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88 Cards in this Set
- Front
- Back
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What is the 3rd step when looking for cholestasis?
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Check for metabolic problems
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What are some metabolic problems that would cause cholestasis?
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CF (newborn screen and sweat test)
a-1-antitrypsin thyroid galactosemia (newborn screen) |
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What is the 4th step with looking for cholestasis?
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check for anatomical problems
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How can you test for anatomical problems that cause cholestasis?
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US
HIDA Liver Biopsy |
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At what point do you biopsy the liver?
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high suspicion of biliary atresia OR alagille syndrome
failing liver extremely prolonged cholestasis with no clear etiology |
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What would cause you to suspect biliary atresia?
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US doesn't show bile duct
stools are white bilirubin continues to climb |
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What component of Alagille Syndrome contributes to cholestasis?
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paucity of bile ducts
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What is indictative of a failing liver?
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Coags increasing
Albumin decreasing ascites? |
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If a pt has no clear etiology for cholestasis, what should you do?
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Biopsy the liver--liver may just have small bile ducts (but not have any other symptoms of alagille syndrome)
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When would you perform a percutaneous biopsy?
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Stable pt
>2kg there is US assistance |
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Why is US assistance helpful?
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Good for assesing size of liver, how far to insert needle and avoid vascular structures and gallbladder
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If a percutaneous biopsy is not possible, what should you do?
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perform an intraoperative biopsy
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What is the risk with a liver biopsy?
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Bleeding
infection trauma pain (localized and to right shoulder) |
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When does bleeding usually occur after surgery and how should you monitor it?
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usually w/in 4hrs
Check the Hg levels in 6hrs |
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What accounts for over 1/2 of all pediatric liver transplants?
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biliary atresia
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What type of bilirubin elevation is Crigler-Najar I/II?
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unconjugated
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Which is less severe Crigler-Najar I or II?
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Crigler-Najar II is less severe
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Does Crigler-Najar I response to Phenobarbital?
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no
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What does phenobarbital do?
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helps enzymes conjugate bilirubin
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What are the levels of unconjugated bilirubin in a person with Crigler-Najar I?
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15-45mg/dL
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When would symptoms of Gilbert's be noticible?
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during stress
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What is the pathological problem in Gilbert's
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a mutation in the gene that codes for Bilirubin UDP glucuronyltransferase.
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What does bilirubin UDP glucuronyltransferase do?
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it binds unconjugated Bilirubin with glucuronic Acid to make it water soluble and be excreted as bile
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What is the first question you should ask?
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Is the bilirubin unconjugated or conjugated.
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What could cause an increase in unconjugated bilirubin?
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hemolysis or extravascular blood
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What is the second question that you would ask when dealing with a jaundice neonate?
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Is it intrahepatic or extrahepatic?
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Why is it important to know if it is intra or extrahepatic?
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It helps with prognosis and determining the need for surgery
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Why can't you eat up to 4 hours before getting imaging on the gallbladder?
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it contracts after eating.
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How can you evaluate the biliary tree?
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use Ultrasound
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What does a HIDA scan (radionucleotide scan) show?
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It shows uptake of the liver and the excretions into the small bowel
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When taking a biopsy, what are you looking at?
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"The way cells are arranged
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What is the idiopathy of intrahepatic jaundice?
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neonatal hepatitis
OR prematurity (in which you may never know) |
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What are some anatomical reasons for intrahepatic jaundice?
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Congential fibrosis
Caroli's |
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What is Caroli's disease/syndrome?
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It is a congenital condition where there is multifocal, segmental dilatation of large intrahepatic bile ducts
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What are some metabolic causes of intrahepatic jaundice?
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AA
Lipid CHO (galactosemia) a-1-antitrypsin CF Hypopit HypoT4 Hemochromatosis TPN/NPO Tyrosinemia Bile Acid |
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What are characteristics of Tyrosinemia?
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progressive liver failure
renal tubular dysfunction hypophosphatemic rickets |
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Biliary atresia is intra or extrahepatic?
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extrahepatic
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Pt is discovered to have choledochal cyst. How should you treat that?
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surgery (surgical emergency)
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Name the different causes of extrahepatic(ductular) jaundice.
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biliary atresia
choledochal cyst spontaneous perforation Mass Bile duct stricture PFIC BRIC Alagille's syndrome Nonsyndromic paucity |
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In a typical workup sequence, what is the first thing you would o?
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Fractionate the bilirubin (direct vs indirect) and get a full hepatic panel
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You order a full hepatic panel--What results will you get?
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"AST, ALT, GGT, albumin, TP (total protein)
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If GGT is elevated--what does that indicate?
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bile duct problems
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Are pediatric's Alkaline phosphatase usually depressed or elevated compared to adults?
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elevated
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Name some tools used to diagnosis anatomical conditions that would cause cholestasis.
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Ultrasound
HIDA liver biopsy |
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What are some infections that would cause cholestasis?
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hepatitis A,B,C
TORCH sepsis |
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What is the order that you would evaluate possible causes of cholestasis?
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1. Fractionate bilirubin, full hepatic panel
2. infectious causes 3. metabolic causes 4. anatomic causes |
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What are some metabolic conditions that would cause cholestasis?
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CF (newborn screen and sweat test)
a-1-antitrypsin thyroid galactosemia (newborn screen) |
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At what point would you choose to biopsy the liver?
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high suspicion of biliary atresia OR Alagille Syndrome
Failing liver Prolonged cholestasis with no clear eitology |
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What would give you high suspicion of biliary atresia?
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US doesn't show bile duct
stools are white bilirubin continues to climb |
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What about Alagille Syndrome contributes to cholestasis?
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The paucity of the bile ducts
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What would cause high suspicion of Alagille's syndrome?
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intrahepatic dropout of bile ducts
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What is indicative of a failing liver?
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CoAg increasing
albumin decreasing ascites |
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What are the requirements for performing a percutaneous biopsy?
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stable pt w/normal coags and platelet function and no significant ascites
At least 2kgs |
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Why would you use US to assist with the biopsy?
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helps with assesing liver size
tells how far to put the needle in helps to avoid vascular structures and gallbladder |
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What would you do if the pt didn't meet the requirements for percutaneous biopsy?
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intraperative biopsy
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What are the risks of biopsy?
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Bleeding
infection trauma pain (referred to right shoulder) |
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How do you control for bleeding with a liver biopsy?
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Most pt will bleed within the first 4hrs, but Hg should be checked after 6hrs
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What accounts for 1/2 of all pediatric liver transplants?
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biliary atresia
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What is the rule of 1/3rds with Kasai surgical procedure?
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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 |
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Who will have a poor prognosis with a kasai?
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Caucasian
cirrhosis no ducts at hilum severe cholestasis |
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What is ascending cholangitis and when is there a risk of it?
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When bacteria migrate up and causes an infection in liver.
It is a risk with the kasai |
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What do you do if the Kasai procedure doesn't work?
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liver transplant
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What causes progressive obliteration of CBD?
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genetics, infection
many hypotheses |
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Fetal biliary atresia occurs with what frequency and in relation to what else?
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10% of total
There is usually other congenital anomalies There usually is not a jaundice free period |
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Postnatal atresia occurs with what frequency and in relation to what else?
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90% of total
usually presents by itself there is a jaundice free interval |
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What is the female to male ratio of choledochal cysts?
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5:1
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What are the five types of choledochal cysts?
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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) |
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What is a major concern with choledochal cyst?
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cholangiocarcinoma
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Which is the most common choledochal cyst and what are it's characteristics?
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Type I:
cyst grows and obstructs bile duct = no flow of bile |
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How do you treat cholestasis?
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surgery
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What are the five characteristics of Alagille's syndrome?
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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 |
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How do you treat Alagille?
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transplant may be needed, but mostly will treat symptomatically (pruritis, bind acids to help excretion)
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What is the genetic basis of Alagille Syndrome?
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jagged 1 gene on short arm of chromosome 20
autosomal dominant with variable penetrance |
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What is PI?
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Protease inhibitor. It is produced in the liver and build up of abnormal proteins causes injury.
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What percentage of people with a-1-antitrypsin deficiency develops liver disease?
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15%
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Where is the mutation that causes a-1-antitrypsin deficiency?
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PI locus on chromosome 14
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With a-1-antitrypsin, what does MM mean?
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normal
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With a-1-antitrypsin, what does ZZ mean?
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disease state
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Of the 10-15% of newborns with a-1 antitrypsin deficiency, how many will do well and how many will need a transplant?
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50% do well
50% need transplant |
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Do infants produce more or less bilirubin than adults/day?
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more: greater RBC mass and shorter RBC lifespan (90 days)
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What is ursodiol?
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selective reabsorbed by intestines competing with toxic bile acids--helps secrete bile
There are very few side effects |
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What should you monitor in infants with cholestasis?
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Stools!
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Why does a lack of intestinal flora effect cholestasis?
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flora converts conjugates to urobilinoids
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Meconium is filled with what?
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lots of bilirubin and B-glucuronidase
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What are some normal risk factors for cholestasis?
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more delta bilirubin
slower secretion of BMG / BDG meconium lack intestinal flora More bilirubin (more RBC, shorter lifespan) bile has more BMG than BDG |
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What should you always do/watch when dealing with cholestasis?
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fractionate bilirubin
check stools rule out infection, obstruction, metabolic initiate enteral feeds as soon as possible ursodiol |
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Why is enteral feeding important for cholestasis?
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30% of daily caloric maintenance can protect the liver
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When should you try to get off TPN?
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as soon as possible
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