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Acute fatty liver of pregnancy

Definition
AFLP is a rare, potentially life-threatening liver disorder that affects pregnant women in the third trimester.
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Pathophysiology
AFLP is associated with defects in fatty acid oxidation secondary to various enzymatic deficiencies, leading to microvesicular fatty infiltration of the liver.
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Epidemiology
The incidence of AFLP in Canada is estimated at 8 per 100,000 person-years.
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Disease course
Clinically, AFLP can present with a range of symptoms, including nausea, vomiting, abdominal pain, and jaundice. In severe cases, it can lead to hepatic encephalopathy and multiorgan failure.
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Prognosis and risk of recurrence
The prognosis largely depends on early diagnosis and prompt delivery. Prompt delivery is recommended, especially in cases of elevated lactate levels and hepatic encephalopathy.
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Key sources
The following summarized guidelines for the evaluation and management of acute fatty liver of pregnancy are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL 2023), the Chinese Society of Obstetrics Gynecology (CSOG 2021), the American Association for the Study of Liver Diseases (AASLD 2021), the American College of Gastroenterology (ACG 2016), and the Italian Association for the Study of the Liver (AISF 2016).
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Guidelines

1.Screening and diagnosis

Indications for screening
Obtain prenatal screening for AFLP in outpatients at 35-37 weeks of gestation.
B
Obtain routine blood tests, liver function, and coagulation function as first-line screening indicators. Obtain testing immediately in pregnant patients with gastrointestinal symptoms, such as nausea and vomiting, and suspected AFLP.
B
Obtain retesting within 24 hours to identify AFLP as soon as possible in patients with suspected AFLP based on initial screening.
B
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  • Diagnosis

2.Classification and risk stratification

Prognosis
Assess postoperative prothrombin activity, TBIL, platelet count, and serum creatinine as prognostic indicators.
B
Evaluate patients by a multidisciplinary team if the abovementioned indicators continue to be abnormal after delivery or the patient is not recovering by 1 week after delivery.
B

3.Diagnostic investigations

Abdominal imaging: obtain abdominal imaging to rule out hepatic hemorrhage, infarction, or rupture in patients with AFLP.
E

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4.Medical management

Setting of care: as per EASL 2023 guidelines, consider managing patients with AFLP developing encephalopathy, elevated serum lactate (> 2.8 mg/dL), a MELD score ≥ 30 or with a score > 7 on the Swansea criteria at level 2 or 3 care (ICU admission).
C

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5.Inpatient care

Serial laboratory assessment: obtain routine monitoring blood tests including glucose, liver function, kidney function, and coagulation function during the treatment period.

6.Therapeutic procedures

Liver support system: offer artificial liver treatment in patients with severe AFLP.
B

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  • Delivery

  • Plasmapheresis

7.Perioperative care

Preoperative risk assessment: use levels of prothrombin activity/INR, TBIL, platelet count, lactic acid, serum creatinine, and disease duration as indicators for preoperative risk assessment.
B
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8.Surgical interventions

Liver transplantation: as per EASL 2023 guidelines, refer patients with AFLP having severe hepatic impairment likely to require transplantation to a transplant center.
B

9.Follow-up and surveillance

Newborn monitoring: as per AASLD 2021 guidelines, screen all newborns of mothers with AFLP at birth for long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency or other fatty acid oxidation defects. Refer affected families to genetic counseling.
E