Liver + Friends Flashcards
What kind of virus is hepatitis A?
An RNA virus
How is hepatitis A transmitted?
Feco-orally
What is a presentation of viral hepatitis in different age groups?
Asymptomatic in children
symptomatic in adults
What is the treatment of viral hepatitis?
A) supportive +/- IVIG or vaccine –> transplant
B) entecavir —-> transplant
C) glecaprevir/pibrentavir —-> transplant
Who is most at risk of getting a severe infection of Hepatitis A?
Anyone with the coinfection of hepatitis B or C
anyone with any other underlying liver disease
What kind of virus is hepatitis B?
DNA virus
Does hepatitis B always progress to chronic liver disease?
No it is often self-limiting requiring no treatment
Who is most at risk of developing chronic liver disease from hepatitis B?
If the virus is acquired parenterally or if infection occurred in childhood
What is the treatment of hepatitis B?
B ET
Antivirals – entecavir
if pregnant - tenofovir
+ supportive
liver transplant if decompensated
How is hepatitis B transmitted?
percutaneous, per mucosal, sexually
What type of virus is hepatitis C?
RNA virus
What is the most common method of spread of hepatitis C?
IV drug users
What proportion of patients develop symptoms of hepatitis C in the acute phase?
1/3
What is management of hepatitis C?
Antivirals - glencaprevir
+ supportive
liver transplant
What are symptoms of viral hepatitis?
Fever and malaise - acute phase
jaundice
ascieties
signs of encephalopathy
If you suspect someone has viral hepatitis what investigations should you perform?
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
What specific diagnostic test can you perform for hepatitis A (apart from viral PCR)?
Hep A IGM
What specific diagnostic test can you perform for hepatitis B (apart from viral PCR)?
Hep B surface antigen test or core antigen test or serum antibody test
What specific diagnostic test can you perform for hepatitis C (apart from viral PCR)?
Hep C antibody immunoassay
What are the three stages of alcoholic liver disease?
Fatty liver – steatosis
alcoholic hepatitis – inflammation and necrosis
cirrhosis
Describe the pathophysiology of alcoholic liver disease?
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
How does alcoholic liver disease usually present?
Right upper quadrant pain – acute hepatitis hepatomegaly weight loss or weight gain malnutrition and wasting jaundice ascites (in cirrhosis)
What bloods do you need to perform in alcoholic 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 and phosphate (–)
U+E (hepatorenal)
Apart from blood what other investigations should you perform in alcoholic liver disease?
Hepatic ultrasound scan: hepatomegaly fatty liver Chirossis ascites portal hypertension splenomegaly
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
What is non-alcoholic fatty liver disease?
Includes a spectrum of conditions characterised by hepatic steatosis/cirrhosis who do not consume excess alcohol
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
what are risk factors associated with non-alcoholic fatty liver disease?
Obesity type II diabetes hyperlipidaemia hypertension rapid weight loss hepatotoxic medications TPN
What medications are hepatotoxic?
Tamoxifen corticosteroids diltiazem nifedipine methotrexate valproate amiodarone
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
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
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
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
What are consequences of cirrhosis?
Portal hypertension
liver failure
hepatocell carcinoma
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
on examination what features may you see of cirrhosis?
Hand and nail features:
leukinochia, spider nevi palmar erythema
facial features:
telangactasia, spider nevi
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
what is portal hypertension?
An increase of pressure within the portal vein due to impeding flow through the liver
What is a common consequence of portal hypertension?
Abdominal/oesophageal varices
What is the presentation of portal hypertension?
haematemesis ascites encephalopathy thrombocytopenia microcytic anaemia - any bleeds
How do you diagnose portal hypertension?
based on the presence of ascites or of dilated veins or varices
What is ascites?
A pathological collection of fluid in the peritoneal cavity most commonly because of cirrhosis
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
How do you diagnose ascites?
Clinical (O/E shifting dullness)
can also be aided by ultrasound scan if unsure
How do you manage ascites?
Diuretics and sodium restriction
What is acute pancreatitis?
A disorder of the exocrine pancreas caused by acing cell injury with local and systemic inflammatory responses
what commonly causes pancreatitis?
I GET SMASHED
idiopathic gallstones ethanol trauma steroids mumps autoimmune scorpion venom (??) hyperlipideamia / calcaemia / hypothermia ERCP + emboli Drugs inc/ HRT
What risk factors are associated with acute pancreatitis?
Alcohol use
history of gallstones
azathioprine and other similar medications
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
What complication is common in acute pancreatitis and what would you find on examination?
Pleural effusion
reduced air entry, dullness on percussion
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
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 (+)
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
How do you manage gallstones with cholangitis (as a cause of acute pancreatitis)?
ERCP within 24 hours plus normal pancreatitis Mx
How do you manage gallstones with bile duct obstruction (as a cause of acute pancreatitis)?
ERCP With sphincterotomy normal pancreatitis Mx
How do you manage acute pancreatitis which is alcohol related?
normal pancreatitis Mx Plus vitamin replacement and alcohol withdrawal programs
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
What is chronic pancreatitis?
progressive pancreatic injury to the pancreas and surrounded structures causing scarring and loss of function
What are the four types of chronic pancreatitis ?
Recurrent acute pancreatitis
idiopathic pancreatitis
chronic relapsing pancreatitis
established chronic pancreatitis
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
What is idiopathic pancreatitis?
No cause has ever been found Usually encompasses chronic relapsing and established chronic pancreatitis
What is chronic relapsing pancreatitis?
Relapsing pain no other relapsing clinical features of pancreatitis
there are pathological changes in tissue specimens
What is established chronic pancreatitis?
All the hallmark clinical features of pancreatitis are present: reduced exocrine functions malabsorption diabetes pancreatic calcifications
What are risk factors associated with chronic pancreatitis?
Alcohol
smoking
family history
coeliac disease
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
What investigations are required to diagnose chronic pancreatitis?
Raised serum amylase
raised blood glucose (occurring import X crying function)
CT
CT unavailable then ultrasound
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
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
What are some complications of chronic pancreatitis?
Pseudo-cysts
Biliary complications
pancreatic stone
intractable pain and pancreatic duct dilatation
how would you manage a Pseudo-cysts in chronic pancreatitis?
Decompression
How would you manage any Biliary complications?
If bilirubin has been raised for one month or more usually surgery will be required
How would you manage pancreatic stones?
Shockwave therapy ESWL
How would you manage pancreatic duct dilatations?
Decompression
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
What is biliary colic commonly associated with?
Gallstones a.k.a. cholelithiasis
What is the presentation of gallstones?
Biliary colic dyspepsia heartburn flatulence bloating
what sign can indicate cholecystitis?
RUQ tenderness/epigastric tenderness
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
How do you diagnose gallstones?
Abdominal ultrasound scan
What should you check for in a patient with gallstones?
Acute pancreatitis (lipase and amylase) liver impact (LFTs) cholangitis, cholecystitis (WCC)
How do stones in gallbladder (cholelithiasis) affects LFTs?
normal LFT
How do stones in bile duct (choledocholithasis) affects LFTs?
+ ALP
+ bilirubin
What is the management of gallstones in gallbladder?
cholecystectomy if symptomatic
otherwise observation
What is the management of gallstones in the bile duct?
ERCP with stenting or balloon dilatation
What is ascending cholangitis?
An infection of the biliary tree most commonly caused by obstruction
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
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
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
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
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
What is primary biliary cholangitis?
A chronic disease of the small intrahepatic bile ducts characterised by progressive bile duct damage and eventual loss
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
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
What is the presentation of primary biliary cholangitis?
itch fatigue dry eyes and mouth (Sjogren's syndrome) postural dizziness the hepatomegaly
How do you diagnose primary biliary cholangitis?
has ONLY increased ALP
is ANA positive!
USS to exclude obstruction like stone
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
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
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
What disease is primary sclerosing cholangitis is commonly associated with?
Inflammatory bowel disease
Summarise the key present team points of primary sclerosing cholangitis?
Pruritus right upper quadrant pain fatigue weight loss fever jaundice
What serum markers/antibodies may be present in primary sclerosing cholangitis?
AN CA
AMA – anti-mitochondrial antibody
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
what test is diagnostic for Primary sclerosing cholangitis?
and what would it show?
MRCP
dilatations and strictures
What drug is used to provide pruritus relief?
colestyramine
What complications can occur due to primary sclerosing cholangitis?
Liver disease/ cirrhosis
hepatic osteopenia/ osteoporosis
strictures
How would you treat hepatic osteoporosis?
alendronic acid and HRT can be used
how would you treat hepatic osteopenia?
Ergocalciferol and calcium carbonate
When would you use immunosuppressant in the treatment of primary sclerosing cholangitis?
Overlapping autoimmune hepatitis
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
What cells does liver cancer derive from?
Hepatocytes
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
How is liver cancer usually diagnosed?
On routine screening as patient almost always have underlying liver conditions
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
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
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
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
What type of cancer is pancreatic cancer usually?
adenocarcinoma
what test is diagnostic of pancreatic cancer?
Pancreatic CT
What would liver function tests show in pancreatic cancer
they will be abnormal because Of obstructive jaundice
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
A patient comes in with painless jaundice what should you immediately think of?
cholangiocarcinoma
but pancreatic cancer can also be a cause
What are the risk factors associated with cholangiocarcinoma?
Over 50 biliary or bile tract disease ulcerative colitis liver disease HIV
What test is diagnostic for cholangiocarcinoma?
Ultrasound scan
What tumour markers are usually present in cholangiocarcinoma?
Ca 19-9
Ca- 125
CEA
What is a liver abscess?
Purulent collections in the liver parenchyma resulting from bacterial fungal or parasitic infections
What pathogens usually cause liver abscesses?
E. coli
klebisella
Streptococcus
but is usually polymicrobial
What is the presentation of liver abscesses?
Constitutional symptoms of infection
right upper quadrant tenderness and hepatomegaly
What is a fairly common complication of liver abscesses?
diaphragmatic irritation causing cough shortness of breath or chest pain
what investigations should you perform for a liver abscess?
Blood cultures
aspirated abscess fluid
liver ultrasound or CT
FBC LFT coag screens
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
What can cause recurrent liver abscesses?
biliary abnormalities
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.
what does ALP stand for?
Alkaline phosphatase
what does ALT stand for?
Alanine transaminase
when is ALT raised
hepatic injury
what does raised ALT and slightly raised ALP mean
hepatic injury
what does raised ALP but slightly raised ALT mean
biliary injury / cholestasis
what does elevated GGT mean ?
suggestive of biliary epithelial damage and bile flow obstruction
also raised in alcohol and drugs like phenytoin
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
Raised ALP and raised GGT meaning?
ALP with a raised GGT is highly suggestive of cholestasis/ biliary causes
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)
what are causes of conjugated hyperbillirubinaemia?
Hepatocellular injury
Cholestasis
what do the symptoms of
Normal urine + normal stools
indicate about the cause of jaundice?
pre hepatic
what do the symptoms of
dark urine + normal stools
indicate about the cause of jaundice?
hepatic
what do the symptoms of
dark urine + dark stools
indicate about the cause of jaundice?
post hepatic
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
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.
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.
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
what does ALT > AST ratio mean?
chronic liver disease
what does AST > ALT ratio mean?
associated with cirrhosis and acute alcoholic hepatitis
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.
a patient presents (20y/o) with: tremor dysarthria dystonia incoordination jaundice
what diagnosis do you suspect?
Wilson’s disease
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
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)
what gene is responsible for Wilsons disease ?
ATP 7B gene
what are all the movement disorders associated with Wilsons disease?
tremour dysarthria dystonia incoordination sloppy/small handwriting dysdidokinesia
what neurological Sx are NOT present in Wilsons disease?
normal sensation
normal muscular strength
normal reflexes
what are the key features of a history of Wilsons disease
young (10-40y)
evidence of hepatic disease PLUS movement disorders of psychiatric disorder
what is the management of Wilsons disease?
zink or trientine (chelating agent)
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
what is a complication of Wilsons?
hepatic failure
ascites
fulminant liver failure
are neurological sx reversible in wilsons?
yes
what is fulminant liver failure?
sudden massive liver necrosis
what is the presentation of fulminant liver failure?
encephalopathy
astirixus
jaundice
septic like symptoms
what is the management of fulminant liver failure?
lactulose for encephalopathy - decreases ammonia absorption, ICU, liver transplant
what is haemochromatosis?
a multisystem disorder of dysregulated dietary iron absorption and increased iron release from macrophages
what risk factors are associated with haemochromatosis?
Middle-aged Male White ancestry family history supplemental iron intake
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
What conditions are Caused by haemochromatosis?
Diabetes mellitus
cirrhosis
How do you diagnose haemochromatosis?
Fasting transferrin saturation and serum ferritin will be raised
final diagnosis is through genetic testing
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
What are cholechondyl cysts?
Congenitalconditions involving cystic die notations of the bile ducts
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
What causes symptoms in choledochal cysts?
Obstructions of bile ducts caused by the cyst
How do you diagnose choledochall cysts?
Can be diagnosed in the antenatal period
otherwise ultrasound scan
What is the management of Choledochal cysts ?
Surgery
What are paediatric causes of liver failure?
Excess paracetamol
viral hepatitis
Wilson’s disease and other metabolic conditions
How do you manage liver failure in paediatrics?
IV dextrose
empirical broad-spectrum antibiotics and antifungals
IV vitamin K and FFP
What are complications of paediatric liver failure?
Cerebral oedema haemorrhage gastritis coagulopathy sepsis pancreatitis
What is biliary atresia?
A progressive idiopathic necro inflammatory process that may involve a segment or the entire extrahepatic biliary tree
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
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 ++
What is the diagnostic investigation for biliary atresia?
cholangiogram showing decreased patency
liver biopsy can be used to differentiate from differential diagnoses
abdominal USS
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
What is a Wilms’ tumour?
nephroblastoma the most common cause of renal malignancy in children
What is the presentation of a Wilms’ tumour?
Abdominal/flank mass/swelling
abdominal distension
abdominal pain - in less than 30% of patients
pallor
What investigations are required for a Wilms’ tumour?
Ultrasound scan will show mass CT shows extents of disease (10% of patients have mets)
biopsy
What will urinalysis and renal function tests reveal in Wilms’ tumour?
Urinalysis= haematuria
renal function will be normal unless there are bilateral tumours
What is a hepatoblastoma?
An uncommon malignant liver cancer originating from immature liver precursor cells
Who is at risk of hepatblastoma?
Children with familial adenomatous polyposis
What is the presentation of hepatoblastoma?
Abdominal mass
what blood test is required in hepatoblastoma?
Alpha-fetoprotein (raised)
What conjenital infections cause neonatal hepatitis?
Cytomegalovirus
rubella
measles
hepatitis ABC
What are the causes of neonatal hepatitis?
Congenital infections
inborn metabolic errors
cystic fibrosis
rarer causes:
intestinal failure
progressive familial intrahepatic cholestasis
galactosaemia
What is familial intrahepatic cholestasis?
Inherited recessive mutation causing decreased bile production
What is the presentation of familial intrahepatic cholestasis?
prolonged new natal jaundice
pruritus
What are the consequences of familial intrahepatic cholestasis if untreated?
Failure to thrive
Ricketts
What is the GGT level in familial intrahepatic cholestasis?
Low
What is the management of familial intrahepatic cholestasis?
uredeoxycholic acide
vitamins ADEK
if v severe : transplant
What is galactosaemia?
High levels of galactose from inability to metabolise sugars causing eventual liver failure
What is a presentation of galactosaemia?
Neonatal jaundice
vomiting
poor feeding
hepatomegaly
What are the consequences of galactosaemia if not treated?
DIC
developmental delayed
cataracts
recurrent bacterial infections
How do you diagnose galactosaemia?
Urine dipstick And your analysis shows increased sugars (galactose)
How do you manage galactosaemia?
Sugar/galactose/ lactose free diet
What are inborn errors of metabolism?
cause faulty biosynthesis causing cholestasis
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)
How do you manage inborn errors of metabolism caused hepatitis?
uredeoxycholic acid
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
What are common causes of neonatal jaundice- conjugated?
Neonatal hepatitis
bile duct obstruction
What are common causes of neonatal jaundice – unconjugated?
Breastmilk jaundice
rubella
measles
hepatitis ABC
when is neonatal jaundice physiological?
Up to 14 days
Why does physiological neonatal jaundice occur?
fetal haemoglobin and red blood cells are replaced
What are treatments of neonatal jaundice?
phototherapy
Exchange transfusion: if bilirubin levels are very high/unstable
What is kernictus?
Occurs when jaundice is left untreated or Billy Rubin is very high
bilirubin deposits in the brain causing brain damage
What are the potential consequences of kernictus?
Cerebral palsy
developmental delay
hearing loss
What are symptoms of kernictus?
Irritable baby lethargic high-pitched cry floppy baby seizures as it progresses
What is the management of kernictus?
Exchange transfusion, supportive care and phototherapy
