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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
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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)

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3
Q

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

A

using bilirubin threshold table

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4
Q

When do you consider intensified phototherapy

A

if serum bilirubin levels are rising rapidly

if bilirubin fails to repost to phototherapy

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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
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6
Q

When do you perform exchange transfusion

A

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

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7
Q

What must you NOT stop during exchange transfusipn

A

Phototherapy

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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)

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9
Q

What is biliary atrasi

A

Progressive fibrosis and obliteration of extra hepatic and intrahepatic biliary tree

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10
Q

What is the cause of biliary atrasia

A

UNKNOWN

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11
Q

What is the outcome for untreated biliary atresia

A

death within TWO years

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12
Q

What is the presentation for biliary atresia

A 
jaundice 
pale stools 
normal BW 
faltering groeth
hepatomegaly, splenomegaly
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13
Q

What are investigations for biliary atrasia

A 
Raised conjugated bilirubin 
abnormal LFT 
Fasting abdo ultrasound 
Cholangiogram 
Liver biopsy
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14
Q

What is management for biliary atrasia

A

Kasai hepatoportoenterosotomy
Liver transplant

Surgical intervention is recommended within 60 days of life

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15
Q

What are complications of biliary atresia

A

growth failure
portal hypertension
cholangitis
ascitis

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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

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17
Q

What is a choledochal cyst

A

Cystic dilatation of extra hepatic biliary system

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18
Q

How does choledochal cyst present

A

neonate: jaundice

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

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19
Q

How do you diagnose choledochal cyst

A

USS /MRCP

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20
Q

How do you treat choledochal cyst

A

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

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21
Q

What is neonatal hepatitis syndrome

A

Prolonged neonatal jaundice
Hepatic inflammation
LBW, faltering growth

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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
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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
what is galactosaemia
metabolic disorder | inability to metabolise galactose
26
\What are features of galactosaemi
When fed milk: - poor feeding - vomiting - jaundice - hepatomegaly
27
What occurs if galactosemia is untreated
liver failure cataracts developmental delay
28
What are investigations for galactosaemia
- galactose in urine | - Galactose-1-Phosphate-Uridyl Trasnferase in red cells
29
How do you manage galactosaemia
GALACTOSE FREE DIET
30
What is acute liver failure
AKA FULMINANT HEPATITIS massive hepatic necrosis with loss of liver function
31
What are causes of acute liver failure in children <2 years old
- infection - metabolic disease - AI hepatitis - drug induced - haemochromatosis
32
What are causes of chronic liver disease in children
Hepatitis B,C AI hepatitis Drug induced hepatitis Non alcoholic fatty liver disease Genetic: - Wilson0s - CF - Alpha 1 antitrypsin deficiency - Sclerosing cholangitis
33
what is Wilson's disease
Reduced synthesis of caeruloplasmin (copper binding protein) > copper accumulation in liver, brain, kidney , cornea
34
How does a child with Wilson's disease present
Deterioration in school , mood and behaviour change EPS eg. incoordination, tremor, dysarthria KEISER - FLEISHER RINGS (after 7 yo)
35
How do you investigate wilson disease
low serum caeruloplasmin Low serum copper increased urinary copper excretion elevated hepatic copper on liver biopsy / identification of gene mutation
36
How do you manage wilson disease
Zinc (blocks copper resorption in intestine) Trientine (increases urinary copper excretion) Pyridoxine (B6) (prevents peripheral neuropathy) Consider liver transplant if end stage disease
37
What are complications of chronic liver disease
NUTRITION (fat malabsorption, protein malnutrition, anorexia) PRURITUS ENCEPALOPATHY
38
How do you manage nutrition problem in children
Supplement fat soluble vitamins Encourage protein intake (unless encephalopathy) NG tube / parenteral feeding if required
39
How do you manage pruritus in children
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
How od you manage encepalopathy
REDUCE NITROGENOUS LOAD - diary protein restriction - lactulose (prevents absorption)
41
WHAT IS Mode of inheritance of alpha 1 antitrypsin
Autosomal rec sessile
42
What occurs in alpha 1 antitrypsin deficiency
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
what are clinical features of alpha 1 antitrypsin def
``` Prolonged neonatal jaundice Bleeding (vit K defi) Hepatomegh Splenomeg Pulmonary disease in ADULT LIFE ```
44
How do you investigate alpha 1 antitrypsin def
measure alpha 1 antitrypsin levels in plasma
45
how do you manage alpha 1 antitrypsin def
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
How many children develop chronic Hep B liver disease
30-50% !!!!
47
When should you wait to start treating Hep C
3 years old | because vertically acquired infections may resolve spontaneously before then
48
Who is Hep E very dfangerous in
Pregnant women Causes fulminant hepatic failure and high mortality rate
49
How do you manage non-alcoholic fatty liver disease
``` Weight loss Tx insulin resistance, diabetes Statins Vit C, E Ursodeoxycholic acid to improve bile flow ```
50
What are complications of chronic liver disease
NUTRITION - fat malabsorption - protein malnutrition - anorexia PRURITYS
51
Why does chronic liver disease cause fat malabsorption
Because without bile you cannot effectively absolve long chain fat
52
Why does CLD cause protein malnutrition
poor intake + high catabolic rate of diseased liver
53
how do you manage autoimmune hepatitis
prednisolone + azathioprine
54
how can you manage hepatic encepalopathy
- Supportive (frequent monitoring of mental status) - Identify and correct precipitating factors - Reduce nitrogenous load (dietary protein restriction, reduce load via LACTULOSE or RIFAXIMIN)
55
What is Reye syndrome
Acute non-inflammatory encepalopathy With microvascular fatty infiltrations in the liver ASSOCIATED WITH ASPIRIN IN CHILDREN

Y5 Paediatrics (30 decks)

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