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Table 45-5

ICPHELLPAFLP
% Pregnancies0.1 (United States)0.2–0.60.005–0.01
Onset25–32 weeks' gestationThird trimester or postpartumThird trimester or postpartum
Family historyOftenNoOccasionally
Presence of preeclampsiaNoYes50%
Typical clinical featuresPruritus
Mild jaundice
Elevated bile acids
Decreased vitamin K
Hemolysis
Thrombo-cytopenia (often <50,000
)
Liver failure with:
  • Coagulopathy
  • Encephalopathy
  • Hypoglycemia
  • DIC
Amino-transferasesMild to 10- to 20-fold elevationMild to 10- to 20-fold elevation300–500 typical but variable
Bilirubin<5 mg/dL<5 mg/dL unless massive necrosisOften <5 mg/dL but higher if severe
Hepatic imagingNormalHepatic infarcts
Hematomas
Fatty infiltration
HistologyNormal to mild cholestasis; no necrosisPatchy to extensive necrosis and hemorrhageMicrovascular fat in zone 3
Maternal mortality rate (%)01–257–18
Fetal or perinatal mortality rate (%)0.4–1.4119–23
Recurrence in subsequent pregnancies (%)45–704–19LCHAD defect

DIC = disseminated intravascular coagulation; LCHAD = long-chain 3 hydroxyacyl CoA dehydrogenase.