Paediatrics Liver

Question 1

WHAT IS NEONATAL HEPATITIS

Answer 1

INFLAMATION OF THE LIVER CAUSING PROLONGED NEONATAL JAUNDICE

Question 2

WHAT ARE SOME NEONATAL FEATURES OF HEAPATITIS AT BIRTH

Answer 2

IUGR

HEPATOSPLENOMEGALY

Question 3

WHAT ARE THE CAUSES OF CONJUGATED NEONATAL JAUNDICE

Answer 3

NEONATAL HEPATITIS

BILE DUCT OBSTRUCTION

Question 4

WHAT ARE THE COMMON CAUSES OF NEONATAL HEPATITIS SYNDROME

Answer 4

CONJENTIAL INFECTIONS

INBORN METABOLIC ERRORS

CYSTIC FIBROSIS

Question 5

WHAT ARE RARER CAUSES ON NEONATAL HEPATITIS SYNDROME

Answer 5

INTESTINAL FAILURE

PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLIESTASIS

GALACTOSEMIA

Question 6

WHAT ARE CONJENITAL INFECTIONS WHICH CAN CAUSE NEONATAL HEPATITIS SYNDROME

Answer 6

CYTOMEGALOIRUS

RUBELLA

MEASLES

HEPATITIS A, B, C

Question 7

WHAT ARE CAUSES OF UNCONJUGATED NEONATAL JAUNDICE

Answer 7

BREAST MILK JAUNDICE

HAEMOLYTIC ANAEMIAS

GI OBSTRUCTIONS

HYPOTHYROIDISM

Question 8

WHAT IS PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS

Answer 8

IN INHERITED RECESSIVE MUTATION WHICH CAUSES DECREASED BILE PRODUCTION

Question 9

WHAT ARE THE PRESENTATIONS OF PROGRESSIVE FAMILIAL INRTAHEPATIC CHOLESTASIS

Answer 9

JAUNDICE

PRURITIS

FAILURE TO THRIVE

DIARRHOEA

RICKETS

Question 10

WHAT IS GGT

Answer 10

GAMMA GLUTAMYL TRANSFERASE, AN ENZYME WHICH RISES IN BILE DUCT OBSTRUCTION

Question 11

WHAT ARE THE LEVELS OF GGT IN FAILIAL INTRAHEPATIC CHOLESTASIS

Answer 11

LOW

Question 12

HOW DO YOU TREAT PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS

Answer 12

UREDYOXYCHOLIC ACID

VITAMINS A D E K

VERY SEVERE = LIVER TRANSPLANT

Question 13

WHAT IS GALACTOSEMIA

Answer 13

HIGH LEVELS OF GALACTOSE FROM INABILITY TO METABOLISE SUGARS

Question 14

WHAT IS THE PRESENTATION OF GALACTOSEMIA

Answer 14

POOR FEEDING

VOMITING

JAUNDICE

HEPATOMEGALY

Question 15

WHAT ARE TEH CONSEQUENCES OF GALACTOSEMIA

Answer 15

CATARACTS

DEVELOPMENTAL DELAY

LIVER FAILURE

RECURRANT BACTERIAL INFECTIONS FROM HIGH BLOOD SUGARS OFTEN RESULTING IN DISSEMINATED INTRAVASCULAR COAGULATION

Question 16

HOW DO YOU DIAGNOSE GALACTOSEMIA

Answer 16

URINE DIPSTICK

• INCREASED GALACTOSE

Question 17

HOW WOULD YOU MANAGE GALACTOSEMIA

Answer 17

GALACTOSE FREE DIET AND TREAT ANY COMPLICATIONS

GALACTOSE IS FORMED FROM LACTOSE BREAKDOWN

(LACTOSE -> GLUCOSE + GALACTOSE )

Question 18

WHAT IS ALPHA-1 ANTITRIPSIN DEFICIENCY

Answer 18

AN AUTOSOMAL RECESSIVE DISEASE HEAVILY ASSICIATED WITH LVER DISEASE AND EMPHESEMA

Question 19

HOW DOES ALPHA -1 ANTITRIPSIN DEFICIENCY PRESENT?

Answer 19

PROLONGED NEONATAL JAUNDICE

BRUISING/PROLONGED BLEEDING

HEPATOSPLENOMEGALY

Question 20

WHAT ARE CONSEQUENCES OF SPNELOMEGALY

Answer 20

PORTAL HYPERTENSION

CIRHOSSIS

Question 21

HOW DO YOU DIAGNOSE ALPHA-1 ANTITRIPSIN DEFICIENCY BE DIAGNOSED

Answer 21

BLOOD PLASMA

• DECREASED A1AT LEVELS

GENOTYPE TESTING

Question 22

WHAT IS THE TREATMENT OF ALPHA-1 ANTITRIPSIN DEFICIENCY?

Answer 22

IV A1AT INFUSION

TREATEMENT OF LIVER FAILURE

• ANTIHYPERTENSIVES
• DIURETICS
• TRANSPLANT

LIFESTYLE ADVICE IE NO SMOKING OR DRINKING

Question 23

WHAT IS THE PROGNOSIS OF ALPHA-1 ANTITRIPSIN DEFICIENCY

Answer 23

50% GOOD PROGNOSIS

50% LIVER DISEASE

Question 24

WHAT IS THE PATHOLOGY OF INBORN ERRORS OF NEONATAL HEPATITIS SYNDROME

Answer 24

INBORN ERRORS CAUSE FAULTY BILE SYNTHESIS CAUSING CHOLESTASIS

Question 25

WHAT ARE INVESTIGATIONS (AND RESULTS) OF DIAGNOSING INBORN ERRORS OF BILE SYNTHESIS

Answer 25

URYNALISIS * ELEVATED CHOLENOIC BILE ACID BLOODS * NORMAL GGT

Question 26

HOW WOULD YOU TREAT INBORN ERRORS IF BILE ACID SYNTHESIS

Answer 26

UREDEOXYCHOLID ACID

Question 27

WHAT ARE CHOLEDUCTAL CYSTS

Answer 27

CYSTIC DILATATIONS OF THE EXTRA HEPATIC BILLARY SYSTEM

Question 28

HOW DO CHOLEDUCTAL CYSTS PRESENT

Answer 28

25% PRESENT IN INFANCY WITH CHOLESTASIS (+JAUNDICE) 75% ABDO PAIN, PALPABLE ABDOMINAL MASS, JAUNDICE, CHOLANGITIS

Question 29

WHAT IS THE DEFINITION OF CHOLESTASIS

Answer 29

IMPAIRED BILE FROM FROM LIVER TO DUODENUM

Question 30

WHAT IS THE DEFINITION OF CHOLANGITIS

Answer 30

INFLAMMSTION OF THE BILE DUCT CAUSING - JAUNDICE - ADBO PAIN - HYPOTENSION - FEVER

Question 31

HOW WOULD YOU DIAGNOSE CHOLEDUCTAL CYSTS

Answer 31

US

Question 32

WHAT IS THE TREATEMENT OF CHOLEDUCTAL CYSTS

Answer 32

SURGICAL EXCISION

Question 33

WHAT RISKS ARE ASSOCIATED WITH CHOLEDUCTAL CYSTS

Answer 33

INCREASED RISK OF CHOLANGITIS AND BILLARY TREE MALIGNANCY

Question 35

what is billary atresia

Answer 35

a congenital or acquired absent/blocked/narrowed bile duct

Question 36

what is the presentation of billary atresia

Answer 36

prolonged neonatal jaundice ADEK malabsorbtion failure to thrive Bleeding and bruising

Question 37

what can billary atresia lead to

Answer 37

portal HTN liver failure cirrosis

Question 38

what are they types of billary atresia

Answer 38

t1= restricted to commn bilde duct t2= involving common hepatic duct t3= involving proximal bile ducts

Question 39

what is the treatement of billary atresia

Answer 39

surgery

Question 40

what is wilsons disease

Answer 40

a genetic mutation of ATP7B gene causing a decrease in billary copper secretion and causing copper buildup

Question 41

where does copper buildup and what does that cause in wilsons disease

Answer 41

liver * chronic liver failure * fulminent liver failure basal ganglia * dementia cornea * kiser-fleischer rings

Question 42

what tests would you perform for wilsons disease and what are the results

Answer 42

check serum cleuroplasmin (decreased) liver biopsy (increased copper) Urynalisis (increased copper)

Question 43

what are the treatements for wilsons disease

Answer 43

penicilllamine zinc liver transplant

Question 44

what can occur due to build up of copper in the liver in wilsons disease

Answer 44

fulminant liver failure

Question 45

what is fulminant liver failure

Answer 45

sudden massive liver necrosis

Question 46

what is the presentation of fulminant liver failure

Answer 46

encephalopathy tremour of hand at extension (asterisks) Jaundice septic like: tachycardia, hypotensive etc

Question 47

how do you manage fulminant liver failure

Answer 47

lactulose (reduces ammonia absorbtion) icu liver transplant

Question 48

what is acute liver failure

Answer 48

development of massive liver necrosis and loss of liver function

Question 49

what can be associated with acute liver failure

Answer 49

hepatic encephalopathy

Question 50

what is hepatic encephalopathy

Answer 50

altered level of conciousness as a result of liver failure

Question 51

what are causes of acute liver failure

Answer 51

XS paracetamol viral hepatitis wilsons and other metabolic conditions reyes syndrome

Question 52

what is the presentation of acute liver failure

Answer 52

Jaundice Encephalopathy Coagulopathy Hyperglycaemia electrolyte disfunction Drowsiness

Question 53

what are complications of acute liver failure

Answer 53

cerebral oedema haemorrage]gastritis coagulopathies sepsis pancreatitis

Question 54

how do you diagnose acute liver failure

Answer 54

bloods * high transaminases, ALP, and plansma ammonia EEG CT head

Question 55

what is the management of acuteliver failure

Answer 55

IV dextrose Empirical Broad spec Abx + ANtifunglas IV Vit K fresh frozen plasma