Miscellaneous Liver and Gastrointestinal Diseases: Gupta Flashcards
What could cause elevated circulating levels of Conjugated bilirubin?
Is accumulation of conjugated bilirubin a big deal?
:: Deficiency of canalicular membrane transporters- Dubin- Johnson syndrome
:: Impaired bile flow from duct obstruction or autoimmune cholangiopathies
Not a big deal, excreted in feces/urine.
What could cause elevated circulating levels of UNconjugated bilirubin?
Is accumulation of unconjugated bilirubin a big deal?
Excess production of bilirubin: -hemolytic anemias, resorption of blood from intestinal hemorrhage, ineffective erythropoesis. Reduced hepatic uptake of bilirubin: - drug interference w/ membrane carriers - Some cases of Gilbert syndrome Impaired bilirubin conjugation -lots of shit
These causes are a big deal! The unbound (to albumin) unconjugated bilirubin is the problem b/c it goes into tissues and accumulates. Kernicterus- deposition in brain of kids.
What are Crigler-Najjar Syndrome Type I and II?
Defects in bilirubin metabolism. –> unconjugated bilirubin buildup
Type I- AR, no UGT1A1 activity (FATAL!)
Type II- AD, reduced UGT1A1 activity (occasional icterus)
What is going on in Dubin-Johnson syndrome?
AR.
CAN’T GET CONJUGATED BILE OUT OF CELLS
Impaired biliary excretion of bilirubin glucuronides due to mutation in canalicular multidrug resistance protein 2 (MRP2)—> pigmented cytoplasmic globules = BLACK LIVER
-Only genetic bile metabolism or transport defect resulting in liver pathology visible under scope.
What is going on in Rotor syndrome?
AR. Not very common, no clinical significance.
Not exactly sure, but decr. hepatic uptake/storage + decr. biliary excretion of bile.
MCC of cholestasis? #2 cause?
#1- Gallstones (extrahepatic) #2- malignancy (biliary tree/pancreas)
You’re looking at histo of an intrahepatic bile duct that is backed up from an obstruction. What can you see?
Inflammatory cells lining the interface between the hepatic parenchyma and the portal tract. Causing periportal edema.
What is the name for the complication of cholestasis that can be caused by bacteria? Which bacteria are the primary culprits? What will you see on histo? What will you see grossly? Symptoms? Tx?
Ascending cholangitis Caused by- Coliforms and Enterococci Neutrophils attacking bile ducts. Pockets of pus, grossly. Fever, chills, abd. pain, jaundice. Treat obstruction and give abx.
What happens if you do not correct cholestasis?
What differentiates this outcome from the same caused by Hep. C or EtOH abuse?
Chronic cholestasis—> periportal fibrosis—> secondary or obstructive biliary cirrhosis.
Irregular, not round regenerative nodules like those found in Hep. C or EtOH cirrhosis.
Also, feathery degeneration of periportal hepatocytes.
- retained bile salts—> foamy hepatocytes
Mallory-Denk bodies. Differs from AFLD/NAFLD b/c M-D bodies are periportal, instead of in zone 3.
ALSO, formation of bile infarcts.
What hepatic pathology associated with bile stasis is a BIG feature of septic shock?
Bile plugs in canals of Hering and bile ductules.
MCC of conjugated hyperbilirubinemia in neonates?
Biliary atresia
Autoimmune cholangiopathies: Primary biliary sclerosis Age? Gender? Clinical course? Assoc. conditions? Serology? Radiology? Duct lesion? Histo? How does the liver compensate?
Primary biliary sclerosis:
Age- older (med. age 50)
Gender- female
Clinical course- progressive
Assoc. conditions- Sjogren’s, scleroderma, thyroid dz.
Serology- 95% AMA, 50% ANA, 40% ANCA
Radiology- normal
Duct lesion- Florid (rosy, pale red color) duct lesions and loss of small ducts only
Histo: neutrophils attacking ducts, can cause rupture–> granuloma formation—> cirrhosis
Liver compensates by incr. number of bile ducts to traffic bile that could not get through damaged ducts.
Autoimmune cholangiopathies: Primary sclerosing cholangitis Age? Gender? Clinical course? Assoc. conditions? Serology? Radiology? Duct lesion?
Primary sclerosing cholangitis
Age- Younger (med. age 30)*
Gender- male*
Clinical course- unpredictable, progressive
Assoc. conditions- IBD- UC (70%)**
Serology- 65% ANCA, 6% ANA
Radiology- Strictures and beading of large bile ducts; pruning of smaller ducts.
Duct lesion- Inflammatory destruction of extrahepatic and large intrahepatic bile ducts; fibrotic obliteration of med./small intrahepatic ducts.
What is a choledochal cyst?
How does it present?
Predisposes to?
What if found in older adults?
Congenital dilations of the common bile duct.
Children younger than 10 as jaundice, recurrent abd. pain, bilary colic.
Predisposes to obstruction w/ stone, stenosis, pancreatitis, obstructive complications within liver.
Older adults at elevated risk for bile duct carcinoma.
Fibropolycystic diseases of the liver. WTF?
Two types:
Heterogenous group of of which primary abnormalities are congenital malformations of biliary tract. Elevated risk for cholangiocarcinoma.
Von Meyenburg complexes- bile duct hamartoma easily confused w/ portal tract. Histo: dilated, irregularly shaped ducts.
Single/multiple intra/extrahepatic biliary cysts- literally exactly what it sounds like.
In isolation, presenting w/ ascending cholangitis- Caroli dz
+ fibrosis- Caroli syndrome
