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Liver + Friends Flashcards

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1
Q

What kind of virus is hepatitis A?

A

An RNA virus

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2
Q

How is hepatitis A transmitted?

A

Feco-orally

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3
Q

What is a presentation of viral hepatitis in different age groups?

A

Asymptomatic in children

symptomatic in adults

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4
Q

What is the treatment of viral hepatitis?

A

A) supportive +/- IVIG or vaccine –> transplant

B) entecavir —-> transplant

C) glecaprevir/pibrentavir —-> transplant

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5
Q

Who is most at risk of getting a severe infection of Hepatitis A?

A

Anyone with the coinfection of hepatitis B or C

anyone with any other underlying liver disease

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6
Q

What kind of virus is hepatitis B?

A

DNA virus

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7
Q

Does hepatitis B always progress to chronic liver disease?

A

No it is often self-limiting requiring no treatment

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8
Q

Who is most at risk of developing chronic liver disease from hepatitis B?

A

If the virus is acquired parenterally or if infection occurred in childhood

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9
Q

What is the treatment of hepatitis B?

A

B ET

Antivirals – entecavir
if pregnant - tenofovir

+ supportive

liver transplant if decompensated

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10
Q

How is hepatitis B transmitted?

A

percutaneous, per mucosal, sexually

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11
Q

What type of virus is hepatitis C?

A

RNA virus

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12
Q

What is the most common method of spread of hepatitis C?

A

IV drug users

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13
Q

What proportion of patients develop symptoms of hepatitis C in the acute phase?

A

1/3

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14
Q

What is management of hepatitis C?

A

Antivirals - glencaprevir

+ supportive

liver transplant

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15
Q

What are symptoms of viral hepatitis?

A

Fever and malaise - acute phase
jaundice
ascieties
signs of encephalopathy

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16
Q

If you suspect someone has viral hepatitis what investigations should you perform?

A

LFT - ALT(especially) and ALP raised, bilirubin,

Coagulation profile - PT raised

U+E - hepatorenal disease, and monitor kidneys if on diuretics

FBC - anaemia of chronic disease, WCC raised in acute infection

Viral PCR or

Hep A IGM
Hep B surface antigen test or core antigen test or serum antibody test
Hep C antibody immunoassay

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17
Q

What specific diagnostic test can you perform for hepatitis A (apart from viral PCR)?

A

Hep A IGM

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18
Q

What specific diagnostic test can you perform for hepatitis B (apart from viral PCR)?

A

Hep B surface antigen test or core antigen test or serum antibody test

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19
Q

What specific diagnostic test can you perform for hepatitis C (apart from viral PCR)?

A

Hep C antibody immunoassay

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20
Q

What are the three stages of alcoholic liver disease?

A

Fatty liver – steatosis
alcoholic hepatitis – inflammation and necrosis
cirrhosis

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21
Q

Describe the pathophysiology of alcoholic liver disease?

A

The breakdown of alcohol inhibits gluconeogenesis and increases fatty acid oxidation

promoting fatty in filtration and producing free radicals

ree radicals and the deficiency of antioxidants (vitamins E- usually from malnutrition) causes oxidative stress

promotes necrosis and apoptosis

free radicals also cause lipid oxidation causing further inflammation and fibrosis

acetyl-aldehyde a metabolite from alcohol breakdown induces inflammation

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22
Q

How does alcoholic liver disease usually present?

A 
Right upper quadrant pain – acute hepatitis
hepatomegaly
weight loss or weight gain
malnutrition and wasting
jaundice 
ascites (in cirrhosis)
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23
Q

What bloods do you need to perform in alcoholic liver disease?

A

LFTs

  • AST ALC serum bilirubin GGT (all +)
  • serum alk phosphate usually normal but can be +
  • serum albumin/protein (-)

PT/INR (+)

FBC (+ WCC thrombocytopenia, microcytic anaemia)
serum electrolytes especially magnesium and phosphate (–)
U+E (hepatorenal)

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24
Q

Apart from blood what other investigations should you perform in alcoholic liver disease?

A 
Hepatic ultrasound scan:
hepatomegaly 
fatty liver
Chirossis
ascites 
portal hypertension 
splenomegaly
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25
What is the management of alcoholic liver disease?
Alcohol abstinence corticosteroids – high bilirubin or hepatic encephalopathy diuretics and sodium restriction – ascites Lifestyle factors: weight loss, smoking cessation, nutritional supplements, immunisation of hepatitis influenza and pneumococcal liver transplant
26
What is non-alcoholic fatty liver disease?
Includes a spectrum of conditions characterised by hepatic steatosis/cirrhosis who do not consume excess alcohol
27
what is the thought pathophysiology Of non-alcoholic fatty liver disease?
Insulin resistance causes an accumulation of triglycerides develops into hepatic steatosis causes inflammation and oxidative injury necrosis occurs
28
what are risk factors associated with non-alcoholic fatty liver disease?
``` Obesity type II diabetes hyperlipidaemia hypertension rapid weight loss hepatotoxic medications TPN ```
29
What medications are hepatotoxic?
``` Tamoxifen corticosteroids diltiazem nifedipine methotrexate valproate amiodarone ```
30
What is the presentation of non-alcoholic fatty liver disease?
Fatigue and malaise - most common initial presenting Sx hepatosplenomegaly right upper quadrant pain if more severe jaundice and ascites
31
How would you investigate non-alcoholic fatty liver disease?
LFTs - AST ALC serum bilirubin GGT (all +) - serum alk phosphate usually normal but can be + - serum albumin/protein (-) PT/INR (+) FBC (+ WCC thrombocytopenia, microcytic anaemia) serum electrolytes especially magnesium, phosphate, sodium (–) U+E (hepatorenal) same pathophysiology as FLD so bloods the same + Hepatic USS
32
What is the management of non-alcoholic fatty liver disease?
Diet and exercise with weight loss intention pharmacology or surgery for weight loss supplemental vitamins E good diabetic control of type II diabetic new in starting if hyperlipidaemia liver transplant
33
what is cirrhosis?
A diffuse pathological process characterised by fibrosis and conversion of normal liver architecture to abnormal nodules it is the final stage of liver disease
34
What are consequences of cirrhosis?
Portal hypertension liver failure hepatocell carcinoma
35
What is the presentation of Cirrhosis?
``` Ascites and peripheral oedema jaundice and pruritus haematemesis or melena right upper quadrant pain muscle wasting and constitutional symptoms ```
36
on examination what features may you see of cirrhosis?
Hand and nail features: leukinochia, spider nevi palmar erythema facial features: telangactasia, spider nevi
37
What is the management of cirrhosis?
Managing the underlying liver disease monitoring for any complications corticosteroids – high bilirubin or hepatic encephalopathy diuretics and sodium restriction – ascites transplant life style factors
38
what is portal hypertension?
An increase of pressure within the portal vein due to impeding flow through the liver
39
What is a common consequence of portal hypertension?
Abdominal/oesophageal varices
40
What is the presentation of portal hypertension?
``` haematemesis ascites encephalopathy thrombocytopenia microcytic anaemia - any bleeds ```
41
How do you diagnose portal hypertension?
based on the presence of ascites or of dilated veins or varices
42
What is ascites?
A pathological collection of fluid in the peritoneal cavity most commonly because of cirrhosis
43
Why does cirrhosis cause ascites?
There is renal dysfunction and abnormal hepatic circulation (splanchnic arterial vasodilation) due to hepatic fibrosis this leads to increased lymph node formation and activation of the RAAS system stimulating the release of antidiuretic hormone this along with increased resistance to portal flow due to cirrhosis causes portal hypertension also causes collateral rain formation and the shunting of blood to systemic circulation
44
How do you diagnose ascites?
Clinical (O/E shifting dullness) can also be aided by ultrasound scan if unsure
45
How do you manage ascites?
Diuretics and sodium restriction
46
What is acute pancreatitis?
A disorder of the exocrine pancreas caused by acing cell injury with local and systemic inflammatory responses
47
what commonly causes pancreatitis?
I GET SMASHED ``` idiopathic gallstones ethanol trauma steroids mumps autoimmune scorpion venom (??) hyperlipideamia / calcaemia / hypothermia ERCP + emboli Drugs inc/ HRT ```
48
What risk factors are associated with acute pancreatitis?
Alcohol use history of gallstones azathioprine and other similar medications
49
What is the presentation of acute pancreatitis?
Nausea and vomiting, – most common made epigastric/LUQ pain radiating to back, constant and severe, sudden onset, exacerbated by movement symptoms of hypovolaemia can be present due to vomiting (oliguria dry mucous membrane decreased skin turgor UTC) can have weight loss due to N+V and decrease intake
50
What complication is common in acute pancreatitis and what would you find on examination?
Pleural effusion reduced air entry, dullness on percussion
51
How do you diagnose acute pancreatitis?
Check serum lipase or amylase imaging not required unless the diagnostic doubt or failure to improve within 48 to 72 hours of treatment
52
Apart from checking pancreatic enzymes what other investigations are useful in acute pancreatitis?
ALT indicates gallstones U+E is elevated in severe cases and shows at risk patients electrolytes should also be checked for imbalances caused by vomiting which can lead to arrhythmias FBC - WCC (+)
53
How do you manage acute pancreatitis?
Fluid recess analgesia and nutritional support (replacing calcium and magnesium) empirical IV antibiotics if infection is suspected plus treating underlying cause
54
How do you manage gallstones with cholangitis (as a cause of acute pancreatitis)?
ERCP within 24 hours plus normal pancreatitis Mx
55
How do you manage gallstones with bile duct obstruction (as a cause of acute pancreatitis)?
ERCP With sphincterotomy normal pancreatitis Mx
56
How do you manage acute pancreatitis which is alcohol related?
normal pancreatitis Mx Plus vitamin replacement and alcohol withdrawal programs
57
What you do if acute pancreatitis is not improving within five – seven days?
``` Perform contrast enhanced CT give ongoing supportive and nutrition treatment do a fine needle aspirate and culture IV antibiotics if suspecting infection catheter draining or debridement ```
58
What is chronic pancreatitis?
progressive pancreatic injury to the pancreas and surrounded structures causing scarring and loss of function
59
What are the four types of chronic pancreatitis ?
Recurrent acute pancreatitis idiopathic pancreatitis chronic relapsing pancreatitis established chronic pancreatitis
60
What is recurrent acute pancreatitis?
There is an identifiable cause of acute pancreatitis which keeps on repairing but does not lead to chronic pancreatitis examples are: gallstones, drugs, hypercalcaemia
61
What is idiopathic pancreatitis?
No cause has ever been found Usually encompasses chronic relapsing and established chronic pancreatitis
62
What is chronic relapsing pancreatitis?
Relapsing pain no other relapsing clinical features of pancreatitis there are pathological changes in tissue specimens
63
What is established chronic pancreatitis?
``` All the hallmark clinical features of pancreatitis are present: reduced exocrine functions malabsorption diabetes pancreatic calcifications ```
64
What are risk factors associated with chronic pancreatitis?
Alcohol smoking family history coeliac disease
65
What is the presentation of chronic pancreatitis?
Abdominal pain – epigastric, dull, radiating to back, occurring 30 minutes post-prandial, SWAB A DECK ``` steatorrhoea weight loss inc. N+V abdo pain bloating ADECK deficiency ```
66
What investigations are required to diagnose chronic pancreatitis?
Raised serum amylase raised blood glucose (occurring import X crying function) CT CT unavailable then ultrasound
67
What findings would you see on a CT scan of chronic pancreatitis?
Pancreatic calcifications local or diffuse enlargement pancreas ductal dilatations can have vascular complications
68
What is the management of chronic pancreatitis?
Analgesia for acute pain + ocreotide is a somatostatin analogue which may help relieve pain pancreatic enzymes PPI dietary modifications including possible enteric feeding for malnutrition
69
What are some complications of chronic pancreatitis?
Pseudo-cysts Biliary complications pancreatic stone intractable pain and pancreatic duct dilatation
70
how would you manage a Pseudo-cysts in chronic pancreatitis?
Decompression
71
How would you manage any Biliary complications?
If bilirubin has been raised for one month or more usually surgery will be required
72
How would you manage pancreatic stones?
Shockwave therapy ESWL
73
How would you manage pancreatic duct dilatations?
Decompression
74
What is biliary colic?
A clinical symptom of pain in the right upper quadrant or epigastric region a constant pain lasting over 30 minutes pain increases with intensity in this time usually has a post-prandial onset
75
What is biliary colic commonly associated with?
Gallstones a.k.a. cholelithiasis
76
What is the presentation of gallstones?
``` Biliary colic dyspepsia heartburn flatulence bloating ```
77
what sign can indicate cholecystitis?
RUQ tenderness/epigastric tenderness
78
What are risk factors associated with gallstones?
``` pregnancy exogenous oestrogen Obesity and diabetes NAFLD prolonged fasting and rapid weight loss TPN terminal highly disease or resection drugs: ocreotide and ceftriaxone ```
79
How do you diagnose gallstones?
Abdominal ultrasound scan
80
What should you check for in a patient with gallstones?
``` Acute pancreatitis (lipase and amylase) liver impact (LFTs) cholangitis, cholecystitis (WCC) ```
81
How do stones in gallbladder (cholelithiasis) affects LFTs?
normal LFT
82
How do stones in bile duct (choledocholithasis) affects LFTs?
+ ALP | + bilirubin
83
What is the management of gallstones in gallbladder?
cholecystectomy if symptomatic | otherwise observation
84
What is the management of gallstones in the bile duct?
ERCP with stenting or balloon dilatation
85
What is ascending cholangitis?
An infection of the biliary tree most commonly caused by obstruction
86
What is the pathophysiology of ascending cholangitis?
Impeded biliary flow allows for bacterial seeding and growth in the biliary tree it can lead to mild inflammation or even sepsis
87
What is the presentation of ascending cholangitis?
RUQ/epigastric pain and tenderness jaundice and fever pale stools and pruritus signs of sepsis such as fever tachycardia
88
What are risk factors for ascending cholangitis?
``` Over 50 history of bile duct stones history of primary/secondary sclerosing cholangitis the structures of the biliary tree trauma to the bile duct ```
89
What investigations should be performed in ascending cholangitis? What are the results?
FBC - + WCC U+E (+cr) - raised in severe disease/may indicate sepsis, Mg and K may be reduced LFT - + Bilirubin, ALT,AST,ALP Blood cultures - positive Abdominal USS - stones and dilated ducts ERCP - diagnostic Sepsis screen
90
What is the management of ascending cholangitis?
IV antibiotics – metronidazole and cefamine supportive care including O2 magnesium potassium and coagulation factors biliary decompression and drainage opioid analgesia
91
What is primary biliary cholangitis?
A chronic disease of the small intrahepatic bile ducts characterised by progressive bile duct damage and eventual loss
92
What is the pathophysiology of primary biliary cholangitis?
Insult to the portal tract caused by inflammation which then becomes chronic causing fibrosis and can lead to cirrhosis
93
What risk factors are associated with primary biliary cholangitis?
``` Female aged 45 – 60 history of autoimmune diseases history of high cholesterol family history of autoimmune diseases ```
94
What is the presentation of primary biliary cholangitis?
``` itch fatigue dry eyes and mouth (Sjogren's syndrome) postural dizziness the hepatomegaly ```
95
How do you diagnose primary biliary cholangitis?
has ONLY increased ALP is ANA positive! USS to exclude obstruction like stone
96
How do you manage primary biliary cholangitis?
urdeoxycholic acid Corticosteroid/immuno modulation (prednisolone) – if there is significant information or overlapped autoimmune hepatitis cholestiramine if itchy liver transplant in end-stage disease
97
A patient presents with right upper quadrant pain pruritus fatigue weight loss and jaundice they say they have an on and off low-grade fever blood tests revealed that they have elevated a LP GGT AST and ALC as well as slightly raised bilirubin what is the diagnosis?
Primary sclerosing cholangitis
98
What is primary sclerosing Cholangitis?
A chronic progressive cholestatic liver disease it is characterised by inflammation and fibrosis of the intra/extra hepatic bile ducts this results in diffuse multifocal structures forming damaging both bile ducts and the liver
99
What disease is primary sclerosing cholangitis is commonly associated with?
Inflammatory bowel disease
100
Summarise the key present team points of primary sclerosing cholangitis?
``` Pruritus right upper quadrant pain fatigue weight loss fever jaundice ```
101
What serum markers/antibodies may be present in primary sclerosing cholangitis?
AN CA | AMA – anti-mitochondrial antibody
102
what blood tests should you run for primary sclerosing cholangitis and what are their results?
ALP + GGT + AST/ALT + Billirubin can be + albumin normal unless advanced disease then - Coagulation screen deranged in advanced liver disease ANCA/AMA
103
what test is diagnostic for Primary sclerosing cholangitis? and what would it show?
MRCP dilatations and strictures
104
What drug is used to provide pruritus relief?
colestyramine
105
What complications can occur due to primary sclerosing cholangitis?
Liver disease/ cirrhosis hepatic osteopenia/ osteoporosis strictures
106
How would you treat hepatic osteoporosis?
alendronic acid and HRT can be used
107
how would you treat hepatic osteopenia?
Ergocalciferol and calcium carbonate
108
When would you use immunosuppressant in the treatment of primary sclerosing cholangitis?
Overlapping autoimmune hepatitis
109
what is the treatment of Primary sclerosing cholangitis?
observation and lifestyle changes – healthy weight diets and limiting alcohol observation of bone mineral density every 2 – 4 years if they have IBD check that with colonoscopy then symptomatic relief end stage disease = transplant
110
What cells does liver cancer derive from?
Hepatocytes
111
What risk factors are associated with liver cancer?
``` Cirrhosis hepatitis B+C chronic heavy alcohol abuse diabetes obesity family history of liver cancer increasing age ```
112
How is liver cancer usually diagnosed?
On routine screening as patient almost always have underlying liver conditions
113
What are the symptoms of liver failure which can develop in liver cancer?
``` Abdominal distension right quadrant pain early satiety and weight loss leg oedema and ascites hepatic encephalopathy the splenomegaly jaundice Asterix's spider naevi Palmar erythema peri-umbilical collateral veins fetur hepaticus ```
114
What blood results are expected in liver failure caused by hepatic cancer?
LFT will be derranged (AST ALT ALP bilirubin - high, albumin - low PT time - prolonged FBC - microcytic anaemia and thrombocytopenia from chronic disease and also variceal bleeding hepatitis screen may be positive alpha-fetoprotein will be elevated in advanced disease
115
Apart from blood tests what further investigations should be performed in liver cancer?
CT (or USS if ct not possible) liver biopsy is not required because of seeding
116
A patient presents with : jaundice non-specific upper abdominal pain which can radiate to the back weight loss what is a possible diagnosis?
Pancreatic cancer
117
What type of cancer is pancreatic cancer usually?
adenocarcinoma
118
what test is diagnostic of pancreatic cancer?
Pancreatic CT
119
What would liver function tests show in pancreatic cancer
they will be abnormal because Of obstructive jaundice
120
What is a management plan for pancreatic cancer?
Because it is a late stage diagnosis usually surgery is no longer possible for palliative care is needed
121
A patient comes in with painless jaundice what should you immediately think of?
cholangiocarcinoma but pancreatic cancer can also be a cause
122
What are the risk factors associated with cholangiocarcinoma?
``` Over 50 biliary or bile tract disease ulcerative colitis liver disease HIV ```
123
What test is diagnostic for cholangiocarcinoma?
Ultrasound scan
124
What tumour markers are usually present in cholangiocarcinoma?
Ca 19-9 Ca- 125 CEA
125
What is a liver abscess?
Purulent collections in the liver parenchyma resulting from bacterial fungal or parasitic infections
126
What pathogens usually cause liver abscesses?
E. coli klebisella Streptococcus but is usually polymicrobial
127
What is the presentation of liver abscesses?
Constitutional symptoms of infection | right upper quadrant tenderness and hepatomegaly
128
What is a fairly common complication of liver abscesses?
diaphragmatic irritation causing cough shortness of breath or chest pain
129
what investigations should you perform for a liver abscess?
Blood cultures aspirated abscess fluid liver ultrasound or CT FBC LFT coag screens
130
What is the management of liver abscesses?
Empirical antibiotics such as the family with metronidazole if haemodynamically unstable consider adding a coverage for MRSA such as vancomycin drainage of abscess
131
What can cause recurrent liver abscesses?
biliary abnormalities
132
What does elevation in serum ALP mean?
ALP elevated in injury to in bile ducts, liver and bones. However is mainly an indication for cholestasis It is raised in liver injury usually secondary to cholestasis but elevation of ALP can indicate injury to any of these structures, usually finding out which one requires looking at other bloods.
133
what does ALP stand for?
Alkaline phosphatase
134
what does ALT stand for?
Alanine transaminase
135
when is ALT raised
hepatic injury
136
what does raised ALT and slightly raised ALP mean
hepatic injury
137
what does raised ALP but slightly raised ALT mean
biliary injury / cholestasis
138
what does elevated GGT mean ?
suggestive of biliary epithelial damage and bile flow obstruction also raised in alcohol and drugs like phenytoin
139
what does ALP raised only (No ALT or GGT)?
Bony metastases or primary bone tumours (e.g. sarcoma) Vitamin D deficiency Recent bone fractures Renal osteodystrophy
140
Raised ALP and raised GGT meaning?
ALP with a raised GGT is highly suggestive of cholestasis/ biliary causes
141
what are causes of unconjugated hyperbillirubinaemia
Haemolysis (e.g. haemolytic anaemia) Impaired hepatic uptake (e.g. drugs, congestive cardiac failure) Impaired conjugation (e.g. Gilbert’s syndrome)
142
what are causes of conjugated hyperbillirubinaemia?
Hepatocellular injury | Cholestasis
143
what do the symptoms of Normal urine + normal stools indicate about the cause of jaundice?
pre hepatic
144
what do the symptoms of dark urine + normal stools indicate about the cause of jaundice?
hepatic
145
what do the symptoms of dark urine + dark stools indicate about the cause of jaundice?
post hepatic
146
why does pre hepatic jaundice not change stools/urine?
Unconjugated bilirubin is water-insoluble and, therefore, doesn’t affect the colour of the patient’s urine
147
what is the function of albumin?
synthesised in the liver and helps to bind water, cations, fatty acids and bilirubin. It also plays a key role in maintaining the oncotic pressure of blood.
148
when would you get low serum albumin?
Liver disease in later stages (e.g. cirrhosis). Inflammation triggering an acute phase response which temporarily decreases the liver’s production of albumin Excessive loss of albumin due to protein-losing enteropathies or nephrotic syndrome.
149
when would PT time be deranged in liver disease?
specifically assessing the extrinsic pathway. In the absence of other secondary causes such as anticoagulant drug use and vitamin K deficiency an increased PT can indicate liver disease and dysfunction - more severe stage
150
what does ALT > AST ratio mean?
chronic liver disease
151
what does AST > ALT ratio mean?
associated with cirrhosis and acute alcoholic hepatitis
152
what does low glucose mean in terms of liver disease
very advanced liver disease Gluconeogenesis tends to be one of the last functions to become impaired in the context of liver failure.
153
``` a patient presents (20y/o) with: tremor dysarthria dystonia incoordination jaundice ``` what diagnosis do you suspect?
Wilson's disease
154
What investigations would you perform for ?Wilsons disease - what would the results be?
LFT - abnormal blood ceruloplasm - low 24h urine copper measurements - high >100 Opthalmological slit lamp - Keyser flesher rings DIAGNOSTIC - liver biopsy - increased copper
155
what is Wilsons disease?
an autosomal recessive disease of copper accumulation leading to copper toxicity caused by mutations in ATP 7B gene (that is part of the biliary excretion pathway)
156
what gene is responsible for Wilsons disease ?
ATP 7B gene
157
what are all the movement disorders associated with Wilsons disease?
``` tremour dysarthria dystonia incoordination sloppy/small handwriting dysdidokinesia ```
158
what neurological Sx are NOT present in Wilsons disease?
normal sensation normal muscular strength normal reflexes
159
what are the key features of a history of Wilsons disease
young (10-40y) | evidence of hepatic disease PLUS movement disorders of psychiatric disorder
160
what is the management of Wilsons disease?
zink or trientine (chelating agent)
161
who is Trientine contraindicated in?
those with neurological Sx as it can worsen symptoms however if Zinc is not tolerated it can be tried transplantation
162
what is a complication of Wilsons?
hepatic failure ascites fulminant liver failure
163
are neurological sx reversible in wilsons?
yes
164
what is fulminant liver failure?
sudden massive liver necrosis
165
what is the presentation of fulminant liver failure?
encephalopathy astirixus jaundice septic like symptoms
166
what is the management of fulminant liver failure?
lactulose for encephalopathy - decreases ammonia absorption, ICU, liver transplant
167
what is haemochromatosis?
a multisystem disorder of dysregulated dietary iron absorption and increased iron release from macrophages
168
what risk factors are associated with haemochromatosis?
``` Middle-aged Male White ancestry family history supplemental iron intake ```
169
What is the presentation of haemochromatosis?
``` Fatigue and weakness arthralgia hypogonadism and loss of libido bronzing skin pigmentation jaundice and other features associated with cirrhosis ```
170
What conditions are Caused by haemochromatosis?
Diabetes mellitus | cirrhosis
171
How do you diagnose haemochromatosis?
Fasting transferrin saturation and serum ferritin will be raised final diagnosis is through genetic testing
172
How do you manage haemochromatosis?
Observation and follow-ups every three years in stable disease or yearly in stage I disease low iron diet hepatitis A and B vaccinations phlebotomy iron chelation therapy therapy using desferrioxamine
173
What are cholechondyl cysts?
Congenitalconditions involving cystic die notations of the bile ducts
174
What is the presentation of a chocholedocal cysts?
Symptomatic within the first year of life abdominal pain jaundice right upper quadrant abdominal mass is sometimes present
175
What causes symptoms in choledochal cysts?
Obstructions of bile ducts caused by the cyst
176
How do you diagnose choledochall cysts?
Can be diagnosed in the antenatal period | otherwise ultrasound scan
177
What is the management of Choledochal cysts ?
Surgery
178
What are paediatric causes of liver failure?
Excess paracetamol viral hepatitis Wilson's disease and other metabolic conditions
179
How do you manage liver failure in paediatrics?
IV dextrose empirical broad-spectrum antibiotics and antifungals IV vitamin K and FFP
180
What are complications of paediatric liver failure?
``` Cerebral oedema haemorrhage gastritis coagulopathy sepsis pancreatitis ```
181
What is biliary atresia?
A progressive idiopathic necro inflammatory process that may involve a segment or the entire extrahepatic biliary tree
182
What is the pathophysiology of biliary atresia?
The the necro inflammation causes fibro of alliterative obstruction of the extrahepatic biliary tree then progresses to the intrahepatic ducts which are developing and utero or during the neonatal period
183
what will the blood tests be like for biliary atresia
Liver function tests- initially normal but v high GGT coagulation initially normal Billirubin may be ++
184
What is the diagnostic investigation for biliary atresia?
cholangiogram showing decreased patency liver biopsy can be used to differentiate from differential diagnoses abdominal USS
185
how do you manage biliary atresia?
surgery + urseodeoxycholic acid if severe enough liver transplant may be needed nutritional support trimethoprim/sulfemathozole prophylactic antibiotics for the 1st year of life
186
What is a Wilms' tumour?
nephroblastoma the most common cause of renal malignancy in children
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What is the presentation of a Wilms' tumour?
Abdominal/flank mass/swelling abdominal distension abdominal pain - in less than 30% of patients pallor
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What investigations are required for a Wilms' tumour?
Ultrasound scan will show mass CT shows extents of disease (10% of patients have mets) biopsy
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What will urinalysis and renal function tests reveal in Wilms' tumour?
Urinalysis= haematuria | renal function will be normal unless there are bilateral tumours
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What is a hepatoblastoma?
An uncommon malignant liver cancer originating from immature liver precursor cells
191
Who is at risk of hepatblastoma?
Children with familial adenomatous polyposis
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What is the presentation of hepatoblastoma?
Abdominal mass
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what blood test is required in hepatoblastoma?
Alpha-fetoprotein (raised)
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What conjenital infections cause neonatal hepatitis?
Cytomegalovirus rubella measles hepatitis ABC
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What are the causes of neonatal hepatitis?
Congenital infections inborn metabolic errors cystic fibrosis rarer causes: intestinal failure progressive familial intrahepatic cholestasis galactosaemia
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What is familial intrahepatic cholestasis?
Inherited recessive mutation causing decreased bile production
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What is the presentation of familial intrahepatic cholestasis?
prolonged new natal jaundice | pruritus
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What are the consequences of familial intrahepatic cholestasis if untreated?
Failure to thrive | Ricketts
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What is the GGT level in familial intrahepatic cholestasis?
Low
200
What is the management of familial intrahepatic cholestasis?
uredeoxycholic acide vitamins ADEK if v severe : transplant
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What is galactosaemia?
High levels of galactose from inability to metabolise sugars causing eventual liver failure
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What is a presentation of galactosaemia?
Neonatal jaundice vomiting poor feeding hepatomegaly
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What are the consequences of galactosaemia if not treated?
DIC developmental delayed cataracts recurrent bacterial infections
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How do you diagnose galactosaemia?
Urine dipstick And your analysis shows increased sugars (galactose)
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How do you manage galactosaemia?
Sugar/galactose/ lactose free diet
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What are inborn errors of metabolism?
cause faulty biosynthesis causing cholestasis
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What with the GGT and liver enzyme levels be like in inborn errors of metabolism?
Normal GGT | abnormal liver enzymes (ALP very high ALT slightly high)
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How do you manage inborn errors of metabolism caused hepatitis?
uredeoxycholic acid
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Splenomegaly appear in neonatal hepatitis and if so what would be the consequences of it?
yes | if untreated can cause portal hypertension and Aids the process of cirrhosis
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What are common causes of neonatal jaundice- conjugated?
Neonatal hepatitis | bile duct obstruction
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What are common causes of neonatal jaundice – unconjugated?
Breastmilk jaundice rubella measles hepatitis ABC
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when is neonatal jaundice physiological?
Up to 14 days
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Why does physiological neonatal jaundice occur?
fetal haemoglobin and red blood cells are replaced
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What are treatments of neonatal jaundice?
phototherapy | Exchange transfusion: if bilirubin levels are very high/unstable
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What is kernictus?
Occurs when jaundice is left untreated or Billy Rubin is very high bilirubin deposits in the brain causing brain damage
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What are the potential consequences of kernictus?
Cerebral palsy developmental delay hearing loss
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What are symptoms of kernictus?
``` Irritable baby lethargic high-pitched cry floppy baby seizures as it progresses ```
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What is the management of kernictus?
Exchange transfusion, supportive care and phototherapy

FINALS (24 decks)

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