ICU · Obs/Gynae
Acute fatty liver of pregnancy (AFLP)
Also known as Acute fatty liver of pregnancy (AFLP) · Acute yellow atrophy of pregnancy · Swansea criteria · Microvesicular fatty liver of pregnancy · Acute yellow atrophy · Mitochondrial fatty-acid-oxidation disorder of pregnancy · LCHAD deficiency in pregnancy · Third-trimester acute liver failure · HELLP mimic
AFLP is a rare (about 5 per 100,000 maternities in the UK national cohort), life-threatening liver disorder occurring in the third trimester. Fatty infiltration of hepatocytes → microvesicular steatosis → acute liver failure. Presents with: nausea, vomiting, abdominal pain, hypertension, jaundice, polydipsia/polyuria (transient central DI), encephalopathy (progressive). Diagnosis: Swansea criteria (6 or more of 14 clinical/laboratory features). Management: URGENT DELIVERY (the only definitive treatment — the liver recovers after delivery), supportive ICU care (coagulopathy, hypoglycaemia, AKI, encephalopathy), NAC may help. Distinguishes from HELLP: AFLP has more severe coagulopathy, encephalopathy, and hypoglycaemia. Mortality: maternal case fatality 1.8 percent and perinatal mortality 104 per 1000 births (UK cohort) — has improved with early recognition and delivery.
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Red flags
- Third trimester + nausea/vomiting + abdominal pain + jaundice = consider AFLP
- URGENT DELIVERY is the definitive treatment — liver function recovers after delivery
- Swansea criteria: 6 or more of 14 features = diagnosis
- Hypoglycaemia is common (liver failure) — check glucose hourly
- The FETUS drives the maternal disease: fetal LCHAD (long-chain 3-hydroxyacyl-CoA dehydrogenase) deficiency → toxic 3-hydroxy-fatty-acid metabolites cross the placenta → maternal hepatic microvesicular steatosis
- AFLP has COAGULOPATHY + HYPOGLYCAEMIA + ENCEPHALOPATHY early; HELLP has THROMBOCYTOPENIA + HAEMOLYSIS dominant — the single most examined distinction
- Unlike non-pregnancy acute liver failure, in AFLP you ARE about to perform a procedure (delivery) → CORRECT coagulopathy BEFORE delivery — DIC is present in about 80 percent and obstetric haemorrhage is the leading killer
- Coexisting transient central DI (polydipsia/polyuria) and pancreatitis (check lipase) are common and easily missed
- Test mother AND baby for LCHAD/fatty-acid oxidation disorder after every AFLP pregnancy — recurrence follows fetal genetics
Meet the patient
A 31-year-old primigravida at 36 weeks arrives with five days of "gastric flu" — nausea, vomiting and epigastric pain — and is now jaundiced and drowsy (GCS 13). Her glucose is 1.9 mmol per L, INR 2.3, fibrinogen 1.2 g per L, platelets 105, AST 380, creatinine 178, ammonia 110. The CTG shows reduced variability. This is the textbook presentation: in the Parkland series of 51 women, the most common complaints were persistent nausea and vomiting (57 percent), hypertension (57 percent) and abdominal pain (53 percent), and more than 90 percent had at least one of these findings.[5]
The question that decides her life and her baby's is not "what is the liver diagnosis" — it is "how fast can I correct the coagulopathy and the glucose and deliver?" That constellation — coagulopathy plus hypoglycaemia plus encephalopathy with only mild thrombocytopenia — is AFLP until proven otherwise, and the definitive treatment is the delivery.[10]
References10ShowHide
- [1]Knight M, Nelson-Piercy C, Kurinczuk JJ, et al. A prospective national study of acute fatty liver of pregnancy in the UK Gut, 2008.PMID 18332072
- [2]Liu J, Ghaziani TT, Wolf JL Acute Fatty Liver Disease of Pregnancy: Updates in Pathogenesis, Diagnosis, and Management Am J Gastroenterol, 2017.PMID 28291236
- [3]Ch'ng CL, Morgan M, Hainsworth I, Kingham JGC Prospective study of liver dysfunction in pregnancy in Southwest Wales Gut, 2002.PMID 12427793
- [4]Ibdah JA, Bennett MJ, Rinaldo P, Zhao Y, Gibson B, Sims HF, Strauss AW A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women N Engl J Med, 1999.PMID 10352164
- [5]Nelson DB, Yost NP, Cunningham FG Acute fatty liver of pregnancy: clinical outcomes and expected duration of recovery Am J Obstet Gynecol, 2013.PMID 23860212
- [6]Nelson DB, Yost NP, Cunningham FG Hemostatic dysfunction with acute fatty liver of pregnancy Obstet Gynecol, 2014.PMID 24901264
- [7]Lee WM, Hynan LS, Rossaro L, et al. Intravenous N-acetylcysteine improves transplant-free survival in early stage non-acetaminophen acute liver failure Gastroenterology, 2009.PMID 19524577
- [8]Murphy N, Auzinger G, Bernel W, Wendon J The effect of hypertonic sodium chloride on intracranial pressure in patients with acute liver failure Hepatology, 2004.PMID 14767999
- [9]Kothari S, Afshar Y, Friedman LS, Ahn J AGA Clinical Practice Update on Pregnancy-Related Gastrointestinal and Liver Disease: Expert Review Gastroenterology, 2024.PMID 39140906
- [10]Nelson DB, Byrne JJ, Cunningham FG Acute Fatty Liver of Pregnancy Clin Obstet Gynecol, 2020.PMID 31725416