hepatology- liver diseases · 2020. 3. 13. · examination of the liver •palpation i. edge: of...

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Hepatology- liver diseases Veronika Vyskočilová

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  • Hepatology- liver diseases

    Veronika Vyskočilová

  • Examination of the liver

    • Surface anatomy

    - seize: 8-12cm

    - weight : 1200- 1500g

    - location: in the dome of diaphragm (75% of liver tissueis on the right side of midline, 25% on the left side)

    - borders: right midclavicular line

    I. expiration: upper border is at the 4-th rib

    II. inspiration: upper border is at the 5-th rib

  • Examination of the liver

    • macroanatomy of the liver

  • Examination of the liver

    • Inspection

    - normal liver cannot be seen

    - a greatly enlarged liver can be seen as total/ circumscribed bulge in the epigastrium + under theright costal margin

  • Examination of the liver• Palpation

  • Examination of the liver

    • Palpation

  • Examination of the liver

    • Palpation

    I. edge: of healthy liver- sharp, resilient

    in pathologic case- round, hard, bumpy

    II. texture: (Naegeli classification) 4 degrees of consistency:

    1) soft, smooth (normal elastic liver tissue)

    2) firmer- harder tissue but still elastic (in congestion,infiltrativeprocess)

    3) hard, non- elastic, non- resilient (liver cirrhosis)

    4) very hard, stone- hard (in cancer)

  • Examination of the liver

    • Palpation

    III. surface: large bumps and nodes (macronodularcirrhosis, tumors, polycystic disease of the liver )

    IV. tenderness: painfull palpation (acute congestion, hepatitis...)

    V. pulsations: transmitted from abd. aorta/ hypertrophy of the right ventricle

    • Hepatojugular reflux : pressure against liver resultsin increased filling of the jugular veins (venostasis)

  • Examination of the liver

    • Percussion

    - examination of the upper border

    - right midclavicular line from the 4-th/ 5-th rib caudad withsharp percussion

    • Auscultation

    - friction rub synchronous with respiration (perihepatitis)

    - vascular murmur synchronous with the pulse(hemangiomas.., )

  • Main symptoms of liver disease

    • Jaundice (Icterus)

    - yellow discoloring of the skin, mucous membranesand sclerae when plasmatic bilirubin > apx. 30umol/l

    a) prehepatic (hemolysis, Gilbert´s syndrome...)

    b) hepatocellular (liver cirrhosis, Hepatitis..)

    c) cholestatic ( CholangioCa, cholelithiasis..)

  • Main symptoms of liver disease

    • Jaundice (Icterus)

  • Main symptoms of liver disease• Portal hypertension

    - ↑ venous pressure in the area of portal vein

    - risks: bleeding from esophag. varices

    ascites

    hepatic encephalopathy

    - types:

    a) prehepatic (blockage of the portal vein)

    b) hepatic pre- sinusoid (multiple infiltrates in the periportalspaces)

    c) hepatic post-sinusoid (most common, liver cirrhosis)

    d) posthepatic (Budd Chiari syndrome...)

  • Main symptoms of liver disease

    • caput medusae

  • Main symptoms of liver disease

    • Hepatic encephalopathy

    - detoxication of nitrogenous products in liver is deficient

    - clinical symptoms: somnolence, slow monosyllabic speech, confusion, apathy, flapping tremor- asterixis, constructiveapraxia, foetor hepaticus...coma

  • Main symptoms of liver disease

    • Ascites

    - most common in decompensated hepatic cirrhosis

    - fluid is yellowish, clear, with a low concentration of protein (< 25g/l )

  • Main symptoms of liver disease

    • differential diagnosis of ascites according to the SAAG

  • Main symptoms of liver disease• Esophageal varices

  • Diagnosis

    • basic biochemical examination in hepatology

    Markes

    ALT, AST enzymes that reflect demage to hepatocytes

    ALP, GMT enzymes that reflect cholestasis

    albumin, prealbumin, globulins ,CHE, coagulation factors...

    biosynthetic function of the liver

    bilirubin, ammonia detoxification and excretoryfunctions of the liver

  • Diagnosis• special examination in hepatologyMarkes

    ↑Transferrin saturation, ↑Fe, ↑Feritinin serum

    Hereditary Hemochromatosis

    ↓Ceruloplasmin, ↓total Cu, ↑free Cu, ↑Cu in urine

    Wilson disease

    PBG , δ-ALA, porfyrines in urine.. Porphyria

    AFP HCC

    anti HAV,anti HEV, anti HCV, anti Hbe, antiHBs, anti HBc,HBsAg, HBeAg, HBV DNA,HCV RNA

    Viral hepatitis A,B, C,E.

    ANA,ASMA, LKM-1, LC-1, pANCA, SLA/ LP Autoimmune hepatitis

    AMA, ANA PBC

    pANCA PSC

  • Diagnosis

    • genetic examination (1)

    Mutation Disease

    HFE gene mutation,(C282Y ,H63D, S65C)

    Hemochromatosis 1.type

    hemojuvelin , hepcidin Hemochromatosis 2.type

    transferrin receptor 2, Hemochromatosis 3.type

    feroportin mutation Hemochromatosis 4.type

  • Diagnosis

    • genetic examination (2)

    Mutation Disease

    ATP7B gene(H1069Q – most frequent, 35-70% in central Europe )

    Wilson disease

    UGT1A1 gene Gilbert´s syndrome

  • Severity of liver cirrhosis

  • Prognosis of liver cirrhosis

  • Other diagnostic tests

    • Ultrasonography

    - normal finding

  • Other diagnostic tests

    • Ultrasonography

    - incipient portal hypertension

  • Other diagnostic tests

    • Ultrasonography

    - micronodular and macronodular liver cirrhosis

  • Other diagnostic tests

    • Ultrasonography

    - hemangioma , cyst

  • Other diagnostic tests

    • Ultrasonography

    - HCC (CT/ MR for „wash in“ and „wash out“ phenomen )

  • Other diagnostic tests

    • CT

    -liver cirrhosis

  • Other diagnostic tests

    • CT

    - HCC

  • Other diagnostic tests

    • CT

    - cyst

  • Other diagnostic tests• percutaneous liver biopsy

    - safe, bedside method with local anesthesia

    - indications:

    1. hepatocellular disease of uncertain cause

    2. prolonged hepatitis with possibility of chronic activehepatitis

    3. unexplained hepatomegaly

    4. unexplained splenomegaly

    5. hepatic filling defects by radiologic imaging

    6. fever of unknown origin

    7. staging of malignant lymphoma

  • Other diagnostic tests

    • percutaneous liver biopsy

    - contraindications:

    1. significant ascites

    2. prolonged INR > 1,4

    3. infection

    4. obstructive icterus

    5. thrombocytopenia < 80

  • Other diagnostic tests

    • percutaneous liver biopsy

    - types:

    1. fine needle Menghini biopsy

    (1,4 mm )

    2. core cut biopsy

  • Other diagnostic tests• percutaneous liver biopsy

  • Liver elastography

    • imaging test that checks the liver for fibrosis ( fibroscan)

    • uses sound waves to measure the stiffness of liver tissue

    • stages of liver stiffness: F0-F4 (Metavir classification)

    • in patients with medium or advanced liver fibrosis F2-F4 has sensitivity 70 % and specificity 80 % , in cirhotic liver is specificity over 90%

    • sensitivity of this method is increased in combinationwith laboratory index for liver (APRI, FIB-4, NAFLD fibrosis score..)

  • TIPS

    • „Transjugular intrahepatic portosystemic shunt“

    • artificial channel within the liver that establishes communication between the inflow portal vein and the outflow hepatic vein

    • HVPG (hepatic venous pressure gradient) measurement( >10-12 torr=clinical significant portal hypertension, e.g. bleeding)

    • decreases portal pressure – reduction of splanchnicvascular resistance – stop variceal bleeding, treatment of refractory ascites and pleural effusion

  • TIPS – technique

    • intervention radiologist

    • cathethrisation via internal jugular vein, superiorvena cava vein, right atrium, IVC, into right or midhepatic vein

    • Colapinto needle – puncture from central part of right hepatic vein into right portal vein

    • ballon cathether – dilatation – insertion of dedicated stentgraft

  • TIPS

    • pros: local anesthesia insertion - also as urgent procedure in critical ill and instablepatient

    • cons: check ups for patency, worsening or new onset of hepatic encephalopathy

  • Complications of TIPS

    • mortality (lower than open surgery shunts) – 1,7% - depends on experience of the centre (> 150 procedures) , intraperitoneal bleeding upon portalvein rupture, hepatic arthery laceration, livercapsule injury

    • worsenig of liver function

    • hepatic encephalopathy

    • volume overload

    • TIPS dysfunction (stenosis, occlusion of stent)

  • TIPS• DUS patency control within 24 hrs after insertion

    hod po zavedení

  • Indications for TIPS

    • Acute: bleeding from portal hypertension(failure to control bleeding or early TIPS, severe Budd-Chiari syndrome, hepatorenalsyndrome?

    • Elective: secondary prevention of bleedingfrom portal hypertension, refractory ascitesand hepatic hydrothorax

  • Contraindications of TIPS

    • poor prognosis: Child-Pugh > 12, MELD > 15-18

    • absolute KI: no evidence of portal hypertension, primary prevention of variceal bleeding, uncontrolledhemorrhagic shock, uncontrolled infection and sepsis, severe tricuspid regurgitation and severe pulmonaryhypertension, unresolved biliary obstruction, polycysticliver, uncontrolled hepatic encephalopathy

    • relative KI: severe coagulopathy (INR >5, Tr

  • Case report (1.1)

    • 52- years old man

    • prior illnesses: middle ear operation in childhood

    • abuses: 0,5-1l wine/ day last 2 yrs , non- smoker

    • medication: none

    • family history: father died 74yrs old – MI

    mother still alive

    • social history: project manager

    • VF: dark urine since yesterday, bright stool, stable weight

    • current complaints: noticed jaundice yesterday, weakness, prostration, nausea, vomiting, lost of appetite, withoutfever and chills

  • Case report (1.2)

    • objectively: icterus, no hepatosplenomegaly, BP 120/80Torr, HR 78/min reg., T: 36,8°C, BMI: 26,6kg/m2

    • Blood count: WBC 10,35 , RBC 4,38, HGB: 154, HTC 0,43, PLT 139,2, MCV 98,41, Neu 87,9%, Lym 7%

    • Coagulation: FBG 2,48, D-Dimer 0,82, INR 1,49, R-aPTT1,11

    • Biochemistry: S-BIL(total)364,56 , S-BILC 319,82, Albumin39,45, S-AST 22,77, S-ALT 20,92, S-GMT 24,61, S-ALP 1,91, S-Fe 38,65 , CRP 12,7

    • Urine : U-Bili 4+

    • Ultrasonography: thickened wall of gall bladder, slightlyenlarged liver, otherwise normal finding

  • Case report (1.3)

    • serology: anti HAV IgG,IgM- negat,

    anti HCV negat,

    antiHBc IgM pozit, HBsAg pozit,

    anti CMV,EBV IgM -negat,

    Autoantibodies – ANA screening negat

    Diagnosis: ???????????????????????????

  • DIAGNOSIS

    Acute viral hepatitis B

  • Case report (2.1)

    • 55- years old woman• prior illnesses: primary hypothyreosis caused by autoimmune

    thyroiditis , DM 2.type, Irritable bowel syndrome, vittiligo, allergicrhinitis, dyslipidemia, Art. hypertension,

    • abuses: non smoker for 10 yrs (before 12cig. per day), alcohol-none

    • medication: Euthyrox, Telmisartan, Concor, Metfogamma, Nolpaza, Stilnox, Valdoxan,

    • family history: father died tragically as 50yrs oldmother died 82yrs old, she had hypothyreosis

    • social history: works in library• VF: dark urine for 7 days, regular stool- normal color and

    consistency, lost of appetite- weight lost 1kg for 1 month• current complaints: noticed jaundice last 7 days, weakness,

    nausea, vomiting 1x per day, lost of appetite, without fever and chills

  • Case report (2.2)

    • objectively: icterus, liver + 1cm in MCL, BP 110/70 Torr, HR 80/min reg., T: 36,6°C, BMI: 23,62 kg/m2

    • Blood count: WBC 9,77, RBC 4,31, HGB: 122, HTC 0,36 PLT 248, MCV 82,8, Neu 74,4%, Lym 12,2%

    • Coagulation: FBG 3,76, D-Dimer 0,27, INR 1,21, R-aPTT1,17

    • Biochemistry: S-BIL(total) 200,42, S-BILC 158,91, Albumin31,7, S-AST 7,88, S-ALT 12,31, S-GMT 3,13, S-ALP 1,88, CRP 19,18,

    • Serum prothein electrophoresis: S- betaglobulin 11,3, S-gamaglobulin 20,7 (chronic hepatopathy)

    • Urine : U-Bili 1+

  • Case report (2.3)

    • Ultrasonography: liver steatosis,polyp in gall bladder, v.sangiomyolipoma of the left kidney, otherwise normalfinding

    • Serology: anti HAV IgG,IgM- negat, anti HCV negat, antiHBcIgM negat, HBsAg negat

    • Autoantibodies: ANA++, ANCA negat, ASMA negat, AMA negat, LKM-1 negat, LC-1 negat

    • CT guided core cut liver biopsy : necroinflammation 8/18, (lymphoplasmacytic infiltration, piecemeal necrosis) fibrosis 3/6 (Ishak)

    • Diagnosis: ???????????????????????????

  • DIAGNOSIS

    Autoimmune hepatitis type 1.

  • Case report (3.1)

    • 55- years old man • prior illnesses: Art. hypertension, DM 1.type, Dyslipidemia,

    ICHS, NSTEMI inferolateral, CHRI, Stroke in past• abuses: smokes 15 cig/day for 20yrs, alcohol (beer,wine)

    occasionally• medication: Inegy (ezetimib+ simvastatin) started using 3

    months ago, Procorolan, Prenessa, Talliton,, ANP, Thiogamma, Humulin N, Humalog

    • family history: father died 51yrs old – strokemother died 80yrs old- colorectalCa

    • social history: automechanic• VF: dark urine during last week, normal stool , stable weight• current complaints: noticed jaundice last 5 days, dark

    urine,weakness last month, lost of appetite, 3 months agostarted using new drug Inegy instead of Torvacard

  • Case report (3.2)

    • objectively: icterus, liver + 2cm in MCL, BP 130/80 Torr, HR 70/min reg., T: 36,9°C, BMI: 22,6kg/m2

    • Blood count: WBC 9,26, RBC 4,21, HGB: 127, HTC 0,38 PLT 166, MCV 82,8, Neu 62,7%, Lym 21,8%

    • Coagulation: INR 0,81, R-aPTT 0,84

    • Biochemistry: S-GLU 16,13, S-BIL(total) 122,44, S-BILC 114,42, Albumin 34,26, S-AST 12,42, S-ALT 12,31, S-GMT 13,12 S-ALP 16,23, TAG 4,23,CRP 0,93

    • Serum prothein electrophoresis: presence ofinflammation

    • Urine : U-Bili 1+, U- Gluk 4+

  • Case report (3.3)

    • Ultrasonography: contraction of gall bladder, otherwisenormal finding

    • Serology: anti HAV IgM negat, IgG pozit, anti HBC IgM negat, anti HBC total negat, anti HCV negat, anti VZV IgM negat, antiEBV IgM negat, anti CMV IgM negat, autoantibodies: ANA +, cANCA++

    • CT guided core cut liver biopsy : hepatic tissue withoutinflammatory changes , without fibrotic changes , lightintrahepatic cholestasis, higher infiltration of eosinophils

    • Diagnosis: ???????????????????????????

  • DIAGNOSIS

    drug induced liver injury – DILI

    (simvastatin,ezetimib)

  • Thank you