Zaman 1-8-08 Gi-6 Gi-7 Liver-General/ Metabolic/biliary Tract

Front 1

FUnction of Pit cells

Back 1

NK, immune

Front 2

Ito cells function

Back 2

Vitamin A storage, fibrosis(alcholism)

Front 3

Why low platelets with portal hypertension?

Back 3

?

Front 4

In general, the A_T is more specific for liver injury than the
A_T (_ = _____).

Back 4

In general, the ALT is more specific for liver injury than the
AST (L = liver).

Front 5

Alkaline phosphatase (AP)
commonly elevated in _____static conditions & also in infiltrative
conditions (chronic passive congestion, infiltrating/metastatic
cancer, etc).
can also be elevated in bone disease, skeletal growth, &
_________.

Back 5

Alkaline phosphatase (AP)
commonly elevated in cholestatic conditions & also in infiltrative
conditions (chronic passive congestion, infiltrating/metastatic
cancer, etc).
can also be elevated in bone disease, skeletal growth, &
pregnancy.

Front 6

Liver synthesizes a variety of proteins such as albumin
& all of the soluble clotting factors except factor ____which is synthesized by the endothelium.

Back 6

Liver synthesizes a variety of proteins such as albumin
& all of the soluble clotting factors except factor VIIIwhich is synthesized by the endothelium.

Front 7

A typical PT is 12 seconds, and a prolongation by _
seconds is considered evidence of severe depletion
of these short lived liver-produced clotting factors.

Back 7

A typical PT is 12 seconds, and a prolongation by 2
seconds is considered evidence of severe depletion
of these short lived liver-produced clotting factors.

Front 8

Jaundice refers to the yellow pigmentation of tissues
and body fluids due to the accumulation of bilirubin.
becomes apparent at a bilirubin level of ___ mg/dl
(normal 0.5-1 mg/dl) & is most noticeable in the sclera
of eyes & skin.

Back 8

Jaundice refers to the yellow pigmentation of tissues
and body fluids due to the accumulation of bilirubin.
becomes apparent at a bilirubin level of 2-3 mg/dl
(normal 0.5-1 mg/dl) & is most noticeable in the sclera
of eyes & skin.

Front 9

Only significance is patient and physician anxiety -worry about
a more serious cause of hyperbilirubinemia.
The defect is thought to be due to reduced levels of hepatic
bilirubin glucuronyl transferase.
There is some evidence that some patients may also have a
reduced ability to transport bilirubin into the hepatocyte.
Up to 5% of blood donors, benign AD inherited condition.
Usually discovered as teenagers or adults.

Back 9

Gilbert's syndrome

Front 10

Rare AR disease in which there is total lack (Type I)
or partial lack (Type II of bilirubin glucuronyl
transferase.
Type I is fatal in first or second year of life as a
result of kernicterus.
Type II carries a danger of kernicterus but patient
usually survives with an elevated unconjugated
hyperbilirubinemia.
33

Back 10

Crigler-Najjar syndrome

Front 11

When the concentration of unconjugated bilirubin reaches a
level that exceeds the capacity of serum albumin to bind it
(typically around 18-20 mg/dl), the child develops ___________.

Back 11

When the concentration of unconjugated bilirubin reaches a
level that exceeds the capacity of serum albumin to bind it
(typically around 18-20 mg/dl), the child develops kernicterus.

Front 12

DUCTULAR
CHOLESTASIS:
Periportal bile
ductules are
hyperplastic, dilated
and contain bile
stained concrements.
The interlobular bile
duct is free. This
sample is from a case
of fungal sepsis
following ____
______ transplant.

Back 12

DUCTULAR
CHOLESTASIS:
Periportal bile
ductules are
hyperplastic, dilated
and contain bile
stained concrements.
The interlobular bile
duct is free. This
sample is from a case
of fungal sepsis
following bone
marrow transplant.

Front 13

CANALICULAR
CHOLESTASIS: There are
bile thrombi in bile
canaliculi, in the central
lobular zone in ml cases
and in the peripheral zone
in severe cases.
Hepatocyte damage, bile
lakes and bile infarcts may
also occur. This sample is
from a case of acute
cholestatic hepatitis _.

Back 13

CANALICULAR
CHOLESTASIS: There are
bile thrombi in bile
canaliculi, in the central
lobular zone in ml cases
and in the peripheral zone
in severe cases.
Hepatocyte damage, bile
lakes and bile infarcts may
also occur. This sample is
from a case of acute
cholestatic hepatitis A.

Front 14

CHOLATE STASIS: The periportal hepatocytes are
swollen, hydropic and foamy, pseudoxanthomatous
cells. These are the changes of so-called "biliary
piece meal necrosis" that occurs in stagnation of bile
flow. The changes are attributed to retention of bile
acids. This sample is from a case of primary _______
_________ (PBC)

Back 14

CHOLATE STASIS: The periportal hepatocytes are
swollen, hydropic and foamy, pseudoxanthomatous
cells. These are the changes of so-called "biliary
piece meal necrosis" that occurs in stagnation of bile
flow. The changes are attributed to retention of bile
acids. This sample is from a case of primary biliary
cirrhosis (PBC)

Front 15

CYTOPLASMIC CHOLESTASIS:
Presence of yellow pigment
throughout the cytoplasm and
not in lysosomes which are
pericanalicular.
Bilirubin granules will stain
green with Hall's method.
The hepatocytes may show
Mallory bodies, eosinophilic
and pseudoxanthomatous
degeneration.
There may be foci of cell
dropout. These changes are
most prominent in the _______
lobular area.

Back 15

CYTOPLASMIC CHOLESTASIS:
Presence of yellow pigment
throughout the cytoplasm and
not in lysosomes which are
pericanalicular.
Bilirubin granules will stain
green with Hall's method.
The hepatocytes may show
Mallory bodies, eosinophilic
and pseudoxanthomatous
degeneration.
There may be foci of cell
dropout. These changes are
most prominent in the central
lobular area.

Front 16

________ bodies: Scattered hepatocytes accumulate
tangled skeins of cytokeratin intermediate filaments
and other proteins, visible as eosinophilic cytoplasmic
inclusions in degenerating hepatocytes
These inclusions are a characteristic but not specific
feature of alcoholic liver disease,

Back 16

Mallory bodies: Scattered hepatocytes accumulate
tangled skeins of cytokeratin intermediate filaments
and other proteins, visible as eosinophilic cytoplasmic
inclusions in degenerating hepatocytes
These inclusions are a characteristic but not specific
feature of alcoholic liver disease,

Front 17

Nonalcoholic fatty liver disease (NAFLD) resembles
alcohol-induced liver disease but occurs in
patients who are not heavy drinkers.
Patients are largely asymptomatic,
NAFL is now considered to be the most common
cause of "______genic" cirrhosis.
The epidemic of _______ in the United States
heightens concern that NAFL will increase in
prevalence.

Back 17

Nonalcoholic fatty liver disease (NAFLD) resembles
alcohol-induced liver disease but occurs in
patients who are not heavy drinkers.
Patients are largely asymptomatic,
NAFL is now considered to be the most common
cause of "cryptogenic" cirrhosis.
The epidemic of obesity in the United States
heightens concern that NAFL will increase in
prevalence.

Front 18

Hemochromatosis results either from a
homozygous-recessive _____ mutation short arm
of chromosome 6p21.3 causing excessive iron
absorption or acquired forms of hemochromatosis
with known sources of excess iron usually
transfusions-secondary hemochromatosis

Back 18

Hemochromatosis results either from a
homozygous-recessive C282Y mutation short arm
of chromosome 6p21.3 causing excessive iron
absorption or acquired forms of hemochromatosis
with known sources of excess iron usually
transfusions-secondary hemochromatosis

Front 19

In the patient with mutant HFE, the inability to downregulate expression of _____, ____,
______in and __1 proteins leads to lifelong excessive absorption of dietary iron.

Back 19

In the patient with mutant HFE, the inability to downregulate expression of Dcytb, DMT1,
ferritin and FP1 proteins leads to lifelong excessive absorption of dietary iron.

Front 20

Wilson disease
AR mutation on ___7B on chromosome 13 marked by
the accumulation of toxic levels of ______ in many
tissues and organs, principally the liver, brain and eye.

Back 20

Wilson disease
AR mutation on ATP7B on chromosome 13 marked by
the accumulation of toxic levels of copper in many
tissues and organs, principally the liver, brain and eye.

Front 21

The biochemical diagnosis of Wilson disease is based on
a decrease in serum _______plasmin, an increase in
hepatic ______ content, and increased urinary excretion
of ______. Serum copper levels are of no diagnostic
value.

Back 21

The biochemical diagnosis of Wilson disease is based on
a decrease in serum ceruloplasmin, an increase in
hepatic copper content, and increased urinary excretion
of copper. Serum copper levels are of no diagnostic
value.

Front 22

A1AT
AR disorder marked by low serum levels of this
protease inhibitor of ________ on chromosome 14, is
very polymorphic, and at least 75 forms have
been identified.
The most common clinically significant mutation is ___;
homozygotes for the ____ protein have circulating

Back 22

A1AT
AR disorder marked by low serum levels of this
protease inhibitor of elastase on chromosome 14, is
very polymorphic, and at least 75 forms have
been identified.
The most common clinically significant mutation is PiZ;
homozygotes for the PiZZ protein have circulating

Front 23

Carcinoma of the gallbladder

I___________ more common -diffuse area of
thickening, scirrhous --fibrous reaction to tumor
growth
F________ -cauliflower growth into lumen with
invasion of wall
Approximately 95% are _____carcinomas -
papillary, infiltrative, poorly differentiated or
undifferentiated
5% are ________ arising in squamous metaplasia
and tend not to metastasize

Back 23

Carcinoma of the gallbladder

Infiltrating more common -diffuse area of
thickening, scirrhous --fibrous reaction to tumor
growth
Fungating -cauliflower growth into lumen with
invasion of wall
Approximately 95% are adenocarcinomas -
papillary, infiltrative, poorly differentiated or
undifferentiated
5% are squamous arising in squamous metaplasia
and tend not to metastasize

Front 24

What is it?

Most common malignant tumor of extrahepatic bile ducts in children; presents
with obstructive jaundice.
Gross: Deceptively polypoid
Micro: Small undifferentiated spindle cells concentrate below an intact
epithelial layer aka “cambium layer”.
Tx: Surgery, radiation, chemo with many longterm survivors.
Mets in 40%, but death due to local effects of tumor.

Back 24

Botryoid RMSA