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Acute fatty liver of pregnancy Aboubakr Elnashar
Benha university, Egypt
ABOUBAKR ELNASHAR
Rare
1 in 7000 to 1 in 20,000 pregnancies
Potentially lethal
for both the mother and fetus, especially if
diagnosis is delayed.
Commoner in:
primigravidae (although this predilection is not
as marked as in pre-eclampsia).
An association with
male fetuses (ratio 3:1)
multiple pregnancy (20% of cases).
ABOUBAKR ELNASHAR
Maternal and fetal mortality rate
lower than originally believed
{milder cases are recognised and appropriately
treated}
Maternal mortality:
10% to 20%
Perinatal mortality:
20% to 30%
UK:
maternal mortality rate
2%
perinatal mortality rate
11%.
ABOUBAKR ELNASHAR
CLINICAL FEATURES
Usually presents after 30 w, and often near term
(35–36 w)
Gradual onset of nausea, anorexia and malaise.
Severe vomiting (60%)
Abdominal pain (60%) should alert the clinician to the diagnosis.
Co-existing features mild pre-eclampsia,
but hypertension and proteinuria are usually mild.
Jaundice usually appears within 2w of the onset of symptoms
±Ascites.
ABOUBAKR ELNASHAR
Liver function
3- to 10-fold elevation in transaminase levels
raised alkaline phosphatase.
DIC
90%
often the presenting feature postpartum
± severe.
ABOUBAKR ELNASHAR
Usually: renal impairment.
± fulminant liver failure with hepatic
encephalopathy.
Hypoglycaemia
70%
± severe
± Polyuria and features of diabetes insipidus (DI)
ABOUBAKR ELNASHAR
Pathogenesis
±variant of pre-eclampsia.
A subgroup of women with AFLP and
Haemolysis, Elevated Liver enzymes and Low
Platelets (HELLP) syndrome is heterozygous for
long-chain 3-hydroxy-acylcoenzyme
A dehydrogenase (LCHAD) deficiency, a
disorder of mitochondrial fatty acid oxidation.
These women may succumb to AFLP or HELLP
syndrome when the fetus is homozygous for -
fatty acid oxidation disorders.
The mechanism of hepatocellular damage may
involve the affected fetus producing abnormal
fatty acid metabolites. ABOUBAKR ELNASHAR
Diagnosis
DD:
HELLP syndrome
Profound hypoglycaemia (70%)
Marked hyperuricaemia (which is out of
proportion to the other features of
preeclampsia (90%)
Coagulopathy (90%) in the absence of
thrombocytopenia.
ABOUBAKR ELNASHAR
ABOUBAKR ELNASHAR
Radiological evaluation
MRI,CT or US:
± hepatic steatosis, but the liver may appear
normal {fat is microvesicular}
CT:
decreased attenuation {fatty infiltration}.
ABOUBAKR ELNASHAR
Liver biopsy with special stains for fatty change
or electron microscopy:
gold standard for diagnosis.
microvesicular fatty infiltration (steatosis) of
hepatocytes, most prominent in the central
zone, with periportal sparing
little or no inflammation or hepatocellular
necrosis.
not always necessary or practical in the
presence of coagulopathy.
ABOUBAKR ELNASHAR
Management
Expeditious delivery:
improved prognosis for mother and baby.
Multidisciplinary team in an intensive care setting.
Coagulopathy and hypoglycaemia
should be treated aggressively before delivery.
Large amounts of 50% glucose may be needed
to correct the hypoglycaemia
fresh frozen plasma and albumin should be
given as necessary.
ABOUBAKR ELNASHAR
The best markers of severity:
– Prothrombin time
– Glucose
– Acidosis and raised lactate
– Encephalopathy.
ABOUBAKR ELNASHAR
Plasmapheresis
has been used in some cases.
N-acetylcysteine (NAC)
an antioxidant and glutathione precursor,
promotes selective inactivation of free radicals
logical tt in hepatic failure
Multiple system failure:
ventilation and dialysis.
ABOUBAKR ELNASHAR
Fulminant hepatic failure and encephalopathy:
referred urgently to a specialist liver unit.
Orthotopic liver transplantation
1. fulminant hepatic failure
2. irreversible liver failure despite delivery of the
fetus and aggressive supportive care.
ABOUBAKR ELNASHAR
Prompt reversal of the clinical and laboratory
findings:
usually follows delivery and may be very
dramatic
Significant morbidity is common (33%)
severe coagulopathy
need for repeated operations to control pp hge
If the woman survives the initial episode:
complete recovery without long-term liver
damage is the norm.
ABOUBAKR ELNASHAR
Recurrence
Has been described:
liver function should be closely monitored in
subsequent pregnancies.
Particularly likely in
women who are heterozygous for disorders of -
fatty acid oxidation:
screening for LCHAD deficiency:
send a neonatal blood spot for
acylcarnitine analysis using tandem mass
spectrometry.
ABOUBAKR ELNASHAR
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.
ABOUBAKR ELNASHAR
The woman is at risk of fulminant hepatic failure
and encephalopathy and may require transfer to a
regional liver unit.
Delivery of the fetus is the correct treatment once
hypoglycaemia, coagulopathy and hypertension
have been controlled.
ABOUBAKR ELNASHAR