Paediatrics Liver Flashcards
WHAT IS NEONATAL HEPATITIS
INFLAMATION OF THE LIVER CAUSING PROLONGED NEONATAL JAUNDICE
WHAT ARE SOME NEONATAL FEATURES OF HEAPATITIS AT BIRTH
IUGR
HEPATOSPLENOMEGALY
WHAT ARE THE CAUSES OF CONJUGATED NEONATAL JAUNDICE
NEONATAL HEPATITIS
BILE DUCT OBSTRUCTION
WHAT ARE THE COMMON CAUSES OF NEONATAL HEPATITIS SYNDROME
CONJENTIAL INFECTIONS
INBORN METABOLIC ERRORS
CYSTIC FIBROSIS
WHAT ARE RARER CAUSES ON NEONATAL HEPATITIS SYNDROME
INTESTINAL FAILURE
PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLIESTASIS
GALACTOSEMIA
WHAT ARE CONJENITAL INFECTIONS WHICH CAN CAUSE NEONATAL HEPATITIS SYNDROME
CYTOMEGALOIRUS
RUBELLA
MEASLES
HEPATITIS A, B, C
WHAT ARE CAUSES OF UNCONJUGATED NEONATAL JAUNDICE
BREAST MILK JAUNDICE
HAEMOLYTIC ANAEMIAS
GI OBSTRUCTIONS
HYPOTHYROIDISM
WHAT IS PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS
IN INHERITED RECESSIVE MUTATION WHICH CAUSES DECREASED BILE PRODUCTION
WHAT ARE THE PRESENTATIONS OF PROGRESSIVE FAMILIAL INRTAHEPATIC CHOLESTASIS
JAUNDICE
PRURITIS
FAILURE TO THRIVE
DIARRHOEA
RICKETS
WHAT IS GGT
GAMMA GLUTAMYL TRANSFERASE, AN ENZYME WHICH RISES IN BILE DUCT OBSTRUCTION
WHAT ARE THE LEVELS OF GGT IN FAILIAL INTRAHEPATIC CHOLESTASIS
LOW
HOW DO YOU TREAT PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS
UREDYOXYCHOLIC ACID
VITAMINS A D E K
VERY SEVERE = LIVER TRANSPLANT
WHAT IS GALACTOSEMIA
HIGH LEVELS OF GALACTOSE FROM INABILITY TO METABOLISE SUGARS
WHAT IS THE PRESENTATION OF GALACTOSEMIA
POOR FEEDING
VOMITING
JAUNDICE
HEPATOMEGALY
WHAT ARE TEH CONSEQUENCES OF GALACTOSEMIA
CATARACTS
DEVELOPMENTAL DELAY
LIVER FAILURE
RECURRANT BACTERIAL INFECTIONS FROM HIGH BLOOD SUGARS OFTEN RESULTING IN DISSEMINATED INTRAVASCULAR COAGULATION
HOW DO YOU DIAGNOSE GALACTOSEMIA
URINE DIPSTICK
- INCREASED GALACTOSE
HOW WOULD YOU MANAGE GALACTOSEMIA
GALACTOSE FREE DIET AND TREAT ANY COMPLICATIONS
GALACTOSE IS FORMED FROM LACTOSE BREAKDOWN
(LACTOSE -> GLUCOSE + GALACTOSE )
WHAT IS ALPHA-1 ANTITRIPSIN DEFICIENCY
AN AUTOSOMAL RECESSIVE DISEASE HEAVILY ASSICIATED WITH LVER DISEASE AND EMPHESEMA
HOW DOES ALPHA -1 ANTITRIPSIN DEFICIENCY PRESENT?
PROLONGED NEONATAL JAUNDICE
BRUISING/PROLONGED BLEEDING
HEPATOSPLENOMEGALY
WHAT ARE CONSEQUENCES OF SPNELOMEGALY
PORTAL HYPERTENSION
CIRHOSSIS
HOW DO YOU DIAGNOSE ALPHA-1 ANTITRIPSIN DEFICIENCY BE DIAGNOSED
BLOOD PLASMA
- DECREASED A1AT LEVELS
GENOTYPE TESTING
WHAT IS THE TREATMENT OF ALPHA-1 ANTITRIPSIN DEFICIENCY?
IV A1AT INFUSION
TREATEMENT OF LIVER FAILURE
- ANTIHYPERTENSIVES
- DIURETICS
- TRANSPLANT
LIFESTYLE ADVICE IE NO SMOKING OR DRINKING
WHAT IS THE PROGNOSIS OF ALPHA-1 ANTITRIPSIN DEFICIENCY
50% GOOD PROGNOSIS
50% LIVER DISEASE
WHAT IS THE PATHOLOGY OF INBORN ERRORS OF NEONATAL HEPATITIS SYNDROME
INBORN ERRORS CAUSE FAULTY BILE SYNTHESIS CAUSING CHOLESTASIS
WHAT ARE INVESTIGATIONS (AND RESULTS) OF DIAGNOSING INBORN ERRORS OF BILE SYNTHESIS
URYNALISIS
- ELEVATED CHOLENOIC BILE ACID
BLOODS
- NORMAL GGT
HOW WOULD YOU TREAT INBORN ERRORS IF BILE ACID SYNTHESIS
UREDEOXYCHOLID ACID
WHAT ARE CHOLEDUCTAL CYSTS
CYSTIC DILATATIONS OF THE EXTRA HEPATIC BILLARY SYSTEM
HOW DO CHOLEDUCTAL CYSTS PRESENT
25% PRESENT IN INFANCY WITH CHOLESTASIS (+JAUNDICE)
75% ABDO PAIN, PALPABLE ABDOMINAL MASS, JAUNDICE, CHOLANGITIS
WHAT IS THE DEFINITION OF CHOLESTASIS
IMPAIRED BILE FROM FROM LIVER TO DUODENUM
WHAT IS THE DEFINITION OF CHOLANGITIS
INFLAMMSTION OF THE BILE DUCT CAUSING
- JAUNDICE
- ADBO PAIN
- HYPOTENSION
- FEVER
HOW WOULD YOU DIAGNOSE CHOLEDUCTAL CYSTS
US
WHAT IS THE TREATEMENT OF CHOLEDUCTAL CYSTS
SURGICAL EXCISION
WHAT RISKS ARE ASSOCIATED WITH CHOLEDUCTAL CYSTS
INCREASED RISK OF CHOLANGITIS AND BILLARY TREE MALIGNANCY
what is billary atresia
a congenital or acquired absent/blocked/narrowed bile duct
what is the presentation of billary atresia
prolonged neonatal jaundice
ADEK malabsorbtion
failure to thrive
Bleeding and bruising
what can billary atresia lead to
portal HTN
liver failure
cirrosis
what are they types of billary atresia
t1= restricted to commn bilde duct
t2= involving common hepatic duct
t3= involving proximal bile ducts
what is the treatement of billary atresia
surgery
what is wilsons disease
a genetic mutation of ATP7B gene causing a decrease in billary copper secretion and causing copper buildup
where does copper buildup and what does that cause in wilsons disease
liver
- chronic liver failure
- fulminent liver failure
basal ganglia
- dementia
cornea
- kiser-fleischer rings
what tests would you perform for wilsons disease and what are the results
check serum cleuroplasmin (decreased)
liver biopsy (increased copper)
Urynalisis (increased copper)
what are the treatements for wilsons disease
penicilllamine
zinc
liver transplant
what can occur due to build up of copper in the liver in wilsons disease
fulminant liver failure
what is fulminant liver failure
sudden massive liver necrosis
what is the presentation of fulminant liver failure
encephalopathy
tremour of hand at extension (asterisks)
Jaundice
septic like: tachycardia, hypotensive etc
how do you manage fulminant liver failure
lactulose (reduces ammonia absorbtion)
icu
liver transplant
what is acute liver failure
development of massive liver necrosis and loss of liver function
what can be associated with acute liver failure
hepatic encephalopathy
what is hepatic encephalopathy
altered level of conciousness as a result of liver failure
what are causes of acute liver failure
XS paracetamol
viral hepatitis
wilsons and other metabolic conditions
reyes syndrome
what is the presentation of acute liver failure
Jaundice
Encephalopathy
Coagulopathy
Hyperglycaemia
electrolyte disfunction
Drowsiness
what are complications of acute liver failure
cerebral oedema
haemorrage]gastritis
coagulopathies
sepsis
pancreatitis
how do you diagnose acute liver failure
bloods
- high transaminases, ALP, and plansma ammonia
EEG
CT head
what is the management of acuteliver failure
IV dextrose
Empirical Broad spec Abx + ANtifunglas
IV Vit K
fresh frozen plasma
