Jaundice 2015

7/23/2019 Jaundice 2015

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JAUNDICE

Khaled Abu – RummanAla’ Eddin Abu – Shareb

-------------------------------------

Prof. Fouad Ammari

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OutlineDefnition

Bilirubin metabolism

ClassifcationDiagnosis

Special investigations

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Jaundice is not a disease but

rather a sign that can occur inmany dierent diseases.

Jaundice is the yellowishstaining of the skin ,sclerae (the whites of the

eyes) and other !ucous!e!"ranes that is caused"y high le#els in "lood ofthe che!ical "iliru"in$

The color o the skin and

sclerae vary depending on thelevel o bilirubin. hen thebilirubin level is mildlyelevated! they are yello"ish.hen the bilirubin level is high!they tend to be bro"n.

hat is &aundice'

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#ormal serum total bilirubin is $$

*+ %mol&' ( * mg&d')

Jaundice is clinically evident "hen serumtotal bilirubin $ *+,- normal (-$ !g.d/)

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0iliru"in !eta"olis!

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0iliru"in 1roduction

2e!e

2e!e o3ygenase

0ili#erdin reductase

2e!oglo"in(+4 to 546)

2e!e 7roteins!yoglo"in, cytochro!es

(-4 to -6)

0ili#erdin

0iliru"inindirectuncon&ugated

7rehe7atic

al"u!in

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0iliru"inE3cretion

0iliru"in diglucuronide

546 8terco"ilinogen

0acterial en9y!es

0iliru"in

0acterial en9y!e- glucuronate

0acterial en9y!e

Uro"ilinogen

:46 li#er

Uro"ilin

*46 kidneys

urine

8terco"ilin feces

intestines

;-46

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2y7er"iliru"inae!iaDisruption of bilirubin metabolism and excretion can

cause hy7er"iliru"inae!ia and su"se<uent &aundice

2y7er"iliru"inae!ia !ay"e uncon&ugated (indirect) orcon&ugated (direct) de7ending on the cause

8o!e inherited syndro!es of "iliru"in handling canresult in hy7er"iliru"inae!ia

=il"ert>s syndro!e ? reduced acti#ity of glucuronyltransferase therefore reduced con&ugated "iliru"intherefore ele#ated uncon&ugated "iliru"in

CrigglerNa&&ar ? reduction in a!ount of glucoronyltransferase therefore ele#ated uncon&ugated "iliru"in

@otor>s.Du"inJohnson syndro!e ? defecti#e e3cretion

of con&ugated "iliru"in into the "iliary cannaliculi

therefore ele#ated con&ugated "iliru"in

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1rehe7atic &aundice2e!olytic disorders

Thalassemias

/0D defciency

1ereditary spherocytosis

2utoimmune hemolytic anemias

3ncompatible blood transusion

42 healthy liver can e-crete /- the normal load obilirubin beore uncon5ugated bilirubin starts toaccumulate6

=il"ert syndro!e, CrigglerNa&&ar syndro!e

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2e7atocellular &aundiceIntra2e7atic

0asically any acute or chronic li#er disease

7iral hepatitis Drug+induced hepatoto-icity 2lcoholic and non+alcoholic liver disease (#2S1) 0BC 0SC 2utoimmune hepatitis1emochromatosis ilson8s disease Cirrhosis 'iver tumors

2ll the causes o hepatitis&cirrhosis (e.g. 2lcohol! viral! auto+immune! primray biliary cirrhosis ! 0rimary sclerosing cholangitihaemochromatosis! "ilsons! alpha+9 antitrypsin defciency ! Drug+induced! )

@otor>s syndro!e, Du"inJohnson syndro!e

Can result in hepatocyte destruction and thereforeunconjugated hyperbilirubinaemia or in bile cannaliculidestruction and therefore conjugated hyperbilirubinaemia or

both

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O"structi#e &aundiceB 1osthe7aticIntralu!inal o"struction: stones ! hydatit cysts "orms

%all 7athologies: Intra-mural

Benign stricture

*+B3'32;< 2T;=S32

-+32T;>=#3C: BILIARY SURGERY , GASTRECTOMY, HEPATIC RESECTION

,LIVER T;2#S0'2#T +3#?'2@@2T>;<A CHOLANGII! " # ANC$%AII! " !CL%$O!ING CHOLANANGII!&

+T;2@2

+3D3>02T13C /+;2D3>T1=;20<

@alignant stricture: cholangiocarcinoma

E3ternal co!7ression: 7ancreatitis + oedema o head o pancreas+ ! tu!or o the head o the pancreas! 1ancreatic

7seudocyst

tumor o the ampulla o 7ater! hilar lymphadenopathy! @irii syndrome !choledocal cyst !

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T1= C>@@>#=ST C2S=8ONE 8/I11IN= INO 2E 0I/IA@F

@EE

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MIRRIZI`s syndrome

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B3'32;< 2T;=S32

NORMAL BILIARY ATRESIA

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C1>'2#3C2;C3#>@2

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C1>'2#3>C2;C3#>@2

LIVER METASTASIS

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Urine "iliru"inNor!ally, tiny a!ount "iliru"in (con&ugated) e3creted in urine

G 1rehe7atic &aundiceB 1aemolysis causes rise in uncon5ugated bilirubin("ater insoluble) and this is not e-creted by the kidney thereore there is norise in urine "iliru"in

G 8o!e causes of 2e7atic &aundice: result in damage to biliary cannaliculiand thereore result in poor biliary drainage and thereore ele#atedcon&ugated "iliru"in le#els in "lood, e3creted into urine (gi#ing dark

urine)G 1ost2e7atic &uandiceB >bstruction to biliary drainage and so con&ugated"iliru"in (water solu"le) le#els in the "lood increase and a77ear in theurine (gi#ing dark urine)

Urine uro"ilinogen1rehe7atic &aundiceB 1aemolysis results in increased bilirubin productionand subseuent increase bilirubin metabolism and urobilinogen in stool andthereore in the urine.

8o!e causes of 2e7atic &aundice B result in hepatocellular destruction andthereore reduced re+e-cretion o re+absorbed urobilinogen (i.e. ;eduction inentero+hepatic circulation o urobilinogen) resulting in elevated levels in urine

1ost2e7atic &aundiceB 'ess bilirubin reaching intestine thereore reduction

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1o" long been 5aundicedE =ver been 5aundiced beoreE 2ny associated evers or abdominal pain or "eight lossE 0ale stool and dark urine (suggests obstructive&post+hepatic

5aundice)E 2ny recent oreign travel (hepatitis! malaria)E 2ny risk actors or hepatitis (tattoos! 37D! high risk

proessions! blood transusions! multiple se-ual partners)E 0@1 o blood disorders (e.g. SCD! thalassemia)E

D1 any ne" medications that can cause 5aundiceE S1 e-cess alcohol intake ?1 o 5aundice (inherited disorders o bilirubin metabolism)

Deter!ining Aetiology of JaundiceB 2istory

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3 5aundice associated "ith background o intermittent ;Fpains think gallstones and choledocholithiasis

3 5aundice associated "ith long history o upper abdominalpain and "eight loss and patient elderly thing 7ancreaticcancer

3 5aundice associated "ith recent oreign travel thinkhe7atitis (A,E) or !alaria

3 5aundice occuring in patient "ith risk actors thinkhe7atitis 0,C

3 5aundice occuring on a background o alcohol abuse think

alcoholic li#er disease 3 5aundice is painless and amily history o blood disorder

think 7rehe7atic &aundice

Deter!ining aetiology of JaundiceB 2istory

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E3a!ination

Jaundice

A"do!inal e3a!ination

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Jaundice

* 8clera

- 8kin

Hucous

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'iver ailure and liver

cirrhosisEarly signs B?inger clubbing

'eukonychiaSpider naevi

0ody hair loss

0almer erythema

Duputyrenscontracture

/ate signs B Jaundice

3ll looking

Teticular atrophy GGG$$$gynaecomastia

2scites

?lapping tremor ( asteri-is )

caput medusaEnce7halo7athy

Intellectual change

Con#ulsions

Co!a

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Early signs B

Leukonychia Finger clubbing

Palmer eryhema

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Du7utyrens contracture

S!ider nae"i

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/ate signs B

ca7ut !edusa Ascites

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=yneco!astia

la77ing tre!or ( asteri3is )

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E3a!inationA"do!inal e3a!ination BB

1al7ation: liver : enlarged or shrunken

tenderness!!

gallbladder splenomegaly

allbladder $$$ enlarged ! palpable ! non tender mild 5aundie :::::: cour#oisires signs ::::

malignant CBD !"tru#tin nt "tn$

1ercussion: liver span !! ascites ..

2uscultation.

0re+hepatic1epatic 0ost+

hepatic

0resent 0resent absent

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#n#estigations/a"s

I!agingB &S ! CT! @;C0! @;3! =;C0! 0TC!

nuclear medicine! angiography

0io7sy

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Test 0re+hepatic 1epatic 0ost+hepatic Total bilirubin H HH HHH

Con5ugatedbilirubin

#ormal 3ncreased Increased

ncon5ugated

bilirubin

Increased 3ncreased #ormal

Total bilirubin and its con5ugated anduncon5ugated levels help to determine

nature o 5aundice

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'iver =nymes 2'T&2ST mainly present in hepatocytes

2'0&T mainly present in bile cannaliculi biliary tree

Deter!ining aetiology of &aundice

est 1rehe7atic 2e7atic 1osthe7atic

2'T&2ST #ormal raised H

2'0&T #ormal H raised

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$e% Pre-he!aic &e!aic Po%-he!aicUrine colour Normal Normal

If Dark (urobilinogen Hcon5ugated bilirubin )

'ark (con5ugated bilirubin )

Urine Bilirubin negative Negative #ncrea%ed

Urine urobilinogen #ncrea%ed Normal present 'ecrea%ed(negai"e

Stool colour Normal Normal Pale

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est 1rehe7atic 2e7atocellular O"structi#e

0lood glucose #ormal 'o" i liverailure

@ay be raised inpancreatic C2

2a7toglo"in 'o" #ormal or lo" iliver ailure

#ormal

1 . IN@ #ormal 0rolonged 0rolonged

C0C and @eticulocyte count ( raised in2e!olysis ) Al"u!in

2e7atitis serologyAnti!itochondrial A" , 0rimary Biliary Cirrhosis

Alfa feto7rotein

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ltrasound Cheap, noninvasive, good sensitivity , no radiation

). $here i% no duc dilaaion eiher in he li"er or in he e*rahe!aic bile duc%. the cause of

the jaundice is at a cellular level or involving microspcopic bile ducts too small to visualize on a

scan. Ex !medical" jaundice such as viral hepatitis, drug induced cholestasis or hepatitis,

metabolic disorders, autoimmune hepatitis, primary biliary cirrhosis and so on.

+A. All of he bile duc% in%ide he li"er ,inrahe!aic and ou%ide of he li"er ,e*rahe!aicare dilaed. #he level of obstruction must be at the lo$er end of the common bile duct. Ex as

ductal gall stones and , pancreatic cancer, stricture secondary to pancreatitis or malignant distal

bile duct cholangiocarcionoma or periampullary cancer.

+. /nly he common bile duc i% dilaed,0)1mm.$hen a gallstone has bloc%ed the lo$er end

of the bile duct but there has not been sufficient time for the intrahepatic bile ducts to become

dilated. #he same could be true of a tumour but usually by the time clinical jaundice is evident both intra and e&traheptic bile ducts $ill be dilated.

2. $he inrahe!aic bile duc% are dilaed ,03mm bu he e*rahe!aic bile duc i% colla!%ed

and non-dilaed.Its rare and implies that the cause of obstruction is at the hilus of the liver. #he

diagnosis that must be considered and e&cluded in this situation is hilar cholangiocarcinoma. #he

other less common alternative diagnoses include primary sclerosing cholangitis, 'irrizzi syndrome

and gall bladder cancer.

I!aging

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Endo%co!ic 4(S provides accurate imaging of stones in CB(, diagnosing and

staging pancreatic and periampullary cancers. )N* can be used $ith the

advantage of avoiding spillage of tumor cells into the peritoneal cavity

=allstone cholangiocarcino!a

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C co!7uted to!ogra7hy scan Triple+phase spiral CT is the gold standard for li#er

i!aging7ery useul or he7atic and 7ancreatic !asses! may be

used to guide biopsy

@;3 may be a good alternative (e.g. patients "ho can8t takecontrast)

/i#er !ets

@;C0

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@;C0

#oninvasive visualiation o biliary andpancreatic ducts

Same uality o =;C0 and 0TC "ithout thepotential complications !!!! #ot therapeutic

Hagnetic resonancecholangio7ancreatogra7hy

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E@C1endoscopy and Iuoroscopy

Endosco7ic retrogradecholangio7ancreatogra7hy

DiagnosticChronic pancreatitis

allstones "ith dilated bile ducts on ultrasonographyBile duct tumorsSuspected in5ury to bile ducts either as a result o trauma oriatrogenicSphincter o >ddi dysunction0ancreatic tumors

hera7eutic=ndoscopic sphincterotomy;emoval o stones3nsertion o stentDilation o strictures (e.g. primary sclerosing cholangitis!

anastomotic strictures ater liver transplantation)

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1Cused to visualie the anatomy o the biliary

tract.

3 ailed =;C0

used to perorm "iliary drainage ! !etalstents can be placed across malignant biliary

strictures to allo" palliative drainage

1ercutaneous ranshe7atic Cholangiogra7hy

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#uclear medicine2IDA (he7ato"iliary i!inodiacetic

acid ) scan

is used or gallbladder abnormalities2cute cholecystitis: B "ill not take isotope in

flling phase

Biliary obstruction: ailure&incomplete e-cretiono isoptope in secretory phase

1E scan is useul or detecting primary

and secondary liver malignancies

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Biopsy&S+guided needle biopsy o the liver may be"arranted i no biliary dilation "as ound todemonstrate hepatic pathologies.

2lso useul or hepatic lesions.

3n the presence o 5aundice! 0T should becorrected beore biopsy

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!anage!ent2l"ays manage the underlying cause

0ruritis can be managed by cholestyramine!antihistamines! ursodeo-colic acid

?at+soluble vitamins

0re+operatively! make sure that the patient is "ell+hydrated and has no coagulopathy

Surgical options'aparoscopic cholecystectomy;esection o neoplastic lesion

Sphinctertomy ! Stent insertion

'iver trnasplant

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T1= =#D

Jaundice 2015

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