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OB
CMECNE

Acute Fatty Liver of Pregnancy: A True Obstetric Emergency

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Learning Objectives and CME/Disclosure Information

This activity is intended for healthcare providers delivering care to women and their families.

After completing this activity, the participant should be better able to:

1. Recall the laboratory findings associated with AFLP
2. Discuss the management of AFLP

Estimated time to complete activity: 0.25 hours

Faculty:

Susan J. Gross, MD, FRCSC, FACOG, FACMG
President and CEO, The ObG Project

Disclosure of Conflicts of Interest

Postgraduate Institute for Medicine (PIM) requires faculty, planners, and others in control of educational content to disclose all their financial relationships with ineligible companies. All identified conflicts of interest (COI) are thoroughly vetted and mitigated according to PIM policy. PIM is committed to providing its learners with high quality accredited continuing education activities and related materials that promote improvements or quality in healthcare and not a specific proprietary business interest of an ineligible company.

The PIM planners and others have nothing to disclose. The OBG Project planners and others have nothing to disclose.

Faculty: Susan J. Gross, MD, receives consulting fees from Cradle Genomics, and has financial interest in The ObG Project, Inc.

Planners and Managers: The PIM planners and managers, Trace Hutchison, PharmD, Samantha Mattiucci, PharmD, CHCP, Judi Smelker-Mitchek, MBA, MSN, RN, and Jan Schultz, MSN, RN, CHCP have nothing to disclose.

Method of Participation and Request for Credit

Fees for participating and receiving CME credit for this activity are as posted on The ObG Project website. During the period from Dec 31 2017 through Dec 31 2021, participants must read the learning objectives and faculty disclosures and study the educational activity.

If you wish to receive acknowledgment for completing this activity, please complete the post-test and evaluation. Upon registering and successfully completing the post-test with a score of 100% and the activity evaluation, your certificate will be made available immediately.

For Pharmacists: Upon successfully completing the post-test with a score of 100% and the activity evaluation form, transcript information will be sent to the NABP CPE Monitor Service within 4 weeks.

Joint Accreditation Statement

In support of improving patient care, this activity has been planned and implemented by the Postgraduate Institute for Medicine and The ObG Project. Postgraduate Institute for Medicine is jointly accredited by the Accreditation Council for Continuing Medical Education (ACCME), the Accreditation Council for Pharmacy Education (ACPE), and the American Nurses Credentialing Center (ANCC), to provide continuing education for the healthcare team.

Physician Continuing Medical Education

Postgraduate Institute for Medicine designates this enduring material for a maximum of 0.25 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Continuing Nursing Education

The maximum number of hours awarded for this Continuing Nursing Education activity is 0.2 contact hours.

Read Disclaimer & Fine Print

CLINICAL ACTIONS:

Acute fatty liver of pregnancy (AFLP) is an uncommon but potentially fatal disease which is unique to pregnancy. The exact pathogenesis of AFLP has yet to be determined. There is no universal standardized approach to diagnosis. Characteristic laboratory findings, imaging and findings may be used for diagnosis when clinical suspicion is present. Liver biopsy is not needed for diagnosis but may be used to decide on early delivery if diagnosis is unclear.

Diagnostic Algorithm When Transaminases are Elevated at > 20 weeks GA

Rule out other potential causes such as the following

  • Viral or autoimmune hepatitis
  • Alcohol related
  • Medications that may cause elevated transaminases or liver injury (e.g., acetaminophen,  allopurinol, omeprazole etc.)
    • Consider herbal preparations such as kava kava (Piper methysticum) and germander (Teucrium chamaedrys)
  • Gallstones
  • Budd-Chiari syndrome (occlusion of hepatic veins, e.g. blood clot)

Review clinical symptoms and signs

  • Abdominal pain, nausea & vomiting
    • May be seen in AFLP but also in other hepatic disorders in pregnancy such as HELLP
  • Hypertension
    • Key feature of HELLP/preeclampsia  (see ‘Related ObG Project Topics’ below)
  • Polydipsia/polyuria, anorexia, lethargy/encephalopathy
    • May be seen in AFLP
    • Not typical of HELLP or preeclampsia
  • Pruritis or jaundice
    • More typical of intrahepatic cholestasis of pregnancy (ICP)

Lab/Imaging

  • Biochemistry
    • Hypoglycemia
      • Seen in AFLP but not typical of HELLP/Preeclampsia or ICP
    • AST and ALT usually <1000
      • Elevated 5 to 10x in ALFP, 1-100x in HELLP/preeclampsia and 1-5x in ICP
    • Elevated serum ammonia | uric acid | hyperbilirubinemia | BUN & Cr
  • Coag studies
    • Prolonged PT/PTT/INR and decreased fibrinogen
  • Hematology
    • Elevated WBC with normal hematocrit
      • Leucocytosis more common in AFLP
      • Thrombocytopenia more likely in HELLP
  • Liver biopsy (gold standard)
    • Characterized by microvesicular fatty infiltration of hepatocytes without inflammation or necrosis
  • Ultrasound findings
    • Described as a “bright” liver and ascites may be present

Management

  • If AFLP diagnosed, deliver promptly and provide supportive care
    • There can be rapid progression to acute liver failure marked by complications such as coagulopathy, hypovolemia and renal failure
  • Patients often requires ICU admission after delivery
  • The use of liver transplantation has been reported for cases of worsening clinical status such as encephalopathy, lactic acidosis and persistent renal failure despite maximal medical therapy

SYNOPSIS:

Acute fatty liver of pregnancy is an obstetric emergency which generally manifests after 30 weeks of gestation. The incidence of AFLP is 1:7000-1:15,000 pregnancies and is much lower than that of preeclampsia and HELLP syndrome. Patients may present with nonspecific symptoms such as nausea and vomiting, abdominal pain, jaundice, polydypsia, polyuria and signs of encephalopathy. The Sawnsea Diagnostic Criteria are presented below, but predictive value may be of limited value if other liver disease in pregnancy (e.g., HELLP) is present.

KEY POINTS:

Risk Factors

  • Multiple gestation (14x risk in twins)
  • Male sex of fetus (3:1 ratio)
  • Coexisting diagnosis of liver disease in pregnancy (HELLP, preeclampsia)
  • Previous episode of AFLP
  • Underlying cause of AFLP is unclear, but an association with mothers and fetuses with mutations in the fatty oxidation pathway has been identified
    • Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency is one example of a fetal fatty acid oxidation defect (FAOD)
    • Pregnancy may stress the placenta sufficiently to result in AFLP

Swansea Criteria

AFLP diagnosis if ≥ 6 criteria are present and no other liver disease of pregnancy (e.g., HELLP) is present

  • Vomiting [60% of patients with AFLP have this abnormality]
  • Abdominal pain [56%]
  • Polydipsia/polyuria [12%]
  • Encephalopathy [9%]
  • Elevated total bilirubin [100%]
    • >0.8 mg/dl (>140 µmol/L)
  • Hypoglycemia [78%]
    • <72 mg/dl (4.0 mmol/L)
  • Elevated uric acid [88%]
    • >5.7 mg/dl (>340 μmol/L)
  • Leucocytosis [98%]
    • >11×109/l
  • Elevated ALT/AST [100%]
    • >42 U/l
  • Elevated Ammonia [50%]
    • >66 µg/dL (>47 µmol/L)
  • Elevated Cr [58%]
    • >1.7 mg/dl (150 µmol/L)
  • Coagulopathy [>50%]
    • PT >14s or APTT >34s
    • Plt count >100×1012 
  • Bright liver on ultrasound [25% ‘bright’ liver or ascites on imaging]
  • Ascites
  • Microvesicular steatosis on liver biopsy

Treatment Notes

  • Treatment of choice is emergent delivery and supportive care
  • Maternal liver recovers quickly after delivery with adequate supportive care
  • Maternal mortality has dropped from 85% in 1980s to <10%
  • Patients are at high risk for DIC and severe postpartum hemorrhage
  • Aggressively manage coagulopathies and hypoglycemia

Learn More – Primary Sources:

The American Journal Of Gastroenterology Volume 112 June 2017 Acute Fatty Liver Disease of Pregnancy: Updates in pathogenesis, diagnosis and management

Acute Fatty Liver of Pregnancy: Pathophysiology, Anesthetic Implications, and Obstetrical Management

HELLP Syndrome or Acute Fatty Liver of Pregnancy: A Differential Diagnostic Challenge

Noninvasive Swansea criteria are valuable alternatives for diagnosing acute fatty liver of pregnancy in a Chinese population

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Related ObG Topics:

Diagnosing Preeclampsia – Key Definitions and ACOG Guidelines
Gestational Thrombocytopenia – a Diagnosis of Exclusion
ACOG Guidance: Emergency Treatment for Severe Hypertension in Pregnancy
SMFM Recommendations: Intrahepatic Cholestasis of Pregnancy

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Computer System Requirements

OBG Project CME requires a modern web browser (Internet Explorer 10+, Mozilla Firefox, Apple Safari, Google Chrome, Microsoft Edge). Certain educational activities may require additional software to view multimedia, presentation, or printable versions of their content. These activities will be marked as such and will provide links to the required software. That software may be: Adobe Flash, Apple QuickTime, Adobe Acrobat, Microsoft PowerPoint, Windows Media Player, or Real Networks Real One Player.

Disclosure of Unlabeled Use

This educational activity may contain discussion of published and/or investigational uses of agents that are not indicated by the FDA. The planners of this activity do not recommend the use of any agent outside of the labeled indications.

The opinions expressed in the educational activity are those of the faculty and do not necessarily represent the views of the planners. Please refer to the official prescribing information for each product for discussion of approved indications, contraindications, and warnings.

Disclaimer

Participants have an implied responsibility to use the newly acquired information to enhance patient outcomes and their own professional development. The information
presented in this activity is not meant to serve as a guideline for patient management. Any procedures, medications, or other courses of diagnosis or treatment discussed or suggested in this activity should not be used by clinicians without evaluation of their patient’s conditions and possible contraindications and/or dangers in use, review of any applicable manufacturer’s product information, and comparison with recommendations of other authorities.

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