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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 is a
little bit yellow and it turns out their
having conjugated rather than
unconjugated hyperbilirubinemia and it's
important in these conditions to know
what exactly is going on
so the definition of conjugated
hyperbilirubinemia is more than one
milligram per deciliter of total if the
total of bilirubin is less than five or
more than 20% of your total bilirubin if
the total bilirubin is more than five so
we have to remember that five cutoff in
terms of how we define hyper cup
bilirubin emia the differential
diagnosis for a child with conjugated
hyperbilirubinemia is generally either
an extra hepatic problem where the bile
can't get out or an intra hepatic
problem elevated conjugated bilirubin is
never normal in a neonate and that's an
incredibly important fact unconjugated
or indirect hyper Billy 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
conjugated hyperbilirubinemia 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 consent when
as 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 a hepatitis and
children
speaking about the delivery and where
there are any complications and
certainly where there are any infectious
exposures 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 the hepatic disease
is affecting them or if this is part of
the syndrome accession 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 Alagille which we'll talk about an
admit 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 and 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 emia that's because while most
children are indirect these children
with conjugated hyperbilirubinemia can't
be missed and should not be lit with
lights as they will develop copper baby
syndrome of course checking the liver
function tests is important and don't
forget the GGT which is useful for
assessing the biliary tree because many
of these children have abnormalities of
the biliary tree synthetic function
tests like pt/ptt INR are important for
understanding whether liver is working
it's not enough to
just know it's inflamed we also want to
know whether it's functioning correctly
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