Acute Fatty Liver, HELLP and Cholestasis of Pregnancy
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Direct answer
Acute fatty liver of pregnancy (AFLP) is a life-threatening microvesicular fatty infiltration of the liver that presents in the third trimester, usually around 36 weeks. HELLP syndrome, with haemolysis, elevated liver enzymes and low platelets, is a severe complication of pre-eclampsia, while intrahepatic cholestasis causes itching with raised serum bile acids. Delivery is the definitive treatment for AFLP and HELLP, and cholestasis is treated with ursodeoxycholic acid and timed delivery.
What you must remember
- AFLP labs: raised bilirubin, hypoglycaemia, prolonged prothrombin time and raised ammonia.
- Swansea criteria: AFLP is diagnosed when six or more of 14 clinical and laboratory features are present.
- Fetal link: the baby may carry long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, which is linked to maternal fatty liver injury.
- Tennessee criteria: platelets at or below 100 × 10^9/L, AST at or above 70 IU/L and LDH at or above 600 IU/L.
- HELLP and blood film: haemolysis on the peripheral smear supports the diagnosis.
- ICP marker: raised serum bile acids, with itching of the palms and soles and no primary rash.
- Definitive treatment: delivery for AFLP and HELLP once the mother is stabilised.
Three liver disorders of late pregnancy
A nulliparous woman at 36 weeks presents with vomiting, jaundice and low blood glucose. Her presentation points to AFLP rather than viral hepatitis, and the examiner expects you to separate the three conditions by timing, labs and management.
| Feature | AFLP | HELLP | Cholestasis of pregnancy |
|---|---|---|---|
| Typical timing | Third trimester | Third trimester, often with pre-eclampsia | Later pregnancy |
| Key findings | Hypoglycaemia, raised ammonia, prolonged PT | Haemolysis, raised enzymes, low platelets | Raised serum bile acids |
| Main symptoms | Vomiting, abdominal pain, jaundice | Right upper pain, malaise, often hypertension | Intense itching |
| Management | Prompt delivery with supportive care | Stabilise, then deliver; magnesium sulphate | Ursodeoxycholic acid and planned delivery |
The table separates three conditions that share jaundice in late pregnancy. In each one, the central management question is when to deliver once the mother is stable.
In AFLP, hypoglycaemia, coagulopathy and encephalopathy can progress quickly, so supportive care with frequent glucose checks, blood products as needed and close monitoring of renal function comes before and alongside delivery.
Where the exam tests obstetric liver disease
Exam questions usually turn on timing, the lab pattern and the role of delivery.
- Trap: treating AFLP with conservative care alone Correct: delivery is definitive once glucose and coagulation are corrected and supportive care is given.
- Trap: overlooking HELLP because blood pressure is normal Correct: HELLP is usually a complication of pre-eclampsia, but a minority of cases lack hypertension or proteinuria, so the platelet count and liver enzymes decide.
- Trap: using ursodeoxycholic acid as a substitute for delivery in severe cholestasis Correct: it relieves itching and lowers bile acids, but delivery timing still reduces stillbirth risk.
Frequently asked questions
When does acute fatty liver of pregnancy usually present?
Acute fatty liver of pregnancy usually presents in the third trimester, often around 36 weeks of gestation. It is more often seen in first pregnancies, and early recognition of vomiting, abdominal pain and jaundice is important.
What are the Tennessee criteria for HELLP syndrome?
The Tennessee criteria for HELLP syndrome are platelets at or below 100 × 10^9/L, AST at or above 70 IU/L and LDH at or above 600 IU/L. Haemolysis on the peripheral blood film supports the diagnosis of HELLP syndrome.
How is intrahepatic cholestasis of pregnancy treated?
Intrahepatic cholestasis of pregnancy is treated with ursodeoxycholic acid, which relieves pruritus and lowers serum bile acids. The timing of delivery is individualised, because stillbirth risk rises with high bile acid levels.
Which features make up the Swansea criteria for acute fatty liver of pregnancy?
The Swansea criteria diagnose acute fatty liver of pregnancy when six or more of 14 features are present. These include vomiting, abdominal pain, hypoglycaemia and encephalopathy, along with raised ammonia and prolonged prothrombin time.