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lek. Anna Skubała Department of Infectious , Tropical Diseases and Parasitoses . Infectious Diseases and Hepatology Clinic. JAUNDICE. Basic classificATION. MECHANICAL JAUNDICE. NON-OBSTRUCTIVE JAUNDICE. Bile ducts ( intra - or extrahepatic ) involved Mechanical blockage

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lek. Anna Skubała

Department of Infectious, TropicalDiseases and Parasitoses.

InfectiousDiseases and HepatologyClinic

JAUNDICE


Basic classification
Basic classificATION

  • MECHANICAL JAUNDICE

  • NON-OBSTRUCTIVE JAUNDICE


Mechanical jaundice

Bile ducts (intra- orextrahepatic) involved

Mechanicalblockage

Cholelithiasis(gall stones) – Cholecystolithiasis – Chledocholithiasis

Pancreatolithiasis

Neoplasmatic tumor aroundduodenum, caputpancreas, duodenalpapilla (Vater’spapilla)

Primarycholangiocarcinoma

Infectionsinthe same places

MECHANICAL JAUNDICE


Mechanical jaundice1
Mechanicaljaundice


Non obstructive jaundice

Infectious (HBV, HCV, HAV, otherhepatotropicviruseslike CMV, EBV)

Autoimmunologic (AIH, PBC, PSC)

Genetic (Wilson’sdisease, hemochromatosis, Gilbert’ssyndrome, Budd-Chiarisyndrome, Crigler-Najjarsyndrome)

Toxic (paracetamol, otherdrugs and chemicals, mushrooms, alcohol)

Non-obstructivejaundice


Non obstructive jaundice1

Alwaystakeintoconsideration a possibility of otherinternaldiseaseslike:

  • Heartinsufficieny

  • Hemolyticanaemia

  • Erytrocytes’ impairement (artificialheartvalve, hemodialysis, DIC, haemolytic-uraemicsyndrome)

  • Severeinfections (sepsis, malaria, toxoplasmosis)

  • Severeburns

  • Hipersplenism

    And oncologicdiseases:

  • HCC hepatocellular carcinoma

  • Metastases to hepar

  • Limphoproliferativesyndromes, lymphomas

Non-obstructivejaundice


Wilson s disease
Wilson’sdisease


copperaccummulation


Autosomalrecessive

Excessivecopperaccummulation, mainlyinliver, due to defectiveprotein, whichisresponsible for transport of Cu inthehepatocytes’ membrane

Cu is not excretedwith bile

Cu accumulateinliver, brain, kidneys and incornea – impairment of thoseorgans


First manifestationsinchildhoodorearlyadulthood

Differentconstelation of signsindifferentpatients

Hepatomegaly, hepatitis, livercirrhosiswithsigns of portal hypertension, acuteliverfailure

Parkinson’ssyndrome(intention tremor, bradykinesis, stiffness, dyzarthria), epilepticseazures, migrainheadache, hypersalivation, sleeplessness, personalitydisturbances, affectivedisturbances, psychoses

Kayser-Fleischer rings, cataract, Fanconisyndrome, cardiomiopathy, heartrythmdisorders, osteoporosis, arthritis, pancreatitis, retardedsexualmaturation, infertility, lack of menstruation, habitualmiscarriages, hypothyroidism, hypoparathyroidism, hemolysiswithjaundice


AST, ALT

Serum ceruloplasmin 

Total serum copperconcentration 

In histologicexaminatoion – uncharacteristicchanges,  Cu concentration


Criteria

Kayser-Fleischer rings

Decreasedceruloplasminconcentration

Liverimpairmentsignsorneurologicmanifestations

Criteria:




Treatment

alcohol membersabstinence

NO: nuts, chocolate, mushrooms, liver, mussels

continouspenicylamine treatment

zinc

Treatment:


In case of acute liver failure or in unstable cirrhosis resistant to treatment – liver transplantation

Regular follow-up visits


Hemochromatosis
Hemochromatosis resistant to treatment – liver transplantation


iron resistant to treatment – liver transplantationaccumulation


autosomal resistant to treatment – liver transplantationrecessive

mutation in the gene responsible for membrane protein

impairment of hepcidine production – protein inhibiting iron absorption in GI tract and iron release from the macrophages

excessive iron absorption causes its accumulation in organs like: liver, pancreas, heart, joints


c resistant to treatment – liver transplantationlinicalmanifestations – more often in men

firstsymptoms > 20 years of age in men and > 40 years of age in women

early symptoms: weakness, libido decline, arthralgia (hands, wrists)

later signs: the effect of chronic hepatitis or liver cirrhosis, cardiomiopathy, pancreas impairment, hormonal disorders (hypopituiarismus, hypothyroidism)

progressive disease

liver cirrhosis

1/3 of the patients reveal hepatocarcinoma (HCC)


Fe resistant to treatment – liver transplantation, ferritin

AST, ALT  (ALT > AST)

CT, MRI – liver cirrhosis


liver bio resistant to treatment – liver transplantationpsy– assessment of disease advance, fibrosis (prognosis); excessive iron accumulation in hepatocytes, fibrosis, cirrhosis

genetic tests – mutations searched by PCR (also 1st degree family members of patients with hemochromatosis)


Treatment1

restrictive resistant to treatment – liver transplantationmeat consumption

alcohol abstinence

avoiding dietary supplements containing iron and vit. C

bloodletting

deferoxamine

Treatment:


A lcoholic l iver d isease
A resistant to treatment – liver transplantationlcoholicliverdisease


alcohol – (oxygenation) – acetic resistant to treatment – liver transplantationaldehyde – acetic acid


Stages

Alcohol resistant to treatment – liver transplantation-relatedsteatosisof theliver (fattydegeneration)– asymptomatic; reversible

Alcohol-relatedhepatitis – fatigue, nausea, vomiting, pain in right subcostal area, hepatomegaly, tenderness of liver (>80%), ascites (up to 80%), jaundice (> 60%), hepatic encephalopathy (45%), temperature (up to 30%)

Cirrhosisof liver due to alcohol abuse

Stages:


Women are more susceptible to negative effects of alcohol resistant to treatment – liver transplantation

smaller dose

faster progress


Diagnosis

GGTP resistant to treatment – liver transplantation

ALT, AST – sometimes (AST/ALT >= 2)

ALP, Fe , bilirubin

prothrombintime – elongation

electrolytedisorders (hypoNa, hypoK; hypoMg)

leucocytosis, macrocyticanaemia, trombocytopaenia

Diagnosis:


Criteria1

rule resistant to treatment – liver transplantationout other possible causes of liver dysfunction

history of alcohol abuse

GGTP 

ultrasonographic picture of liver

Criteria:


Treatment2

alcohol resistant to treatment – liver transplantationabstinence

treatment of malnutrition and other defficiences connected with alcohol abuse

treatment of electrolytes disorders

glycocorticosteroidsif severe course of the disease with encephalopathy

treatment of cirrhosis and liver failure complications

Treatment:


N on a lcoholic s teato h epatitis
N resistant to treatment – liver transplantationon-alcoholicsteatohepatitis


chronic resistant to treatment – liver transplantation, progressive hepatitis in people who do NOT abuse alcohol

histopatologicchanges similar to those in alcoholic liver disease


Causes
Causes resistant to treatment – liver transplantation:

A. Metabolic disorders:

  • metaboicsyndrome

  • DM

  • obesity

  • protein malnutrition

  • excessive weigh loss (zespoleniaomijającejelitacienkiegoiżołądka, excision of a large part of intestine, long-lasting starvation, malabsorption syndrome, celiakia, unspecific enteritis, pancreas diseases)

  • total, long-lasting parenteral feeding

  • lipids metabolism disorders

  • chorobyspichrzeniowe (Wolmans disease, Niemann-Pick disease)

  • Tay-Sachs disease

  • Gaucherdisease

  • Wilson's disease

  • hemochromatosis

  • glikogenoses

B. Drugs:

  • Amiodaron

  • Diltiazem

  • Tamoxifen

  • GKS

  • Warfarin

  • ARV drugs

  • antibiotics (tetracycline, bleomycin)

  • Cytostatics

  • large doses of vit. A

  • Metotrexat

  • salicylanes, ASA

    C. Hepatotoxins:

  • carbon tetrachloride

  • Phosphorus

  • alpha-amanitin

    D. Infections:

  • HCV

  • HDV

    E. Other:

  • Reye's syndrome

  • pregnancy complication

  • eclampsia


Symptoms

usually resistant to treatment – liver transplantationasymptomatic

fatigue

weakness

discomfort in right upper abdominal quadrant

hepatomegaly(<75%) or splenomegaly (<25%)

other signs of portal hypertension (rarely)

Symptoms:


Diagnosis1

AST resistant to treatment – liver transplantation, ALT (AST/ALT <1)

dyslipidaemia

hyperglycaemia

hypoalbuminaemia

prothrombintime elongation

Fe, ferritin

GGTP, bilirubin

liver biopsy

Diagnosis:


Treatment3

causative resistant to treatment – liver transplantation– e.g. metabolicsyndrome

treatment of liver cirrhosis complications

Treatment:


Budd chiari syndrome
Budd- resistant to treatment – liver transplantationChiarisyndrome:


hepatic resistant to treatment – liver transplantationveins thrombosis

could also contain VCI thrombosis

impaired blood drainage from the liver


Causes1

myeloproliferative resistant to treatment – liver transplantationneoplsms (polycythaemia, nadpłytkowość)

stanynadkrzepliwości

oral contraceptives

idiopathic

Causes:


If comprises one of the hepatic veins and the resistant to treatment – liver transplantationcollateralcirculation is well developed – asymptomatic

If all 3 hepatic veins involved – acute liver failure, with fast growing ascites

Usually sub-acute or chronic: hepatomegaly, ascites, jaundice, liver failure symptoms, peripheral oedema


Diagnosis2

doppler resistant to treatment – liver transplantationultrasonography

CT

angio-MRI

Diagnosis:


Treatment4

causative resistant to treatment – liver transplantation– ifpossible

chronicanticoagulanttreatment

acute: liver transplantation

Treatment:


A uto i mmunologic h epatitis
A resistant to treatment – liver transplantationutoimmunologichepatitis


chronic resistant to treatment – liver transplantationnecrotico-inflammatory process of liver

unknownethiology

increased serum gamma-globulin concentration

presenceof autoantibodies


no resistant to treatment – liver transplantationage limit, most common in okrespokwitana and between 40. - 60. years of age

4 times more common in women


asymptomatic resistant to treatment – liver transplantation

acute or chronic hepatitis


most common symptom – fatigue resistant to treatment – liver transplantation

jaundice

rarely acute liver failure

more aggressive and treatment resistant in children and young adults

co-existingotherautoimmunologicdiseases


Diagnosis3

AST resistant to treatment – liver transplantation, ALT 

bilirubina 

ALP – normalor

prothrombintime elongation

Hypoalbuminaemia

mild leucopenia, eosynophilia, normocyticanaemia, thrombocytopaenia

serum gamma-globulins (mainly IgG)

other autoantibodies: ANA, ASMA, anti-actin, anty-LKM1, pANCA

liver biopsy

Diagnosis:


Overlapping syndromes

AIH resistant to treatment – liver transplantation+ PBC

AIH + PSC

AIH + chronic viral hepatitis

Overlapping syndromes:


Treatment5

i resistant to treatment – liver transplantationmmunosupresive– prednisolon, prednisone p.o.

Azathioprine (allows to reduce steroids doses)

liver transplantation

alcohol abstinence

Treatment:


P rimary b iliary c irrhosis
P resistant to treatment – liver transplantationrimarybiliarycirrhosis


Unknown resistant to treatment – liver transplantationetiology

Chronic, progressive

Associated withdestruction of small bile ducts


Asymptomatic resistant to treatment – liver transplantation

Fatigue, pruritus


Hepatomegaly resistant to treatment – liver transplantation

Xanthelasma

Jaundice

Liverfailuresymptomsinadvanceddisease


ALP, GGTP resistant to treatment – liver transplantation

ALT, AST 

Bilirubin 

Hypercholesterolaemia

Characteristicautoantbodies AMA

IgM

Liverbiopsy


Only resistant to treatment – liver transplantationapprovedtreatment: ursodeoksycholicacid

Livertransplantation


Osteoporosis resistant to treatment – liver transplantation

Fat-solublevitaminsdeficiency

HCC


Gilbert s syndrome
Gilbert’s resistant to treatment – liver transplantationsyndrome


c resistant to treatment – liver transplantationongenitalhyperbilirubinaemia

1,5-7x moreoftenin men

usuallyasymptomatic

slightjoundiceperiodically

accidentaldiagnosis

do not needtreatment


Crigler najjar syndrome
Crigler-najjar resistant to treatment – liver transplantationsyndrome


c resistant to treatment – liver transplantationongenital, autosomalrecessive

Severejaundicein first daysafterbirth

otherlabolatorytests – normal

jaundice of subcorticalnuclei

neonataldeath

treatment: long-lastongphototherapy, plasmapheresis, livertransplantation


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