In this lecture, we’re going
to discuss conjugated
hyperbilirubinemia and liver
diseases in children.
This happens every now and then, a child
is born who’s a little bit yellow
and it turns out they’re
rather than unconjugated
And it’s important in these conditions
to know what exactly is going on.
So the definition of
is more than 1 mg per deciliter, if
the total bilirubin is less than 5.
Or more than 20% of your total bilirubin
if the total bilirubin is more than 5.
So we have to remember that 5 cut off in
terms of how we define hyperbilirubinemia.
The differential diagnosis for a child
with conjugated hyperbilirubinemia
is generally either an
where the bile can’t get out
or an intrahepatic problem.
Elevated conjugated bilirubin
is never normal in a neonate
and that’s an incredibly
Unconjugated or indirect hyperbili is
very common and we see it all the time.
But when it’s conjugated, there
is definitely a problem.
So when you see a patient with
it’s important to
take a good history.
Family history is critical because
there are inherited syndromes
where children are more
prone to liver disease.
Asking about consanguinity is
always important as well because
obviously with consanguineous families,
recessive disorders are
more likely to show up.
Understanding if there were any pregnancy
complications can be helpful as well
as some of these conditions are
syndromic and there may be a problem.
Or even more likely, the mother may have
obtained an infection during pregnancy.
Some of the TORCH infections can
cause hepatitis in children.
Asking about the delivery and whether
there were any complications
and certainly whether there were any infectious
exposure to the child are important.
It’s critical to do a good
physical exam on these children.
Specifically, knowing their growth parameters
and plotting them on a growth curve,
both height, weight and head circumference
is critical to understanding
how this child is developing and if
their hepatic disease is affecting them
or if this is part of a
Understanding vital signs and making
sure they’re normal are critical.
And of course, a skin evaluation will
give you a sense of just how bad
the hyperbilirubinemia might be prior
to actually getting the lab value.
Conjunctival icterus is common in these
patients and it may be how you find it.
The best place to look for
jaundice may be under the tongue,
around the frenulum area.
Patients, if they have a cardiac condition
and there are some conditions like
Alagilles which we’ll talk about in a bit,
that are associated
with cardiac findings.
So understanding a cardiac murmur and
listening for one is important as well.
The abdominal exam is
of course critical.
Especially feeling for that liver
edge or doing the scratch test,
which may be an even better way to
assess for hepatomegaly in children.
Of course, labs are the mainstay and
so obtaining a bilirubin is important.
Remember especially in babies, you
can’t just get a total bilirubin,
you need to break it down into
direct and indirect bilirubin.
That’s because while most
children are indirect,
these children with conjugated
hyperbilirubinemia cant’ be missed
and should not be lit with lights as
they will develop copper baby syndrome.
Of course, checking the liver
function test is important
and don’t forget the GGT, which is
useful for assessing the biliary tree
because many if these children have
abnormalities of the biliary tree.
Synthetic function tests
like PT, PTT and INR
are important for understanding
whether the liver is working.
It’s not enough to just know it’s inflamed,
we also want to know whether
it’s functioning correctly