The Yellow Baby Flashcards
(62 cards)
1
Q
Function of the liver
A
Produces Essential proteins - albumin - clotting factors Absorption and metabolism of food - fat - carbohydrate Storage - glycogen - other substrates and minerals Metabolism and excretion of toxins
2
Q
LFTs
A
Bilirubin - Total - Split ALT / AST Alkaline phosphatase GGT
3
Q
What is “split bilirubin”?
A
Direct (conjugated) and indirect (unconjugated)
4
Q
What does bilirubin indicate?
A
How well you liver is clearing bile
5
Q
What are ALT / AST elevated in?
A
Hepatocellular damage (hepatitis) Other processes e.g. muscle diseases
6
Q
What are ALT / AST?
A
Transaminases
7
Q
When is alkaline phosphatase elevated?
A
Biliary disease
8
Q
Which LFT is usually ignored in children and why?
A
Alkaline phosphate
Because it is affected by bone growth and so can fluctuate and be high but nothing to do with their liver at al
9
Q
When is GGT elevated?
A
Biliary disease
10
Q
What does GGT stand for?
A
Gamma glutamyl transferase
11
Q
Tests to assess liver function
A
Coagulation - prothrombin time PT/INR - APTT Albumin Bilirubin Blood glucose Ammonia
12
Q
Presentation of paediatric liver disease
A
JAUNDICE abnormal incidental finding on blood test Chronic ; Encephalopathy Epistaxis Spider naevi Varices with portal HTN muscle wasting from malnutrition bruising and petechiae splenomegaly with portal HTN Hypersplenism Hepatorenal failure Liver palms Clubbing Loss of fat stores secondary to malnutrition Hypotonia Ascites Peripheral neuropathy
13
Q
What is jaundice?
A
A yellow discolouration of skin and tissues due to accumulation of bilirubin
14
Q
Where Is jaundice usually most obvious?
A
Sclera
15
Q
At what level is bilirubin usually visible?
A
> 40 - 50 umol/l
16
Q
Bilirubin metabolism
A
- Break down of RBCs into biliverdin
- Converted into unconjugated bilirubin by biliverdin reductase of tissues which happens all over the body
- Bilirubin goes to the liver and is converted to conjugated bilirubin - which goes to bile and gallbladder and is converted in small intestine into urobillogen
- either excreted by kidneys or going in stercobilin and is excreted into stool
17
Q
What does stercobilin do?
A
Makes faeces the colour they are
18
Q
Solubility of unconjugated bilirubin
A
Water insoluble
Fat soluble
19
Q
Features of unconjugated bilirubin
A
Cant be directly excreted into kidneys or stool
20
Q
What is neonatal jaundice classified by?
A
Age
21
Q
What class of neonatal jaundice is ALWAYS pathological?
A
Early ( < 24 hrs old)
22
Q
Causes of early ( <24 hours old) jaundice
A
Haemolysis
Sepsis
23
Q
How old is intermediate neonatal jaundice?
A
24 hours - 2 weeks
24
Q
Causes of intermediate neonatal jaundice
A
Physiological Breast milk Sepsis Haemolysis Abnormal conjugation of bilirubin
25
How long is prolonged neonatal jaundice?
> 2 weeks old
| 3 weeks for pre term infants
26
Causes of prolonged neonatal jaundice
Extrahepatic obstruction
Neonatal hepatitis
Hypothyroidism
Breast milk
27
Why do infants get neonatal jaundice?
Shorter RBC life span in infants (80-90 days)
Relative polycythaemia (180-200)
So loads of unconjugated bilirubin is produced
Relative immaturity of liver function
28
When does physiological jaundice occur?
AFTER the first day of life
29
What type of jaundice is physiological jaundice?
Unconjugated jaundice
30
What type of jaundice Is breast milk jaundice?
Unconjugated jaundice
31
How long can breast milk jaundice persist for?
up to 12 weeks
32
Why does sepsis cause jaundice?
Increases haemolysis
| Slows down liver function
33
Causes of haemolysis causing jaundice in the neonate
```
ABO incompatibility
- excessive haemolysis
Rheus disease
- excessive haemolysis
Bruising / cephallhaematoma from birth trauma
- excess of bilirubin production
Red cell membrane defects e.g. spherocytosis
- excessive haemolysis
Red cell enzyme defects e.g. G6PD
- excesshaemolysis
```
34
What causes abnormal conjugation of bilirubin?
Gilberts Disease
| Criler-Najjar syndrome
35
What is kernicterus a complication of?
Neonatal unconjugated jaundice
36
Pathology of kernicterus
Unconjugated bilirubin is fat soluble and so can cross the BBB
Neurotoxic and deposits in brain
- especially basal ganglia
37
Presentation of kernicterus
```
Early signs - encephalopathy
- poor feeding
- lethargy
- seizures
Late consequences
- severe choreathetoid CP
- LDs
- sensorineural deafness
```
38
Which type of bilirubin can cross the BBB?
Unconjugated
39
Treatment of neonatal jaundice
Phototherapy
40
What type of jaundice does phototherapy treat?
Unconjugated
41
What does phototherapy do?
Visible light (NOT UV) converts unconjugated bilirubin to water soluble isomer (photoisomerization)
42
What is exchange transfusion?
When phototherapy sometimes doesn't bring it down efficiently, and have to do this more excessively, then may need a blood transfusion past this threshold
43
What type of jaundice always requires further investigations?
Prolonged
44
Causes of unconjugated prolonged neonatal jaundice
Hypothyroidism
| Breast milk jaundice
45
Causes of conjugated prolonged neonatal jaundice
```
Anatomical (biliary obstruction)
- Biliary atresia
- choledochal cyst
- alagille syndrome
Neonatal hepatitis
- viral hepatitis
- parental nutrition
- glycogen storage disorders
- haemochromatosis
- urea cycle defects
```
46
Presentation of biliary atresia causing neonatal jaundice
Conjugated jaundice
| Pale stools
47
Investigations for biliary atresia
Split bilirubin
Stool colour
USS
Liver biopsy
48
What is a choledochal cyst?
Developmental condition where there is a cystic abnormality in bile ducts so get obstructive picture
49
What kind of jaundice is a cholechondral cyst?
Conjugated jaundice
50
What does a cholechondral cyst present with?
Pale stools
| Jaundice
51
Investigations of a cholechondral cyst
Split bilirubin
Stool colour
USS
52
Pathology of Alagille syndrome
Congenital damage to intrahepatic bile ducts
53
Presentation of Alagille Syndrome
Intrahepatic cholestasis
Dysmorphism
Congenital heart disease
54
Investigations of alagille syndrome
Dysmorphism
| Genotype
55
What type of jaundice in infants is ALWAYS abnormal?
Conjugated
56
What is the most important test in prolonged jaundice?
Split bilirubin
57
What should always be done along with split bilirubin as an investigation for prolonged jaundice in infants?
Pale stools In babies
58
What is biliary atresia?
Congenital fibro-inflammatory disease of bile ducts leading to destruction of extra-hepatic bile ducts
- so there is no flow of bile into small intestine out of the liver
59
Presentation of biliary atresia
Prolonged, conjugated jaundice
Pale stools
Dark urine
60
What can biliary atresia progress to if not identified and treated?
Liver failure
61
What is the most common indication for liver transplantation in children?
Biliary atresia
62
Treatment of biliary atresia
Kasai Portoenterostomy
