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Acute fatty liver of pregnancy
1. ACUTE FATTY LIVER OF PREGNANCY
VS
HELLP SYNDROME
R.M Thusitha Bandara(MBBS/EUSL)
References
01. https://www.uptodate.com/contents/acute-fatty-liver-of-pregnancy
02. Medscape.com/article/1562425-overview
03. https://www.rcog.org.uk/en/guidelines-research
2. ACUTE FATTY LIVER OF PREGNANCY
• Acute fatty liver of pregnancy (AFLP) is an obstetric emergency.
• Rare,
1 in 7000 to 1 in 20,000 pregnancies
• Potentially lethal,
For both the mother and fetus,
• Commoner in,
primi gravida
• An association with,
M:FM(Fetus) = 3:1
Multiple pregnancy (20% of cases).
Low body mass index (BMI <20 kg/m2)
This disease has been linked to an abnormality in fetal fatty acid metabolism.
3. Pathogenesis acute fatty liver of pregnancy
• The pathogenesis (AFLP) is unclear.
• But defects in fatty acid metabolism during pregnancy appear to play
a role.
• particularly late in gestation.
• Fetal long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD)
deficiency
• Maternal-fetal fatty acid metabolism is defective, intermediate
products of metabolism can accumulate in maternal blood and
hepatocytes, with deleterious effects on maternal hepatocytes.
• This contributes to long-chain metabolites accumulating in maternal
blood and hepatocytes, resulting in toxic effects
4. • Clinical presentation of acute fatty liver of pregnancy (AFLP) is nonspecific
patient can present with the following complaints:
• Nausea and vomiting (70%)
• Hypoglycemia 70%
• Right hyper-quadrant and epigastric pain (50-80%)
• Malaise
• Polyuria
• Polydipsia
• Acute renal failure
• Pancreatitis
• Fulminant liver failure with hepatic encephalopathy
CLINICAL FEATURES
5. • Hypertension
• Confusion and altered mental status
• Jaundice
• Abdominal tenderness
• Ascites
• Bleeding (DIC)
• AKI
On physical examination
6. The diagnosis of AFLP is usually made clinically, based upon the presentation and
compatible laboratory results.
Laboratory tests
•Elevated aminotransferases (5 to 10 times the upper limit of normal)
•Elevated serum bilirubin
•Elevated prothrombin time
•Elevated urate level
•Elevated ammonia level
•Elevated creatinine
•Elevated white blood cell count
•Low serum glucose
•Low fibrinogen
INVESTIGATIONS
7. Management
• Urgent delivery of fetus - once mother stabilized -(usually be emergency LSCS)
• Multidisciplinary team in an intensive care setting.
-Full monitoring
-CTG/Ultrasound
• Coagulopathy and hypoglycaemia Mx
-Should be treated aggressively before delivery.
-Large amounts of 50% glucose may be needed to correct the hypoglycemia
-Fresh frozen plasma and albumin should be given as necessary.
• hematological resuscitation (Blood products, vitamin K)
• Intravascular volume correction
• Hypoglycemia treatment
• Antidotes/specific Treatments
• IV MgSO4 (adjust in renal failure)
• lactulose to decrease ammonia production
• liver transplantation
8. Conclusion
• Rare, but potentially fatal.
• The diagnosis should be considered and liver function measured,
especially if there is vomiting and abdominal pain.
• Differential diagnosis includes HELLP syndrome.
• Liver dysfunction is usually marked with hypoglycaemia,
hyperuricaemia, renal impairment and coagulopathy.
• The woman is at risk of fulminant hepatic failure and encephalopathy
and may require ICU Mx.
• Delivery of the fetus is the correct treatment once hypoglycaemia,
coagulopathy and hypertension have been controlled.
9. (Hemolysis, Elevated Liver Enzymes ,Low Platelets)
• HELLP syndrome thought to be a severe form of pre-eclamptic liver dysfunction
• But it can occur in normotensive patients as well.
Differentials
Can be confused with many medical conditions
Acute fatty liver of pregnancy
Biliary colic and cholecystitis
ITP
GORD and Peptic ulcer disease
Appendicitis
Cerebral hemorrhage
HELLP syndrome
10. • RHC pain or pain around stomach
• Nausea
• Headache
• Malaise
Eclampsia
Abruption of placenta
DIC
Acute renal failure
Severe ascites
Cerebral edema
Pulmonary edema
• RHC tenderness
• Increased BP
• Proteinuria
• Edema
Complications
SYMPTOMS Signs
HELLP syndrome
11.
12. Radiological evaluation
MRI,CT or US:
± hepatic steatosis, but the liver may appear
normal {fat is microvesicular}
CT: decreased attenuation {fatty infiltration}.
Liver biopsy :Gold standard for diagnosis.
HELLP SYNDROME VS AFLD
13. Ward patient
27yr
Primi
EM/LSCS day 1
• Hx- Nausea and vomiting
Hypoglycemia
Polyuria
Polydipsia
Lethargy
Headache
Shivering
• O/E – afebrile
not pale
Icteric +
PR-100bpm
BP- 120/90mmHg
AB- Soft