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Jaundice
Dorota Mańkowska -Wierzbicka, MD,
PhD
Jaundice
Definition
Accumulation of yellow pigment in the
skin and other tissues (Bilirubin)
Jaundice definition
It arises from the abnormal accumulation
of bilirubin in body tissues, which occurs
when the serum bilirubin level exceeds
3 mg/dL or 50 umol/L.1 Excess bilirubin
causes a yellow tinting to the skin, sclera,
and mucous membranes.
Jaundice
The presence of jaundice can indicate a
transient hepatitis, biliary obstruction, or
progressive deterioration in a patient
awaiting liver transplantation. Thus, the
appropriate management of the patient with
jaundice depends on localizing the site and
nature of the problem.
Jaundice
Hyperbilirubinemia is defined as a total
bilirubin level greater than 1.5 mg/dL, an
unconjugated level greater than 1 mg/dL, or
a conjugated bilirubin level greater than 0.3
mg/dL.
Jaundice
Jaundice- Causes Of Hyperbilirubinemiaunconjugated
Hemolysis
Ineffective erythropoesis
Neonatal causes
Uridine diphosphate glucuronoyltransferase
deficiencies
Gilbert syndrome
Crigler-Najjar syndromes
Miscellaneous causes (drugs, hypothyroidism,
thyrotoxicosis, pulmonary infarct, fasting)
Gilbert Syndom
Defective UDPG Transferase
Uridine diphosphate glucuronoyltransferase
Usually asymptomatic
Serum BR < 3 mg%
↑ BR-monoglucuronide
Benign
Crigler –Najjar Syndrome Type I
No UDPGT
No BR conjugation
Neonatal kernicterus, death
Autosomal recessive
Crigler–Najjar Syndrom Type II
Reduced UDPGT activity
Jaundice, 2d-3d decade of life
Mostly monoglucuronide in bile
Usually benign
Treatment: Phenobarbital, induces
more UDPGT
Jaundice- Causes Of Hyperbilirubinemiaconjugated
Congenital causes
Rotor syndrome
Impaired biliary excretion of conjugated BR. Jaundice
appearing in childhood. Autosomal recessive.
Uncommon.Benign
Dubin-Johnson syndrome
Defective transporter for conjugated BR. Uncommon. Benign.
Conjugated BR in blood & urine
Choledochal cysts
Familial disorders
Benign recurrent intrahepatic cholestasis
Cholestasis of pregnancy
Jaundice- Causes Of Hyperbilirubinemiaconjugated
Hepatocellular defects
Alcohol abuse
Viral infection
Cholestatic
Primary biliary cirrhosis
Primary sclerosing cholangitis
Biliary obstruction
Pancreatic disease
Jaundice- Causes Of Hyperbilirubinemiaconjugated
Systemic disease
Infiltrative disorders
Postoperative complications
Renal disease
Sepsis
Drugs
Jaundice
Unconjugated hyperbilirubinemia exists if more
than 80% to 85% of the total bilirubin is
unconjugated. Conjugated hyperbilirubinemia
exists if greater than 30% of the total bilirubin
level is conjugated. Overproduction of bilirubin
results in unconjugated hyperbilirubinemia. Mild
hyperbilirubinemia may require blood or urine
tests for detection, as jaundice usually does not
become apparent until the bilirubin level exceeds
3 mg/dL.
Pathophysiologic classification of
Jaundice
Hemolytic Jaundice
Hepatic Jaundice
Obstructive Jaundice
Hemolytic Jaundice
Pathogenesis
Overproduction
Hemolysis (intra and extra vascular)
inherited or genetic disorders
acquired immune hemolytic anemia
(Autoimmune hemolytic anemia)
nonimmune hemolytic anemia
(paroxysmal nocturna Hemoglobinruia)
Ineffective erythropoesis
Overproduction may overload the liver with UB
Hemolytic Jaundice
Symptoms
weakness, Dark urine, anemia,
Icterus, splenomegaly
Lab
UB↑
↑ without bilirubinuria
fecal and urine urobilinogen↑
↑
hemolytic anemia
hemoglobinuria (in acute intravascular hemolysis)
Reticulocyte counts↑
↑
Hepatic Jaundice
Due to a disease affective hepatic
tissue either congenital or acquired
diffuse hepatocellular injury
Hepatic Jaundice
Pathogenesis
Impaired or absent hepatic conjugation of bilirubin
Familiar or hereditary disorders
•
decreased GT activity (Gilbert‘s syndrome)
hereditary absence or deficiency of UDPGT (Grigler-Najjar
Syndrome)
Dubin-Johnson Syndrome
Rotor syndrome
Acquired disorders
hepatocellular necrosis
intrahepatic cholestasis
(Hepatitis, Cirrhosis, Drug-related)
Hepatocellular defects
Viral hepatitis
Hepatitis A
Hepatitis B
Hepatitis C
Hepatitis D
Hepatitis E
Hepatitis G
Hepatocellular defects
Alcohol
Liver
abuse
steatose (fatty liver)
Hepatitis
Cirrhosis of the liver
Obstructive Jaundice
Pathogenesis
it is due to intra- and extra hepatic obstruction
of bile ducts
intrahepatic Jaundice: Hepatitis, PBC, Drugs
Extra Hepatic Biliary Obstruction: Stones,
Stricture, Inflammation, Tumors, (Ampulla of
Vater)
Cholestasis
PBC
PSC
Biliary obstruction
Pancreatic disease
PBC
The clinical features include jaundice, pruritus,
steatorrhea, xanthomas, and osteopenia. In
advanced disease, signs of portal hypertension will
occur with esophageal varices, ascites, and other
conditions.
Primary biliary cirrhosis is a chronic progressive
cholestatic disorder typically, although not
exclusively, found in women in the fourth to fifth
decade of life.
PBC
laboratory abnormalities:
a characteristic isolated alkaline phosphatase
elevation out of proportion to other liver
function tests,
Elevated
serum IgM,
cholesterol,
antimitochondrial antibody (AMA) .
PBC
laboratory abnormalities:
a characteristic isolated alkaline phosphatase
elevation out of proportion to other liver
function tests,
Elevated
serum IgM,
cholesterol,
antimitochondrial antibody (AMA) .
PSC
Progressive cholestatic liver disease, which is
found mostly in men of middle age.
Inflammation of both intrahepatic and
extrahepatic bile ducts
Clinically, patients present with recurrent
abdominal pain, fever, and intermittent jaundice
and later with symptoms of portal hypertension.
Characteristic narrowing or "beading" occurs in
the hepatic ducts, as demonstrated by
cholangiogram.
PSC vs. UC
Chronic inflammatory bowel diseases, such
as ulcerative colitis or Crohn , are
associated in 30% to 75% of primary
sclerosing cholangitis patients
Of all patients with ulcerative colitis, 5% to
10% will have primary sclerosing
cholangitis.
Obstructive Jaundice
symptoms
Pruritus
Jaundice may vary in intensity
Chill+fever+gall bladder enlargement
→stone+cholangitis
Obstructive Jaundice
Lab Findings
Serum Bilirubin↑
↑
Feceal urobilinogen↓
↓ (incomplete obstruction)
Feceal urobilinogen absence (complete
obstruction)
urobilinogenuria is absent in complete
obstructive jaundice
bilirubinuria ↑
ALP ↑ , GGTP ↑
cholesterol ↑
Evaluation to the patient with jaundice
The investigation of a patient with jaundice
begins with a thorough review of the history
of presentation, medication use, past
medical history, physical examination, and
evaluation of liver function tests.
Evaluation to the patient with jaundice
Several questions must be answered initially:
1.
Is the elevated bilirubin conjugated or unconjugated? In
general, most jaundiced patients will not have an isolated
unconjugated hyperbilirubinemia.
2.
If the hyperbilirubinemia is unconjugated, is it caused by
increased production, decreased uptake, or impaired
conjugation?
3.
If the hyperbilirubinemia is conjugated, is the problem
intrahepatic or extrahepatic?
4.
Is the process acute or chronic?
Evaluation to the patient with jaundice
Patients with conjugated hyperbilirubinemia
usually have acquired disease- identify an
intrahepatic or an obstructive cause.
Acute disease usually can be differentiated from
chronic disease by the patient's history, physical
examination (xanthelasma, spider angioma,
ascites, or hepatosplenomegaly), and laboratory
tests (hypoalbuminemia, thrombocytopenia, and
an uncorrectable prolongation of the prothrombin
time).
Evaluation to the patient with jaundice
Chronic cholestasis may arise from such
diseases as cirrhosis, primary sclerosing
cholangitis, primary biliary cirrhosis,
secondary biliary cirrhosis, or carcinoma or
from drugs.
Patients with chronic cholestasis usually do
not have hepatitis or gallstones.
Evaluation to the patient with jaundice
The presence of fever, right upper quadrant
pain, tenderness, hepatomegaly, and newonset bilirubinuria usually indicates acute
disease.
Patients older than 50 years of age with
asymptomatic cholestasis and mild
hepatomegaly may have carcinoma of the
pancreas or biliary tree.
Evaluation to the patient with jaundice
Women aged over 30 years of age are more
likely to have choledocholithiasis.
Gallstones
cholelithiasis
the formation or
presence of stones in
the gallbladder or
common bile duct.
chole = bile, lith =
stone, iasis =
condition
Gallstones
biliary colic: pain associated with gallstones that have entered the
common bile duct.
choledocholithiasis the presence of gallstones in the common bile
duct.
cholecystitis inflammation of the gallbladder.
cholangitis inflammation of the bile ducts.
cholecystectomy : surgical removal of the gallbladder.
Gallbladder Disorders
Risk factors for cholelithiasis
a. Age
b. Family history, also Native Americans and
persons of northern European heritage
c. Obesity, hyperlipidemia
d. Females, use of oral contraceptives
e. Conditions which lead to biliary stasis:
pregnancy, fasting, prolonged parenteral nutrition
f. Diseases including cirrhosis, ileal disease or
resection, sickle-cell anemia, glucose intolerance
Gallbladder Disorders
Manifestations of cholelithiasis
a.
Many persons are asymptomatic
b.
Early symptoms are epigastic fullness after meals or
mild distress after eating a fatty meal
c.
Biliary colic (if stone is blocking cystic or common bile
duct): steady pain in epigastric or RUQ of abdomen lasting
up to 5 hours with nausea and vomiting
d.
Jaundice may occur if there is obstruction of common
bile duct
Gallbladder Disorders
Manifestations of acute cholecystitis
a. Episode of biliary colic involving RUQ
pain radiating to back, right scapula, or
shoulder; the pain may be aggravated by
movement, or deep breathing and may last 12
– 18 hours
b. Anorexia, nausea, and vomiting
c. Fever with chills
Evaluation to the patient with jaundice
An adult with asymptomatic, isolated
unconjugated hyperbilirubinemia who is
not taking any drugs and has no evidence of
hemolysis probably has Gilbert syndrome
and can be monitored with bilirubin
determinations for 12 months. If no
abnormality develops, no further evaluation
is needed.
Evaluation to the patient with jaundice
Fever, leukocytosis, and hypotension point
to ascending cholangitis
Asterixis, confusion, or stupor may indicate
severe hepatocellular dysfunction or
fulminant hepatocellular failure
Clinical symptoms
Fever
Chills
Weight loss
Flu like symptoms
Abdominal pain
Anorexia
Nausea, and vomiting
Pruritus
Physical examination
General appearance
cachexia, muscle wasting, palmar erythema,
Dupuytren contracture, abnormal nails,
parotid enlargement, or xanthelasmas,
gynecomastia, spider nevi, or dilated veins.
Physical examination
The size and consistency of the liver
shrunken, nodular liver - cirrhosis,
a palpable mass - an abscess or malignancy.
If the liver span is greater than 15 cm- fatty infiltration,
congestion, other infiltrative diseases, or malignancy.
Liver tenderness may denote acute disease but is
generally not helpful.
The presence of a friction rub or bruit suggests
malignancy.
Clinical examination
Spider angioma, palmar erythema, and distended abdominal veins
Ascites in the presence of jaundice (cirrhosis, malignancy and severe
acute disease, such as viral or alcoholic hepatitis)
Splenomegaly (infections, infiltrative diseases, viral hepatitis, or
cirrhosis)
A palpable, distended gallbladder suggests malignant biliary
obstruction.
Asterixis in fulminant hepatic failure and end-stage liver disease.
Patients with biliary colic or infection may have fever.
Laboratory tests
TB (conjugated, unconjugated)
AspAT, AlAT
GGTP
AP
TP + (albumin, gammaglobulin)
Prothrombin
Laboratory tests
TCh
AMA
5’-NT
LAP
Alfa1-antitrypsin
Iron levels
Ceruloplasmin
Alfa-fetoprotein (AFP)
Antinuclear antibody
Noninvasive tests
US
CT
Radionuclide imaging is an excellent means
of detecting cystic duct obstruction. It is the
test of choice if acute cholecystitis is
suspected, but it has little value in
differentiating intrahepatic from
extrahepatic causes of cholestasis.
Invasive tests
ERCP - endoscopic retrograde
cholangiopancreatography
PTC - percutaneous transhepatic
cholangiography
liver biopsy
ERCP
ERCP is 90% successful regardless of the
presence or absence of ductal dilation and can
localize the site of obstruction in more than
90% of patients. It is particularly helpful in
diagnosing patients with common duct stones.
Because it has therapeutic capabilities, it
allows some patients to avoid surgery. ERCP
is also helpful if a stricture due to chronic
pancreatitis is suspected.
PTC
PTC visualizes the biliary tree in 90% to 100% of patients
with dilated ducts and localizes the site of obstruction in
90% of cases. It locates the obstruction in 50% to 90% of
patients without dilated ducts, as determined by
ultrasound. PTC is less expensive than ERCP and often is
easier to perform. The patient must have a prothrombin
time of less than 16 seconds, a platelet count greater than
50,000, and no ascites. Minor complications occur in 30%
of patients. Major complications, including sepsis,
bleeding, biliary leak, pneumothorax, arteriovenous
fistula, hematoma, abscess, and peritonitis, occur in 1% to
10% of patients who undergo PTC.