Acute fatty liver of pregnancy: Difference between revisions
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==Background== | <languages/> | ||
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==Background== <!--T:1--> | |||
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[[File:Sobo 1906 389.png|thumb|Inferior view of the liver with surface showing lobes and impressions.]] | |||
*Rare, potentially fatal complication that presents in second half of pregnancy or (less commonly) early postpartum | *Rare, potentially fatal complication that presents in second half of pregnancy or (less commonly) early postpartum | ||
*Exact etiology unclear, but thought to involve abnormal fetal fatty acid metabolism | *Exact etiology unclear, but thought to involve abnormal fetal fatty acid metabolism | ||
*Fat vesicles accumulate within hepatocytes, interfering with liver function | *Fat vesicles accumulate within hepatocytes, interfering with liver function | ||
==Clinical Features== | |||
==Clinical Features== <!--T:3--> | |||
<!--T:4--> | |||
*Usually presents in 3rd trimester, but may occur any time in 2nd half of pregnancy to early postpartum | *Usually presents in 3rd trimester, but may occur any time in 2nd half of pregnancy to early postpartum | ||
*[[Nausea/vomiting]] (commonly severe) | *[[Special:MyLanguage/Nausea/vomiting|Nausea/vomiting]] (commonly severe) | ||
*[[Jaundice]] | *[[Special:MyLanguage/Jaundice|Jaundice]] | ||
*Findings consistent with [[preeclampsia]] in some women: | *Findings consistent with [[Special:MyLanguage/preeclampsia|preeclampsia]] in some women: | ||
**[[Hypertension]] | **[[Special:MyLanguage/Hypertension|Hypertension]] | ||
**Edema | **Edema | ||
**Proteinuria | **[[Special:MyLanguage/Proteinuria|Proteinuria]] | ||
*[[Hypoglycemia]] | *[[Special:MyLanguage/Hypoglycemia|Hypoglycemia]] | ||
*Often, signs/symptoms of [[DIC]] | *Often, signs/symptoms of [[Special:MyLanguage/DIC|DIC]] | ||
*+/- [[encephalopathy]], [[ascites]] | *+/- [[Special:MyLanguage/encephalopathy|encephalopathy]], [[Special:MyLanguage/ascites|ascites]] | ||
==Differential Diagnosis== | |||
==Differential Diagnosis== <!--T:5--> | |||
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{{Postpartum emergencies DDX}} | {{Postpartum emergencies DDX}} | ||
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{{Jaundice DDX}} | |||
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=== | ==Evaluation== <!--T:6--> | ||
=== | ===Workup=== <!--T:7--> | ||
<!--T:8--> | |||
*[[Special:MyLanguage/LFTs|LFTs]] | |||
*[[LFTs]] | |||
**ALT/AST usually in 300-500 range, alk phos usually elevated in pregnancy | **ALT/AST usually in 300-500 range, alk phos usually elevated in pregnancy | ||
**[[Hyperbilirubinemia]]- more pronounced than in preeclampsia | **[[Special:MyLanguage/Hyperbilirubinemia|Hyperbilirubinemia]]- more pronounced than in preeclampsia | ||
*BMP | *BMP | ||
**[[Hypoglycemia]] | **[[Special:MyLanguage/Hypoglycemia|Hypoglycemia]] | ||
*[[DIC]] labs | *[[Special:MyLanguage/DIC|DIC]] labs | ||
**Low fibrinogen, [[coagulopathy]] | **Low fibrinogen, [[Special:MyLanguage/coagulopathy|coagulopathy]] | ||
**DIC present in as many as 70% of patients<ref>Ko H, Yoshida EM (2006). Acute fatty liver of pregnancy. Canadian Journal of Gastroenterology. 20 (1): 25–30</ref> | **DIC present in as many as 70% of patients<ref>Ko H, Yoshida EM (2006). Acute fatty liver of pregnancy. Canadian Journal of Gastroenterology. 20 (1): 25–30</ref> | ||
*CBC | *CBC | ||
**Often shows leukocytosis | **Often shows [[Special:MyLanguage/leukocytosis|leukocytosis]] | ||
*[[UA]] | *[[Special:MyLanguage/UA|UA]] | ||
**Proteinuria | **[[Special:MyLanguage/Proteinuria|Proteinuria]] | ||
*[[Special:MyLanguage/RUQ US|RUQ US]] | |||
**Non-specific; the liver can even be normal in echotexture | |||
**Useful to rule out other causes of obstructive biliary tract pathology. | |||
===Diagnosis=== <!--T:9--> | |||
<!--T:10--> | |||
*Often initially misdiagnosed as [[Special:MyLanguage/preeclampsia|preeclampsia]]/[[Special:MyLanguage/HELLP|HELLP]] | |||
**Hypoglycemia, jaundice, ascites, hypofibrinogenemia all ''more'' common in AFLP | |||
==Management== | |||
====Swansea criteria<==== <!--T:11--> | |||
<!--T:12--> | |||
At least six of the following findings, in the absence of another cause:<ref>Dey M, Reema K. Acute Fatty liver of pregnancy. N Am J Med Sci. 2012;4 (11): 611-2. doi:10.4103/1947-2714.103339</ref> | |||
*[[Special:MyLanguage/Vomiting|Vomiting]] | |||
*[[Special:MyLanguage/Abdominal pain|Abdominal pain]] | |||
*Polydipsia/polyuria | |||
*Encephalopathy | |||
*[[Special:MyLanguage/Elevated bilirubin|Elevated bilirubin]] | |||
*[[Special:MyLanguage/Hypoglycemia|Hypoglycemia]] | |||
*Elevated urea | |||
*Leukocytosis | |||
*[[Special:MyLanguage/Ascites|Ascites]] or bright liver on ultrasound scan | |||
*Elevated transaminases (AAT or ALT) | |||
*Elevated ammonia | |||
*Renal impairment: elevated creatinine | |||
*Coagulopathy: elevated prothrombin time or PT | |||
*Microvesicular steatosis on liver biopsy | |||
==Management== <!--T:13--> | |||
<!--T:14--> | |||
*Emergent Ob/Gyn consult | *Emergent Ob/Gyn consult | ||
**Delivery typically results in rapid hepatic recovery | **Delivery typically results in rapid hepatic recovery | ||
*[[Dextrose]] for hypoglycemia | *[[Special:MyLanguage/Dextrose|Dextrose]] for hypoglycemia | ||
*[[FFP]], [[cryoprecipitate]], and/or [[platelets]] for [[coagulopathy]] (see [[DIC]]) | *[[Special:MyLanguage/FFP|FFP]], [[Special:MyLanguage/cryoprecipitate|cryoprecipitate]], and/or [[Special:MyLanguage/platelets|platelets]] for [[Special:MyLanguage/coagulopathy|coagulopathy]] (see [[Special:MyLanguage/DIC|DIC]]) | ||
==Disposition== | |||
==Disposition== <!--T:15--> | |||
<!--T:16--> | |||
*Admit ICU or transfer to center with Ob | *Admit ICU or transfer to center with Ob | ||
==See Also== <!--T:17--> | |||
==External Links== <!--T:18--> | |||
==References== <!--T:19--> | |||
<!--T:20--> | |||
<references/> | <references/> | ||
[[Category:OBGYN]] [[category:GI]] | [[Category:OBGYN]] [[category:GI]] | ||
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Latest revision as of 23:25, 12 January 2026
Background
- Rare, potentially fatal complication that presents in second half of pregnancy or (less commonly) early postpartum
- Exact etiology unclear, but thought to involve abnormal fetal fatty acid metabolism
- Fat vesicles accumulate within hepatocytes, interfering with liver function
Clinical Features
- Usually presents in 3rd trimester, but may occur any time in 2nd half of pregnancy to early postpartum
- Nausea/vomiting (commonly severe)
- Jaundice
- Findings consistent with preeclampsia in some women:
- Hypertension
- Edema
- Proteinuria
- Hypoglycemia
- Often, signs/symptoms of DIC
- +/- encephalopathy, ascites
Differential Diagnosis
3rd Trimester/Postpartum Emergencies
- Acute fatty liver of pregnancy
- Amniotic fluid embolus
- Chorioamnionitis
- Eclampsia
- HELLP syndrome
- Mastitis
- Peripartum cardiomyopathy
- Postpartum endometritis (postpartum PID)
- Postpartum headache
- Postpartum hemorrhage
- Preeclampsia
- Resuscitative hysterotomy
- Retained products of conception
- Septic abortion
- Uterine rupture
Jaundice
Indirect Hyperbilirubinemia
- Hemolytic
- G6PD
- Drug related
- Autoimmune hemolytic anemia
- Hematoma resorption
- Ineffective erythropoiesis
- Gilbert's
Direct (Conjugated) Hyperbilirubinemia
- Choledocholithiasis
- Cholecystitis
- Ascending cholangitis
- AIDS cholangiopathy
- Stricture
- Neoplasm
- Pancreatic head
- Gallbladder
- Primary liver (e.g. hepatocellular carcinoma)
- Metastatic
- Obstructing AAA
Hepatocellular damage
Patient will have severely elevated AST/ALT with often normal Alkaline Phosphatase
- Viral hepatitis
- Fulminant hepatic failure
- alcoholic hepatitis
- Ischemic hepatitis
- Toxins
- Isoniazid
- Phenytoin
- acetaminophen
- Ritonavir
- Halothane
- Sulfonamide
- Autoimmune hepatitis
- Primary biliary cirrhosis
- HELLP Syndrome
- Congestive Hepatopathy
Pregnancy Related
Transplant Related
Pediatric Related
- Inborn error of metabolism
- Neonatal jaundice (physiologic)
Additional Differential Diagnosis
- Reye syndrome
- TPN
- Heatstroke
- Budd-Chiari (with acute ascites)
- Wilson's disease
- Sarcoidosis
- Amyloidosis
Masqueraders
Only bilirubin stains the sclera
- Carotenemia
- Quinacrine ingestion
- Dinitrophenol, teryl (explosive chemicals)
Evaluation
Workup
- LFTs
- ALT/AST usually in 300-500 range, alk phos usually elevated in pregnancy
- Hyperbilirubinemia- more pronounced than in preeclampsia
- BMP
- DIC labs
- Low fibrinogen, coagulopathy
- DIC present in as many as 70% of patients[1]
- CBC
- Often shows leukocytosis
- UA
- RUQ US
- Non-specific; the liver can even be normal in echotexture
- Useful to rule out other causes of obstructive biliary tract pathology.
Diagnosis
- Often initially misdiagnosed as preeclampsia/HELLP
- Hypoglycemia, jaundice, ascites, hypofibrinogenemia all more common in AFLP
Swansea criteria<
At least six of the following findings, in the absence of another cause:[2]
- Vomiting
- Abdominal pain
- Polydipsia/polyuria
- Encephalopathy
- Elevated bilirubin
- Hypoglycemia
- Elevated urea
- Leukocytosis
- Ascites or bright liver on ultrasound scan
- Elevated transaminases (AAT or ALT)
- Elevated ammonia
- Renal impairment: elevated creatinine
- Coagulopathy: elevated prothrombin time or PT
- Microvesicular steatosis on liver biopsy
Management
- Emergent Ob/Gyn consult
- Delivery typically results in rapid hepatic recovery
- Dextrose for hypoglycemia
- FFP, cryoprecipitate, and/or platelets for coagulopathy (see DIC)
Disposition
- Admit ICU or transfer to center with Ob
