Liver Flashcards
What are causes of unconjugated prolonged jaundice
Breastmilk infection Haemolytic anaemia Hypothyroidism High GI obstruction Crigler-Najjar syndrome
What are causes of conjugated prolonged jaundice
Bile duct obstruction (biliary atresia, cholechodal cyst)
Neonatal hepatitis syndrome (infection, error of metabolism, alpha-1-antitrypsin, galactosaemia)
How do you determine what kind of intervention a baby needs for jaundice?
using bilirubin threshold table
When do you consider intensified phototherapy
if serum bilirubin levels are rising rapidly
if bilirubin fails to repost to phototherapy
How do you assess for underlying disease in jaundice
- 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
When do you perform exchange transfusion
If above threshold on graph OR baby shows signs of bilirubin encephalopathy
What must you NOT stop during exchange transfusipn
Phototherapy
What resources can you give for neonatal jaundice
NHS Choices Neonatal Jaundice Factsheet
The Breastfeeding Network (information and support for breastfeeding mothers)
Bliss (premature/sick babies)
What is biliary atrasi
Progressive fibrosis and obliteration of extra hepatic and intrahepatic biliary tree
What is the cause of biliary atrasia
UNKNOWN
What is the outcome for untreated biliary atresia
death within TWO years
What is the presentation for biliary atresia
jaundice pale stools normal BW faltering groeth hepatomegaly, splenomegaly
What are investigations for biliary atrasia
Raised conjugated bilirubin abnormal LFT Fasting abdo ultrasound Cholangiogram Liver biopsy
What is management for biliary atrasia
Kasai hepatoportoenterosotomy
Liver transplant
Surgical intervention is recommended within 60 days of life
What are complications of biliary atresia
growth failure
portal hypertension
cholangitis
ascitis
How do you manage complications of biliary atresia
ursodeoxycholic acid (promote bile flow)
fat soluble vitamins
prophylactic antibiotics e.g. co-trimoxazole to prevent cholangitis
What is a choledochal cyst
Cystic dilatation of extra hepatic biliary system
How does choledochal cyst present
neonate: jaundice
older children: abdo pain, palpable mass, jaundice, cholagitis
How do you diagnose choledochal cyst
USS /MRCP
How do you treat choledochal cyst
surgical excision of cyst with Roux en Y anastomosis to biliary duct
What is neonatal hepatitis syndrome
Prolonged neonatal jaundice
Hepatic inflammation
LBW, faltering growth
What is Alagille Syndrome - clinical its
Rare autosomal dominan
Clinica fts:
- triangular facies
- skeletal abnormalities
- congenital heart disorders
- renal tubular disorders
- defects of eye
How do you manage Alagille syndrome
Nutrition and fat soluble vitamins
May require liver transplant
Most survive to adulthood
What are two autosomal recessive causes of jaundice
Familial intrahepatic cholestasis
Alpha 1 antitrypsin deficiency
what is galactosaemia
metabolic disorder
inability to metabolise galactose
\What are features of galactosaemi
When fed milk:
- poor feeding
- vomiting
- jaundice
- hepatomegaly
What occurs if galactosemia is untreated
liver failure
cataracts
developmental delay
What are investigations for galactosaemia
- galactose in urine
- Galactose-1-Phosphate-Uridyl Trasnferase in red cells
How do you manage galactosaemia
GALACTOSE FREE DIET
What is acute liver failure
AKA FULMINANT HEPATITIS
massive hepatic necrosis with loss of liver function
What are causes of acute liver failure in children <2 years old
- infection
- metabolic disease
- AI hepatitis
- drug induced
- haemochromatosis
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
what is Wilson’s disease
Reduced synthesis of caeruloplasmin (copper binding protein) > copper accumulation in liver, brain, kidney , cornea
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)
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
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
What are complications of chronic liver disease
NUTRITION (fat malabsorption, protein malnutrition, anorexia)
PRURITUS
ENCEPALOPATHY
How do you manage nutrition problem in children
Supplement fat soluble vitamins
Encourage protein intake (unless encephalopathy)
NG tube / parenteral feeding if required
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)
How od you manage encepalopathy
REDUCE NITROGENOUS LOAD
- diary protein restriction
- lactulose (prevents absorption)
WHAT IS Mode of inheritance of alpha 1 antitrypsin
Autosomal rec sessile
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) \
what are clinical features of alpha 1 antitrypsin def
Prolonged neonatal jaundice Bleeding (vit K defi) Hepatomegh Splenomeg Pulmonary disease in ADULT LIFE
How do you investigate alpha 1 antitrypsin def
measure alpha 1 antitrypsin levels in plasma
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)
How many children develop chronic Hep B liver disease
30-50% !!!!
When should you wait to start treating Hep C
3 years old
because vertically acquired infections may resolve spontaneously before then
Who is Hep E very dfangerous in
Pregnant women
Causes fulminant hepatic failure and high mortality rate
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
What are complications of chronic liver disease
NUTRITION
- fat malabsorption
- protein malnutrition
- anorexia
PRURITYS
Why does chronic liver disease cause fat malabsorption
Because without bile you cannot effectively absolve long chain fat
Why does CLD cause protein malnutrition
poor intake + high catabolic rate of diseased liver
how do you manage autoimmune hepatitis
prednisolone + azathioprine
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)
What is Reye syndrome
Acute non-inflammatory encepalopathy
With microvascular fatty infiltrations in the liver
ASSOCIATED WITH ASPIRIN IN CHILDREN
