Chapter 2 Flashcards
1
Q
The structure of the liver can be broken into which 3 categories
A
- hepatic vascular system
- biliary tree
- hepatic lobules
2
Q
Why is the liver considered a vascular organ?
A
it has a dual blood supply, and at any given time contains 13% of the bodies blood
3
Q
Describe the dual blood supply of the liver
A
Arerial blood supplied by hepatic artery and portal vien brings the liver al the blood that was previously passed through the small intestine and spleen.
4
Q
What are sinusoids in the liver?
A
vascular channels lined with highly fenestrated endothelial cells and surrounded by hepatocytes.
5
Q
what provides a major portion of the body lymps?
A
plasma that is filtered into the space between the endothelium and hepatocytes
6
Q
describe the flow of blood int the liver
A
hepatic portal vein + hepatic artery»_space; sinusoids —^plasma—>central vein of each lobule—-> hepatic veins > (leaves liver)—> inferior vena cava.
7
Q
What is the biliary system?
A
a series of channels and ducts that transport bile from the liver into the small intestine.
8
Q
What cells produce and secrete the bile?
A
hepatocytes
9
Q
What happens to bile once it reaches the gallbladder?
A
its concentrated and stored until it is needed for digestive sxs. it re-enters the common bile duct to form ampulla of vater.
10
Q
What controls the ampullary opening into the duodenum?
A
muscle sphincter of Oddi.
11
Q
What is the hepatic lobule consisted of?
A
hexagonal arrangement of plates of hepatocytes radiating outwards from a central vein. It encompasses the liver tissue that is served by a single branch of the central vein.
12
Q
What are portal triads?
A
regions of connective tissue that include branches of the bile duct, portal vein and hepatic artery.
13
Q
How many hepatic lobules are in a normal liver?
A
100K
14
Q
What is the primary purpose of the liver?
A
to maintain homeostasis by
- detoxification
- metabolism
- synthesis of lipoproteins and cholesterol
- synthesis of plasma proteins
- synthesis of immune factors
- digestive function
- excretion of bilirubin
- storage
15
Q
Define liver metabolism
A
hepatic cells assimilate carbs, fats, and proteins
they convert glucose to glycogen
16
Q
what Glucogenesis?
A
production of glucose from sources other than carbs- can be carried out by the liver.
17
Q
Name the plasma proteins produced by the liver
A
- albumin
- fibrinogen
- certain globulins (transport proteins)
18
Q
What do the phagocytes in the liver produce in response to the inflammatory process, tissue repair and immune cell activities?
A
they produce acute-phase protein in response to microbes
19
Q
What is the purpose of bile? what does it do?
A
digests and aids in the absorption of fats. The liver adds a bicarbonate-rich solution of inorganic ions, which helps neutralize acid in the duodenum
20
Q
What is bilirubin?
A
results from the breakdown of the hemoglobin in the RBC and is excreted into the bile by hepatocytes.
21
Q
what happens when bilirubin cannot be removed from the blood quickly enough?
A
Jaundice
22
Q
What vitamins and proteins are stored in the liver?
A
- glucose in form of glycogen
- fats
- iron
- copper
- vit K, A, D, B12
23
Q
What are some primary issues that are associated with the use of FLT’s for liver pathology screening?
A
- non-specific to Liver function
- LFTs have low sensitivity and specificity
- results can be affected by several factors
- structural or functional damage can evade detection using BW
24
Q
LFTs are tested to evaluate the specific aspects of the liver. They can be categorized based on their ability to what (5)
A
- detect injury to hepatocytes
- determine hepatic biosynthetic capacity
- measure excretory function
- detect chronic inflammation of the liver, and hep
- serve as tumour markers.
25
Name the enzymes that are sensitivity indicators of hepatic injury
Serum aminotransferases.
ATL, AST
ALT is more specific since it is mainly found in liver, where AST i found in many tissues
26
Why is LDH not typically concerning in the u/w process?
it adds little evaluation of suspected hepatic dysfunction.
27
When goes elevation of GGT occur?
```
with minor subclinical hepatocellular damage, in association in ALT.
CAD
renal failure
MI
pancreatic disease
DM
alcohol/medications
```
28
What are GGT enzymes?
they are involved in the transfer of amino acids across cellular membranes.
29
What is bilirubin?
the main bile pigment that is formed from the breakdown of hemoglobin in red blood cells.
30
Serum Bilirubin levels reflect the livers ability to do what?
take up, process, and secrete bilirubin into the bile.
31
What is urobilinogens?
this is the chemical compound that emmerges when the bilirubin is acted on by bacteria in the small intesting entrance.
32
What gives feces it dark color? What can result from the absence of bilirubin in the intestine?
uribilinogen
clay-colored stools
33
Bilirubin is chemically different after it goes through the conjugation process in the liver, how do lab test will differentiate between them?
The unconjugated (indirect) and conjugated (direct) bilitybin reflects how it reacts to certain dyes added to the blood specimen.
34
Unconjugated hyperbilirubinemia is caused by?
1. increase production of bilirubin- hemolytic anemia
| 2. decreased conjugation- familial hyperbilirubinemia.
35
Conjugated hyperbilirubinemia is caused by?
1. decreased secretion of bilirubin by the liver- cirrhosis, hep,
2. cholestasis- found in biliary obstruction, cholesdoscholithiasis, stricture, neoplast,
36
In the liver, what causes increased AP levels?
rise of bile acids, early marker of cholestasis- which results in increased synthesis and secretion of alk Phos
37
If the AP is elevated d/t hepatic pathology, Will GGT and/or bilirubin also be elevated?
yes
38
What are the most common causes of AP elevations?
1. liver- bilary obstruction, cholestatis
2. bone diease-
2. malignant tumors
4. renal disease
5. primary hyperthyroidism
6. polycythemia vera
7. pregnancy
39
Where is albumin synthesized?
liver,
40
What causes Changes in albumin levels?
progressive hepatocellular injury, hepatic synthetic capacity.
heavy alcohol intake and chornic inflammation inhibit albumin synthesis.
41
What is Alpha-fetoprotein (AFP)
a glycoprotein synthesized in the yolk sac, liver, and GI of the futur. It is a major protein in fetaol serum.
As fetal liver matures it AFP levels decline.
42
Do normal livers in non-pregnant adults still produce small levels of AFP?
no
43
Tumor markers can be used for what 4 purposes?
1. cancer screening
2. making a dx of caner
3. determining prognosis
4. monitoring effectiveness of cancer treatments.
44
AFP is a tumor marker for what?
1. hepatocellular carcinoma. AFP caused by abN cell proliferation in the liver.
2. heptoblastoma and nonseminomatous germ cell tumors-
3. cirrhosis and viral hep
45
What does prothrombin time (PT)measure?
the rate of conversion of prothrombin to thrombin after activation of the extrinsic coagulation pathway.
prolonged= coagulation deficiency/ acute liver disease
46
Which coagulation factors are synthesized by the liver?
factor 1, 2, 5, 7, 9, 10
47
What are some non-hepatic causes of prolonged PT times?
Vit K deficiency, coagulopathies, inherited deficiency of a coagulation factor, or medications that antagonize the prothrombin complex (warfarin)
48
What is Carbohydrate-deficient transferrin (CDT)?
a blood test use to help detect heavy alcohol consumption.
49
What is transferrin, and what happens to it when you ingest ++ ETOH?
Plasma transport protein for iron.
| ETOH causes the make-up of the protein.
50
What is Hemoglobin-associated acetaldehyde (HAA) assay
A specific confirmation test that distinguishes alcohol-related from non-alchohol-related LFT elevations. ITs a metabolite of Ethanol.
51
What can be detected through an abdo x-ray (in terms of liver
on occasion, calcificatoins d/t gallstones, cytst, or scarring.
tumors, or vascular lesions
52
What can be detected through an abdo ultrasound?
first choice study- many hepatobiliary disorders: focal lesions, biliary dilation, stones, and facilitate biopsies or solid hep masses..
in expensive
non invasive,
portable
53
What can be detected through an abdo CT?
becoming preferred technique for hepatobiliary system- not for GB
-detects masses, and differentiates types, cavernous hemangiomas, and neoplastic vascular invasion. Cirrhosis, and portal HTN, and changes in liver.
54
What can be detected using an MRI?
characterizing and staging liver leisons seen on other imaging tests.
55
What is a (Transient Elastography) fibroscan and why is it used?
non invasive procedure that utilizes an ultrasound probe to measure shear wave velocity in order to determine the severity of hepatic fibrosis. Less expensive then biopsy.
- less reliable in people with low grade fibrosis and with acute liver inflammation
56
What id the purpose of a liver biopsy
confirm dx of specific liver disease.
| provides information about structural integrity of the liver, type and degree of injury.
57
What are the methods for obtaining liver tissue
```
percutanrous **
transjugular
laproscopic
ultrasound
CT-guided fine needle aspiration
```
58
List the informaiton of the liver the biopsy will provide.
1. evaluation of abN dx findings and hepatomegaly
2. confirm dx and prognosis
3. confirm suspected hep neoplasm
4. dx cholestatic liver disease
5. evaluate infiltration or granulomatous disease
6. evaluate and stage chornic hep
6. identify and staging of alc. liver disease
8. evaluate effectiveness of tx of liver disorders.
59
What are some risks associated with completing a liver biopsy. What are the limitations
pain, hemorrage, biliary peritonitits,
bacteremia
sampling error, d/t adequacy or location
60
What is Fatty liver?
what are the classifications?
What is the severity?
when more than 5% of the liver mass is made of fat, usually trigs.
Classified as alcoholic or non-alcoholic
mild stenosis(fatty liver) to inflammation (setathohepatitis) to fibrosis or cirrhosis.
61
What is NASH?
Non alcoholic steatohepatitis, and can be associated with progressive fibrosis, cirrhosis, and liver failure.
62
Hepatic stenosis can be caused by what detects in the movement of fatty acids through the liver.
1. ^ peripheral mobilization of fatty acids into the liver
2. ^ hepatic synthesis of fatty acids
3. impaired hepatic catabolism of fatty acids
4. impaired synthesis and excretion of VLDL from the liver
5. necroinflammatory changes
63
How are the most advanced stages of NAFLD (nonalcoholic fatty liver disease) marked?
disposition of collagen in the liver, results in -> fibrosis and eventually cirrhosis.
64
What are the causes of NAFLD?
```
poorly controlled diabetes
insulin resistance
metabolic syndrome
hyperlipidemia
obesity
acute starvation
post-bariatric surgery
medications
```
65
How is NAFLD detected?
most people are Asxs
-usually indicated by mildy elevated ALT and/or AST.
Ferritin levels are also increased
- U/s, CT/ and MRI are reliable to detect moderate to severe Fatty changes in liver.
66
Which immaging test is able to distinguish between simple steanosis and NASH or indicating the stage of fribrosis?
none
| Liver biopsy- dx the presence and extend of NAFLD.
67
If a disease progresses from simple steatosis to NASH, what would you find on the liver boipsy?
1. stetosis- fat droplets within hepatocytes
2. inflammation
3. mallory bodies
4. glycogen nuclei
5. fibrosis- chicken wire pattern
6. cirrhosis
68
NAFLD is not curable, but what can help prevent the progression?
weight loss, diet modifications to reduce carbs and fats, and tighter control of hyperlipidemia and diabetes resistance.
69
The pathology of alcoholic liver injury comprises which 3 major components?
1. fatty liver,
2. alcoholic hepatitis
3. cirrhosis
70
What are 7 risk factors that affect alcoholic liver disease>
1. quantity of ETOH
2. female gender
3. hep C (HCV)
4. genetic variability in alcohol-metabolizing enzymes
5. malnutrition
6. co-exposure to drug toxins
7. immunologic dysfunction.
71
How does the Wold Health Organizations define cirrhosis?
1. a diffuse process characterized by fibrosis and the conversion of normal liver architecture into structurally abN nodules that lack normal lobular organizations.
>> an irreversible` chronic injury of the hepatic parenchyma and includes extensive fibrosis in association with the formation of regenerative nodules.
72
What are the 5 criterias used to determnine a dx of cirrhosis?
1. pronounced, insufficiently repaired necroses of the parenchyma
2. diffuse connective tissue proliferation
3. varying degrees of nodular parenchymal regeneration
4. loss and transformation of the lobular structure within the liver as a whole
5. impaired intrahepatic and intra-acinar vascular supply.
73
Why is cirrhosis classified based on ethology of the disease?
1. morphologic classification is more difficult to determine d/t the overlap of findings amoung different etiologies, and
2. tx and prognosis are based on etiology.
74
What are the major causes of cirrhosis?
```
alchohol,
chronic infection
cholestasis
autoimmune hep
chemical agents
venous congestion
hemochromatosis
NASH
```
75
What does cirrhosis cause? sxs of the impairement
```
jaundice
portal HTN
esophageal varices
ascites
spintaneous bacterial perionitits
hepatorenal syndrome
hepatic encephalopathy
coagulopathy.
```
76
What is Jaundice and the 3 classes of cause for it?
Its the excess levels of bilirubin
1. pre-hepatic or hemolytic jaundice- d/t increased breakdown of RBC
2. hepatic jaundice
3. extrahepatice Jaundice( obstructive)
77
What is portal HTN?
abN high pressure in the portal circulation, its caused by increased resistance to flow through a cirrhosised liver.
Leads to the development of collateral veins that divert blood flow bypassing the liver. Which means detoxification doesnt occur.
78
What are major site of collateral flow?
veins around the cardioesophageal junction
rectum
retroperitoneal space
falciform ligament of the liver.
79
What is varicosities?
the blood vessels become engorged and dialted, while the vessel walls become thing and fragil
80
What are the major clinical manifestations that result from portal HTN?
1. gastroesophageal varices,
2. slenomegaly with hyperplenism
3. ascites
4. acute and chronic hep encephalopathy.
81
What is esophageal varices?
dilated blood vessels within the wall of the esophagus that develop as a result of portal HTN.
diameters are 0.5 to 1cm lager.
82
What is Ascites?
The pathological accumulation of fluid within the peritoneal cavity.
- caused by cirrhosis, other liver diseases, malignancy, heart failure, infection, pancreatitis.
83
What is the most common primary source of infection for peple with ascitic fluid infection?
spontaneous bacterial peritonitis
84
What is Hepatorenal syndrome (HRS)?
refers to the develoment of acute renal failure in individuals wiht advanced chronic liver disease and fulminant hep, who have portal HTN and ascites.
85
How is HRS characterized?
1. marked decrease in GFR and renal plasma flwo in the absence of other identifiable causes of renal failure
2. marked AbN in systemic hemodynamics
3. activation of endogenous vasoactive systems
86
What is Hepatic Encephalopathy (HE)?
(HE) is a complex neuropsychiatric syndrome that occurs in the setting of significant liver disease.
87
How is HE characterised
disturbances in consciousness and behaviour, personality changes, fluctuating neurological signs. flapping tremour, and electroencephalographic changes.
88
Can thrombocytopenia result from hyperplenism?
yes
89
In cirrhosis which coagulation factor that is first to be depleted?
factor 7
90
What is hepatitis?
inflammation of the liver.
91
What can cause hepatitis?
viral, bacterial, fungal, parasitic organism
| medication, toxins, and autoimmune disorder
92
Almost all cases of viral hepatitis is caused by one of which five viral agents?
```
hep A (HAV)
hep B (HBV)
Hep C (HCV)
HBV- associated delta agent (HEP D_ HDV
Hep E (HEV)
```
93
Which Hep viruses are RNA and which are DNA viruses?
RNA: all except B, which is DNA.
94
Agents of viral hep can be broadly classified into two which groups?
1. viruses that spred via enteric (oral-fecal) transmission and blood-borne agents
95
Which hep viruses are enterically-transmitted?
HAV and HEV
| - self-limiting infection
96
Which hep viruses are blood-born?
HBV, HCV and HDV are assocoiated with persistent infection, prolonged viremia and develppment of chronic liver disease
97
What is acute hep vs chronic ?
self-limiting inflammation of the liver that does not lead to fibrosis vs persistent infection for at least 6 mo which can lead to long-term disease.
98
Is there a vaccine for HAV?
yes
99
How is HBV trasnmitted
exposure to infectious blood or body fluids. ie unprotected sex, blood transfusion, and re-use of bad needles.
100
What are the four stages of HBV?
1. incubation period-
2. prodromal state
3. clinical stage- Jauncie
4. convalescence
101
Does the presence of chronic HBV significantly decrease or increase the risk of developing hepatocellular carcinoma?
increases
| Co-infection with hep C and D further increases risk of both cirrhosis and liver cancer
102
What are factors that suggest progression to chronic infection include
1. lack of complete resolution of clinical symptoms and persistence of hepatomegaly
2. presence of bridhing or multi-lobular hep necrosis on liver biopsy during protracted severe acture viral hep
3. failure of the serum aminotransamine, bilirubin, and globulin levels to return to normal within 6-12 months after the acute illness
4. persistence HBeAg beyond 3 months or HBsAg beyond 6 mo after acute hep
103
Describe the Serology of Hep B surface antigen (HBsAg)
1. earliest marker of acute infection
2. appears before the onset of sxs and the evalation of serum thransaminases
3. should clear by convalenscence stage of acute infection
4. persistence for more than 6 mo indicates prgression to carrir state or chronic HBV
104
Describe the Serology of Hep e antigen (HBeAg)
1. marker for highlyy infection state and active viral replication
2. found only in presence of HBsAg
2. should clear by convalescence stage of acute infection
4. Persistence for more than 10 weeks, suggests progression to chronic state
105
Describe the Serology of HBV core antibody (HBcAb or anti-HBcAb)
1. indicates exposure to HBV and viral replication
2. appears shortly after HBsAg in acute infection and persist for life
3. not a good marker for acute diease or cure
106
Describe the Serology of HBV surface antibody (HBsAb or anti-HBsAb)
1. a protective antibody that neuralized HBV
2. if present with -ve HBsAg it represent cure from acute infection and immunity
3. if present with -ve HBcAb, it represents immunity as a result of vaccine
107
Describe the Serology of HBV e antibody (HBeAb or anti-HBeAb)
indicates decreasing infectivity
108
Describe the Serology of HBV DNA
1. measured viral load
| 2. useful for the assessment of those with chronic HBV as candidates for antiviral tx and to track response to tx.
109
What are the goals for chronic Hep B therapy?
sustained virologic suppression reducing th rate of disease progression, and decreasing the risk of complications.
110
What are some anti-viral agents currently being used for HVB tx
```
lamivudine
adefovir
tenofovir
entecavir
telbivudine
interferon alfa
pegylated interferon alfa
```
111
How is Hep C virus transmitted?
via blood and body fluids.
| targets hepatocytes
112
There are 6 distinct HVC genotypes and 13 subtypes. Which is the most common
genotype 1, accounts for 40-80%.
| 1a and 1 b are prevalent in the US
113
What is the most common way to aquire Hep C virus (in the us)
those who use intravenous drugs, and less than 20% through sec.
114
Describe the 3 stages of hep C.
1. incubation period- 6-12 weeks, no sxs,
2. acute phase- mild sxs maybe
3. chronic HCV - progression silent, sxs absent until scaring of liver occured
115
What are the 3 mechanism that cause the pathology associated with HCV?
1. direct damage to hepatocytes
2. immune-medicated hepatocyte inflammation and destruction
3. viral-induced autoimmunity
116
What are some factors that are reported to influence the speed of HCV progression>?
```
Age,
gender (male>female),
alchohol
HIV co-infection
insulin resistance
metabolic synfrome
fatty liver
```
117
T or F
| Anti-HCV antibodies indicate exposure to the virus but can not determine if on going infection is present
T
118
How is HCV detected?
detected by PCR, it also measures the effectiveness of the tx.
119
How do you tx HCV?
combination of pegulated interferon alpha and the antiviral drug ribavirin for a period of 24-48 weekls.
120
What makes HDV unique in terms of infection requirements?
it requires the helper function of HBV for its replication and expression
in combinaiton this is the highest mortality hep infection
121
How can you be infected by HDV? two types
Co-infection- via simultaneous HBV
| Superinfection- via infection of an individual previously infected with HBV
122
How is HEV infected?
through fecal contamination of water supplies or good
123
Who does HEV infected the worst?
pregnant women, specially in 3rd trimester
124
define Autoimmunie Hepatitis (AIH)
a chronic disorder characteristed by continuing hepatocellular necrosis and inflammation, usually fibrosis, which commonly progresses to cirrhosis and liver failure.
125
What causes the progressive liver injury in individuals with autoimmune hepatitis?
the result of cell-mediated immunologic attack directed against hepatocytes.
the pre disposition is inherited. but the specific injury is triggered by environmental factors.
126
How is autoimmune hepatitis most effectively treated?
Combination of prednisone and azathioprine.
127
Define Toxic Hepatitis
liver injury following the inhalation, ingestion or prenternal administration of a number of pharmacologic and chemical agents.
128
What are the most commonly used drugs and chemicals that can precipitate toxic hepatitis?
```
acetaminophen
isoniazid
oral contreceptives
androgens
chlorpromazine
allopurinol
aminodarone,
hydralazine,
alcohol,
valproic acid,
phenytoin
helathane
carbamazephine
methotexate
```
129
How do you tx Toxic hepatitis?
discontinuation of the suspected agent
| sxs usually subside after removal of the agent
130
What is Hereditary hemochromatosis>? (HH)
is an adult-onset impairment of iron metabolism that results in the abN accumulation of iron in parenchymal organs leading to toxicity and fibrosis.
caused by mutation that disrupts the regulation of the intestinal absorption of iron > ++ absorbed.
131
T or F HH is the most comon inherited liver disease in caucasians and the most common autosomal recessive genetic disorder?
T
132
What are the 3 important mediators that regulate iron absorption in the human body?
1. transferring
2. transferring receptor
3. ferritin
133
What are the two mutations of the HFE gene associated with hemochromatosis?
C282Y
| H63D
134
What is the major site (tissue) of excess iron deposition?
liver
| its stored in the hepatocytes in form of ferritin and hemosiderin. Too much = toxic d/t free radicals
135
What are the primary causes of death from HH ?
1. hepatocellular carcinoma
2. complications of cirrhosis
3. cardiomyopathy, congestive heart failure, arrhythmia
4. complications of DM
5. bacterial and viral infection
136
What tests are useful in the dx of HH
1. serum ferritin level
2. transferring saturation
3. genetic testing for HFE mutation
4. liver biopsy
137
What are the treatment options for HH?
phlebotomy, to mobilize and remove excess iron levels.
| monitoring AFP is always required.
138
what are the most common benign solid tumors of the liver?
1. hepatocellular adenoma
2. hemangioma
3. focal nodular hyperplasia (FNH)
139
What is thx option for a hepatocellular adenoma
remove surgically. d/t risk of bleeding, rupture and malignant transformation.
140
What are giant/ cavernous hemangiomas?
hemangiomas larger than 4 cm
141
what are the sxs of hemangiomas?
none usually, but a giant one can cause thrombosis.
| - they have no potential to become malignant.
142
how are hepatic cysts identified?
incidentally on imaging studies
143
What complications may arise from larger cysts"
spontaneous hemorrhage
rupture into the peritoneal cavity
compression of biliary ducts
rupture into the biliary tree
144
what is a simple cyst?
cyst formation that contain clear fluid and do not communicate with the intrahepatic biliary tree
solitary but occasionally multiple cyst can be found.
145
What is primary hepatocellular carcinoma (HCC)?
most common non-metastatic malignancy of the liver
146
HCC is attributed to what diseases?
Hep C, Hep B
147
What are risk factors for the development of HCC?
cirrhosis, chronic hep B and or C, NASH, alcohol and tobacky, chemical agents, Rx and radiation.
148
What are the most frequent sites of origin for hepatic metastases?
lung
breast
GI
Genitourinary
149
What is portal vein thrombosis?
form of venous thrombosis that affects the portal veins in the liver. Blood flow is impleded and caues hepatic inflow obstruction and increases pressure in vascular bed. -
- main cause of non-cirrhotic portal HTN
150
What causes portal vein thrombosis?
```
cirrhosis
malignancy
pancreatitis
hypercoagulable disorders
sepsis
```
151
What are complications associated with portal vein thrombosis?
variceal bleeding- #1
hepatic encephalopathy
ascites
152
When is prognosis for portal vein thrombosis favourable?
in those who do not have cirrhosis or a malignancy as an etiologic factor
153
What is wilsons' disease?
rare, autosomal recessive inherited disorder of copper metabilism.
primarily affects the liver, brains, kidneys and eyes and joints.
154
T or F. Copper is known to be hepatoxic at low levels
F. Excessive levels.
| causes 2 main patterns of liver damage: cirrhosis, Chronic hep, or fulminant hepatic failure
155
What is the primary complication associated with ^^ coper levels in the body?
destruction of basal ganglia in the brain.
- areas of coordinate movement are affected
- causes dystonia, incorrdination, and tremor
- psychiatric ft. can also be present
156
What is the prognosis of wilson's disease
depending on severity, but if discovered early and recovered during the first two years of tx- okay.
untx'ed is fatal
157
What are the diagnostic testings for wilson's
```
serum ceruloplasmin
24 urine copper excretion
hepatic copper concentration
genetic testing
imaging studies
liver biopsy
```
158
What is the treatment for wilson's
chelating agents, when enhance Cu excretion
and inhbit absoption
diet changes
its life long
159
What is Glycogen storage disease (GSD)?
group of disorders that develop as a result of any one of several inborn erros of metabolism. These occur becuase of enzyme defects that affect the process of glycogen synthesis.
160
What is the most common GSD?
type 1 disorder- glucose-6-phosphate deficiency
161
What happens over time to the liver for people with GSD?
store glucose as glucogen but cant release it, so it builds up in the liver causing hepatomegaly as well as kidney enlargement.
162
What are some sxs/complications of GSD?
```
hypoglycemia > seziures
cyanosis
apnea
low BS->> delayed growth and development
hepatic adenomas
hepatocellular carcinoma
renal insufficiency
coma/ brain damage
anemia (severe)
chronic pancreatitis
chronic IBD
crohns
```
163
What is the treatment for Type 1 GSD
maintain blood glucose levels,
max growth and development
prevent complications.
164
What is Gilberts syndrome?
biliary tract disorder
| most common herediary cause of unconjugated hyperbilirubinemia (mild)
165
What are the sxs of Gilberts syndrome?
Jaundice in the absence of hemolysis or underlining liver disease
- its a benign condition
166
What are sxs of hyperbilirubinemia?
```
dehydration
fasting or stressors
vague abdo discomfort
general fatigue
- episodes resolve spintaneously and no tx required
```
167
What is cholangitis?
localized or diffuse inflammatory changes affecting the intrahepatic and extrahepatic bile ducts
168
What are the main factors in the pathogenesis of acute cholangitis?
biliary tract obstruction, elevated intraluminal pressure and infection of the bile
169
what are the most common causes of bacterial cholangitis?
common bile duct stones
tumors
strictures
complications from endoscopic retrograde cholangiopancreatography (ERCP)
170
What is the prognosis of Bacterial cholangitis?
```
mild forms > tx with ABD
severe- poor, complications are
liver failure
sepsis with spetic schock
DIC
infected portal thrombosis
acute renal failyre
hepatic abscesses
metastatic abscesses
catheter-related complications related to tx
```
171
What is Primary sclerosing Cholangitis (PSC)
chronic cholestatic liver disease of unknown etiology that primarily affects young to middle-aged males.
- assoc. with IBD, UC + crohns
172
what are the sxs and characteristics of PSC?
fibrosing inflammation of both the intrahepatic and extrahepatic biliary tree.
-results in irreversable damage to the bile ducts and ultimately leads to cholestasis, cirrhosis and liver failure.
173
what is the tx for those with PSC
there is none, liver transplant is the only effective option for individuals with end-stage liver disease from PSC
174
what is the most likely etiology of the diseasE?
an autoimmune response causing damage to the biliary tree
175
What is biliary cirrhosis and how is it characterized?
a rare form of liver cirrhosis caused by disease or defects of the bile ducts.
cholestasis and progressive liver diease
176
What are the two types of biliary cirrhosis'
primary and secondary
177
Describe primary biliary cirrhosis (PBC)
chornic and progressive cholestatic liver disease that prodominantly affects middle aged women
- etiology unknown- ?autoimmune
-
178
What is the pathology of PBC?
continuous destruction fo small and medium bile ducts resulting in chronic cholestaiss
179
What is the prognosis of the Primary biliary cirrhosis disease?
for those who are Asxs when dx'ed its slow
| those who are sxs- 5.5-12 yr survival.
180
Describe secondary biliary cirrhosis
occurs as a result of prolonged bile duct obstruction, narrowing or clsoure
- most common cause is scleorsing cholangitis... also bile duct structures gallstones, stumors, biliary atresia cystic firbosis
181
What is the prognosis of secondary biliary cirrhosis?
similar to PBC unless underlying cause is eliminated.
182
what is alfa 1- anturypsin deficiency?
a genetic disorder caused by mutations to the A1AT gene. decreases A1At activity in the blood and lungs and deposition of excessive A1AT proteins in liver.
- leads to pulmnary disease and primarily emphysema
183
What is the function of A1AT gene in the liver?
protect the lungs from enzymes that can damage connective tissue and destroy alveoli
184
What is the therapy suggested for A1AT deficiency?
none,
try with supportive care to reduced pulmonary sxs. In presence of cirrhosis, liver transplantation is the only corrective therapy.
185
What is Gauchers disease?
Its genertic and affects the gene that regulates lipid storage. Characterized by the deposition of a glycolipid that is accumulated in the body.
186
Gauches disease exists in 3 clinical forms, define them.
type 1- non-neuronopathic, most common, features: hepatoscplenomegaly, thombocytopenia and pathologic bone fracture
Type 2- Infantile or acute neuronopathic, features: hepatosplenomegaly and progressive neurologic deterioration
type 3- juvenile or subacute neuronopathic, features: progress slowly.
187
What disorders results for abN accumulation of the glycolipid in the bone marrow, liver, spleen, lungs and other organs
pancytopenia,
massive hepatosplenomegaly
occasionally diffuse infiltrative pulmonary disease.
188
The fats that contribute to neurologic involvement in Gaucher's type 3 and 2 disease may be related to an accumulation of what>
cytotoxic glycolipids in the brain
189
What is Reye's syndrome?
rare disorder characterized by acute noninflammatory encephalopathy and hepatic failure.
190
What is the etiology of Reyes syndrome?
unknown, but typically occurs after a viral illness, specially upper respiratory, and is associated with the use of aspiring during the illness
191
Death by Reyes usually occurs as a result of what?
cerebral edema, or increase intracranial pressure.
myocardial dysfunction,
cardio vascular collapse
respiratory failure renal failure GI bleeding
status epilepticus
sepsis
