ObstetricsTier 2Disease (DEADMAN)

Gestational liver disease

Description

Liver dysfunction unique to pregnancy spans a spectrum by trimester and severity: hyperemesis-associated (1st trimester), intrahepatic cholestasis of pregnancy (ICP) (2nd-3rd trimester), and pre-eclampsia-associated (HELLP, AFLP) (3rd trimester)

ICP: ~0.7-1.5% of pregnancies (higher in Chilean/Scandinavian populations); AFLP is rare (~1:10,000-20,000); HELLP complicates ~10-20% of severe pre-eclampsia

ICP - impaired biliary transport (genetic susceptibility - ABCB4/ABCB11 variants) under rising oestrogen/progesterone -> bile acid accumulation -> pruritus and hepatotoxicity
AFLP - microvesicular fatty infiltration, associated with fetal LCHAD deficiency - a maternal heterozygote carrying an affected fetus cannot clear fetal fatty acid metabolites
HELLP - a variant/complication of pre-eclampsia: microangiopathic haemolysis + hepatic sinusoidal fibrin deposition + consumptive thrombocytopenia

ICP: pruritus (classically palms/soles, worse at night) + raised bile acids (>=10 micromol/L) +/- mildly deranged LFTs, otherwise normal liver; a diagnosis of exclusion
AFLP: Swansea criteria (>=6 of: vomiting, abdominal pain, polydipsia/polyuria, encephalopathy, raised bilirubin/urate/WCC/ammonia/creatinine, deranged LFTs, coagulopathy, imaging/biopsy evidence of fatty liver)
HELLP: haemolysis (schistocytes, raised LDH, low haptoglobin) + AST/ALT elevation + platelets <100x10^9/L

ICP: ursodeoxycholic acid for symptom/biochemical control; timed birth (~37-39 weeks depending on bile acid level) given stillbirth risk rises with bile acids >=100 micromol/L
AFLP and HELLP: expedited delivery is definitive treatment - supportive care (correct coagulopathy, treat hypoglycaemia in AFLP, manage as severe pre-eclampsia in HELLP), ICU-level monitoring
Distinguish from viral hepatitis, gallstone disease, and pre-existing liver disease unmasked by pregnancy

ICP - increased stillbirth risk (bile-acid dependent), meconium-stained liquor, preterm birth
AFLP/HELLP - DIC, acute kidney injury, hepatic rupture/infarction (rare, catastrophic)

All resolve postpartum (AFLP can take days-weeks for liver recovery; rare fulminant cases may need liver transplant)
ICP recurs in ~45-90% of subsequent pregnancies

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