Liver + Gallbladder robbins Flashcards
1
Q
What are the most common type of gallstones?
A
Cholesterol (80% in western world)
2
Q
What are Pigment (black + brown) stones made up of?
A
Billirubin and Calcium salts = Radio opaque
**INcreased Unconjugated billirubin= Hemolysis
BROWN= Due to Infection in Bile tree
**COMMON in Asian countries
3
Q
What are some risk factors for Cholesterol stones?
A
Gallbladder Hypomotility Mucus hypersecretion= traps cholesterol crystals AGE FEMALE (hormones) Obesity (Insulin resistance) Rapid Weight reduction Dyslipidemias Inborn errors of Bile metabolism
4
Q
What kind of Gallstones are seen in a patient with SCD or hereditary spherocytosis or mechanical heart valves?
A
Calcium Billirubinate = PIGMENT stones
5
Q
Yellow pale radiolucent gallstone is most likely?
A
Cholesterol stone
6
Q
Enlarged, tense gallbladder, with thickened walls, and edema caused by a gallstone?
A
Acute Cholecystitis
7
Q
Patient with Severe RUQ pain lasting 6hrs. Pain radiates to shoulder, Fever, nausea, leukocytosis, and Hyperbillirubinemia?
A
Acute calculous Cholecystitis
**Jaundice= Blockage of CB duct
8
Q
Recurrent attacks of steady epigastric pain, N/V, and intolerance to fatty foods?
A
Chronic cholecystitis
9
Q
What is choledocholithiasis?
A
Gallstone in the biliary tree (NOT gallbladder)
10
Q
What is Cholangitis and MCC?
A
Inflammation of the Bile ducts
Caused by Bacterial infection (e coli)
11
Q
Pt with Right upper abdominal pain, fever, chills, and jaundice?
A
Bacterial cholangitis
12
Q
What consequence of chronic obstruction of extrahepatic biliary tree is seen in the Liver?
A
Secondary Biliary Cirrhosis–>
Secondary inflammation
Initiates periportal fibrogenesis
Leads to Scarring and nodule formation
13
Q
Infant with pale stool, malabsorption and inflammation and fibrosis of CB duct and progressive destruction of intrahepatic biliary tree. Liver biopsy shows Portal tract edema, fibrosis, and parenchymal cholestasis. Eventually Cirrhosis and liver failure?
A
Biliary atresia
14
Q
What are the serum tests for Hepatocyte integrity?
A
AST
ALT
LDH
15
Q
What are the serum tests for biliary excretion function?
A
bilirubin (serum + urine)
bile acids
ALK
GGT
16
Q
What are the serum tests for hepatocyte function?
A
Albumin PT (INR) Ammonia Aminopyrine breath test (hepatic demethylation) Galactose elimination (IV)
17
Q
Pt with hepatic insufficiency that progresses to Hepatic encephalopathy in 2-3 wks. What are the MCCs?
A
Acute liver failure–> massive hepatic necrosis
**MCC–> Drugs or Viral hepatitis
18
Q
What are the signs and symptoms of Hepatic failure?
A
Jaundice (Bilirubin >2.0) Hypoalbuminemia--> peripheral edema Palmar erythema Spider angiomas Hypogonadism or Gynecomastia- Increased Estrogen Coagulopathy
19
Q
What is defined as the systemic retention of Billirubin, bile salts, and cholesterol?
A
Cholestasis
20
Q
What is the pathogenesis for physiological jaundice of the newborn?
A
Hepatic conjugation machinery is not fully developed–> Mild Unconjugated Hyperbilirubinemia
21
Q
Pt develops icterus during long periods of fasting or stress caused by a decreased level of Glucoronosyltransferase (GST)?
A
Gilbert syndrome
22
Q
What is Dubin Johnson syndrome?
A
AR defect in Transport protein responsible for Hepatocellular excretion of bilirubin glucuronides into Caniculi.
- *Conjugated hyperbilirubinemia
- **BLACK LIVER
23
Q
Patient with black liver has what genetic deficiency?
A
Conjugated bilirubin transport protein
24
Q
What are the causes of Jaundice due to excess production of UNconjugated bilirubin?
A
Hemolytic anemia
Pernicious anemia
Thalassemia
25
What are the causes of jaundice due to reduced hepatic uptake of Unconjugated bilirubin?
Drugs
| Diffuse Hepatocellular disease (Viral or drug induce hepatitis, Cirrhosis)
26
What is the cause of jaundice due to impaired bilirubin conjugation?
Physio of Newborn
| Gilbert syndrome
27
What are the causes of jaundice caused by Conjugated bilirubinemia due to decreased Hepatocellular excretion?
Def in canalicular membrane transporter (Dubin johnson syndrome)
Drugs (OCT , cyclosporines)
Hepatocellular damage (viral or drug hepatitis)
Gallstone
Carcinoma in head of pancreas
28
What are some of the signs and symptoms of Cholestasis?
```
Jaundice
Pruritus
Skin Xanthomas (increased Cholesterol)
Elevated ALK
Malabsorption of vitamins ADEK
```
29
MCCs of jaundice due to excess conjugated bilirubin?
Hepatitis
| Intra-extra hepatic obstruction of Bile
30
MCC of jaundice due to excess unconjugated bilirubin?
Hemolytic anemia
31
What are some distinctions between Acute and chronic hepatitis?
Acute= Less inflammation but More hepatocyte death than Chronic
**Both induce T-cell response
32
Which Hepatitis virus is transferred by Fecal-Oral route and does NOT have a chronic or Carrier state?
HAV + HEV
| **Hepatitis A + E only cause AcutE infections
33
Hepatitis virus that is ssRNA and related to Picornaviruses. What is the laboratory diagnostic test?
Serum Anti-HAV IgM antibodies
34
What are the most likely outcomes of a HBV infection?
60-65%--> Subclinical= 100% recovery
20-25%--> Acute= 99% recover (1% fulminant)
5-10% --> Carriers
4%--> Chronic= Hepatocellular carcinoma
35
What is genome and route of infection for the Hepadnavirus?
HBV--> dsDNA
ROI= Parenteral, Sexual, perinatal
**NOT FECAL
36
Which viral hepatitis is associated with Daycare centers and Jails?
HAV
37
Which viral hepatitis is associated with Dialysis, Needle-sticks, and IVDA?
HBV
38
Which part of the HBV is important for establishing Persistent infection? Immunogenic? Developing hepatocellular carcinoma?
HBeAg-> secreted to establish persistant infection
HBsAg--> secreted and is immunogenic
HBV X protein--> HC carcinoma
39
Describe the clinical course of HBsAg in a HBV infection?
HBsAg appears before onset of Symptoms (1st)
Peaks during Overt disease
Disappears 3-6 post infection
**Anti-HBsAg does NOT rise until Acute infection is over and wks after HBsAg disappears= GAP
40
What Antibody conferes immunity to HBV infection and is basis for current vaccines?
Anti-HBsAg
41
What is the significance of HBeAg, HBV-DNA, and persistent HBeAg?
Both signify ACTIVE viral replication
Persistent HBeAg--> progression to Chronic
**Identify INFECTIVE STATE
42
Ground Glass cells in liver signify?
HBV infection--> Hepatocytes with ER stuffed with HBsAg
43
What are the potential outcomes of Acute HCV infection?
15%--> Resolve
| 85%--> Chronic (80%stable) (20%>cirrhosis>HC carcinoma> death)
44
Why is it hard to develop HCV vaccines?
Highly variable due to infidelity of RNA replication
| **Quasispecies--> infected person can carry many HCV species at once
45
Which Hepatitis virus is associated with Fatty change, PROMINENT Lymphoid infiltrates, and Bile duct injury?
HCV
46
This hepatitis virus ONLY causes infection when it is encapsulated by HBsAg?
HDV--> NEEDS co-infection with HBV
| **D for defective= Needs another virus
47
What is the best indication of co-infection by HDV and HBV?
IgM against both HDV Ag a& HBcAg
| **IgM= denotes current infection which is required for HDV replication
48
Which Hepatitis virus has a HIGH mortality rate in Pregnant women (20%)?
HEV
49
Which type of Viral hepatitis is most frequently causes acute hepatitis?
HBV
50
Which type of virus almost Never cause Fulminant hepatitis?
HCV
51
What type of Hep Virus is notorious for causing a Chronic hepatitis evolving into Cirrhosis?
HCV
52
Which type of viral hepatitis is associated with Cryoglobulinemia?
HCV
53
Carrier states are prevalent in what type of viral hepatitis?
HBV--> infections acquired in Childhood (95%)
| **Those acquired in adulthood only 10% become carriers due to Increased Maturity of Cellular immunity
54
Councilman bodies are seen in what liver infection?
Yellow Fever
| **Councilman bodies are Eosinophilic apoptotic Hepatocytes
55
Which hepatitis virus is transferred by Blood, Birthing, and Bonking?
HBV
56
A chronic liver disease patient presents with rigidity, seizures, confusion and Asterixis?
Hepatic encephalopathy
57
What can cause Asterixis?
Severe loss of Hepatocellular function
| Shunting of blood around chronic liver
58
What is the pg of hepatic encephalopathy?
Elevated ammonia= impairs neuronal function and promotes generalized brain EDEMA
59
Diffuse fibrosis and conversion of normal liver architecture into structurally abnormal nodules?
Cirrhosis
60
What are the manifestation of ETOH on the liver?
Steatosis
Steatohepatitis
Cirrhosis
61
What is nonalcoholic fatty liver disease associated with?
```
METABOLIC SYNDROME
Diabetes
Obesity
Hypertension
dyslipidemia
```
62
What is the earliest change in Alcoholic liver disease?
Fatty liver--> accumulation begins around centrilobular hepatocytes and extends to central vein
**Soft yellow greasy liver
63
What are some features of steatohepatitis?
hepatocyte ballooning-> swelling and necrosis
Mallory Denk Bodies-> Tangled intermediate filaments as cytoplasmic inclusions
PMN infiltration
64
What characteristic feature is seen in Alcoholic HEPATITIS?
Mallory Denk bodies
65
What is the histological appearance of Fatty liver disease?
Fatty hepatocytes making a "CHICKEN WIRE FENCE" appearance
66
What is the "burned-out" stage of alcoholic cirrhosis?
Replacement of Fat with Fibrosis= Small nodules
67
Causes liver cell necrosis, inflammation, fatty change. and Mallory Bodies?
Alcoholic hepatitis
68
What is the pathogenesis of alcoholic Fatty liver?
1. Excess reduced NAD from ethanol metabolism= increases Lipid synthesis
2. Impaired assembly and secretion of lipoproteins
3. Increased peripheral catabolism of fat
69
What substance directly affects cytoskeleton organization and mitochondrial functions of hepatocytes?
Alcohol
70
What are the causes of alcoholic hepatitis?
ETOH= Mito and cytoskeleton poison
Acetaldehyde= induces peroxidation disrupting cytoskeleton and membranes
ROS from oxidation of ethanol
Cytokines (TNF)
71
What is the treatment for an alcoholic with hepatomegaly, mildly elevated bilirubin and ALK?
Fatty change== STOP ETOH reverses
72
Patient with malaise, anorexia, wght loss, RUQ pain, hepatomegaly, and fever. High bilirubin, ALK, Neutrophilic leukocytosis, and elevated AST>ALT?
Alcoholic cirrhosis
73
Chronic alcohol use is associated with what vitamin deficiencies?
Thiamine (B1)
B12
Folate
74
What is considered the alcohol consumption threshold for developing alcoholic liver disease?
50-60 g/day
75
What is the mechanism of NAFLD in patients with insulin resistance and obesity?
impaired oxidation of FA
Increased synthesis and uptake of FA
decreased hepatic secretion of VLDL cholesterol
76
What is the genetic cause of disorder characterized by accumulation of body iron depositing in organs (Liver, heart, pancreas)?
Hemochromatosis--> HFE gene mutation
77
What are the MCC of secondary hemochromatosis?
Acquired= Transfusions + beta Thalassemia + myelodysplastic syndromes
78
Hereditary disorder causing cirrhosis, Diabetes, and skin pigmentation?
Hemochromatosis
79
What is the function of hepcidin?
Down regulates the efflux of iron from intestines and macrophages into plasma and inhibit iron absorption
80
What is the mechanism of damage in hemochromatosis once iron is above 20g?
Lipid peroxidation by iron catalyzed free radicals
Simulation of collagen formation
Direct interaction btwn iron and DNA
81
AR disorder marked by accumulation of Toxic levels of Copper in Liver, brain, and eyes?
Wilsons disease
82
What is the genetic abnormality in wilsons disease?
mutated ATP7B gene == encoding ATPase metal ion transporter
83
What is the plasma transporter of Copper>
Albumin
84
What accounts for 90% of the copper in the Blood?
Copper- alpha2 globulin complex= Ceruloplasmin
85
What is the primary elimination route of Copper?
Bile
86
What is a Kayser-Fleischer ring?
Eye lesion caused by Excess Copper in WILSON disease
87
5 yo with ascites, jaundice, psychosis, and a dark green rings encirculing the iris?
Wilson disease--> Elevated copper (Ceruloplasm)
88
What is the function of Alpha 1 anti-trypsin?
inhibition og proteases (PMN elastases during inflammation)
89
What is the Pathogenesis of A1 antitrypsin deficiency mediated damage in the Liver?
Mutant A1AT cannot be secreted by hepatocytes= accumulation in ER and triggers Unfolded protein response causing APOPTOSIS
90
What does AAT mutation ZZ cause?
Pulmonary emphysema and Liver injury
91
Liver biopsy shows severe hepatocyte necrosis, marked inflammation with monos and Plasma cells, and burned-out cirrhosis?
AI Hepatitis
92
What types of Abs are seen in AI hepatitis?
ANA
Anti smooth muscle
anti microsomal
93
Most common cause of Acute liver failure necessitating transplantation In US caused by toxic metabolite produced by CYPs in acinus of zone 3 hepatocytes?
Acetaminophen
94
What substances are known to cause hepatocellular necrosis of Zone 3 cells?
```
Methyldopa
Phenytoin
Acetaminophen
Halothane
Isoniazid
```
95
What substances are known to cause Macrovesicular Steatosis?
Ethanol
methotrexate
Corticosteroids
96
What substances are associated with causing Microvesicular steatohepatitis and Mallory Bodies?
Amiodarone
| Ethanol
97
3 yo child with permicious vomiting, and lethargy, and hepatomegaly following viral infection?
Reye syndrome--> microvesicular steatosis
98
What are the two predisposing factors to Reye syndrome?
Aspirin
| Viral infection
99
What are the key morphological features of Reyes syndrome?
Hepatocellular microvesicular steatosis
Pleomorphic enlargement of matrices + loss of dense bodies.
**CEREBRAL EDEMA= swollen astrocytes
*Skeletal muscle + kidneys+ heart also show microvesicular fatty change
100
What is the most common hepatic neoplasms?
Metastasis from:
Colon
Lung
breast
101
What is a childhood hepatocellular tumor?
Hepatoblastoma
102
What liver tumor is associated with exposure to Vinyl chlorides + Arsenic?
Angiosarcoma
103
Well circumscribed endothelial cell lined vascular channels and intervening stroma lesions?
cavernous hemangiomas= benign
104
Bile duct strictures separated by bland, densely collagenized stroma. No malignant potential but chains are associated with PCKD?
Von Meyenburg complexes
105
WEll demarcated but poorly encapsulated lesions of hyperplastic hepatocytes with central stellate fibrosis in otherwise normal liver?
Focal Nodular Hyperplasia
* *response to abnormal vascular flow or vascular anomaly
* *MC in females bc grow with Estrogen or OCT
106
What liver lesions is MC in women of child bearing age that grows in response to OCP and regresses with discontinuation?
Hepatic adenoma
107
What risks are associated with Hepatic adenomas?
1. Rupture causing hemorrhage esp in Pregnant females due to HIGH Estrogen
2. Malignant transformation in those harboring Beta Catenin mutations
108
What are the genetics associated with Hepatic adenomas?
90% somatic mutation of HNF1A or CYP1B1
| 10% Beta catenin= Malignant potential
109
What is the precursor lesion to HCC?
MC-> Chronic liver failure / cirrhosis == Dysplasia over time
110
What are the 2 forms of Hepatocellular dysplasia?
Large cell change= large, pleomorph, Large nuclei
| Small cell= small, hyperchromatic, angulated nuclei
111
What two things are associated with HIGH risks of HCC?
```
HBV (East) HCV (West)
Aflatoxin
Alcoholic liver
NAFLD
AAT def
Hemachromatosis
hereditary tyrosinemia
```
112
What disorder is Most likely to give rise to HCC?
hereditary tyrosinemia
113
What toxin is found in "moldy" grains and peanuts and can covalently bind cellular DNA causing TP53 mutations and subsequent HCC?
Aflatoxin--> Aspergillus
114
Where do most HCC arise from?
Large or small dysplastic lesions in Cirrhotic livers
115
What are some factors involved in the pathogenesis of HCC?
Inflammation and regeneration= mutations
Beta catenin or TP53 muations
HBV X antigen
Defects in DNA repair
116
What is the most common path of metastasis for HCC?
Hematogenous--> invades Hepatic vein
117
PT with rapid increase in liver size, worsened ascites, appearance of bloody ascites, fever and pain?
HCC
118
What is the MC serological marker for HCC?
Alpha feto-protein
**also used in YOLK SAC tumors
119
Neonate with jaundice prior to 2wk is most likely?
Physiological Jaundice of newborn
120
Neonate with jaundice post 2wks is most likely?
Biliary atresia
121
Newborn presents with jaundice, dark urine, acholic stools, and hepatomegaly?
Neonatal cholestasis
| **physiological + Atresia
122
Caused by nonsuppurative destruction of small and medium sized intrahepatic bile ducts leading to inflammation, fibrosis, cirrhosis, and liver failure?
Primary biliary cirrhosis
123
What serological marker is seen in 45yo female with elevated ALK, pruritis, and history of Sjogren?
PBC--> Anti-mitochondrial antibodies
124
What is the "Florid duct lesion?"
PBC lesion--> in portal tract
lymphocyte infiltrate
plasma cells
Granuloma
125
35 yo male with bloody hx of bloody diarrhea, has "patchy" inflammatory/fibrotic destruction of bile duct system?
Primary Sclerosing Cholangitis
126
Bile Duct beading (strictures and dilations) are seen in association with what GI disease?
Primary Sclerosing cholangitis--> IBD (UC)
127
What is seen under microscopy of primary sclerosing cholangitis?
ONION skinning fibrosis of bile ducts
128
What drugs are associated with cholestasis
| (Bland) ?
Anabolic steroids
Contraceptive steroids
Antibiotics
129
What is the most common cause of cholangitis>
Ascending infection caused by E coli, Klebsiella, Entercocci, Clostridium due to Obstruction
130
What are some common causes of parasitic cholangitis?
Fasciola hepatica
schistosomiasis
cryptosporidiosis (HIV)
131
What is the MCC of neonatal death caused by liver failure?
Biliary atresia
132
Platypnea (easier to breath laying down) & Orthodeoxia (decrease in BP with upright posture) are Pathognominic for what?
Hepatopulmonary syndrome
133
Impaired blood flow at what level of the liver will produce Eso varices, splenomegaly, and intestinal congestion only?
Inflow impairment
| **Portal vein obstruction
134
Genetic disease resulting in accumulation of glucocerbrosides within phagocytes leading to distended cytoplasms and cytoplasm looking like "wrinkled tissue paper?"
Gaucher Disease Type 1
**Glucocerebrosidase def
135
Child splenomegaly and Lytic bone lesions, osteonecrosis, and absence of CNS deficiency has what lysosomal genetic disease?
Gaucher disease
* *Ashkenazi Jews
* *Types 2+3 CNS involvement
136
What is the deficiency in Von Gierke Disease?
glucose-6-phosphatase def
