II. Epidemiology

  1. Incidence: 1 in up to 7,000 pregnancies

III. Pathophysiology

  1. Idiopathic condition
  2. Abnormal hepatic mitochondrial function
  3. Results in buildup of fat droplets (microvesicular fatty infiltration) in hepatocytes

IV. Risk Factors

  1. First pregnancy
  2. Multiple Gestation

V. Symptoms

  1. Onset in third trimester
  2. Vomiting (76%)
  3. Upper Abdominal Pain (43%)
  4. Anorexia (21%)
  5. Jaundice (16%)

VI. Signs

VII. Labs

  1. Liver Function Tests
    1. Serum Bilirubin increased, but <5 mg/dl
    2. Prothrombin Time (aPT and INR) increased
    3. Serum Transaminases (AST, ALT) <500 IU/L
      1. Contrast with much higher in Viral Hepatitis
    4. PTT and INR/ProTime prolonged
  2. Platelet Count decreased mildly: 100,000 to 150,000
    1. Contrast with HELLP Syndrome in which Platelet Count is much lower
  3. Chemistry panel
    1. Serum Creatinine increased
    2. Hypoglycemia

VIII. Imaging

  1. All imaging tests have high False Positive Rate
  2. Abdominal Ultrasound
    1. Evaluate for hepatic infarct, hematoma and Acute Cholecystitis

IX. Differential Diagnosis

  1. See Acute Hepatitis
  2. Hyperemesis Gravidarum
    1. Liver transaminases (AST, ALT) may be over 200 IU/L
    2. Alkaline Phosphatase may be increased up to twice normal
    3. Serum Bilirubin may be increased enough to cause visible Jaundice
  3. HELLP Syndrome
    1. May be most difficult to distinguish from Acute Fatty Liver of Pregnancy
    2. Often associated with Preeclampsia with Severe Hypertension and Proteinuria
    3. Most commonly occurs in third trimester and immediately postpartum
  4. Acute Fatty Liver of Pregnancy
    1. Associated with more severe liver failure and Renal Insufficiency
    2. May be difficult to distinguish with HELLP Syndrome
  5. Intrahepatic Cholestasis of Pregnancy
    1. Most common liver disease in pregnancy (second and third trimester)
    2. Significantly elevated Bilirubin levels risk fetal demise and preterm delivery
  6. Other Acute Liver Disease
    1. See Acute Hepatitis
    2. Hepatic infarct
    3. Trauma (Liver Hematoma)
    4. Acute Cholecystitis

X. Differential Diagnosis: Non-pregnancy related

XI. Management

  1. Distinguish from HELLP Syndrome (and other causes of liver disease in pregnancy)
    1. See Differential Diagnosis above
    2. Severe hepatic insufficiency is more likely in Acute Fatty Liver of Pregnancy (AFLP) than HELLP
      1. Encephalopathy, coagulopathy and Hypoglycemia are more common in AFLP
  2. Delivery is critical and should be performed as soon as possible
    1. Delay may result in disease progression with risk of maternal mortality
    2. Delivery regardless of Gestational age
  3. Avoid Hepatotoxins (e.g. certain general anesthetics)
  4. Treat concurrent Disseminated Intravascular Coagulation
    1. Correct coagulopathy with Blood Products
  5. Correct Hypoglycemia
    1. D10W infusion and
    2. Dextrose 50% boluses as needed

XII. Course

  1. Fulminant hepatic failure if untreated
  2. Disease usually remits within days of delivery (although lab abnormalities may persist)

XIII. Prognosis

  1. Maternal mortality: <10% (previously as high as 92%)
  2. Infant mortality: Previously as high as 50%

XV. References

  1. Swencki (2015) Crit Dec Emerg Med 29(11):2-10
  2. Castro (1999) Am J Obstet Gynecol 181:389-95 [PubMed]

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Ontology: Acute fatty liver of pregnancy (C1455728)

Concepts Disease or Syndrome (T047)
MSH C537957
SnomedCT 91162000
LNC LA16929-4
English AFLP - Ac fatty liver of pregn, FATTY LIVER OF PREGNANCY ACUTE, Acute fatty liver, gestational, acute fatty liver pregnancy, fatty liver of pregnancy, fatty liver pregnancy, acute fatty liver of pregnancy, Acute fatty liver of pregnancy, AFLP - Acute fatty liver of pregnancy, Fatty liver of pregnancy
Italian Steatosi epatica acuta in gravidanza
Japanese 妊娠時の急性脂肪肝, ニンシンジノキュウセイシボウカン
Czech Akutní steatóza jater v těhotenství
Hungarian Acut terhességi zsírmáj
Portuguese Fígado gordo agudo da gravidez
Spanish Hígado graso agudo del embarazo
Dutch acute leververvetting tijdens de zwangerschap
French Stéatose hépatique aiguë de la grossesse
German akute Fettleber bei Schwangerschaft