: Regeneratknoten, die keine oder exzentrische Zentralvenen einschliessen 2. Septen: Kollagenfaserreiche Bindegewebsstreifen, die die Portalfelder untereinander oder mit der Zentralvene verbinden 3. Entzündungszeichen 4. Zeichen der Progredienz: Unscharfe Grenzen zwischen Septen und Parenchym 5. Proliferation von Pseudogallengängen (Cholangiolen) in den Portalfeldern und Septen 6. Weitere Veränderungen: Verfettung, Siderose, Mallory-Körperchen, Cholestase, etc.
• Cirrhosis Condition caused by chronic damage to the liver. Etiology:- Hepatotoxicity→ Long-standing alcohol abuse (one of the most common causes of chronic liver disease in the USA)→ Medications (e.g., acetaminophen, amiodarone or chemotherapy drugs such as methotrexate)→ Ingesting aflatoxin created by Aspergillus- Inflammation→ (Chronic) viral hepatitis B, C, and D → Primary biliary cirrhosis→ Primary sclerosing cholangitis→ Autoimmune hepatitis- Parasitic infections (e.g., schistosomiasis, leishmaniasis, malaria)- Metabolic disorders→ Non-alcoholic steatohepatitis (NASH) → Hemochromatosis→ Wilson's disease→ Alpha‑1 antitrypsin deficiency- Hepatic vein congestion or vascular anomalies→ Budd-Chiari syndrome→ Cardiac cirrhosis (congestive hepatopathy) Classification: Child-Pugh score- Parameters: Serum albumin, serum bilirubin, INR, ascites, hepatic encephalopathy Pathology:- Fibrosis- Bile duct proliferates- Replacement of normal liver tissue with collagenous regenerative nodules (histological staging is based on the size of the regenerative nodules)Micronodular = 1-3 mm→ Following a chronic active process, e.g. chronic hepatitis B or C; alcoholic hepatitisMacronodular = > 3 mm→ Following diffuse parenchymal necrosis with relapses or acute course; e.g. relapse or fulminant viral hepatitis; intoxications (e.g., death cap poisoning)
• Cholestase Intra- und/oder extrazelluläre Ablagerung von Galle.- Das morphologische Korrelat eines Ikterus (Bilirubinkonzentration >20 mg/dl) 1. Gallepigment: v.a. läppchenzentral. Gallenthromben sind rundliche, amorphe, gelbbraune bis schwarze Massen zwischen den Leberbälkchen. 2. Schwellung und Netznekrosen der Hepatozyten 3. Entzündliche Veränderungen: Granulozyten, Lymphozyten, Histiozyten 4. Gallenseen: Rupturierte, stark ausgeweitete und mit Galle angefüllten portalen Gallengängen. Ruft eine Portalfibrose hervor. 5. Ductulusproliferation am Rand der Portalfelder.
• Akute Virushepatitis Viren:Hepatitis A-E, EBV, CMV, Gelbfieber, Coxsackie, Herpes, verschiedene Formen des hämorrhagischen Fiebers 1. Leberzelldegeneration: Nekrotische Hepatozyten werden als Councilman-Körper bezeichnet. 2. Ballonzellen: Hydroptisch geschwollene Leberzellen 3. Eosinrotes Zytoplasma 4. Lymphoplasmazelluläre Infiltration; Kupffer-Sternzellen (Sternzellknötchen)
• Autoimmune hepatitis - Most often in young women- Associated with HLA-DR3 and DR4, Hashimoto's thyroiditis, Grave's disease Symptoms:Nonspecific symptoms: Fatigue, abdominal pain, weight loss- Signs of acute liver failure (∼ ⅓ of patients): Jaundice, RUQ pain, fever- Hepatosplenomegaly- ↑ transaminases (ALT > AST) Type 1: 80% of cases- Anti-neuclear-antibodies (ANAs)- Anti-smooth-muscle antibodies (ASMA)- ↓ Albumin, ↑ Prothrombin time Type 2: - Anti-LKM-1 (liver kidney microsome-1), anti-LC (liver cytosol-1), anti-SLA (soluble liver antigen)- Often young women Differential diagnoses:- Viral hepatitis (e.g., hepatitis C)- Primary sclerosing cholangitis- Primary biliary cirrhosis- Alcohol-related hepatoxicity Therapie:- Initial therapy: combination of prednisone and azathioprine- Maintenance therapy: azathioprine or prednisolone
• Autoimmunhepatitis Diagnosestellung 1. Antikörper:- ANA: anti-nuclear antibodies- SMA: Smooth-muscle antibodoes- LKM: Liver-kidney microsomal antibodies- SLA/LP: Soluble liver antigen and liver-pancreas antigen 2. IgG 3. Leberhistologie vereinbar mit/typisch für Autoimmunhepatitis 4. Abwesenheit einer Virushepatitis Summe: ≥6: Wahrscheinliche Autoimmunhepatitis≥7: Definitive Autoimmunhepatitis
• Primary biliary cholangitis Chronic progressive liver disease of autoimmune origin that is characterized by destruction of the intralobular bile ducts. - Associated with autoimmune hepatitis and Sjögren's syndrome- F: M = 9:1 Clinical features:- Fatigue (usually the first symptom)- Marked generalized pruritus- Hepatomegaly, dull lower margin, RUQ discomfort- Splenomegaly- Jaundice- Maldigestion- Xanthomas and xanthelasma Diagnosis:- ↑ Anti-mitochondrial antibodies (AMAs) (> 95%), ↑ ANA (up to 70%), ↑ IgM- ↑ Cholestasis parameters (ALP, γ-GT, conjugated bilirubin) Histopathological stages:Stage I: lymphocytic infiltration of portal areas and periductal granulomasStage II: bile duct ductopenia, progressive fibrosisStage III: bridging fibrosisStage IV: liver cirrhosis Treatment:- Ursodeoxycholic acid- Pruritus treatment: Cholestyramin- Liver transplant
• Primär-biliäre Zirrhose Stadium I: Gallengangsläsion in den Portalfeldern Stadium II: Proliferation der Ductuli Stadium III: Faservermehrung mit septaler Narbenbildung- Schwund der Gallengänge (vanishing bile duct) Stadium IV: Zirrhose Mallory-Körperchen können auch hier vorkommen.- Bei PBC → Läppchenperipher- Bei Alkoholhepatitis → Zentroazinär
• Viral hepatitis 5 types: - Hepatitis A: Contaminated water; travelers- Hepatitis E: Undercooked seafood, water, fulminant in pregnant women- Hepatitis C: Childbirth, sex, drug-users; chronic; diagnose with RNA level- Hepatitis B: Childbirth, sex, drug-user; chronic in 20% but increased in children; associated with HCC- Hepatitis D: Can only infect if host has HBV Symptoms:- Fever, malaise, nausea- Hepatomegaly, pain- Jaundice Hepatocytes undergoing apoptosis = Councilman bodies Diagnosis:- ALT > AST, ↑ unconjugated and conjugated bilirubin → brown urine (urobilinogen)- Atypical lymphocytosis (large from antigen stimulation) If it lasts longer than 6 months → chronic 
• Hemochromatosis Unusually high absorption of iron (4 mg/day; normal = 1 mg/day). - Age ~50 years (slighly later in women) Types:- Primary (hereditary) hemochromatosis: Genemutation in HFE on chromosome 6 → C282Y or H63D mutation. Associated with HLA-A3- HFE mutation → defective binding of transferrin to its receptor → liver stops producing hepcidin → unregulated ferroportin causes ↑ iron reabsorption in duodenal enterocytes- Secondary hemochromatosis: Frequent blood transfusions, hemolytic diseases, porphyria cutanea tarda Iron can cause free radicals using the Fenton reaction → cellular damage → cell death → tissue fibrosis. Clinical features:- Asymptomatic in 75% of cases- Abdominal pain, hepatomegaly → liver cirrhosis (+ hepatocellular carcinoma)- Fatigue, lethargy- Hyperpigmented, bronze skin - Signs of diabetes mellitus (polydipsia, polyuria)- Arthralgia, chondrocalcinosis- Erectile dysfunction, testicular atrophy, loss of libido, amenorrhea- Cardiomyopathy (restrictive or dilated) → congestive heart failure, arrhythmias - Type I diabetes melitus (destruction of islet cells) or malabsorption (destruction of exocrine pancreas) Diagnosis:- ↑ iron, ↑ transferrin saturation (normal: 15-45%), total iron binding capacity ↓, ↑ ferritin- Liver biopsy: Brown spots (DD: Lipofuscin) → Confirm with Prussian blue stain Treatment: Phlebotomy, deferoxamine
• Eisenüberladung Frage an Pathologie:- Bestätigung einer Eisenüberlatung (Genotyp vs. Phänotyp)- Ausmass der Eisenüberladung und Fibrose- Behandlungseffekte Eisenpigment: Grobkörniges, intrazytoplasmatisches Pigment von rostbrauner Eigenfarbe. Lässt sich selektiv mit der Berliner-Blau-Reaktion darstellen (im Gegensatz zu Lipofuszin)
• Alpha-1 antitrypsin deficiency Mode of inheritance: autosomal codominant.- Gene is called SERPINA1 on chromosome 14. - Normal is M (50%)/M (50%); PiMM = 100% function→ S Mutation → 25% function; PiSS = 50% function→ Z Mutation → 10% funtion; PiZZ = 20% function→ 0 mutations → Absent protein Pathophysiology:- Liver: Impaired secretion of α1-antitrypsin by hepatocytes → intracellular accumulation of α1-antitrypsin → hepatocyte destruction → hepatitis and liver cirrhosis- Lungs: deficiency of α1-antitrypsin in plasma → increased protease activity in the lungs → destruction of the pulmonary architecture → panacinar emphysema Clinical presentation:- Severe form: Prolonged neonatal jaundice, hepatitis, cirrhosis, and pulmonary emphysema- Mild form: Manifests in adolescence, primarily with pulmonary disease; hepatic symptoms may also be present.- Patients are at increased risk for hepatocellular carcinoma. Diagnosis: - Electrophoresis (decreased alpha-1 peak), serum antitrypsin level, liver biopsy- Chest x-ray or CT (hyperinflation), pulmonary function tests (obstructive pattern) Microscopy: Stain with PAS- PAS ⊕ (Periodic acid shiff that stains glycoproteins pink) → stains because stuck in ER- Diastase (destroys glycoproteins) → resistant because cannot reach ER Therapy: - IV infusion of normal A1AT Protein- Inhalers, supplemental oxygen
• Morbus Wilson Normal consumption of copper: 1-2 mg/day. Extra is excreted in bile (90%) and urine. Genetic defect in autosomal recessive ATP7B transport protein (chromosome 13) that results in the excess copper being kept in the body. - Presents in late childhood Role of ATP7B:1. Binds Cu2+ to apoceruloplasmin → ceruloplasmin. 2. Packages copper into vesicles for excocytosis to bile → excretion. Buildup of copper can react with superoxide and create free radicals. Free copper circulates and deposits in other tissues.- Liver → Acute hepatitis → Cirrhosis- Basal ganglia → Movement disorders- Cerebral cortex → Dementia- Descemet's membrane → Kayser-Fleischer ring Diagnosis:- ↓ Ceruloplasmin, ↑ Cu (also in urine)- Hepatosplenomegaly- Renal damage- Hemolytic anemia- Biopsy: Rhodanin staining Treatment: Penicillamine, liver transplant
• Leberherd - Differentialdiagnose Fokale Läsion:- Zyste, Abszess- Echinokokkose- Hämatom- Hämangiom Hepatozelluläre Läsion:- Fokal noduläre Hyperplasie- Partielle noduläre Transformation- Hepatozelluläres Adenom- Hepatozelluläres Karzinom Nicht-hepatozelluläre Läsion:- Metastase- Biliäres Adenom- Cholangiokarzinom- Seltene Tumoren: Lymphangiom, Angiomyolipom, Angiosarkom, embryonales Sarkom, ...
• Melanom Färbung - HMB-45 - S100
• Lebermetastase Differenzierungsmarker CDX2: Adenokarzinom intestinaler Herkunft TTF-1: Pulmonale und Schilddrüsen-Adenokarzinome PSA: Prostata Calcitonin: Medulläres Schilddrüsenkarzinom CD31: Endothelialer Herkunft (Angiom, SLL)
• Malignant hepatic tumors Metastatic liver disease- Most common malignant lesion of the liver- Primary tumor site: gastrointestinal tract (colon, stomach, pancreas), lungs, and breasts Intrahepatic cholangiocellular carcinoma (CCC)- Second most common primary hepatic malignancy after HCC Hepatic angiosarcoma- Third most common primary hepatic malignancy- High rate of recurrence- Poor prognosis- Etiology: associated with exposure to vinyl chloride, arsenic, and thorium dioxide- Resistant to treatment (chemotherapy and radiotherapy)- Biopsy showing endothelial cells positive for PECAM-1 (CD31) Primary hepatic lymphoma
• Benign liver tumors 1. Cavernous hemangioma- Most common- Mass of blood vessels- Usually <1.5 cm, asymptomatic- If large → rupture & intraperitoneal bleeding- Diagnosis: US, CT scan, MRI 2. Focal nodular hyperplasia (FNH)- Localized aggregates of rapidly reproducing liver cells- Women > men, any age- Nodules of benign hepatocytes- Centralized blood vessel - Centralized fibrous scar tissue- Diagnosis: CT scan; most often found incidentally 3. Hepatocellular adenoma- Enlarged, non-functional hepatocytes- Highly-vascularized tissue- Absent bile ducts and portal areas- Highly associated with estrogen-based drugs, steroids 4. Hepatic cyst5. Arteriovenous malformation6. Hepatic lipoma
• Hepatozelluläres Karzinom 1. Zellatypien: Mitosen, trabekuläre/glanduläre/solide ANordnung 2. Vaskularisierung, Gewebseinblutungen 3. Tumoreinbruch in Blutgefässe 4. Tumormarker- CD10- HepPar1- Alpha-Fetoprotein
• Zirrhose Mikronoduläre Leberzirrhose:- 1-3 mm grose Parenchymregeneratknoten- Chronische Hepatitis B oder C, alkoholbedingte Fettleberhepatitis Makronoduläre Leberzirrhose:- Regeneratknoten >3 mm gross- Wenn Hepatitis (Virushepatitis, Wilson, Intoxikationen) in Schüben verläuft 1. Regeneratknoten 2. Bindegewebsvermehrung 3. Entzündliches Infiltrat 4. Pseudogallengänge im Bereich der Septen, als Zeichen einer Regeneration
• Cholangiokarzinom Risikofakoren: - Chronische Cholangitis, primäre sklerosierende Cholangitis, - Infektionen: Clonorchis sinensis Extrahepatisch (80%)Intrahepatisch (20%) Immunhistochemie: HepPar1 (wie HCC), CK7 (vgl. CD10 bei HCC) 1. Unifokal (HCC meist multifokal), bevorzugt in Hilumnähe 2. Adenokarzinom  3. Metastasierung: Lymphangiosis carcinomatosa
• Budd-Chiari syndrome Etiology:- Secondary to conditions associated with hypercoagulability (most common):→ Polycythemia vera (most important cause) → Paraneoplastic thrombocytosis→ Pregnancy→ Clotting disorders (e.g., factor V Leiden thrombophilia, antiphospholipid syndrome)→ Side effect of medication (e.g., hormonal contraception)- Secondary to invasion or compression of the hepatic veins (less common):→ Hepatocellular carcinoma, renal carcinoma→ Chronic infections and hepatic lesions (e.g., amebiasis, aspergillosis, syphilis, tuberculosis) Clinical presentation:- Abdominal pain- Tender hepatomegaly- Ascites and abdominal distention- Jaundice- Signs of increased perfusion of portocaval anastomoses Diagnostics:- Blood analysis: often nonspecific findings; possible elevated aminotransferases- Ascites fluid analysis: WBC count < 500 /μL; Serum ascites albumin gradient > 1.1- Imaging (confirmatory test): Doppler ultrasound; CT, MRI with contrast Treatment:- Anticoagulation (to prevent propagation of the thrombus)- Restore blood flow: Localized thrombolysis; Balloon angioplasty and stenting- TIPS (transjugular intrahepatic portosystemic shunt) if complications of portal hypertension occur
• Cholestasis Any condition affecting bile formation or secretion (nonobstructive intrahepatic cholestasis), or leading to biliary obstruction. Etiology:- Inherited disorders: Dubin-Johnson syndrome, Rotor's syndrome- Intrahepatic cholestasis→ Liver disease (e.g., hepatitis or cirrhosis)→ Primary biliary cholangitis→ Infiltrative process (e.g., tuberculosis, sarcoidosis, and amyloidosis)- Extrahepatic cholestasis (biliary obstruction)→ Choledocholithiasis→ Tumors (pancreatic cancer, cholangiocellular carcinoma, gallbladder cancer)→ Primary sclerosing cholangitis→ Malformations of the bile ducts (e.g., postoperative/inflammatory strictures, and biliary cysts)→ Parasitic infection (e.g., liver flukes) Clinical features:- Jaundice- Pale, clay-colored (acholic) stool- Dark urine- Pruritus- Fal malabsorption (steatorrhea, weight loss)
• Primary sclerosing cholangitis Progressive chronic inflammation of both the intrahepatic and extrahepatic bileducts.- M > F- ∼90% of PSC patients have IBD (from these patients, 87% have ulcerative colitis and 13% have Crohn's disease) Clinical featuers:- Signs of cholestasis→ Jaundice/scleral icterus→ Pruritus→ Fatigue- Acute cholangitis (fever, chills, right upper quadrant pain)- Later stages: signs of cirrhosis→ Hepatomegaly→ Portal hypertension→ Liver failure  Diagnostics:- Perinuclear anti-neutrophil cytoplasmic antibodies (pANCA) in up to 80% of cases- ↑ ALP, GGT, conjugated bilirubin- Potentially, ↑ transaminases (however, they are usually < 300 U/L)- Magnetic resonance cholangiopancreatography (MRCP)- Ultrasound- Following diagnosis → colonoscopy- Liver biopsy: "onion skin" scarring and fibrosis of bile ducts Treatment:- Ursodeoxycholic acid and immunosuppressives (e.g., tacrolimus)- Treatment of pruritus (e.g., cholestyramine)- Supplementation of fat-soluble vitamins- Liver transplantation Complications:- Steatorrhea and deficiency of fat-soluble vitamins- Liver cirrhosis- Cholangiocarcinoma (∼10-15% of cases)- Increased risk of hepatocellular, colorectal, pancreatic, and gallbladder cancer
• Hepatocellular carcinoma Malignant, most often solitary tumor of the liver. Etiology:- Liver cirrhosis (80% of cases)- Risk factors independent of cirrhosis: → Chronic hepatitis B or C virus infection→ Nonalcoholic steatohepatitis (NASH)→ Hemochromatosis→ Wilson's disease→ Alpha-1 antitrypsin deficiency→ Schistosomiasis→ Glycogen storage disease→ Chronic ingestion of food contaminated with aflatoxin Clinical features:- Usually asymptomatic - Metastasis is uncommon and usually only occurs in advanced stages- Possible symptoms in advanced disease: Weight loss, cachexia, hepatomegaly and right upper quadrant tenderness, ascites and jaundice Diagnostics:- ↑ Serum alpha-fetoprotein (AFP)- ↑ LFTs, positive hepatitis serologies, ↓ coagulation factors- Paraneoplastic syndromes: erythrocytosis, hypoglycemia, hypercalcemia- Ultrasound: first test - Abdominal CT (confirmatory test) Treatment:- Curative therapeutic options→ Surgical resection→ Liver transplantation→ Ablative therapies
• Echinococcosis Parasitic disease caused by small tapeworms of the genus Echinococcus.- Echinococcus granulosus causes cystic echinococcosis.- Echinococcus multilocularis causes alveolar echinococcosis. - Definitive hosts: foxes, dogs, and cats- Intermediate hosts: hoofed animals Cystic echinococcosis:- Single hepatic cyst (hydatid cyst) - Hepatomegaly → RUQ pain- Malaise, nausea, vomiting- Lung involvement in 25% of cases → chest pain, cough, dyspnea, hemoptysis Alveolar echinococcosis: - Infiltrative grown- Hepatic cyst→ Hepatomegaly → RUQ pain→ Malaise, weight loss, nausea, vomiting- Cyst that invades and destroys the liver and surrounding tissue→ Portal hypertension, cholestatic jaundice, cholangitis→ Budd-Chiari syndrome→ Liver cirrhosis→ May resemble hepatocellular carcinoma- Metastasis to other organs (especially lungs, brain, spleen) in ∼13% of cases Diagnostics:- Laboratory tests: mild eosinophilia, leukopenia or thrombocytopenia, liver function abnormalities- Serology: positive ELISA- Ultrasonography- CT scan Treatment:- Albendazole- Ultrasonography/CT-guided percutaneous drainage- Surgery
• Liver tissue architecture Zone I – periportal zone:- Viral hepatitis- Toxins (eg, cocaine) Zone II – intermediate zone:- Yellow fever Zone III – pericentral vein (centrilobular) zone:- Affected 1st by ischemia- High concentration of cytochrome P-450- Most sensitive to metabolic toxins (eg, ethanol, CCl4, halothane, rifampin)ƒ - Alcoholic hepatitis
• Cholangiocarcinoma Risk factors:- Chronic inflammation of the gallbladder, bile ducts, or liver→ Chronic cholangitis, especially primary sclerosing cholangitis (most common risk factor)→ Chronic liver disease- Congenital abnormalities of the bile ducts (e.g., choledochal cysts, congenital hepatic fibrosis)- Parasitic infection of the biliary tracts (e.g., Clonorchis sinensis)- Diabetes, obesity, and alcohol consumption Classification: Bismuth- Extrahepatic cholangiocarcinoma are most common (80% of cases): further divided into perihilar and distal extrahepatic cholangiocarcinomas- Intrahepatic cholangiocarcinoma involves the intrahepatic bile ducts. Clinical features:- Intrahepatic bile duct carcinoma→ Nonspecific abdominal symptoms (e.g., weight loss, nausea) → Dull right upper quadrant (RUQ) pain→ Signs of cholestasis are rare- Extrahepatic bile duct carcinoma→ Nonspecific symptoms (e.g., weight loss, fever) → Signs of cholestasis: jaundice, pale stools, dark urine, pruritus, hepatomegaly→ Possibly a positive Courvoisier's sign

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