Liver Flashcards
1
Q
Metabolic functions of the liver
A
Carbohydrate metabolism - Gluconeogenesis Glycolysis Glycogenesis Glycogenolysis Fat metabolism and synthesis of - Cholesterol and triglycerides Lipoproteins Phospholipids Ketogenesis (In starvation) Protein metabolism - Transamination and deamination of amino acids Convert amino acids into urea
2
Q
Hormonal metabolism in the liver
A
Degradation of - Insulin, glucagon, ADH, steroid hormone Activation - Deiodination of thyroxine T4 to more active triodothronine T3 Vitamin D3 to 25(OH)D
3
Q
Storage function of liver
A
Stores vitamin A, D, E, K, B12
Coagulation of factor II, VII, IX and X. protein C & S
Synthesis of plasma proteins (albumin)
4
Q
Which factors require post translational modifciation by vitamin K dependant - carboxylation
A
2, 7, 9 10
5
Q
Protective function of liver
A
Kupffer cells (liver phagocytes) that digest/destroy cellular debris (RBCs) and invading bacteria Produce immune factors
6
Q
Detoxification function of liver
A
Endogenous substances - Make sure proteins of haemolyses are detoxified, bilirubin
Exogenous substances - Drugs, alcohol
7
Q
Where is bile stored between meals
A
Gallbladder, sphincter of Oddi is closed
8
Q
What stimulates secretion of bile
A
Chyme on duodenum stimulates release of CCK from enteroendocrine or I cells in duodenum sense the presence of lipids. Sphincter of Oddi opens and gall bladder contracts to release bile into the duodenum.
9
Q
How is bile concentrated
A
By removal of Cl- and water.
10
Q
Cause of cholelithiasis
A
Excess cholesterol relative to bile results in acids and lecithin being precipitated into micro crystals that aggregates to galls stones
11
Q
pH of bile
A
Slightly alkaline to neutralize acidic chyme from stomach. Also allows intestinal enzymes to function at their optimum pH.
12
Q
Primary bile acids vs secondary bile acids
A
Primary bile acids - Cholic and chenodeoxycholic acids
Secondary - Deoxycholic and Lithocholic acids
13
Q
How is primary bile acid converted to secondary
A
Hepatocytes release primary bile juice into the bile canaliculi which drains into the bile duct via cystic duct. This enters the duodenum. Intestinal bacteria dehydroxylate primary bile acids to form secondary bile acids. These acids are reabsorbed across intestinal wall and taken back to the liver where they can be re-secreted into the bile.
14
Q
What gives faeces brown appearance
A
Bilirubin, dark urine and pale stool can indicate poor liver function
15
Q
Composition of bile
A
Bile acids mainly cholic and chenodeoxycholic acid, water and electrolytes, lipids/phospholipids. cholesterol, IgA, bilirubin
16
Q
Most common pathology of biliary tract
A
Cholelithiasis
17
Q
Treatment for symptomatic stones
A
Laparoscopic cholecystectomy
18
Q
Treatment for unimpaired gallbladder function having small/medium sized radiolucent stones
A
Ursodeoxycholic acid
19
Q
What stones may be radiolucent and not detectable by x-ray
A
Large stone made up of purely cholesterol
20
Q
How can morphine worsen biliary colic pain
A
Morphine constricts the sphincter of Oddi, increasing intrabiliary pressure and making the pain worse. Atropine may be administered together to make the pain better
21
Q
Alternatives to morphine in biliary colic
A
Buprenorphine and pethidine
22
Q
What is biliary colic
A
Gallbladder/gallstone attack is pain due to a gallstone blocking the bile duct
23
Q
Treatment for relief of biliary spasm
A
Atropine or Glyceryltrinitrate (GTN)
24
Q
Transporters involved in reabsorption of bile salts
A
Active transport in the ileum by hepatocytes via Na coupled transport. Enterohepatic recycling takes place
25
What synthesized more bile if too much is lost in faeces
Cholesterol
26
What are bile acid sequestrants
Group of resins used to bind to certain components of bile in GI tract. They disrupt enterohepatic circulation of bile acids and prevent reabsorption
27
Examples of bile acid sequestrants
Colesevelam, colestipol and colestyramine. These prevent reabsorption of bile thereby promoting hepatic conversion of cholesterol into bile acid, leading to a lowered plasma cholesterol.
28
What does hepatic conversion of cholesterol into bile acids cause
Increase in LDL-receptor activity of hepatocytes increasing clearance of LDL-cholesterol from plasma
29
Use of bile acid sequestrants
Hyperlipidemia, cholestatic jaundice (depleting cholesterol leads to less cholesterol available to be precipitated out to form gallstones), bile acid diarhoea (eliminate bile acids from body)
30
Drawbacks of bile acid sequestrants
Unpalatable, inconvenient (large doses needed), diarrhoea, deficiency of fat soluble vitamins due to reduced absorption
31
What does drug metabolism act to do
Convert parent drug to more polar metabolites so they aren't readily reabsorbed by the kidney
Convert drugs to metabolites that are usually pharmacologically less active than parent
32
Main organ of drug metabolism
Liver but GI tract, lungs, plasma have activity too
33
Aspirin metabolism
Possess different type or spectrum of action -
| Aspirin has antiplatelet and anti-inflammatory activity whereas its metabolite salicylic acid is anti-inflammatory
34
Phases of drug metabolism (usual)
Phase 1 - Mediated by cytochrome P450 enzyme
A more reactive polar group is added to the parent compound, this can involve oxidation/reduction/hydrolysis
Phase 2 - Conjugation
Adds an endogenous compound increasing polarity
35
Why do drugs have to be polarized and metabolized
Most drugs are lipophilic and difficult for the kidneys to be excreted as they are reabsorbed by the kidney easily. Hence adding a polar group makes excretion of the drug easier.
36
What are cytochrome (CYP) P450 family of monooxygenases
Haem proteins located in endoplasmic reticulum of liver hepatocytes mediating oxidation reductions (phase 1) of lipid soluble drugs
37
Main gene families of cytochrome P450 monooxygenase
CYP1, CYP2 and CYP3
38
What is hepatic encephalopathy (HE)
Decline in brain function due to insuffiiency in detoxification of ammonia to urea resulting in raised ammonia (NH3) levels
39
Therapeutic option in hepatic encephalopathy
Lactulose - Semisynthetic fructose + lactose
Not digested or absorbed in ileum
Breakdown products in colon are acidic - reduce pH
This converts ammonia into ammonium which isn't absorbed and safely excreted
Antibiotics - Neomycin and Rifaximin
Minimally absorbed kind, suppress colonic flora, inhibit ammonia generation
40
Solid liver lesions in elderly
More likely to be malignant with metastases, more common than primary liver cancer in the absence of liver disease
41
Solid liver lesions in chronic liver patients
More likely to be primary lung cancer than metastases or benign tumours.
42
Solid liver lesion in non cirrhotic younger patients
Haemangioma
43
What is a hepatic haemangioma
Benign tumour of the liver composed of hepatic endothelial cells
44
Benign tumours of liver
Haemangioma
Focal nodular hyperplasia
Adenoma
Liver cyst
45
Malignant tumour of liver
```
Hepatocellular carcinoma
Cholangiocarcinoma
Fibrolamellar hepatocellular carcinoma
Hepatoblastoma
Metastases
```
46
What is fibrolamellar hepatocellular carcinoma
Form of hepatocellular carcinoma that typically affects young adults, histologically shows laminated fibrous layers between tumour cells
47
Features of haemangioma
Females > males, hypervascular tumour. Usually single, small and well-demarcated capsule. Asymptomatic
48
Management of haemangioma
No treatment needed, no need for fine needle aspiration biopsy. US/CT scan and MRI diagnostic
49
How does haemangioma show up in a CT scan
Lights up with a contrast as it's a very vascular tumour
50
Second most common benign tumour of the liver
Focal nodular hyperplasia (FNH)
51
What causes focal nodular hyperplasia
Congenital vascular anomaly
52
Classical presentation of focal nodular hyperplasia
Central scar containing a large artery, radiating branches to the periphery
53
Features of focal nodular hyperplasia (FNH)
Asymptomatic usually, rarely grows or bleeds, no malignant potential. More common in young-middle aged females with sinusids, bile ductules and kupffer cells on histology
54
Treatment for focal nodular hyperplasia
No treatment required
55
Liver disease associated with contraceptive hormones and anabolic steroids
Hepatic adenoma, more common in females than males, 10:1 ratio
56
Clinical features of hepatic adenoma
Mainly asymptomatic but may have RUQ pain and may progress to rupture, haemorrhage or malignancy (rare)
57
Malignant transformation of hepatic adenoma is higher in which gender
Males than females
58
Is the portal tract present in hepatic adenoma
No portal tract, central vein or bile duct present in hepatic adenoma
59
Glycogen storage disease can lead to
Formation of multiple adenomas (adenomatosis)
60
Diagnosis of hepatic adenoma
Stop hormones (oral contraceptives/anabolic steroids)
Weight loss
Males - Surgical excision
Females - Imaging after 6 months, if;
<5 cm or reducing in size - annual MRI
>5 cm or increasing in size - surgical excision
61
What is a simple cyst
Liquid collection in a sac lined by an epithelium
62
Clinical features of a simple cyst
Asymptomatic with no malignant potential
| If symptomatic, may be related to intracystic haemorrhage, infection, rupture (rare) or compression
63
Management of simple cyst
No follow up necessary, image 3-6 months if in doubt
| Surgical resection if symptomatic or uncertain of diagnosis
64
What causes hydatid cysts
Infection due to ingestion of the larvae of the tapeworm - Echinococcus granulosus, not common in the UK
65
How is diagnosis of hydatid cyst made
History, appearance (may be jaundiced, weight loss, abdominal pain), serological testing by detection of anti-Echinococcus antibodies
66
Cyst commonly found in dogs that consume organs or meat of infected sheep
Hydatid cyst
67
Mangement of hydatid cyst
Conservative surgery - Open cystectomy, marsupialization
Radical surgery - Pericystectomy, lobectomy
Drugs - Albendazole
68
Option for patients unresponsive to surgery for hydatid cyst
PAIR - Percutaneous aspiration-injection reaspiration drainage
69
What can embryonic ductal plate malformation of the intrahepatic biliary tree lead to
Formation of numerous cysts through the liver -
Polycystic liver disease
von Meyenburg complex (VMC)
Autosomal dominant polycystic kidney disease
70
What are von Meyenburg complexes
Also known as multiple biliary hamartomas, rare cause of multiple benign hepatic lesions throughout the liver.
71
What is polycystic liver disease (PCLD)
Numerous cysts throughout the liver with liver function preserved. Symptoms depend on size and involves a mutation in PCLD gene - PRKCSH and SEC63
72
What is autosomal dominant polycystic kidney disease (ADPKD)
Renal failure due to polycystic (multiple cysts) in the kidney. Non-renal extra hepatic features and massive hepatic enlargement are common. Due to a mutation in the ADPKD genes - PKD1 and PKD2
73
One of the most lethal and prevalent monogenic human disorder
Autosomal dominant polycystic kidney disease
74
Examination findings of polycystic liver disease
Abdominal pain, abdominal distension, atypical compression of adjacent structures due to voluminous cysts
75
Management of polycystic liver disease
Conservative treatment to halt cyst growth and allow abdominal decompression and ameliorate symptoms
76
When are invasive procedures called for in polycystic liver disease
In selective patient groups such as those with advanced PCLD, ADPKD or liver failure
77
Pharmacological therapy and polycystic liver disease
By somatostatin analogues can relieve symptoms and reduce liver volume
78
Patient with a abdominal or biliary infection, recent dental procedure. High fever, leucocytosis and RUQ abdominal pain.
Liver abscess
79
Management of liver abscess
Empirical broad spectrum antibiotics
Aspiration/drainage percutaneously
Echocardiogram
80
Management of liver abscess if no improvement
Operation - Open drainage or resection
81
Most common primary liver cancer mostly found in men
Hepatocellular carcinoma (HCC)
82
Cause of hepatocellular carcinoma
Hepatitis B, hepatitis C, alcohol, aflatoxins
83
What are aflatoxins
Family of toxins produced by certain fungi found on agriculture crops such as maize, peanuts, corn. Main fungi producing this are Aspergillus flavus and Aspergillus parasiticus
84
Most common feature of hepatocellular carcinoma
Right upper quadrant pain and weight loss
85
Examination of hepatocellular carcinoma (HCC)
Signs of cirrhosis, hard enlarged RUQ mass, rare liver bruit
86
Where can hepatocellular carcinoma metastasize to
Rest of liver, portal vein, lymph nodes, lung, bone, brain
87
How is hepatocellular carcinoma invstigated for
Alfa fetoprotein and ultrasound. Elevation of alfa fetoprotein is seen in 60-80% of patients.
88
Why is biopsy avoided in diagnosing HCC
Biopsy may lead to seeding of cells along the route of resection.
89
Best treatment for HCC
Liver transplant only is single tumour < 5cm or less than 3 tumours less than 3 cm each
90
When is resection done in HCC
Small tumours with preserved liver function, i.e. no jaundice or portal hypertension. Recurrence rate is high
91
Treatment for patients without resectable livers and advanced liver cirrhosis in HCC
Local ablation using percutaneous ethanol injection or radiofrequency. Not a good choice for tumours beside major blood vessels, diaphragm or bile duct. It is a temporary measure and rate of recurrence is high
92
Palliative treatment for unresectable HCC in patients with early cirrhosis but well compensated
TransArterial ChemoEmbolization (TACE), it restricts the tumours blood supply. Chemotherapeutic drugs are injected into the artery supplying the tumour through a catheter. These drugs not only block blood supply but also induce cytotoxicity.
93
Systemic therapy for HCC
Sorafenib -
Kinase inhibitor drug, inhibits vascular endothelial growth factor receptor, platelet-derived growth factor receptor and Raf
94
Young patient showing typical stellate scar with radial septa showing persistent enhancement on CT scan. Alfa-fetoprotein blood test is normal
Fibro-lamellar carcinoma
95
Treatment options for fibro-lamellar carcinoma
```
Surgical resection or transplantation.
TransArterial ChemoEmbolization (TACE) for unresectable tumours
```
96
Most common site for blood borne metastases
Liver
97
Areas of a lobule
1.Periportal zone closest to the portal triad. 2.Mid acinar or midzonal and 3.centrilobular or pericentral zone is closest to the central vein
98
Cells of which zone are the most prone to injury
Zone 3 - Pericentral
99
Normal progression of liver disease
Insult to hepatocytes - Inflammation - Fibrosis - Cirrhosis
100
What is acetaminophen poisoning
Paracetamol poisoning, excessive use of paracetamol. Feeling of tiredness, abdominal pain or nausea. Can progress onto a yellowish skin, blood clotting problems and confusion. Can lead to death
101
How can uncomplicated jaundice be treated in a newborn
Exposed the undressed infant to filtered sunlight
102
Do all patients with liver disease get jaundice
No
103
Is liver disease the only cause of jaundice
No
104
Cause of pre-hepatic jaundice
Too much haem breakdown due to excess haemolysis. Leads to formation of unconjugated bilirubin
105
Cause of hepatic jaundice
```
Dead or injured liver cells -
Acute liver injury (virus, drugs, alcohol)
Alcoholic hepatitis
Cirrhosis (decompensated)
Bile duct loss (atresia/PBC/PSC)
Pregnancy
```
106
What is primary biliary cirrhosis commonly known as now
Primary biliary cholangitis
107
Primary biliary cholangitis vs primary sclerosing cholangitis
PBC - Slow, progressive autoimmune destruction of the small bile ducts of the liver
PSC - Slow, progressive disease of liver and gallbladder affecting the medium and large bile ducts inside and outside the liver causing sclerosis (hardening)
108
Post hepatic jaundice
Bile can't get into the bowel
109
Cause of post hepatic jaundice
Congenital biliary atresia
Gallstones blocking common bile duct
Strictures of common bile duct
Tumours
110
Is cirrhosis reversible
No, fibrous bands form separating regenerative nodules of hepatocytes. Hepatic microvasculature is altered causing a loss in hepatocyte function
111
Common causes of liver cirrhosis
```
Alcohol
Hepatitis B and C
Iron overload
Gallstones
Autoimmune liver disease
```
112
Why is chronic liver failure inevitable after cirrhosis
Hepatic microvasculature is altered causing a reduced blood supply to hepatocytes. Hence the liver can't detox nor synthesize
113
Why are lower oesophageal varices seen in portal hypertension
Lower oesophagus enters portal circulation via the left and right gastric veins to the hepatic portal vein
114
Complications of cirrhosis
```
Portal hypertension causing -
Oesophageal varices
Caput medusa
Haemorrhoids
Ascites
Liver failure
```
115
What causes caput medusae
Portal hypertension causes the superficial epigastric vein to become engorged and distended. Paraumbilical vein, which normally closes within one week of birth, becomes recanalised due to portal hypertension
116
Causes of portal hypertension
```
Liver cirrhosis
Budd-Chiari syndrome
Portal fibrosis
Sarcoidosis
Schistosomiasis
Portal vein thrombosis
```
117
What is Budd-Chiari syndrome
Rare condition, caused by occlusion of hepatic veins that drain the liver to IVC. Presents with classical triad of abdominal pain, ascites and liver enlargement
118
Pathophysiological basis of oedema due to liver failure
Reduced albumin synthesis resulting in hypoalbuminemia due to reduction in oncotic pressure. This pulls leads to excess fluid buildup in tissues
119
Pathophysiological basis of haematemesis due to liver failure
Ruptures oesophageal varice
120
Pathophysiological basis of purpura and bleeding due to liver failure
Reduced clotting factor synthesis
121
Pathophysiological basis of infection due to liver failure
Reduced kuffper cell number and function
122
What happens in liver after ingesting alcohol
Alcohol causes increased peripheral release of fatty acids, increased synthesis of fatty acids and triglycerides within hepatocytes. The major product of alcohol metabolism, acetaldehyde is mainly responsible for cell injury by formation of Mallory's hyaline. Fibroblasts also increase synthesis of collagen
123
Is hepatitis reversible
Yes
124
Histological difference between alcoholic fatty liver and alcoholic hepatitis
Alcoholic fatty liver - Fat vacoules seen in hepatocytes
| Alcoholic hepatitis - Mallory bodies and pericellular fibrosis seen, neutrophils and hepatocyte necrosis seen
125
Histology shows fat vacuoles in hepatocyte with collagen (stained blue)
Alcoholic fibrosis
126
What stain can be used to see alcoholic cirrhosis clearly
Masson's trichrome - Three colour staining procedure that helps distinguish cells from surrounding connective tissue
127
Non-alcoholic steatohepatitis generally occurs in
Patients with diabetes, obesity or hyperlipidaemia
128
Disease pathologically identical to alcoholic liver disease but in non-drinkers
Non-alcoholic steateohepatitis (NASH)
129
What kind of hepatitis can Hepatitis viruses cause
Interface hepatitis or piecemeal necrosis
130
What is piecemeal necrosis
Refers to necrosis that occurs in fragments. It is necrosis of the limiting plates in the liver
131
What is the periportal limiting plate
In smaller portal triads, the vessels lie in a network of connective tissue and are surrounded on all sides by hepatocytes. This ring is the periportal limiting plate
132
What is a councilman body
It is an apoptotic body, a globule of cells that represents a dying hepatocyte often surrounded by normal parenchyma
133
Mallory bodies vs councilman body
Mallory bodies - Intracytoplasmic inclusion and mainly seen in alcoholic hepatitis. Formed by aggregation of cytoskeletal intermediate filaments
Councilman body - Seen in viral hepatitis, complete cell is involved and formed by apoptosis
134
Primary biliary cirrhosis is mainly found in which gender
Females (90%), autoimmune
135
Why is biopsy doen in primary biliary cirrhosis
To stage the disease
136
Chronic inflammation affecting intra and extra-hepatic bile ducts associated with ulcerative colitis
Primary sclerosing cholangitis
137
Primary sclerosing cholangitis is seen more in gender?
Male
138
What is haemochromatosis
Accumulation of iron in the body
139
Primary vs secondary haemochromatosis
Primary - Genetic condition, increase iron absorption
| Secondary - Iron overload from diet, transfusion, iron therapy
140
What is hereditary or primary haemochromatosis
Inherited autosomal recessive condition causing excess absorption of Iron from intestine, abnormal iron metabolism
141
How does primary haemochromatosis affect the liver
Iron is deposited in the liver due to excess absorption and abnormal iron metabolism. This is eventually deposited in portal connective tissue, stimulating fibrosis. This can lead to cirrhosis and predispose to carcinoma if untreated
142
What stain can be used to confirm iron deposition in hepatocytes in primary haemochromatosis
Perls stain
143
Inherited autosomal recessive disease of Copper metabolism
Wilson's diseaese
144
Wilson's disease can lead to accumulation of copper in the eye forming dark rings around the iris called?
Kayser-Fleischer rings
145
What protein is deficient in Wilson's disease
Ceruloplasmins, copper-carrying protein
146
Why does Wilson's disease cause chronic hepatitis and neurological deterioration
Due to accumulation of copper in the liver and brain (basal ganglia)
147
What is alpha-1-antitrypsin deficiency
Inherited autosomal recessive disorder of production of an enzyme inhibitor. Causes excessive trypsin
148
Hepatocellular adenoma is more common in what gender
Females
149
What is chronic liver diseaes
Duration of liver disease greater than 6 months
150
What are hepatic stellate cells
Pericytes found in perisinusoidal space of the liver also known as space of Disse. They store vitamin A as retinol ester in their cell bodies. During an injury, the amount of vitamin A stored decreases progressively. This activates stellate cells causing secretion of collagen scar tissue.
151
Function of senescent stellate cells
Role as liver resident antigen presenting cells to natural killer cells
152
What are hepatic stellate cells also known as
Perisinusoidal cells or Ito cells
153
What switches off hepatic stellate cells
Tissue inhibitor of metalloproteinase
154
Chronic liver disease vs disease affecting liver that is chronic
Chronic liver disease can lead to cirrhosis
155
Non-alcoholic steatohepatitis is also known as
Non-alcoholic fatty liver disease
156
Pathogenesis of non-alcoholic steatohepatitis
2 hit paradigm -
1st hit - Excess fat accumulation
2nd hit - Intrahepatic oxidative stress, lipid peroxidation, TNF-a, cytokine cascade
157
Investigating simple steatosis
Investigate by ultrasound
158
Investigating NASH
Investigate by liver biopsy
159
Is primary sclerosing cholangitis autoimmune related
Disease showing auto-immune features but cause in unknown. Related to UC
160
How does primary biliary cholangitis present
Middle aged woman, asymptomatic/incidental find, may have fatigue/itch without rash or xanthelasma or xanthoma
161
Investigating primary biliary cirrhosis
Positive antimitochondrial-antibody test (AMA)
Cholestatic LFT's
Liver biopsy
162
Treatment of primary biliary cirrhosis/cholangitis
Ursodeoxycholic acid - Is a secondary bile acid, produced as a byproduct of intestinal bacteria
Liver transplant
163
Autoimmune hepatitis is also known as
Chronic active hepatitis
| Lupoid hepatitis
164
Type 1 vs type 2 autoimmune hepatitis affecting population
Type 1 - More in children
| Type 2 - Any age but often in young adults
165
Presence of LKM-1 antibody suggests what type of hepatitis
Type 2 autoimmune hepatitis
166
Antibody found in autoimmune hepatitis type 2
anti-liver kidney microsomal antibody (LKM-1)
| anti mitochondrial antibody (AMA)
167
Antibodies found in autoimmune hepatitis type 1
Autonuclear antibodies (ANA)
Anti-smooth muscle antibody (ASMA)
anti-soluble liver antigen (SLA)
168
Presentation of autoimmune hepatitis
```
Spider angioma
Jaundice
Scleral icterus
Palmar erythema
Gynaecomastia
Ascites
Encephalopahty
Asterixis
Hepatomegaly
Splenomegaly
Malaise, nausea, fatigue, lethargy, abdominal pain, anorexia
```
169
Stigmata of liver disease
```
Spider angioma
Jaundice
Scleral icterus
Palmar erythema
Gynaecomastia
Ascites
Encephalopahty
Asterixis
```
170
LFT finsings of autoimmune hepatitis
Elevated AST and ALT
| Elevated PT
171
Pattern of autoimmune hepatitis
Piecemeal necrosis and lobular involvement with interface hepatitis and numerous plasma cells
172
Treatment of autoimmune hepatitis
Oral Prednisolone and Azathioprine (immunosuppresant)
173
Dosage of drugs in autoimmune hepatitis
Prednisolone - Start at 30mg daily, taper down to 15mg at week 4 and maintain 10mg daily until therapy end
Azathioprine - 50-100mg daily
174
Commonest liver disease in Scandinavia
Primary sclerosing cholangitis, more common in men
175
Clinical presentation of PSC
Recurrent cholangitis
176
Investigating PSC
Imaging
177
Treatment of PSC
Maintain bile flow
| Monitor for cholangiocarcinoma and colorectal cancer
178
Mutations causing haemochromatosis
C282Y or H63D mutations in Human Haemochromatosis Protein (HFE) gene
179
Common presentation of haemochromatosis
Bronze diabetic -
| Darkening of skin and hyperglycaemia
180
Why does haemochromatosis have a late presentation
As the Iron accumulates in the liver showing no symptoms. Takes many years for it to have an effect
181
Clinical presentation of alpha-1-antitrypsin deficiency
Lung emphysema,deposition of mutant protein in lungs causing SOB, wheeze and increased risk of infection. May also have COPD, cirrhosis and panniculitis.
182
Mutation found in alpha-1-anti-trypsin deficiency
A1AT gene
183
Treatment of A1AT deficiency
Supportive management
184
Investigating Budd-Chiari syndrome
Ultra-sound of hepatic veins
185
Treatment of Budd-Chiari syndrome
Recanalization or TIPS
186
Patients with Budd-Chiari syndrome often have what protein deficiency
Protein C and S, increased thrombotic potential
187
Methotrexate and liver
Can cause dose dependant progressive fibrosis of liver
188
Treatment for methotrexate liver fibrosis
Stop methotrexate
189
What is cardiac cirrhosis
Hepatic derangement that occur due to setting of right-sided heart failure. Signs and symptoms of right sided heart failure dominate
190
Deficiency of this affect lung and the liver
Alpha-1-anti-trypsin deficiency
191
What organs contribute to portal vein inflow
Spleen, distal end oesophagus, stomach, pancreas, small and large intestine
192
Hepatic blood flow direction
Liver sinusoids - Central vein - Hepatic vein - Inferior vena cava - Right atrium of heart
193
What is considered portal hypertension
Portal vein pressure above 5 - 8 mm Hg
194
2 factors that can cause portal hypertension
Increase resistance to portal flow
| Increase portal venous inflow
195
Prehepatic cause of portal hypertension
Blockage of portal vein before liver due to thromboembolus (Budd-Chiari syndrome or occlusion secondary to congenital portal venous abnormalities)
196
Intrahepatic causes of portal hypertension
Presinusoidal - Schistosomiasis or non-cirrhotic portal hypertension
Postsinusoidal - Cirrhosis, alcoholic hepatitis, congenital hepatic fibrosis
197
Compensated vs decompensated liver cirrhossi
Compensated is clinically normal and found incidentally on imaging. Portal hypertension may be present
Decompensated cirrhosis is deterioration in liver function in patient with cirrhosis usually leading to development of jaundice, ascites, variceal haemorrhage, hepatic enchephalopathy or infections
198
Most common liver cause of ascites
Decrease in albumin synthesis decreasing oncotic pressure
199
Complications of cirrhosis
Jaundice, encephalopathy, variceal bleeding, liver failure
200
Treatment of cirrhosis
Feed early and quickly as patietns tend to undergo catabolism
Identify and treat infections
Remove or treat cause
201
What vitamin supplementation is mandatory in alcohol excess cirrhosis
Vitamin B1 - Thiamine
202
Why should Calcium be administered in cirrhosis
Osteoporosis (fragile bone) and osteomalacia (soft bones). 1000mg of Ca supplement and 20 microg of D
203
Cirrhosis due to PBC and PSC can cause deficiency of
Fat soluble vitamines - A, D, E, K
204
How can ascites be diagnosed
Ultrasound - Dark
| Shifting dullness - Percuss on GI examination
205
Treatment of ascites
```
Improve liver disease
Look for and treat infection
No NSAID
Reduce salt intake
Diuretics - Spironolactone
Paracentesis
TIPS
Transplantation
```
206
Spironolactone vs Amiloride in ascites treatment
Spironolactone (aldosterone antagonist) is more effective than Amiloride (K-sparring loop diuretic)
207
What should be frequently monitored after dose change or paracentesis
U&E
208
Infection of ascitic fluid
Spontaneous bacterial peritonitis (SBP)
209
Investigating spontaneous bacterial peritonitis
Tap in ascites and neutrophil count (>250 cells/mm3)
210
Treatment of spontaneous bacterial peritonitis
Antibiotics
Terlipressin for vascular instability
Maintain renal perfusion
Hepatorenal syndrome development
211
Alcohol withdrawal vs encephalopathy flap
Alcohol withdrawal gives a tremor whereas encephalopathy gives a liver flap
212
Pathophysiology of hepatic encephalopathy
Ammonia is generated in the intestines from diet. This ammonia is metabolised to urea in the liver and excreted out by the kidney in urine.
Portal systemic shunts and liver failure cause a rise in blood ammonia as this ammonia is taken into systemic circulation directly rather than passing through the liver. This affects brain function via astrocytes and disturbs neurotransmitter trafficking.
213
Should protein and energy intake be reduced in encephalopathy
No as this may cause more harm by adversely affecting the nutritional status
214
Treatment of encephalopathy
Look for cause such as infection, drugs, liver failure or metabolic and treat
Lactulose to reduce transit time - Rifaximin
Small frequent meals and carbohydrate containing late evening snack to reduce fasting period overnight and reduce catabolism
Transplant
215
Pathophysiology of variceal bleeding
Increased portal pressure
Increased variceal size
Decreased vessel wall thickness
Increased variceal wall tension
216
Primary prophylaxis for variceal bleeding
Non-selective beta-blockers;
Propanolol, Carvedilol
Variceal ligation
217
Treatment for acute variceal bleeding
```
Resuscitation
Pharmacological therapy
Timing of therapy
TIPS
Transection/shunt surgery
```
218
Possible endoscopic therapies for variceal bleeds
Sclerotherapy or variceal ligation
| Variceal ligation preferred due to lower morbidity and quicker eradication
219
What is sclerotherapy
Procedure to treat blood vessels or blood vessel malformations by injecting a medicine into vessels to make them shrink
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What can be done to 'buy time' for a TIPS or transfusion
Balloon tamponade
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Drawback of TIPSS
Can cause encephalopathy due to formation of porto-systemic shunt. This can lead to ammonia entering the systemic circulation directly, bypassing the liver for conversion to urea and safe excretion
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What can be used to prioritise liver transplantation
United Kingdom Model for End-Stage Liver Disease (UKELD) predicts prognosis in chronic liver disease and is used to prioritise transplants
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Threshold to be eligible for liver transplant
49 or higher on UKELD score unless hepatocellular carcinoma
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Variant syndromes
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Diuretic resistant ascites
Hepatopulmonary ascites
Chronic hepatic encephalopathy
Intractable pruritus
Polycycstic liver disease
Familial amyloidosis
Primary hyperlipidaemia
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The first sign of jaundice is seen in which organ
The eyes
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Clinically apparent hyperbilirubinemia
Jaundice
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Duration of liver damage to be classified as chronic
> 6 months
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True liver function tests consists of
Bilirubin, albumin and prothrombin. These mark liver function
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What are markers of liver damage
Alkaline phosphatase (ALP), Alanine amino transferase (ALT) and Gamma Glutamly Transferase (GGT)
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Markers of cholestatic problems
Alkaline phosphatase (ALP) and Gamma GT
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Markers of hepatocyte damage
Alanine amino transferase (ALT)
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Not dilated bile duct common cause
Drug induced, common cause if Flucloxacillin
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What can cause abnormal liver function tests
Obesity, diabetes, alcohol, drugs
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Degree of liver dysfunction can be classified via
Child-Pugh classification
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What does high portal pressure and low albumin lead to
Ascites
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Shunting of portal brain affects the brain. Why?
Toxins bypass the liver and enter straigth into the systemic circulation.
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Albumin and plasma volume
Low albumin leads to low plasma volume as oncontic pressure is lowered
