Hepatology I Flashcards
DM
Prednisolone
Autosomal recessive
The severity of cirrhosis can be graded using the []
The severity of cirrhosis can be graded using the Child-Pugh score.
What are the arrows pointing to? [1]
What pathology does this indicate? [1]
Mallory bodies are highly eosinophilic and thus appear pink on H&E stain. The bodies themselves are made up of intermediate keratin filament proteins that have been UBIQUINATED, or bound by other proteins such as heat shock protein
Found in ALCOHOLIC HEPATITIS
What are signs & symptoms of alcoholism associated with:
- CNS [6]
CNS:
- Reduced memory
- cortical atropy
- fits & falls
- wide based gait (cerebellar dysfunction)
- Korsakoffs (memory disorder that results from vitamin B1 deficiency and is associated with alcoholism)
- W.Enceph.
What are signs & symptoms of alcoholism associated with the blood [3]
- Increased MCV: non-megaloblastic macrocytic anaemia
- anaemia from marrow depression
- Folate deficiency: decreased intake; inhibits folate absorption
What are signs & symptoms of alcoholism associated with reproduction [4]
- testicular atrophy
- decreased testosterone and progesterone
- increased oestrogen
- fetal alcohol withdrawal
Which score is used to calculate risk of alcohol dependence? [1]
AUDIT
Describe the treatment plan for alcohol withdrawal [2]
Benzodiazapenes:
- Chlordizepoxide: give for 1st 3D 10-50mg/6hrs, then plan on weaning
- Lorazepan (for patients with underlying cirrhosis)
Describe the different symptoms for alcohol withdrawal symptoms for the following time periods:
6-12 hours: [1]
12-24 hours [1]
24-48 hours: [1]
24-72 hours: [1]
6-12 hours: tremor, sweating, headache, craving and anxiety
12-24 hours: hallucinations
24-48 hours: seizures
24-72 hours: delirium tremens
TOM TIP: Both the NFS and FIB-4 scores use the AST:ALT ratio to assess the severity of liver fibrosis.
The normal ratio is []/
A ratio [] in NAFLD suggests advanced fibrosis
. An AST:ALT ratio [] indicates alcohol-related liver disease rather than NAFLD.
TOM TIP: Both the NFS and FIB-4 scores use the AST:ALT ratio to assess the severity of liver fibrosis.
The normal ratio is less than 1.
A ratio greater than 0.8 in NAFLD suggests advanced fibrosis.
An AST:ALT ratio greater than 1.5 (meaning a disproportionately high AST) indicates alcohol-related liver disease rather than NAFLD.
Name 3 drugs that are associated with increasing risk factor for NAFLD [3]
Name a surgery that is associated associated as a risk factor for NAFLD [1]
Describe nutrition that is associated with increasing risk factor for NAFLD [2]
Drugs:
- Steroids
- Amiodarone
- Methotrexate
Weight reducing surgery:
- jejuno-ileal by pass
Nutrition
* Protein calorie malnutrition & TPN
Describe the treatment algorithm for NAFLD [6]
- Weight loss: diet & exercise
- Consider vitamin E
- Consider pioglitazone (reduces peripheral insulin resistance)
- Consider weight loss medication: orlistat
- Consider bariatric surgery if BMI > 35 and one other obesity co-morbid / BMI > 40.
- Liver transplant
BMJ BP
Diagnosis of NAFLD? [3]
The diagnosis requires the presence of
- ultrasound findings of a fatty liver
- risk factors
- excluding other causes of liver disease with a careful alcohol history and full non-invasive liver screen.
- Liver biopsy is the gold standard test.
Describe the natural history of Hep B (HBV) [3]
- Majority (65%) exposed to acute HBV infection have asymptomatic recovery
- 25% have acute hepatitis but recover
-
10% have chronic infection (HBV antigens >6months)
i) 90% these: asymptomatic
ii) 10% lead to chronic hepatitis; cirrhosis; HCC
Which test for HBV is present in serum 1-3 months after acute illness and infers high infectivity? [1]
HbeAg
How would you infer that a patient has had a vaccination and no infection? [1]
solely having antibodies to HBsAG: HBsAB
Surface AB: Safe
Explain how E antigen (HBeAg) is used in understanding the level of virus in patient? [4]
E antigen (HBeAg): marker of viral replication and implies high infectivity:
Where the HBeAg is present it implies the patient is in an acute phase of the infection where the virus is actively replicating.
The level of HBeAg correlates with their infectivity
If the HBeAg is higher, they are highly infectious to others
Describe the treatment for HBV [3]
Nucleoside analogues:
- Entecavir
- Tenofovir
AND
PEG-IFN (peginterferon alfa 2a)
If cirrhosis - just E & T
Describe symptoms of acute HBV infection [6]
Asymptomatic (majority)
Jaundiced
RUQ pain
N&V
Arthralgia
urticaria (hives)
How do you investigate for HCV? [5]
Test for:
- HCV antibody IgG
- HCV RNA (6 genotypes; can change treatment strategies)
- LFTs
- HCC screen (given higher risk): USS and AFP
- Liver biopsy (rare) or Fibroscan to investigate stage of fibrosis}
How do you confirm an ongoing HCV infection? [1]
What would be the next mangament step if found to be positive [1]
HCV PCR [1]
Procede to have biospy to see extent of damage
Describe HCV treatment:
- Length? [1]
- Therapies? [3]
8-12 weeks
Therapies:
- ARVs: aim sustained virological response (SVR; undetectable serum HCV RNA six months after the end of therapy)
- Combination dependent on genotype and stage of fibrosis
- currently a combination of protease inhibitors (e.g. daclatasvir + sofosbuvir or sofosbuvir + simeprevir) with or without ribavirin are used
Which gene is associated with haemochromatosis? [1]
Describe the inheritance pattern [1]
How does this gene mutation cause the pathogenesis of the disease? [5]
- human haemochromatosis protein (HFE) gene
- autosomal recessive
- HFE mutation causes decreased hepcidin activity (regulates iron stores); increased duodenal/jejunal iron absorption AND release of iron from bone marrow macrophages → iron deposition in cells → Fenton reaction and hydroxyl free radicals → DNA, lipid and protein damage → organ dysfunction
Fenton reaction:
Iron freely undergoes oxidation in cells from Fe2+ to Fe3+ as part of the Fenton reaction
In this process, hydroxyl free radicals are generated which then cause oxidative damage
Tx for haemochromatosis? [2]
Venesection (regularly removing blood to remove excess iron – initially weekly)
Iron chelation therapy (iron chelator) works by binding to the extra iron so that it can be removed from the body): Oral: Deferasirox and parenteral agents Desferrioxamine.
What presentation of haemochromatosis may be highlighted via XR imaging? [1]
Chondrocalcinosis: causes stressed joints to deteriorate faster than resting joints
How is Wilson’s disease diagnosed? [4]
- reduced serum caeruloplasmin
- reduced serum copper(counter-intuitive, but 95% of plasma copper is carried by ceruloplasmin)
- Liver bx
- MRI brain
- increased 24hr urinary copper excretion
- haemolytic anaemia
Tx of Wilson’s diease? [3]
Copper chelation using either:
* Penicillamine
* Trientine
Zinc salts (inhibit copper absorption in the gastrointestinal tract)
Liver transplantation
If have high risk of HCC (have liver cirrhosis), what screening is undertaken to ID risk? [2]
- USS: 6 months
- test for AFP - tumour marker
The MELD Score is used to measure severity of? [1]
Cirrhosis
The Model of End-Stage Liver Disease (MELD)
Score measures the severity of cirrhosis based on five parameters: serum bilirubin, INR, sodium, creatinine and need for dialysis.
What is the first line treatment for hepatic encephalopathy? [1]
What is the secondory prophylaxis of hepatic encephalopathy? [1]
NICE recommend lactulose first-line
rifaximin for the secondary prophylaxis of hepatic encephalopathy
[] is the first line treatment for hereditary haemochromatosis.
[] may be used second-line
Venesection is the first line treatment for hereditary haemochromatosis.
Desferrioxamine may be used second-line (medicine that removes excess iron that builds up after having regular blood transfusions)
What is pneumonic for remembering the factors that influence Child-Pugh score? [5]
ABCDE
A - albumin
B - bilirubin
C - clotting
D - distention (ascites)
E - encephalopathy
What is the triad of hepatorenal syndrome? [3]
Cirrhosis
Ascites
AKI not attributable to any other cause.
How do you treat type 1 HRS? [3]
terlipressin, have a growing evidence base supporting their use. They work by causing vasoconstriction of the splanchnic circulation
volume expansion with 20% albumin
transjugular intrahepatic portosystemic shunt
(still have a v poor prognosis)
What is Budd-Chiari syndrome? [1]
What is Budd-Chiari syndrome associated with? [2]
Budd–Chiari syndrome of hepatic vein thrombosis
Associated with pregnancy and being postpartum
Describe the extra-hepatic symptoms of Wilsons disease [5]
Blue nails
Kayser fleischer ring
Haemolytic anaemia
Cardiomyopathy
Neurological:
- Cognitive decline
- Lack of co-ordination leading to PD like symptoms
- Dementia
- Pyschosis
-
What are the major clinical manifestations of haemochromatosis? [5]
Cirrhosis of the liver
Diabetes mellitus
Arthritis
Cardiomyopathy
Hypogonadotropic hypogonadism
Describe the impact of haemochromatosis on reproductive system [3]
Amenorrhoea
Erectile dysfunction
Testicular atrophy
Describe the presentation of Budd-Chiari syndrome [3]
abdominal pain: sudden onset, severe
ascites → abdominal distension
tender hepatomegaly
Name an iatrogenic risk factor for Budd-Chiaria syndrome [1]
The oral contraceptive pill
What would the HBV serology for a vaccine responder look like? [1]
Anti-HBsAg +ve only
What would the HBV serology for someone suffering from an acute infection look like? [2]
HBsAg +ve
Anti-HBcAg IgM +ve
What would the HBV serology for someone suffering from a chronic infection look like? [2]
HBsAg +ve
Anti-HBcAg IgG +ve
