7. CHOLESTASIS
● Histological demonstration of bile in liver tissue
● Measurable reduction in hepatic secretion of solutes
and water
● Demonstrable accumulation in blood of substances
normally excreted in bile(bilirubin, cholesterol, bile acids)
11. PROGNOSIS
● No maternal mortality
● Fetal mortality 1-2%
● Recurs in 60-70% of subsequent pregnancies
12. MANAGEMENT
● UDCA : 500 mg -1.5 g daily(10 mg/kg/day)
● Pruritus: antihistamines
● Vitamin K supplementation
● Lifestyle advices
● Ultimate treatment: delivery
● Consider early delivery in severe cases:
○ Unbearable maternal pruritus or risk of fetal
distress/death
○ Deliver at 38 weeks if mild, at 36 weeks for severe
cases-if jaundice
14. PATHOPHYSIOLOGY
● Deficiency of the enzyme long chain 3 hydroxyacyl
coenzyme A dehydrogenase
● Defect in β-oxidation of fatty acids in mitochondria
● Accumulation of long chain 3 hydroxyacyl metabolites
● Micro vesicular fatty infiltration of hepatocytes
● Fat droplet formation in hepatocytes
16. CLINICAL PRESENTATION
● 1-2 week history of nausea, vomiting, abdominal pain and
fatigue
● Hypoglycaemia
● Coagulopathy
● Reduced antithrombin III activity
● May progress rapidly to liver failure with
encephalopathy
● 50% will have signs of preeclampsia
● DIC in 80-100%
22. MANAGEMENT
● Delivery of the baby
● Supportive care to the mother
● Plasmaphoresis
● If hepatic functions fail to recover/ fulminant, liver
transplant
● Screen child for genetic defects in fatty acid oxidation
26. RISK FACTORS
● Advanced maternal age
● Multiparity
● Nulliparity
● Late pregnancy/ post partum patients who did not have
hypertension/ proteinuria in t2/ t3