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88 Cards in this Set

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What is the 3rd step when looking for cholestasis?
Check for metabolic problems
What are some metabolic problems that would cause cholestasis?
CF (newborn screen and sweat test)
galactosemia (newborn screen)
What is the 4th step with looking for cholestasis?
check for anatomical problems
How can you test for anatomical problems that cause cholestasis?
Liver Biopsy
At what point do you biopsy the liver?
high suspicion of biliary atresia OR alagille syndrome
failing liver
extremely prolonged cholestasis with no clear etiology
What would cause you to suspect biliary atresia?
US doesn't show bile duct
stools are white
bilirubin continues to climb
What component of Alagille Syndrome contributes to cholestasis?
paucity of bile ducts
What is indictative of a failing liver?
Coags increasing
Albumin decreasing
If a pt has no clear etiology for cholestasis, what should you do?
Biopsy the liver--liver may just have small bile ducts (but not have any other symptoms of alagille syndrome)
When would you perform a percutaneous biopsy?
Stable pt
there is US assistance
Why is US assistance helpful?
Good for assesing size of liver, how far to insert needle and avoid vascular structures and gallbladder
If a percutaneous biopsy is not possible, what should you do?
perform an intraoperative biopsy
What is the risk with a liver biopsy?
pain (localized and to right shoulder)
When does bleeding usually occur after surgery and how should you monitor it?
usually w/in 4hrs

Check the Hg levels in 6hrs
What accounts for over 1/2 of all pediatric liver transplants?
biliary atresia
What type of bilirubin elevation is Crigler-Najar I/II?
Which is less severe Crigler-Najar I or II?
Crigler-Najar II is less severe
Does Crigler-Najar I response to Phenobarbital?
What does phenobarbital do?
helps enzymes conjugate bilirubin
What are the levels of unconjugated bilirubin in a person with Crigler-Najar I?
When would symptoms of Gilbert's be noticible?
during stress
What is the pathological problem in Gilbert's
a mutation in the gene that codes for Bilirubin UDP glucuronyltransferase.
What does bilirubin UDP glucuronyltransferase do?
it binds unconjugated Bilirubin with glucuronic Acid to make it water soluble and be excreted as bile
What is the first question you should ask?
Is the bilirubin unconjugated or conjugated.
What could cause an increase in unconjugated bilirubin?
hemolysis or extravascular blood
What is the second question that you would ask when dealing with a jaundice neonate?
Is it intrahepatic or extrahepatic?
Why is it important to know if it is intra or extrahepatic?
It helps with prognosis and determining the need for surgery
Why can't you eat up to 4 hours before getting imaging on the gallbladder?
it contracts after eating.
How can you evaluate the biliary tree?
use Ultrasound
What does a HIDA scan (radionucleotide scan) show?
It shows uptake of the liver and the excretions into the small bowel
When taking a biopsy, what are you looking at?
"The way cells are arranged
What is the idiopathy of intrahepatic jaundice?
neonatal hepatitis
prematurity (in which you may never know)
What are some anatomical reasons for intrahepatic jaundice?
Congential fibrosis

What is Caroli's disease/syndrome?
It is a congenital condition where there is multifocal, segmental dilatation of large intrahepatic bile ducts
What are some metabolic causes of intrahepatic jaundice?
CHO (galactosemia)
Bile Acid
What are characteristics of Tyrosinemia?
progressive liver failure
renal tubular dysfunction
hypophosphatemic rickets
Biliary atresia is intra or extrahepatic?
Pt is discovered to have choledochal cyst. How should you treat that?
surgery (surgical emergency)
Name the different causes of extrahepatic(ductular) jaundice.
biliary atresia
choledochal cyst
spontaneous perforation
Bile duct stricture
Alagille's syndrome
Nonsyndromic paucity
In a typical workup sequence, what is the first thing you would o?
Fractionate the bilirubin (direct vs indirect) and get a full hepatic panel
You order a full hepatic panel--What results will you get?
"AST, ALT, GGT, albumin, TP (total protein)
If GGT is elevated--what does that indicate?
bile duct problems
Are pediatric's Alkaline phosphatase usually depressed or elevated compared to adults?
Name some tools used to diagnosis anatomical conditions that would cause cholestasis.
liver biopsy
What are some infections that would cause cholestasis?
hepatitis A,B,C
What is the order that you would evaluate possible causes of cholestasis?
1. Fractionate bilirubin, full hepatic panel
2. infectious causes
3. metabolic causes
4. anatomic causes
What are some metabolic conditions that would cause cholestasis?
CF (newborn screen and sweat test)
galactosemia (newborn screen)
At what point would you choose to biopsy the liver?
high suspicion of biliary atresia OR Alagille Syndrome
Failing liver
Prolonged cholestasis with no clear eitology
What would give you high suspicion of biliary atresia?
US doesn't show bile duct
stools are white
bilirubin continues to climb
What about Alagille Syndrome contributes to cholestasis?
The paucity of the bile ducts
What would cause high suspicion of Alagille's syndrome?
intrahepatic dropout of bile ducts
What is indicative of a failing liver?
CoAg increasing
albumin decreasing
What are the requirements for performing a percutaneous biopsy?
stable pt w/normal coags and platelet function and no significant ascites

At least 2kgs
Why would you use US to assist with the biopsy?
helps with assesing liver size
tells how far to put the needle in
helps to avoid vascular structures and gallbladder
What would you do if the pt didn't meet the requirements for percutaneous biopsy?
intraperative biopsy
What are the risks of biopsy?
pain (referred to right shoulder)
How do you control for bleeding with a liver biopsy?
Most pt will bleed within the first 4hrs, but Hg should be checked after 6hrs
What accounts for 1/2 of all pediatric liver transplants?
biliary atresia
What is the rule of 1/3rds with Kasai surgical procedure?
1/3 do fine:never need liver transplant
1/3 immediately will not work and need a transplant
1/3 don't need a liver transplant until later
Who will have a poor prognosis with a kasai?
no ducts at hilum
severe cholestasis
What is ascending cholangitis and when is there a risk of it?
When bacteria migrate up and causes an infection in liver.

It is a risk with the kasai
What do you do if the Kasai procedure doesn't work?
liver transplant
What causes progressive obliteration of CBD?
genetics, infection

many hypotheses
Fetal biliary atresia occurs with what frequency and in relation to what else?
10% of total
There is usually other congenital anomalies
There usually is not a jaundice free period
Postnatal atresia occurs with what frequency and in relation to what else?
90% of total
usually presents by itself
there is a jaundice free interval
What is the female to male ratio of choledochal cysts?
What are the five types of choledochal cysts?
Type I: CBD
Type II: diverticulum of CBD and/or GB
Type III: choledochocoele
Type IV: multiple intra or extrahepatic cysts
Type V: fusiform intrahepatic dilitation (caroli's disease)
What is a major concern with choledochal cyst?
Which is the most common choledochal cyst and what are it's characteristics?
Type I:
cyst grows and obstructs bile duct = no flow of bile
How do you treat cholestasis?
What are the five characteristics of Alagille's syndrome?
1. Cholestasis
2. PPS (peripheral pulmonic stenosis) septal defects, TOF, etc
3. Butterfly vertebrae (hemivertebrae)
4.Posterior embryotoxom (eyes)
5. Triangular face
a.frontal bossing
b.deep-set eyes
c.bulbous tip of nose
d.pointed chin
How do you treat Alagille?
transplant may be needed, but mostly will treat symptomatically (pruritis, bind acids to help excretion)
What is the genetic basis of Alagille Syndrome?
jagged 1 gene on short arm of chromosome 20

autosomal dominant with variable penetrance
What is PI?
Protease inhibitor. It is produced in the liver and build up of abnormal proteins causes injury.
What percentage of people with a-1-antitrypsin deficiency develops liver disease?
Where is the mutation that causes a-1-antitrypsin deficiency?
PI locus on chromosome 14
With a-1-antitrypsin, what does MM mean?
With a-1-antitrypsin, what does ZZ mean?
disease state
Of the 10-15% of newborns with a-1 antitrypsin deficiency, how many will do well and how many will need a transplant?
50% do well
50% need transplant
Do infants produce more or less bilirubin than adults/day?
more: greater RBC mass and shorter RBC lifespan (90 days)
What is ursodiol?
selective reabsorbed by intestines competing with toxic bile acids--helps secrete bile

There are very few side effects
What should you monitor in infants with cholestasis?
Why does a lack of intestinal flora effect cholestasis?
flora converts conjugates to urobilinoids
Meconium is filled with what?
lots of bilirubin and B-glucuronidase
What are some normal risk factors for cholestasis?
more delta bilirubin
slower secretion of BMG / BDG
lack intestinal flora
More bilirubin (more RBC, shorter lifespan)
bile has more BMG than BDG
What should you always do/watch when dealing with cholestasis?
fractionate bilirubin
check stools
rule out infection, obstruction, metabolic
initiate enteral feeds as soon as possible
Why is enteral feeding important for cholestasis?
30% of daily caloric maintenance can protect the liver
When should you try to get off TPN?
as soon as possible