00:02
In this lecture, we’re going to
discuss conjugated hyperbilirubinemia
and liver diseases in children.
00:09
This happens every now and then,
a child is born who’s a little bit yellow
and it turns out they’re
having conjugated
rather than unconjugated
hyperbilirubinemia.
00:19
And it’s important
in these conditions
to know what
exactly is going on.
00:25
So the definition of
conjugated hyperbilirubinemia
is more than 1 mg per deciliter,
if the total bilirubin is less than 5.
00:37
Or more than 20% of your total bilirubin
if the total bilirubin is more than 5.
00:44
So we have to remember that 5 cut off in
terms of how we define hyperbilirubinemia.
00:52
The differential diagnosis for a child
with conjugated hyperbilirubinemia
is generally either an
extrahepatic problem
where the bile can’t get out
or an intrahepatic problem.
01:06
Elevated conjugated bilirubin
is never normal in a neonate
and that’s an incredibly
important fact.
01:13
Unconjugated or indirect hyperbili is
very common and we see it all the time.
01:19
But when it’s conjugated,
there is definitely a problem.
01:23
So when you see a patient with
conjugated hyperbilirubinemia,
it’s important to
take a good history.
01:30
Family history is critical because
there are inherited syndromes
where children are more
prone to liver disease.
01:38
Asking about consanguinity is
always important as well because
obviously with
consanguineous families,
recessive disorders are
more likely to show up.
01:48
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.
01:56
Or even more likely, the mother may have
obtained an infection during pregnancy.
02:02
Some of the TORCH infections
can cause hepatitis in children.
02:08
Asking about the delivery and
whether there were any complications
and certainly whether there
were any infectious exposure
to the child are important.
02:16
It’s critical to do a good
physical exam on these children.
02:19
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 syndromic situation.
02:36
Understanding vital signs and making
sure they’re normal are critical.
02:39
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.
02:49
Conjunctival icterus is common in these
patients and it may be how you find it.
02:54
The best place to look for
jaundice may be under the tongue,
around the frenulum area.
03:01
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.
03:12
So understanding a cardiac murmur and
listening for one is important as well.
03:17
The abdominal exam is
of course critical.
03:20
Especially feeling for that liver
edge or doing the scratch test,
which may be an even better way to
assess for hepatomegaly in children.
03:30
Of course, labs are the mainstay and
so obtaining a bilirubin is important.
03:36
Remember especially in babies,
you can’t just get a total bilirubin,
you need to break it down into
direct and indirect bilirubin.
03:45
That’s because although most
children with neonatal jaundice
have elevated indirect or
unconjugated bilirubin.
03:52
The children with direct or conjugated
hyperbilirubinemia can't be missed
because they should not
be given phototherapy.
04:00
Otherwise, they may develop
bronze baby syndrome.
04:04
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.
04:18
Synthetic function tests like
PT, PTT and INR
are important for understanding
whether the liver is working.
04:26
It’s not enough to just
know it’s inflamed,
we also want to know whether
it’s functioning correctly.