Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Y5 Paediatrics > Liver > Flashcards

Liver Flashcards

1
Q

What are causes of unconjugated prolonged jaundice

A 
Breastmilk
infection 
Haemolytic anaemia 
Hypothyroidism 
High GI obstruction 
Crigler-Najjar syndrome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are causes of conjugated prolonged jaundice

A

Bile duct obstruction (biliary atresia, cholechodal cyst)

Neonatal hepatitis syndrome (infection, error of metabolism, alpha-1-antitrypsin, galactosaemia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How do you determine what kind of intervention a baby needs for jaundice?

A

using bilirubin threshold table

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

When do you consider intensified phototherapy

A

if serum bilirubin levels are rising rapidly

if bilirubin fails to repost to phototherapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How do you assess for underlying disease in jaundice

A
  • measure serum bilirubin
  • Htc
  • Blood group
  • DAT
  • Find out if mother received prophylactic anti D
  • FBC, blood film
  • blood G6PD
  • LFT
  • Sepsis screen if infection suspected
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

When do you perform exchange transfusion

A

If above threshold on graph OR baby shows signs of bilirubin encephalopathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What must you NOT stop during exchange transfusipn

A

Phototherapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What resources can you give for neonatal jaundice

A

NHS Choices Neonatal Jaundice Factsheet
The Breastfeeding Network (information and support for breastfeeding mothers)
Bliss (premature/sick babies)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is biliary atrasi

A

Progressive fibrosis and obliteration of extra hepatic and intrahepatic biliary tree

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the cause of biliary atrasia

A

UNKNOWN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the outcome for untreated biliary atresia

A

death within TWO years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the presentation for biliary atresia

A 
jaundice 
pale stools 
normal BW 
faltering groeth
hepatomegaly, splenomegaly
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are investigations for biliary atrasia

A 
Raised conjugated bilirubin 
abnormal LFT 
Fasting abdo ultrasound 
Cholangiogram 
Liver biopsy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is management for biliary atrasia

A

Kasai hepatoportoenterosotomy
Liver transplant

Surgical intervention is recommended within 60 days of life

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are complications of biliary atresia

A

growth failure
portal hypertension
cholangitis
ascitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How do you manage complications of biliary atresia

A

ursodeoxycholic acid (promote bile flow)
fat soluble vitamins
prophylactic antibiotics e.g. co-trimoxazole to prevent cholangitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is a choledochal cyst

A

Cystic dilatation of extra hepatic biliary system

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

How does choledochal cyst present

A

neonate: jaundice

older children: abdo pain, palpable mass, jaundice, cholagitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

How do you diagnose choledochal cyst

A

USS /MRCP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

How do you treat choledochal cyst

A

surgical excision of cyst with Roux en Y anastomosis to biliary duct

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is neonatal hepatitis syndrome

A

Prolonged neonatal jaundice
Hepatic inflammation
LBW, faltering growth

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What is Alagille Syndrome - clinical its

A

Rare autosomal dominan

Clinica fts:

  • triangular facies
  • skeletal abnormalities
  • congenital heart disorders
  • renal tubular disorders
  • defects of eye
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

How do you manage Alagille syndrome

A

Nutrition and fat soluble vitamins
May require liver transplant
Most survive to adulthood

24
Q

What are two autosomal recessive causes of jaundice

A

Familial intrahepatic cholestasis

Alpha 1 antitrypsin deficiency

25
Q

what is galactosaemia

A

metabolic disorder

inability to metabolise galactose

26
Q

\What are features of galactosaemi

A

When fed milk:

  • poor feeding
  • vomiting
  • jaundice
  • hepatomegaly
27
Q

What occurs if galactosemia is untreated

A

liver failure
cataracts
developmental delay

28
Q

What are investigations for galactosaemia

A
  • galactose in urine

- Galactose-1-Phosphate-Uridyl Trasnferase in red cells

29
Q

How do you manage galactosaemia

A

GALACTOSE FREE DIET

30
Q

What is acute liver failure

A

AKA FULMINANT HEPATITIS

massive hepatic necrosis with loss of liver function

31
Q

What are causes of acute liver failure in children <2 years old

A
  • infection
  • metabolic disease
  • AI hepatitis
  • drug induced
  • haemochromatosis
32
Q

What are causes of chronic liver disease in children

A

Hepatitis B,C
AI hepatitis
Drug induced hepatitis
Non alcoholic fatty liver disease

Genetic:

  • Wilson0s
  • CF
  • Alpha 1 antitrypsin deficiency
  • Sclerosing cholangitis
33
Q

what is Wilson’s disease

A

Reduced synthesis of caeruloplasmin (copper binding protein) > copper accumulation in liver, brain, kidney , cornea

34
Q

How does a child with Wilson’s disease present

A

Deterioration in school , mood and behaviour change
EPS eg. incoordination, tremor, dysarthria
KEISER - FLEISHER RINGS (after 7 yo)

35
Q

How do you investigate wilson disease

A

low serum caeruloplasmin
Low serum copper
increased urinary copper excretion
elevated hepatic copper on liver biopsy / identification of gene mutation

36
Q

How do you manage wilson disease

A

Zinc (blocks copper resorption in intestine)
Trientine (increases urinary copper excretion)
Pyridoxine (B6) (prevents peripheral neuropathy)

Consider liver transplant if end stage disease

37
Q

What are complications of chronic liver disease

A

NUTRITION (fat malabsorption, protein malnutrition, anorexia)

PRURITUS

ENCEPALOPATHY

38
Q

How do you manage nutrition problem in children

A

Supplement fat soluble vitamins
Encourage protein intake (unless encephalopathy)
NG tube / parenteral feeding if required

39
Q

How do you manage pruritus in children

A

loose cotton clothing
keep nails short
emollients

Medicitions :

  • phenobarbital (stimulates bile flow)
  • cholestyramine (bile salt resin to absorb bile salts)
  • ursodeoxycholic acid (solubilises bile)
  • rifampicin (enzyme infucer)
40
Q

How od you manage encepalopathy

A

REDUCE NITROGENOUS LOAD

  • diary protein restriction
  • lactulose (prevents absorption)
41
Q

WHAT IS Mode of inheritance of alpha 1 antitrypsin

A

Autosomal rec sessile

42
Q

What occurs in alpha 1 antitrypsin deficiency

A

alpha 1 antitrypsin is abnormally folded > cannot leave liver as it normally would > cannot act systemically

  • LIVER: hepatic damage
  • Lungs: emphysema (as neutrophile elastase is not neutralised) \
43
Q

what are clinical features of alpha 1 antitrypsin def

A 
Prolonged neonatal jaundice
Bleeding (vit K defi) 
Hepatomegh 
Splenomeg 
Pulmonary disease in ADULT LIFE
44
Q

How do you investigate alpha 1 antitrypsin def

A

measure alpha 1 antitrypsin levels in plasma

45
Q

how do you manage alpha 1 antitrypsin def

A

avoid smoking and pollution
Limit alcohol
Treat pulmonary problems like COPD (bronchodilators, ICS, pulm rehab, vaccine)
Treat liver manifestations like other liver diseases (monitor for coagulopathy, diuretics for ascites, OGD for varices, liver transplant)

46
Q

How many children develop chronic Hep B liver disease

A

30-50% !!!!

47
Q

When should you wait to start treating Hep C

A

3 years old

because vertically acquired infections may resolve spontaneously before then

48
Q

Who is Hep E very dfangerous in

A

Pregnant women

Causes fulminant hepatic failure and high mortality rate

49
Q

How do you manage non-alcoholic fatty liver disease

A 
Weight loss 
Tx insulin resistance, diabetes
Statins 
Vit C, E 
Ursodeoxycholic acid to improve bile flow
50
Q

What are complications of chronic liver disease

A

NUTRITION

  • fat malabsorption
  • protein malnutrition
  • anorexia

PRURITYS

51
Q

Why does chronic liver disease cause fat malabsorption

A

Because without bile you cannot effectively absolve long chain fat

52
Q

Why does CLD cause protein malnutrition

A

poor intake + high catabolic rate of diseased liver

53
Q

how do you manage autoimmune hepatitis

A

prednisolone + azathioprine

54
Q

how can you manage hepatic encepalopathy

A
  • Supportive (frequent monitoring of mental status)
  • Identify and correct precipitating factors
  • Reduce nitrogenous load (dietary protein restriction, reduce load via LACTULOSE or RIFAXIMIN)
55
Q

What is Reye syndrome

A

Acute non-inflammatory encepalopathy
With microvascular fatty infiltrations in the liver

ASSOCIATED WITH ASPIRIN IN CHILDREN

Y5 Paediatrics (30 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions