presentation jaundice

1. By
Dr. Abdul Majid Siddiqui

2.  Definition: yellow discolouration of skin and sclera as a result
of hyperbilirubinaemia
 Bilirubin >35mmol/L for jaundice to be visible on
examination
 Sclera first place to become jaundiced

3.  Bilirubin is a product of metabolism of haemoglobin (80%) and other haem
containing proteins (e.g. Myoglboin, cytochrome P450: 20%)
 Degredation of haemoglobin into bilirubin takes place in macrophages. Bilirubin
is then excreted into plasma and binds with albumin
Haemoglobin
Globin Haem (iron + porphyrin)
Amino
Acids Biliverdin
Bilirubin
(unconjugated)
Bilirubin binds to albumin in
the plasma
RBC breakdown

5.  Pre-hepatic: pathology occuring prior to the liver
 Any cause of increased haemolysis (e.g. Spherocytosis,
thalassaemia, sickle cell disease, transfusion reaction, auto-
immune, malaria etc.) and some drugs
 Causes unconjugated hyperbilirubinaemia
 Intra-Hepatic: pathology occuring within the liver
 All the causes of hepatitis/cirrhosis (e.g. Alcohol, viral, auto-
immune, primray biliary cirrhosis etc)
 Can result in hepatocyte destruction and therefore
unconjugated hyperbilirubinaemia or in bile cannaliculi
destruction and therefore conjugated hyperbilirubinaemia or
both

6. Post-hepatic:
Pathology occuring after conjugation of bilirubin within the
liver (also known as obstructive jaundice)
•Any cause of biliary obstruction (e.g. Gallstones)
•Causes conjugatedhyperbilirubinaemia

7. PHYSIOLOGICAL JAUNDICE
Usually mild form of jaundice appears in some newborn children on
the second or third day of life,called jaundice of newborn or Neonatal
jaundice.
CAUSE---
Excessive destruction of R B Cs after birth causing increase in
serum bilirubin.
Due to hepatic immaturity-- During intrauterine life bilirubin formed
is mainly eliminated via the placenta and to lesser extent by liver.
Immediately after birth the liver has to eliminate all the bilirubin
formed, therefore, liver is unable to deal adequately with this increase
amount of bilirubin during the first 10 days of life causing
development of jaundice
When S. Bilirubin risebeyond 5mg/dL clinical jaundice apperars
Treatment----Phototherapy

8. Test Pre-hepatic Hepatic Post-hepatic
Total bilirubin + ++ +++
Conjugated
bilirubin
Normal Increased Increased
Unconjugated
bilirubin
Increased Increased Normal
 Total bilirubin and its conjugated and unconjugated levels help
to determine nature of jaundice

9.  Liver Enzymes
 ALT/AST mainly present in hepatocytes
 ALP/GGT mainly present in bile cannaliculi biliary tree
 Derrangement of particular liver enzymes in
association with jaundice can determine nature of
the jaundice
Test Pre-hepatic Hepatic Post-hepatic
ALT/AST Normal +++ +
ALP/GGT Normal + +++

10. Hemoglobinopathy is a genetic disorder caused by
abnormal polypeptide chains of hemoglobin.
Some of the hemoglobinopathies are:
i. Hemoglobin S: It is found in sickle cell anemia.
In this, the α-chains are normal and β-chains are abnormal.
ii. Hemoglobin C: The β-chains are abnormal. It is
found in people with hemoglobin C disease, which
is characterized by mild hemolytic anemia and splenomegaly.
Hemoglobinopathies

11. iii. Hemoglobin E: Here also the β-chains are abnormal.
It is present in people with hemoglobin E disease which is also
characterized by mild hemolytic anemia and splenomegaly.
iv. Hemoglobin M: It is the abnormal hemoglobin
present in the form of methemoglobin. It occurs due to mutation
of genes of both in α and β chains, resulting in abnormal
replacement of amino acids. It is present in babies affected by
hemoglobin M disease or blue baby syndrome. It is an inherited
disease, characterized by methemoglobinemia