Vous êtes sur la page 1sur 45

Dr.

MABEL SIHOMBING SpPD-KGEH


Dr.ILHAMD SpPD
DPERTEMEN ILMU PENYAKIT DALAM
What RS.HAM/FK-USU MEDAN
?
Who?
PERUBAHAN WARNA ( KULIT,SKLERA MATA
ATAU JARINGAN LAIN ) MENJADI KUNING
DISEBABKAN PENINGKATAN KONSENTRASI
BILIRUBIN DALAM SIRKULASI DARAH.
BILIRUBIN TERBENTUK DISEBABKAN OLEH
PEMECAHAN CINCIN HEM PADA METABOLISME
SEL DARAH MERAH.
KATA IKTERUS/JAUNDICE BERASAL DARI
PERANCIS JAUNE BERARTI KUNING.
IKTERUS/JAUNDICEKADAR BILIRUBIN >3
MG/DL
Bilirubin Metabolism
- Bilirubin breakdown of mature RBCs in the RES.

- 15% of bilirubin comes from the catabolism of other


haem-containing proteins, such as myoglobin.

- 250 300 mg of bilirubin are produced daily.

- Biliverdin is formed from the haem and this is reduced to


form bilirubin.
- The bilirubin produced is unconjugated and is transported to
the liver attached to albumen.
Bilirubin Metabolism
RES (SPLEN,LIVER,BONE MARRAOW)
* Increased bil. production (e.g.
haemolysis), or resorption of haematoma.
Inability of the hepatocytes to take up
bilirubin from the blood e.g. Gilbert &
Crigler-Najjar syndromes.
By (for example) biliary calculi causes
backup and reabsorption of
conjugated bilirubin.
Fisiologi produksi dan transportasi
bilirubin
Jaundice can occur in four
different ways:
1- Increased bilirubin load as in haemolysis.
2- Disturbance in the hepatic uptake &
transport
of bilirubin within the hepatocytes
3- Defects in conjugation.
4- Defects in the excretion of conjugated
bilirubin across the canalicular cell
membrane or an obstruction of the large
biliary channels.
Pathophysiologic
classification of Jaundice

Hemolytic Jaundice

Hepatic Jaundice

Obstructive
Jaundice(Cholestasis)

Congenital Jaundice
Jaundice classification
predominantly unconjugated
hyperbilirubinaemia

predominantly conjugated
hyperbilirubinaemia
Causes:
1. Increased bilirubin
production
Lead to increases in
2. Reduced bilirubin uptake
free (unconj.) bilirubin
by hepatic cells
3. Disrupted intracellular
conjugation
4. Disrupted secretion of
bilirubin into bile
canaliculi Result in rise in conj.
5. Intra/extra-hepatic bile bilirubin levels
duct obstruction
Causes of Jaundice
Pre-hepatic unconjugated
hyperbilirubinaemia

Haemolysis
Congenital defects:
Gilbertssyndrome
(uptake/conjugation defect)
Crigler-Najar (conjugation defect)
Isolated elevation of serum Bilirubin

Unconjugated Hyperbilirubinaemia
* Increase bil. production (e.g. haemolysis,
resorptionof haematoma)
* Decrease hepatocellular uptake (e.g.
rifampicin)
* Decrease conjugation (Gilbert S, Crig.
Nagar S)
Conjugated Hyperbilirubinemia
* Dubin-Junson syndrome
* Roter syndrome
Causes of Jaundice
Hepatocellular
Acute Chronic
Viral hepatitis A, B, C.. Viral hepatitis B, C
Other viruses: EBV,
Chronic AI hepatitis
CMV
End-stage liver
Drugs

Dose-dependant e.g. disease (of any


paracetamol cause)
Idiosyncratic Alcoholic

Toxins Hepatitis B, C
Autoimmune hepatitis
Autoimmune
Alcoholic hepatitis
Haemochromatosis
Tumours
Wilsons disease
Causes of Jaundice

Cholestatic
Extra-hepatic Intra-hepatic

Gallstones Drugs
Carcinoma of head of Primary biliary
pancreas
Benign stricture cirrhosis
Congenital Cholestatic phase
Traumatic of viral hepatitis
iatrogenic
Alcoholic hepatitis
Carcinoma of
ampulla of Vater or Primary or
bile ducts secondary cancer
Sclerosing Lymphoma
Cholangitis
pancreatitis Pregnancy
Clinical symptoms and
signs
History:
- The onset of Jaundice in viral hepatitis is
associated with a prodrome of ANV,
malaise & myalgia.
- The onset of cholestasis is insidious, it is
associated with pruritus.
- A history of fever with rigors, Rt upper
abd. pain or a past history of biliary
surgery suggest cholangitis.
- Dark urine & pale stool exclude the
possibility of haemolytic jaundice.
(related autoimun).
- A history of multiple sex partners, travel,
ethanol intake, drugs, bl. transfusion,
needlestick exposure & tattooing is also
important.
- Recent surgery with subsequent jaundice
after one week may suggest halothane
toxicity.
- Previous biliary surgery with subsequent jaundice
may suggest stricture, residual stones or hepatitis.
- A family history of jaundice or liver disease
suggests the possibility of hereditary
hyperbilirubinaemia or genetic disorder such
as Wilson disease.
- Painless jaundice in older person with epigastric
mass & weight loss = biliary obstruction from
malignancy
The clinical assessment & basic biochemical
parameters lead to three broad subgroups of
patients:
1- Isolated elevation of s. bilirubin: when AST,
ALT & ALP levels are normal.
2- Hepatocellular jaundice: when the AST & ALT
levels are elevated out of proportion to the ALP
levels.
3- Cholestatic jaundice: when the ALP level is
elevated out of proportion to the AST & ALT
levels.
Examination
Pale yellow vs. deep yellow
Signs of cirrhosis,Sp.Nevy,Caput
Medusa,Gynecomasty,ascites,Palmar
Erytema,.
Liver tender, enlarged, firm, shrunken,
irregular
Gallbladder tender (Murphys sign),
palpable
splenomegaly
Stigmata of Chronic Liver Disease

Palmar eryth. Spider nevi Parotid enlag Gynecomastia

Muscle atrop.
atrop Astraxis WHITE NAIL EXTR. edema
Spooning
Laboratory Tests
Serum bilirubin level CBC
(total and direct) PT
Liver Other labs pertinent
aminotransferase to history
levels,Albumin Coombs test
Alk. Phos
Hgb electrophoresis
U/A for bilirubin and
Viral hepatitis panel
urobilogen
U/S Gallbladder
Investigations
Pre-hepatic Hepatic Post-hepatic

Urine No Bilirubin ? Bilirubin Bilirubin


Urobilinogen Urobilinogen Urobilinogen

Faeces Dark Pale Pale

Blood FBC - Bilirubin Bilirubin (up to


Reticulocyte mixed 1000mol/L)
count conjugated & conjugated
Coombs test unconjugated ALP, GT
Bilirubin (up to ALP, GT PT
100mol/L) AST, ALT correctable with
unconjugated PT not Vit K
ALP Normal correctable with
PT Normal Vit K
RADIOLOGI
CHEST RONTGEN

ULTRASONOGRAPY :
EUS:ENDOS-ULTRASONO
ENDOSKOPY: GASTROSKOPY,ERCP
CT-ABDOMEN/MRI/MRCT
BIOPSI
Imaging Procedures:

Radiological imaging is important for


the diagnosis of a focal liver mass or
biliary disease. However, imaging
plays little role in the evaluation of
diffuse hepatocellular e.g. hepatitis
Ultrasonography (US):
It is a valuable but operator-dependent
investigation.
It has sensitivity of 55-91% &
specificity of 82-95% for biliary
obstruction.
Although US may not detect stones in
the extrahepatic bile duct, which may
be obscured by overlying gas, it
reliably establishes the presence of a
dilated bile ducts
Ultrasonography (US): Cont

Beside it can differentiate


intrahepatic from extrahepatic
cholestasis, US can also detect the
associated abnormalities such as
portal hypertension, focal lesions &
fatty liver.
USG ACUTE HEPATITIS

ENLARGE LIVER

NORMAL

HEPATIC PARENCHYMAL
ECHODENSITY DECREASED.

PORTAL TRIADE PROMINENCE


Gall Bladder Carcinoma

Doppler: internal vascularity within the


material
Stone and non shadowing echogenic
material

Doppler wave form


analysis
documents arterial
flow
within the mass wich
histologically
Computerized Tomography (CT):
CT has a sensitivity of 63-96% & a specificity
of 93-100% to detect biliary obstruction.

Non-calcified cholestrol gall stones can be


easily missed on CT because they may be
isodense with bile.
Endoscopic retrograde
cholangiopancreaticography (ERCP):

ERCP not only permits direct


visualization of the biliary tree but
also allows therapeutic intervention
e.g. removal of CBD stones or biliary
stenting. It is the gold standard test for
the evaluation of extrahepatic biliary
disease causing jaundice.
Benign distal CBD stricture
Magnetic resonance
cholaniopancreaticography: MRCP
MRCP is superior to US & CT in
detecting biliary obstruction. It has a
sensitivity of 82-100% & a specificity
of 92-98% to detect biliary
obstruction.
Liver Biopsy:
It has relatively low risk, it is needed in
only a minority of cases with hepatic
dysfunction.

Major indications include chronic


hepatitis, cirrhosis, unexplained
liver enzyme abnormalities,
hepatosplenomegaly of unknown
aetiology, suspected infiltrative
disorder, suspected
granulomatous disease.
Liver Biopsy: Cont

Relative contraindications include a


tendency for clinical bleeding,
INR>1.5 or PT >3 sec above the
control, severe thrombocytopenia &
marked ascites.

The risk of fatal haemorrhage in


patients undergoing LB is 0.4% if
they have a malignancy & 0.04% if
they have non-malignant disease.
Management
ANEMIA HEMOLITIC HEMATOLOGY
DIVISION TREATMENT

GENETICAL DISORDER GEN


THERAPY AND SYMPTOMATIC
THERAPY

HEPATIC CELLULER ICTERIC


ANTIVIRAL THERAPY
Management (cont)
Symptom relief
Pain, itch
Fluid resuscitation
Correction of coagulopathy
Treat secondary complications
Sepsis, bleeding, anaemia
Treat underlying cause
Medical or surgical
ENDOSCOPY TREATMENT AND
Surgical Management
Relieve obstruction
Definitive or temporising, Curative or palliative

ERCP / PTC
Remove stones
Stent or dilate stricture

Surgery

Cholecystectomy with bile duct exploration

Resection of obstructing tumour

Whipples procedure

Bypass of irresectable lesion


ERCP and stent insertion for obstructing cholangiocarcinoma
Thank You