Episode Overview
Theme:
This episode of Dr. Chapa’s OBGYN Clinical Pearls delves into the diagnostic challenges and key clinical distinctions between Acute Fatty Liver of Pregnancy (AFLP) and preeclampsia with severe features/HELLP syndrome. Dr. Chapa leverages a recent real-world patient case and pop culture references to discuss the criteria, underlying pathophysiology, and management approaches for these rare but critical peripartum liver disorders.
Dr. Chapa’s signature tone—engaging, clinically focused, and conversational—makes the topic accessible to medical students, residents, and practicing clinicians.
Key Discussion Points & Insights
1. Real-World Clinical Scenario: Diagnostic Dilemma
- Dr. Chapa sets the stage with a patient readmitted 6 days post-C-section with elevated blood pressure, headache, and rising transaminases (>900s), but normal platelets. The core question: Is this preeclampsia with severe features, or could it be AFLP?
- Quote @02:35: “The two conditions, guys, preeclampsia with severe features, AFLP, they can look the same, ... So how do we figure this out and are we missing that now?”
- Distinguishing features are critical because AFLP can be rapidly fatal if missed.
2. Pathogenesis & Epidemiology of AFLP
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AFLP is rare—about 1 in 10,000 to 1 in 20,000 pregnancies (08:45).
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Often occurs in the third trimester or postpartum.
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Genetic basis: Linked to mitochondrial fatty acid oxidation defects, especially the G1528C mutation—but absence doesn’t rule out AFLP due to possible fetal or maternal carriage.
- Quote @09:53: “So when somebody asks you, is there a genetic cause to this? Yeah, no question. It's G1528C. ... But just because the patient or the child are negative doesn't put them in the clear.”
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Risk factors:
- More common with multiple gestations (twins).
- More likely in pregnancies with male fetuses.
- Quote @09:07: “Much more common in twins and much more common in male fetuses than in pregnancies with female fetuses.”
3. Clinical Distinction: Preeclampsia/HELLP vs AFLP
Symptoms
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AFLP:
- Systemic malaise, nausea, vomiting, abdominal pain, jaundice, hypoglycemia, confusion/encephalopathy, coagulopathy, and renal dysfunction.
- Quote @13:37: “These patients... tend to have hypoglycemia, they have coagulopathy, they have renal dysfunction, and their ammonium can also be... abnormally elevated...”
- Systemic malaise, nausea, vomiting, abdominal pain, jaundice, hypoglycemia, confusion/encephalopathy, coagulopathy, and renal dysfunction.
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Preeclampsia with severe features:
- Elevated BP, headache, possible transaminase rise, but absence of hypoglycemia, hyperammonemia, and coagulopathy.
- Proteinuria helps clinch the diagnosis.
Labs & Features
- AFLP hallmarks:
- Elevated transaminases (often >1000)
- Hyperbilirubinemia (jaundice)
- Hypoglycemia
- High blood ammonia
- Coagulopathy (abnormal PT/PTT, low fibrinogen)
- Preeclampsia/HELLP:
- Transaminase elevation (typically less extreme), hypertension, proteinuria, thrombocytopenia (with HELLP), normal glucose/ammonia.
Notable Clinical Pearl
- Quote @24:30: “Hypoglycemia is not normal with preeclampsia with severe features... Having high ammonium is not part of preeclampsia with severe features.”
4. Diagnostic Criteria: The Swansea Criteria
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Swansea Criteria for AFLP: If 6 out of 15 features are met, diagnosis is likely—liver biopsy generally not needed.
- Features include: vomiting, abdominal pain, polydipsia/polyuria, encephalopathy, elevated bilirubin, hypoglycemia, elevated uric acid, leukocytosis, elevated transaminases, hyperammonemia, renal impairment, coagulopathy, ascites/bright liver on imaging, and microvesicular steatosis on liver biopsy.
- Quote @17:14: “Six or more of this is 15 total criteria, has pretty darn good sensitivity and specificity for acute fatty liver of pregnancy.”
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Mnemonic: Keep AFLP possibilities open especially if the presentation doesn’t “fit” a textbook diagnosis, or if there’s clinical deterioration.
5. Management Principles
Prompt Delivery Is Critical
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Delivery (usually C-section) is the only definitive intervention, as placental removal reverses the metabolic derangement. Multidisciplinary care (OB, anesthesia, hepatology, neonatology) is essential.
- Quote @26:55: “Prompt delivery is game changing here. For some reason, removal of the placenta from the mama helps the recovery immensely.”
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Monitor post-delivery, as recovery can take days to weeks. Rarely, patients may need liver transplant or plasmapheresis (for refractory cases), but these are not standard.
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Plasmapheresis?
- Only for select refractory cases post-delivery—not first-line.
- Quote @28:53: “There is some data that plasmapheresis in those who have already had delivery and are just not getting better... can help... But that is not the first line.”
- Only for select refractory cases post-delivery—not first-line.
Notable Quotes & Memorable Moments
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On distinguishing features:
“Jaundice is a big deal, guys, because preeclampsia with severe features don't turn your patient yellow.” (16:13) -
On clinical mindset:
“It's very easy. Is she hypoglycemic? Is she coagulopathic? Because coagulopathy is not typical preeclampsia with severe and neither is altered glucose metabolism.” (29:37) -
On nomenclature & clinical humility:
“Whether you want to call it Swansea, which I've heard, or Swansea, I don't care. Just know the criteria.” (22:24)
(And the comic detour on mispronouncing “Guillain Barre” as “Gillian Barr” in Texas.) -
Summary of the challenge:
“AFLP versus preeclampsia with severe features or help can be a clinical conundrum. And I hope we've done it justice.” (30:43)
Key Segment Timestamps
| Time | Segment | |:--------:|-------------------------------------------------------| | 00:38 | Patient clinical case setup | | 02:30 | Diagnostic challenge: AFLP vs preeclampsia discussion | | 08:45 | AFLP epidemiology & risk factors | | 09:40 | Genetic mutation & inheritance explained | | 16:00 | Introduction to Swansea Criteria | | 17:10 | Clinical and laboratory distinctions | | 24:30 | Clinical pearls for distinguishing AFLP | | 26:55 | Management: prompt delivery/emergency management | | 28:53 | Role of plasmapheresis | | 29:37 | Final clinical pearls & reflection |
Clinical Pearl Summary
- Always consider AFLP in third-trimester or postpartum patients with hepatic dysfunction, malaise, or jaundice—especially if labs show hypoglycemia, coagulopathy, or elevated ammonia.
- Use the Swansea Criteria (6/15) for practical diagnosis.
- Prompt delivery is life-saving—placental removal is curative.
- Monitor post-delivery for ongoing hepatic/renal dysfunction; involve multidisciplinary teams.
- Plasmapheresis is a rescue tool only for refractory cases.
Dr. Chapa’s Bottom Line:
“AFLP versus preeclampsia with severe features or help can be a clinical conundrum... Keep your eyes open for this.” (30:43)
