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Liver diseases Flashcards

1
Q

Types of jaundice?

A

Pre- hepatic
Hepatocellular/ hepatic
Post hepatic

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2
Q

Pre- hepatic?

A

Excess red cell breakdown overwhelms liver

More unconjugated

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3
Q

Hepatocellular/ hepatic jaundice?

A

Liver cells injured/ dead
Liver loses conjugating ability
Can be mixed conjugated and unconjugated

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4
Q

Post- hepatic jaundice?

A

Obstruction biliary drainage
Bile cannot escape into bowel
Still conjugated in the liver

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5
Q

Normal urine + normal stool but jaundice?

A

Pre- hepatic cause

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6
Q

Dark urine + pale stools?

A

Post hepatic cause

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7
Q

Gilbert’s syndrome?

A

Mild disorder of bilirubin processing in liver
Mutation decreases activity of liver enzyme that processes bilirubin
Increased unconjugated bilirubin in blood with normal LFT’s

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8
Q

Haemolytic anaemia?

A

Abnormal breakdown of RBC’s
Fatigue, SOB
Jaundice

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9
Q

Acute hepatocellular jaundice?

A

Posioning e.g. paracetamol
Infection e.g. Hep A, B
Liver ischaemia

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10
Q

Chronic hepatocellular jaundice?

A 
AFLD
NASH
Cirrhosis
Chronic infection (Hep B, C)
PBC
Pregnancy
AI hep
PSC< haemochromatosis, Wilsons
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11
Q

Obstructive jaundice?

A

Gallstones
Strictures
Tumours
Congenital biliary atresia

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12
Q

How does liver injury occur?

A

Hepatic stellate cells in space of Disse usually in quiescent state are activated by injury and cause fibrosis

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13
Q

NAFLD?

A

Non alcoholic fatty liver disease

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14
Q

NAFL ?

A

Steatosis - no inflammation or fibrosis

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15
Q

NASH?

A

Non- alcoholic steatohepatitis

- Steatosis & inflammation and scarring

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16
Q

Pathway from steatosis to cirrhosis?

A

Steatosis –> Steatohepatitis –> steatohepatitis with fibrosis –> cirrhosis

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17
Q

2 hit pathogenesis of liver disease?

A

1st = excess lipid accumulation in liver
2nd = Oxidative stress and lipid peroxidation
Pro- inflammatory cytokine release, lipopolysaccharide, ischaemia

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18
Q

NAFLD?

A 
4 stages:
1 - Simple fatty liver (steatosis) (most common)
2 - NASH
3 - Fibrosis
4 - Cirrhosis
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19
Q

Clinical features/ history of NAFLD?

A 
Asymptomatic
Fatigue
RUQ pain
Alcohol, drugs, sexual activity
Obesity 
Slightly deranged LFT's 
Diabetes
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20
Q

Diagnosis of NAFLD?

A

Usually incidentally
Suspect if abnormal USS or LFT derangement > 3 months
Usually diagnosed by ultrasound
Biopsy for staging
Signs of advanced liver disease? - jaundice, ascites, spiders

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21
Q

Ultrasound finding for NAFLD?

A

Steatosis in absence of injurious causes e.g. alcohol

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22
Q

Risk factors for NAFLD?

A

Obesity
Hypertension
Type 2 diabetes
Hyperlipidaemia

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23
Q

Treatment of NAFLD?

A

Weight loss, exercise
Nutrition
Vitamin E
Liver transplantation

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24
Q

NASH diagnosis?

A

Liver biopsy:

  • Mallory bodies
  • Ballooning
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25
Risks of NASH?
Can progress to cirrhosis
26
Treatment of NASH?
Weight loss, exercise
27
Alcoholic liver disease?
Result of chronic, heavy alcohol consumption | Inc release of fatty acids and TAGs in hepatocytes
28
What is responsible for damage in ALD?
Acetaldehyde (ethanal)
29
Microscopic appearance of fatty liver?
Fat vacuoles in hepatocyes
30
Microscopic appearance of alcoholic hepatitis?
Hepatocytes necrosis Neutrophils Mallory bodies Fibrosis
31
Microscopic appearance of alcoholic fibrosis?
Collagen laid down around cells
32
Microscopic appearance of alcoholic cirrhosis?
Micro- nodular | Bands of fibrosis separating regenerating nodules
33
Alcoholic fatty liver disease history/ clinical features?
``` Fatigue RUQ pain Alcohol, drugs, sexual activity Obesity Clear history if excess alcohol ```
34
Diagnosis of AFLD?
May be asymptomatic LFTs Liver screen - rule out other causes USS/ fibro scan, MRI, CT
35
Treatment of AFLD?
NO ALCOHOL Corticosteroids may be used in acute inflammation Benzodiazepenes (acute withdrawal) Transplant (6-12 months abstinence)
36
Complications of AFLD?
``` Many e.g. Acute liver failure Cirrhosis Hepatocellular carcinoma Oesophageal varices ```
37
Cirrhosis?
Final common end point of liver disease Irreversible Bands of fibrosis separating regenerative nodules of hepatocytes
38
Common causes of cirrhosis?
``` Alcohol NAFLD Hep C PBC AI Hepatitis Hep B ```
39
Chronic liver disease?
>6 months | Can lead to cirrhosis
40
Signs and symptoms of cirrhosis? - Liver dysfunction
``` Spider nevi Palmar erythema Gynecomastia Ascites Shrunk, large liver Jaundice Itching Acute kidney injury Abnormal bruising Hypogonadism Encephalopathy etc ```
41
Signs and symptoms of cirrhosis? - Portal hypertension
Splenomegaly Oesophageal varices Caput medusa Anorectal varices
42
Cirrhosis history?
- Chronic alcohol abuse - NAFLD - Chronic infection - AI, inherited disorders
43
Cirrhosis diagnosis?
Liver biopsy - regenerating nodules of hepatocytes, fibrosis/ connective tissue between nodules Liver screen LFTs USS (elastography)
44
How does fibrosis lead to portal hypertension?
Fibrosis leads to destruction of other structures within liver (sinusoids, space of Disse, vascular structures) --> leads to inc resistance and inc portal pressure
45
Compensated cirrhosis?
Asymptomatic stage LFTs show damage Some back pressure may be visible Palmar erythema, clubbing, gynecomastia, hepato/ splenomegaly
46
Decompensated cirrhosis?
``` Symptomatic - Ascites Jaundice Variceal haemorrhage Easy bruising Hepatic encephalopathy ```
47
Grading cirrhosis?
CHILD PUGH score | MELD
48
Cirrhosis management?
Cannot be reversed - stop progression and complications | Healthy diet, no alcohol
49
Symptom management cirrhosis - ascites?
Diuretics - spironolactone 1st line, furosemide
50
Symptom management cirrhosis - itch?
Bile salt resins e.g. cholestyramine
51
Symptom management cirrhosis ?
Caution with meds e.g. paracetamol Treat cause to prevent progression Transplantation
52
Liver transplant?
``` Event based (ascites, varies) Liver funciton based UKELD score (49 = min) ```
53
Portal hypertension?
Portal hepatic pressure gradient >5 Inc in hydrostatic pressure within portal vein or tributaries Results from inc resistance to portal flow and inc portal Venous inflow
54
What is portal vein made from?
Superior mesenteric vein Splenic vein Gastric vein Part from inferior mesenteric veins
55
Portal blood supply?
Carries deoxygenated blood from gut to liver | Drains blood from organs, back through liver for processing
56
Hepatic blood flow?
Twin/ dual blood supply O2 blood from hepatic artery (circulation) Nutrient rich, deox blood from hepatic portal vein
57
Pathway of hepatic blood flow?
O2 blood from hepatic artery and deox, nutrient blood from hepatic portal vein --> liver sinusoids --> central vein --> IVC --> RA of heart
58
What happens to blood supply in portal hypertension?
Anastomosis in portal venous system (w/ systemic blood supply) become enlarged, dilated, varicosed & rupture
59
Effect on blood pathway in portal hypertension?
Inc pressure in portal vein --> inc pressure in portal circulation --> inc pressure in systemic circulation --> blood flows at inc volume down anastomosis --> dilate --> rupture
60
Pre- hepatic causes of portal hypertension?
Blockage of portal vein before the liver | Due to - portal vein thrombosis, occlusion secondary to congenital portal vein abnormalities
61
Intra- hepatic causes of portal hypertension?
Due to distortion of liver architecture - Pre- sinusoidal (schistomasis) - Post sinusoidal (cirrhosis) - Budd chair syndrome, veno- occlusive disease
62
Budd chairi syndrome?
Occlusion of hepatic veins which drain the liver
63
Budd chairi syndrome clinical features?
``` Assoc. w/ pregnancy Sudden RUQ pain Rapid ascites Hepatomegaly Jaundice Acute kidney injury +/- fulminant liver failure ```
64
Diagnosis of Budd Chairi?
``` LFTs - mild elevation Urea and creatine - renal impairment? Coagulation profile USS, CT, MRI venography ```
65
Definitive management of Budd Chairi?
Liver transplant
66
Veno- occlusive disease?
Small veins in liver are obstructed
67
Clinical presentation of Veno- occlusive disease?
RUQ pain Painful hepatomegaly Ascites Abnormal LFT's
68
Diagnosis of Veno- occlusive disease?
Anyone who has undergone HCT then develops liver dysfunction | Clinical ground - Modified Seattle Criteria
69
Pharmacological treatment of BCS and VOD?
Correct underlying disorder Anticoagulation - enoxaparin and SC warfarin Thrombophilia Symptoms control - furosemide
70
Surgical treatment of BCS and VOD?
VOD - prevention, pharmacologic prophylaxis w/ ursodeoxycholic acid or low dose heparin Supportive care - Radiologic intervention (UVC balloon, hepatic angioplasty, TIPS),surgical shunting, liver transplant
71
Acites?
- Pathological accumulation of fluid in peritoneal cavity
72
How do ascites occur?
Cirrhosis causes portosystemic shunting --> inc hydrostatic pressure in splanchnic vessels --> release of vasodilators (NO mediated) --> vasodilation causes dec blood volume (baroreceptors) --> RAAS, sympathetic system and ADH --> sodium and water retention
73
Spontaneous bacterial peritonitis?
Neutrophil >250 cells/mm3 Bacterial infection in peritoneum, no obvious source Diagnostic paracentesis tap promptly as acute management & check cell count E. coli - most common cause Broad spectrum antibiotic
74
Diagnosis of ascites?
Shifting dullness USS Diagnostic tap for pts with undetermined cause of ascites
75
Treatment for ascites?
``` SPIRONOLACTONE 1st line Treat underlying disease Infection? Furosemide No NSAIDS Paracentesis TIPPS if long term recurrent Transplant ```
76
Hepatorenal syndrome?
Result of cirrhosis/ portal hypertension Kidney failure in those with severe liver damage Look for altered liver function, abnormalities in circulation and kidney failure Treat - transplant, TIPPS
77
Mechanism for hepatorenal syndrome?
Portal hypertension --> splanchnic vasodilation --> decreased effective circulatory volume --> activation of RAAS --> renal vasoconstriction --> hepatorenal syndrome
78
Hepatic encephalopathy?
Decline in brain function due to severe liver disease | Liver failure causes hyperammonaemia - toxic to CNS
79
Symptoms of Hepatic encephalopathy?
``` LIVER FLAP Confusion Non- coordination, shaking Drowsiness, coma Slurred speech Seizures Cerebral oedema ```
80
Treatment of Hepatic encephalopathy?
Lactulose - decreases colonic pH, prevents absorption of NH3, NH3 can be converted to NH4 and excreted Antibiotics - neomycin, rifaxamin (suppress colonic flora, inhibits ammonia)
81
Causes of Hepatic encephalopathy?
Drugs, infection renal, GI bleed, electrolyte disturbances
82
Acute liver failure?
Any insult to liver causing damage in previously normal liver < 6 months duration Causing encephalopathy & impaired protein synthesis
83
Clinical features of acute liver failure?
``` None Jaundie Lethargy, arthralgia N&V, anorexia RUQ pain Itch ```
84
Diagnosis of acute liver failure?
- Physical exam (jaundice, ascites). - History - Infections, alcohol, pregnancy. - Mental changes, coagulopathy. - Abnormal LFTs.
85
Investigations for acute liver failure?
``` Alcohol? Drugs, paracetamol? Possible toxins LFTs Virology Investigations for chronic liver disease ```
86
Treatment of acute liver failure?
``` Rest - 3-6 months recovery Fluids No alcohol Inc calorie intake Regular observation Monitor and supplement ```
87
How can NSAIDs damage the liver?
Decrease renal PGE synthesis Worsens renal impairment by increasing renal vasoconstriction and sodium retention Increase risk of hepatorenal syndrome
88
How can diuretics damage the liver?
o Furosemide – Decreases intravascular volume – hypokalaemia, hepatorenal syndrome. o Thiazide – Same as furosemide. o Spironolactone. Combats secondary aldosteronism.
89
Oesophageal varices?
Abnormally swollen veins in oesophagus Formed when blood flow in liver is compromised Band ligation during acute bleeding BB for prophylaxis
90
Hepatitis A?
Faecal oral spread Most common viral hep worldwide Poor hygiene, overcrowding No chronic state, only acute
91
Clusters in which Hep A is prevalent?
Gay men | PWID
92
Clinical features of hepatitis A?
Acute hepatitis Older children, young adults Unwell with no specific symptoms - nauseam anorexia, headache, myalgia arthralgia
93
Jaundice in Hep A?
Pts may recover and not become jaundice (lifetime immunity/ aninteric infection) After 1-2 weeks some become jaundice (most infectious just before jaundice)
94
Lab marker on Hep A?
Acute infection - Hep A IgM (HAV IgM) (acute infection) IgG HAV - present in those who have been vaccinated LFTs - serum bilirubin reflects jaundice, serum AST and ALT rise before onset of jaundice
95
Management?
Stop alcohol consumption Supportive treatment Vaccine Hygiene
96
Hep E?
``` Similar to HEP A Tropical countries Faecal oral - Pigs - Contaminated water Acute infection will only progress to chronic in some immunocompromised ```
97
Clinical presentation of Hep E?
``` Almost identical to Hep A Nausea and anorexia Jaundice Vomiting Altered mental state Enlarged liver, splenomegaly ```
98
Investigations for Hep E?
Hep E Viral RNA (HEV RNA) - detected by PCR in stool or serum
99
Management of HEP E?
Stop alcohol | Supportive treatment
100
Hep B transmission ?
3 B's Bone (sex) Blood Baby (mother to child)
101
When is chronic Hep B infection more likely to result?
If first exposure is in childhood
102
Clinical features of Hep B?
``` May be asymptomatic V similar to Hep A Illness more severe Itchy rashes Arthritis Fever Diarrhoea, abdo pain ```
103
Hep B surface antigen (HBsAg)
In all infectious individuals | - If > 6 months = chronic
104
Hep B e antigen (HBeAg)?
Present in highly infectious individuals Also indicates acute infection Continues infectious state Allows for replication of virus in blood
105
Hep B virus DNA (HBV DNA)?
Present in highly infectious individuals | Also used to measure response to anti-viral therapy
106
Hep B IgM?
Recently infected cases
107
Anti Hbs?
Present in immunity (vaccine, previous infection)
108
Hep B c IgM titre levels?
High titre - acute/ recent infection | Low titre - Chronic infection
109
Hep B IgG?
Past exposure to Hep B (vaccine) | HBsAg will be negative
110
Anti HBs?
Hep B surface antibody (HbsAb) - recovery/ immunity to HBV | Successful vaccination
111
Best indication of Hep B prognosis?
HBV DNA
112
Control of Hep B?
Minimise exposure Pre- exposure vaccines (children, older children and adults) Post exposure prophylaxis - vaccine and plus HBIG
113
Prognosis?
Self resolving most of the time Full recovery Or chronic infection
114
Management of HBV?
If symptomatic Constant HBV marker monitoring Antivirals (only suppress, don't cure) Antecavir, tenofavir, in v ill
115
Hep D?
Only found with HBV Unable to replicate on it own - activated by HBV Parasite of a parasite Exacerbates HBV infection
116
Hep C?
Chronic infections more common | Transmission - blood, sex and mother to child (BBB)
117
Clinical features of Hep C?
Acute - asymptomatic, mild flu- like, jaundice, raised amino transferases Chronic - more common
118
Investigations for HCV?
Initially test for antibody (anti- HCV) If positive - either past infection or active Test for HCV RNA by PCR - determines if active or past
119
HCV RNA?
Tests usually positive 1-8 weeks after infection Current active infection Dec --> recovering Levels stay same --> chronic?
120
Management of Hep C?
Continually monitor HCV- RNA If viral load falls - treatment may not be req Spontaneous clearing If not - anti- viral therapy during acute phase
121
General treatment of acute Hepatitis infection?
``` If symptomatic No antivirals Monitor for encephalopathy Monitor for resolution Notify public health immunisation of contacts ```
122
Chronic hepatitis?
Caused by HBV and HCV
123
When to treat chronic HBV infection?
Raised ALT and high HBV DNA
124
When to treat chronic HCV infection?
Always straight away | Patients with advanced fibrosis and cirrhosis first
125
Therapy for chronic HBV?
Suppressive anti viral drug - E.g. entecavir, tenofavir Peginterferon alone
126
Chronic HVC therapy?
Aim for undetectable HCV RNA 12 weeks after treatment completion Direct antivirals - sofosbuvir, ledipasvir Reg screen for hepatocellular carcinoma No alcohol
127
Autoimmune hepatits?
Females > males T cells directed against hepatocyte surface antigens Type 1 & 2
128
Type 1 Ai hepatitis?
Age 10-20, 45-70 | Teen girls, young women
129
Type 2 AI hepatitis?
Young kids/ adults
130
Young female with deranged LFT's taking oral contraceptive?
Think AI hepatitis
131
Clinical features of AI hepatitis?
Hepatomegaly Jaundice Signs of chronic liver disease May present similarly to acute onset hepatitis
132
Investigations for AI hepatitis?
Antibodies Raised LFTs? Raised IgG Liver biopsy to confirm severity
133
Antibodies for type 1 AI hepatitis?
ASMA and ANA positive
134
Antibodies for type 2 AI hepatitis?
LKM positive | ASMA and ANA negative
135
Management of AI hepatitis?
Corticosteroids and azathioprine | Eventual liver transplant
136
Benign focal lesions of the liver?
Haemangioma Focal nodular hyperplasia Adenoma Liver cysts
137
Malignant lesions of liver?
Primary liver cancers (hepatocellular carcinoma, cholangiocarcinoma) Metastases
138
Haemangioma?
``` Most common liver tumour Female > male Hypervascular tumour Well demarcated capsule Asymptomatic ```
139
Diagnosis of haemangioma?
- USS: echogenic spot, well demarcated - CT: venous enhancement from periphery to center - MRI: high intensity area
140
Treatment for haemangioma?
No treatment
141
Focal nodular hyperplasia ?
Benign Central scar containing large artery, radiating branches to periphery Middle aged women ASympto, some pain
142
Diagnosis of FNH?
- US: Nodule with varying echogenicity - CT: Hypervascular mass with central scar - MRI: Iso or hypo intense - FNA: Normal hepatocytes and Kupffer cells with central core
143
Treatment of FNH?
None
144
Features of FNH?
``` Hypervascular Contains all liver ultrastructure (RES and bile ductules) Pain Central scar No malignant risk ```
145
Hepatic adenoma?
Benign neoplasm composed of normal hepatocytes no portal tract, central veins or bile ducts Right lobe >> Females Contraceptive hormones, anabolic steroids
146
Symptoms of hepatic adenoma?
Incidental finding Asymptomatic RUQ pain, bleeding
147
Diagnosis of hepatic adenoma?
- US: Filling defect - CT: Diffuse arterial enhancement - MRI: Hypo or hyper intense lesion - FNA: May be needed
148
Treatment of hepatic adenoma?
Stop taking oral contraceptive, hormones, steroids Weight loss Males = surgical excision (as malignant transformation risk higher) Females = (<5cm or reducing, annual MRI) (>5cm, surgical excision)
149
Cystic lesions in liver?
``` Simple Hyatid Atypical Polycystic Pyogenic or amoebic abscess ```
150
Simple cyst?
Liquid collection lined by epithelium
151
Symptoms of simple cyst?
Most asymptomatic but may include: - RUQ pain - Fever
152
Investigations for simple cyst?
USS - 1st line | Follow up imaging in 3-6 months if in doubt
153
Treatment of simple cyst?
None | If symptomatic/ uncertain diagnosis - surgical intervention may be req
154
Hyatid cyst?
Caused by tapeworm echinococcus granulosus Live stock Farming communities Cysts can erode into adjacent structures
155
Clinical features of hyatid cysts?
Often asymptomatic | May cause dull ache and swelling in right hypochondrium
156
Investigations for heated cysts?
Serology (check for anti- echinococcus antibodies) AXR - cyst calcification US/ CT - cysts and daughter cysts = diagnostic sign
157
Management of hyatid cysts?
``` Surgery - Conservative (open cystectomy, marsupialization) - Radical (peri-cystectomy, lobectomy) Medical - Albendazole anti worm meds Percutaneous drainage (PAIR) ```
158
Polycystic liver disease?
Numerous cysts throughout liver parenchyma | 3 types - VMC, polycystic liver disease, autosomal dominant polycystic kidney disease
159
Von Meyenburg complexes (VMC)?
Benign cystic nodules throughout the liver | Incidental finding
160
Polycystic liver disease (PCLD)?
o Liver function preserved and kidneys functioning normally o Symptoms depend on size of cysts – may cause RUQ pain and distension o PCLD gene – PRKSCH and SEC63
161
Autosomal dominant polycystic kidney disease
o Renal failure due to polycystic kidneys and non- renal extra- hepatic features common Massive hepatic enlargement o Marked reduction in kidney function but liver function should be preserved o ADPKD genes – PDK1 and PDK2
162
Clinical features of polycystic liver disease?
- Abdominal pain - Abdominal distension - Atypical symptoms - Signs of liver failure
163
Investigations of polycystic liver disease?
- Gene studies - Polycystic disease shown clearly on CT - Kidney function tests
164
Management of polycystic liver disease?
``` - Conservative treatment o Halt cyst growth o Allow abdo decompression o Ameliorate symptoms - Surgical procedures if advanced PCLD, ADPKD, liver failure: o Aspiration/ defenestration o Liver transplantation - Pharmacologic therapy: o Somatostatin analogues (sandostatin) - Symptomatic relief - Reduce liver volume ```
165
Liver abscess?
Pus filled mass in the liver | Can be pyogenic (pus forming) or caused by protozoa
166
Clinical features of liver abscess?
- High fever - Leukocytosis - Abdominal pain - Complex liver lesion - Nausea and vomiting - Jaundice: o If so may be pleural rub in right lower chest - Malaise lasting several months
167
History for liver abscess?
- Abdominal or biliary infection | - Dental procedure
168
Investigations for liver abscess?
- Check for leucocytosis - CXR may show a raised right hemi-diaphragm - US – detects an abscess - CT – more useful in complex and multiple lesions - Echocardiogram 
169
Management for liver abscess?
Broad spectrum antibiotics (amoxicillin, metronidazole gentamicin) IV ASpiration/ drainage percutaneously Echocardiogram Operation - open drainage, resection 4 weeks repeat antiB, repeat imaging
170
Primary tumours in liver?
Rare
171
Secondary tumours in liver?
More common than primary
172
Hepatocellular carcinoma?
Most common primary cancer Men>> Malignant
173
Risk factors for hepatocellular carcinoma?
Cirrhosis Carriers of HBV, HCV Male
174
Clinical features of hepatocellular carcinoma?
- Weight loss and RUQ pain (most common) - Malaise, fever, anorexia - Asymptomatic - Worsening of pre- existing chronic liver disease - Acute liver failure - Known liver cirrhosis + rapid symptoms
175
Examination for hepatocellular carcinoma?
- Signs of cirrhosis - Hard enlarged RUQ mass - Liver bruit (rare)
176
Investigations for hepatocellular carcinoma?
Serum alpha- fetoprotein (AFP) - may be raised (HCC tumour marker) USS - filling defects CT - HCC MRI - if smaller than 1cm
177
Treatment of hepatocellular carcinoma?
``` 1 - Resection (if possible) 2 – Liver transplantation 3 – Local ablation if assoc. disease means liver transplant not possible, temp measure 4 – TACE (early cirrhosis) 5 – Systemic therapies - sorafenib ```
178
Fibrolamellar carcinoma?
``` Young Not related to cirrhosis AFP = normal CT - stellate scar with radial septa Surgical resection or transplantation TACE if unresectable ```
179
Secondary liver mets?
Most common site for blood Bourne mets Colon, breast, lung, stomach, pancreas, melanoma Mild cholestatic picture Imaging or FNA
180
Haemochromatosis?
Inherited disease characterised by excess iron deposition in liver Defect in iron absorption and metabolism Autosomal recessive
181
Common inherited gene for Haemochromatosis?
HFE gene on chromosome 6
182
Clinical features of Haemochromatosis?
Early - lethargy, arthralgia Classic triad = bronze skin, hepatomegaly, diabetes mellitus Cardiac arrhythmias, HF Liver fibrosis, failure, cirrhosis
183
Investigations for haemochromatosis?
Serum iron >30 LFT's - normal 1st degree fam - screened
184
Management of haemochromatosis?
Avoid iron rich foods Venesections (2x weekly until adequate iron removed) Desferrioxamine (iron chelation therapy)
185
Wilson's disease?
Autosomal recessive disorder leading to reduced ceruloplasmin (copper transporter) --> copper accumulates in liver and brain
186
Gene defect in Wilson's disease?
ATP78 gene defect on chromosome 13
187
Symptoms of Wilson's disease?
Kayser Fleischer rings Signs of liver disease CNS signs - tremor, involuntary movements (Vomiting, weakness, abdominal fluid, leg swelling, yellowish skin and itchiness)
188
Investigations for Wilson's disease?
LFTs Ceruloplasmin (reduced) Serum copper - reduced
189
Management of Wilson's disease?
Penicillamine
190
Alpha- 1 antitrypsin deficiency?
Autosomal recessive | AAT = mainly produced by liver
191
Signs and symptoms of AAT deficiency?
o Lung – emphysema | o Liver – cirrhosis and HCC
192
Investigations for AAT deficiency?
Alpha-1 antitrypsin levels = low | Spirometry = obstructive
193
Management of AAT deficiency?
Never smoke, drink COPD treatment if lung involved AAT deficiency augmentation therapy Organ transplan
194
Fulminant hepatic failure?
Jaundice and encephalopathy in someone with previously normal liver
195
Causes of FHF?
Drug overdose | Viral
196
Management of paracetamol overdose?
o Acetylcysteine o <8 hours can add activated charcoal o Anti- emetic (odansetron) o Liver transplant
197
Bile?
When chyme enters duodenum --> CCK stimulates gallbladder to contract and release bile Bile is secreted via sphincter of ODDI at D2
198
Types of gallstnes?
Cholesterol (usually) Pigmented black stones (too much bilirubin in bile) Brown stones (parasitic infection)
199
Cholelithiasis ?
Gallstones
200
Cholecystolithiasis ?
gallstone in gallbladder
201
Choledocholithiasis ?
Gallstones in bile duct
202
Uncomplicated gallstones - clinical features?
``` Colicky pain --> back and shoulder Worse after eating fatty meal Nausea & vomiting Itch Dark urine, pale stool and/ or jaundice ```
203
Investigations for uncomplicated gallstones?
LFTs - raised ALP and GGT USS MRCP
204
Management of uncomplicated gallstones?
Supportive Ursodoxycholic acid for itch ERCP
205
Biliary colic?
Temporary obstruction of cystic/ common bile duct by a gallstone 2-6 hours (crescendo of pain then plateaus) Pain caused by contraction around stone
206
Signs and symptoms of biliary colic?
RUQ/ epigastric colicky pain (sharp, localized GI pain) - may radiate to right shoulder Assoc. w/ indigestion and high fat foods Mid-evening, early morning, lying flat Nausea and vom
207
Investigations for biliary colic?
Clinical diagnosis | USS
208
Management of biliary colic?
Lifestyle modifications Paracetamol, NSAIDs Diclofenac IM (severe pain) Stone will either pass in faeces orgs back into GB
209
Gallstone ileus?
Form of bowel obstruction caused by gallstones lumen of small bowel Large stone Thinning of GB wall Fistulas form
210
Signs, symptoms of gallstone ileus?
Abdo distension N&V Recurrent RUQ pain (due to chronic cholecystitis) Dehydration
211
Investigations for gallstone ileus?
- Rigler’s triad (via radiography) o Pneumobilia (air in biliary tree) o Evidence of small bowel obstruction o Evidence of gallstone outside gallbladder
212
Management of gallstone ileus?
IV fluid resuscitation NG tube Gallstone removal
213
Cholangitis?
Cholangitis means inflammation of bile duct system
214
Ascending cholangitis
Inflammation of bile duct due to bacterial infection on obstruction of biliary tree
215
Clinical features of Ascending cholangitis?
- Charcot’s triad: o Fever, jaundice, RUQ pain - Reynold’s pentad: o Fever, jaundice, RUQ pain, hypotension, confusion
216
Causes of ascending cholangitis?
``` Anything causing blockage Gallstones Iatrogenic in surgery chronic pancreatitis Malignancy ```
217
Investigations for ascending cholangitis?
- ERCP - gold standard - Bloods - raised WCC, raised CRP - LFTs - raised ALP, bilirubin, GGT (consistent with obstruction) - Blood culture - USS - MRCP
218
Management of ascending cholangitis?
- IV Abx amoxicillin, metronidazole and gentamicin (Vancomycin, metronidazole and gentamicin if penicillin allergic) - |Step down to oral Co-trimoxazole and metronidazole
219
Complications of ascending cholangitis ?
Sepsis
220
Cholecystitis?
Inflammation of gallbladder
221
Clinical features of cholecystitis?
- RUQ pain spreading to back/ shoulder - RUQ tenderness - Fever - Nausea and vomiting - + Murphy’s sign - Obstructive jaundice
222
Murphys sign?
Take in and hold deep breath while palpating right subcostal area If pain occurs on inspiration - inflamed gallbladder comes into contact with hand = positive
223
Causes of cholecystitis?
- Anything causing a blockage - Gallstones - PSC - Iatrogenic during surgery - Malignancy
224
Investigations for cholecystitis?
``` History High ALP Abdo USS - stones, thickened GB wall? MRCP ERCP ```
225
Management of cholecystitis (GB inflammation)?
Symptoms <72 hours - cholecystectomy (GB removal) >72 hours - medical management, follow up for cholecystectomy & days oral Ab - amici, metronidazole, gentamicin
226
Complications of cholecystitis?
perforation Fistula Peritonitis Sepsis
227
Acalculous cholecystitis?
Inflammation of GB in absence of gallstone
228
signs and symptoms of acalculous cholecystitis?
Same as calculous
229
Investigations for acalculous cholecystitis?
Raised ALP High CRP USS - thickened GB wall, no stones
230
Management of calculous cholecystitis ?
``` Cholecystectomy Percuataneous cholecystostomy (stoma in GB) ```
231
Primary biliary cholangitis/ cirrhosis?
Autoimmune condition - T cells attack small bile ducts in liver causing bile to leak into interstitium --> chronic inflammation of bile ducts
232
Clinical features of PBC?
``` Middle aged women, 45- 60 Asymptomatic Symptoms: - Fatigue - Itch, no rash - Jaundice - Joint pain - Xanthelasma and xanthoma - Abdo pain ```
233
Associated doseases with PBC?
Sjogren's rheumatoid arthritis Hypothyroidism
234
Investigations for PBC?
2/3: - Positive AMA (anti- mitochondiral antibody) - Cholestatic LFTs (raised ALP, GGT) - Liver biopsy (USS< MRCP, high IgM, cholesterol High)
235
Treatment for PBC?
First line = urseodoxycholic acid | 2nd = obeticholic acid
236
Primary sclerosing cholangitis?
Long term progressive disease of liver and gallbladder - inflammation and scarring of bile ducts (normally allow bile to drain into GB)
237
Cholestasis?
decrease in bile flow
238
Clinical features of PSC?
``` Male Asymptomatic History of UC Abdo - RUQ pain Itch Fatigue Jaundice Weight loss Acute presentation of hepatitis- like infection - fever, jaundice, cholangitis ```
239
Investigations for PSC?
``` Examination - jaundice, weight loss? Lots - raised ALP and GGT USS MRCP - standard for diagnosis (beaded appearance) ERCP - biopsy, onion skin May have ANCA bodies ```
240
Management of PSC (if symptomatic)?
Liver transplant Manage symptoms good diet Endoscopic interventions - ERCP for balloon dilation or stent placement
241
Biliary strictures?
Narrowing of bile duct
242
Congenital biliary atresia?
Condition babies born with --> absence or deficiency of extra- hepatic biliary tree Leads to cholestasis and eventual liver cirrhosis from back pressure of bile
243
Symptoms of congenital biliary atresia?
3 months of birth - Jaundice - Dark urine, pale stools - Growth delay
244
Investigations for congenital
Blood tests - LFTs, bilirubin USS - fibrous tissue, change to hepatic blood flow HIDA scan
245
Management for congenital biliary atresia?
Excision of fibrous tissue | Liver transplant
246
Cholangiocarcinoma?
Cancer of bile duct | Intrahepatic (less) or extra hepatic
247
Most common form of cholangiocarcinoma?
Hilar cholangiocarcinoma
248
Risk factors for cholangiocarcinoma?
Age (>65) SE Asia Prev biliary disease e.g. PSC, gs, cysts Genetic predisposition
249
Symptoms of cholangiocarcinoma?
``` Asymptomatic Jaundice (dark urine, pale stools) Abdo pain Weight loss, fatigue, loss of appetite Symptoms of cholangitis - Charcot's triad and Reynolds pentad RUQ pain, jaundice, fever ```
250
Investigations for cholangiocarcinoma?
LFTs - abnormal bilirubin, marked elevation of ALP and GGT Contrast MRI!!! CT for staging
251
Management of cholangiocarcinoma?
Surgical resection Stenting Adjuvant chemotherapy and radiotherapy
252
carcinoma of ampulla of vater?
Rare cancer - forms at junction of CBD and main pancreatic duct Extensive surgery

GI (9 decks)

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