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Phase 3a > Paediatrics Liver > Flashcards

Paediatrics Liver Flashcards

1
Q

WHAT IS NEONATAL HEPATITIS

A

INFLAMATION OF THE LIVER CAUSING PROLONGED NEONATAL JAUNDICE

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2
Q

WHAT ARE SOME NEONATAL FEATURES OF HEAPATITIS AT BIRTH

A

IUGR

HEPATOSPLENOMEGALY

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3
Q

WHAT ARE THE CAUSES OF CONJUGATED NEONATAL JAUNDICE

A

NEONATAL HEPATITIS

BILE DUCT OBSTRUCTION

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4
Q

WHAT ARE THE COMMON CAUSES OF NEONATAL HEPATITIS SYNDROME

A

CONJENTIAL INFECTIONS

INBORN METABOLIC ERRORS

CYSTIC FIBROSIS

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5
Q

WHAT ARE RARER CAUSES ON NEONATAL HEPATITIS SYNDROME

A

INTESTINAL FAILURE

PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLIESTASIS

GALACTOSEMIA

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6
Q

WHAT ARE CONJENITAL INFECTIONS WHICH CAN CAUSE NEONATAL HEPATITIS SYNDROME

A

CYTOMEGALOIRUS

RUBELLA

MEASLES

HEPATITIS A, B, C

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7
Q

WHAT ARE CAUSES OF UNCONJUGATED NEONATAL JAUNDICE

A

BREAST MILK JAUNDICE

HAEMOLYTIC ANAEMIAS

GI OBSTRUCTIONS

HYPOTHYROIDISM

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8
Q

WHAT IS PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS

A

IN INHERITED RECESSIVE MUTATION WHICH CAUSES DECREASED BILE PRODUCTION

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9
Q

WHAT ARE THE PRESENTATIONS OF PROGRESSIVE FAMILIAL INRTAHEPATIC CHOLESTASIS

A

JAUNDICE

PRURITIS

FAILURE TO THRIVE

DIARRHOEA

RICKETS

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10
Q

WHAT IS GGT

A

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

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11
Q

WHAT ARE THE LEVELS OF GGT IN FAILIAL INTRAHEPATIC CHOLESTASIS

A

LOW

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12
Q

HOW DO YOU TREAT PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS

A

UREDYOXYCHOLIC ACID

VITAMINS A D E K

VERY SEVERE = LIVER TRANSPLANT

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13
Q

WHAT IS GALACTOSEMIA

A

HIGH LEVELS OF GALACTOSE FROM INABILITY TO METABOLISE SUGARS

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14
Q

WHAT IS THE PRESENTATION OF GALACTOSEMIA

A

POOR FEEDING

VOMITING

JAUNDICE

HEPATOMEGALY

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15
Q

WHAT ARE TEH CONSEQUENCES OF GALACTOSEMIA

A

CATARACTS

DEVELOPMENTAL DELAY

LIVER FAILURE

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

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16
Q

HOW DO YOU DIAGNOSE GALACTOSEMIA

A

URINE DIPSTICK

  • INCREASED GALACTOSE
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17
Q

HOW WOULD YOU MANAGE GALACTOSEMIA

A

GALACTOSE FREE DIET AND TREAT ANY COMPLICATIONS

GALACTOSE IS FORMED FROM LACTOSE BREAKDOWN

(LACTOSE -> GLUCOSE + GALACTOSE )

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18
Q

WHAT IS ALPHA-1 ANTITRIPSIN DEFICIENCY

A

AN AUTOSOMAL RECESSIVE DISEASE HEAVILY ASSICIATED WITH LVER DISEASE AND EMPHESEMA

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19
Q

HOW DOES ALPHA -1 ANTITRIPSIN DEFICIENCY PRESENT?

A

PROLONGED NEONATAL JAUNDICE

BRUISING/PROLONGED BLEEDING

HEPATOSPLENOMEGALY

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20
Q

WHAT ARE CONSEQUENCES OF SPNELOMEGALY

A

PORTAL HYPERTENSION

CIRHOSSIS

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21
Q

HOW DO YOU DIAGNOSE ALPHA-1 ANTITRIPSIN DEFICIENCY BE DIAGNOSED

A

BLOOD PLASMA

  • DECREASED A1AT LEVELS

GENOTYPE TESTING

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22
Q

WHAT IS THE TREATMENT OF ALPHA-1 ANTITRIPSIN DEFICIENCY?

A

IV A1AT INFUSION

TREATEMENT OF LIVER FAILURE

  • ANTIHYPERTENSIVES
  • DIURETICS
  • TRANSPLANT

LIFESTYLE ADVICE IE NO SMOKING OR DRINKING

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23
Q

WHAT IS THE PROGNOSIS OF ALPHA-1 ANTITRIPSIN DEFICIENCY

A

50% GOOD PROGNOSIS

50% LIVER DISEASE

24
Q

WHAT IS THE PATHOLOGY OF INBORN ERRORS OF NEONATAL HEPATITIS SYNDROME

A

INBORN ERRORS CAUSE FAULTY BILE SYNTHESIS CAUSING CHOLESTASIS

25
Q

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

A

URYNALISIS

  • ELEVATED CHOLENOIC BILE ACID

BLOODS

  • NORMAL GGT
26
Q

HOW WOULD YOU TREAT INBORN ERRORS IF BILE ACID SYNTHESIS

A

UREDEOXYCHOLID ACID

27
Q

WHAT ARE CHOLEDUCTAL CYSTS

A

CYSTIC DILATATIONS OF THE EXTRA HEPATIC BILLARY SYSTEM

28
Q

HOW DO CHOLEDUCTAL CYSTS PRESENT

A

25% PRESENT IN INFANCY WITH CHOLESTASIS (+JAUNDICE)

75% ABDO PAIN, PALPABLE ABDOMINAL MASS, JAUNDICE, CHOLANGITIS

29
Q

WHAT IS THE DEFINITION OF CHOLESTASIS

A

IMPAIRED BILE FROM FROM LIVER TO DUODENUM

30
Q

WHAT IS THE DEFINITION OF CHOLANGITIS

A

INFLAMMSTION OF THE BILE DUCT CAUSING

  • JAUNDICE
  • ADBO PAIN
  • HYPOTENSION
  • FEVER
31
Q

HOW WOULD YOU DIAGNOSE CHOLEDUCTAL CYSTS

A

US

32
Q

WHAT IS THE TREATEMENT OF CHOLEDUCTAL CYSTS

A

SURGICAL EXCISION

33
Q

WHAT RISKS ARE ASSOCIATED WITH CHOLEDUCTAL CYSTS

A

INCREASED RISK OF CHOLANGITIS AND BILLARY TREE MALIGNANCY

34
Q
A
35
Q

what is billary atresia

A

a congenital or acquired absent/blocked/narrowed bile duct

36
Q

what is the presentation of billary atresia

A

prolonged neonatal jaundice

ADEK malabsorbtion

failure to thrive

Bleeding and bruising

37
Q

what can billary atresia lead to

A

portal HTN

liver failure

cirrosis

38
Q

what are they types of billary atresia

A

t1= restricted to commn bilde duct

t2= involving common hepatic duct

t3= involving proximal bile ducts

39
Q

what is the treatement of billary atresia

A

surgery

40
Q

what is wilsons disease

A

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

41
Q

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

A

liver

  • chronic liver failure
  • fulminent liver failure

basal ganglia

  • dementia

cornea

  • kiser-fleischer rings
42
Q

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

A

check serum cleuroplasmin (decreased)

liver biopsy (increased copper)

Urynalisis (increased copper)

43
Q

what are the treatements for wilsons disease

A

penicilllamine

zinc

liver transplant

44
Q

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

A

fulminant liver failure

45
Q

what is fulminant liver failure

A

sudden massive liver necrosis

46
Q

what is the presentation of fulminant liver failure

A

encephalopathy

tremour of hand at extension (asterisks)

Jaundice

septic like: tachycardia, hypotensive etc

47
Q

how do you manage fulminant liver failure

A

lactulose (reduces ammonia absorbtion)

icu

liver transplant

48
Q

what is acute liver failure

A

development of massive liver necrosis and loss of liver function

49
Q

what can be associated with acute liver failure

A

hepatic encephalopathy

50
Q

what is hepatic encephalopathy

A

altered level of conciousness as a result of liver failure

51
Q

what are causes of acute liver failure

A

XS paracetamol

viral hepatitis

wilsons and other metabolic conditions

reyes syndrome

52
Q

what is the presentation of acute liver failure

A

Jaundice

Encephalopathy

Coagulopathy

Hyperglycaemia

electrolyte disfunction

Drowsiness

53
Q

what are complications of acute liver failure

A

cerebral oedema

haemorrage]gastritis

coagulopathies

sepsis

pancreatitis

54
Q

how do you diagnose acute liver failure

A

bloods

  • high transaminases, ALP, and plansma ammonia

EEG

CT head

55
Q

what is the management of acuteliver failure

A

IV dextrose

Empirical Broad spec Abx + ANtifunglas

IV Vit K

fresh frozen plasma

56
Q
A

Phase 3a (22 decks)

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