acute fatty liver of pregnancy (aflp)
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Dr KABERA Ren
Family Physician
Rwanda
ACUTE FATTY LIVER OF PREGNANCY (AFLP)
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INTRODUCTION
Acute fatty liver of pregnancy (AFLP) is a rare complication
of the third trimester. Sheehan described the pathology in 1940 as an "acute yellow
atrophy" of the liver, then thought to be related to delayed
chloroform poisoning.
Early recognition and termination of the pregnancy (delivery)
and extensive supportive therapy have reduced the mortality
rate to approximately 20%.
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PATHOPHYSIOLOGY
A familial deficiency of long-chain 3-hydroxyacyl-CoA
dehydrogenase (LCHAD) caused by a single codon mutation
Recessively inherited mitochondrial abnormalities of fatty acid
oxidation.
The mitochondrial dysfunction in the oxidation of fatty acidsleads to an accumulation in hepatocytes.
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PHYSIOPATHOLOGY
The microvesicular steatosis in the liver.
The infiltration of fatty acids causes acute liver insufficiency.
It is associated with a high maternal and neonatal morbidity and
mortality
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EPIDEMIOLOGY
Rare complication (1/13000)
Unique for pregnancy
For inexplicable reasons, the disease is more common in
nulliparas.
It likely is more common with a male fetus
15 percent of cases have a multifetal gestation
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CLINICAL PRESENTATION
Complaints
Malaise
Nausea and vomiting (70%); this may present for the first time in
the third trimester
Bleeding
Abdominal pain
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CLINICAL PRESENTATION
Physical
Hypertension(50%)
Bleeding
Confusion and altered mental status (encephalopathy)
Jaundice
Right upper-quadrant and epigastric pain (50-80%)
Edema
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DIFFERENTIALS
Drug-Induced Hepatotoxicity
Eclampsia
HELLP Syndrome
Hepatitis, Viral
Preeclampsia
Toxicity, Acetaminophen
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DIAGNOSIS
Aspartate transaminase (AST) and alanine transaminase (ALT) can be
elevated due to the hepatic injury. Decreased gluconeogenesis and, therefore, decreased blood glucose
levels.
Liver detoxification is also affected, resulting in elevated levels of
blood ammonia, especially late in the disease course leading to
encephalopathy.
Disseminated intravascular coagulation (DIC) with prolongation of
prothrombin time, low fibrinogen, and low antithrombin levels DIC is not due to consumption of the clotting factors but to decreased
production by the damaged liver.
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DIAGNOSIS
Bilirubin levels are elevated. This elevation is primarily the
conjugated form, with levels exceeding 5 mg/dL. Some patients may develop pancreatitis, which can result in
elevated amylase, lipase, and increased blood sugars.
In 50 % blood creatine and uric acid can become elevated,leading to metabolic acidosis.
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DIAGNOSIS
Imaging Studies
Imaging studies have a low sensitivity for diagnosing AFLP and should notbe used to exclude the diagnosis.
Liver ultrasonographic examination may reveal increased echogenicity in
severe cases.
A computed tomography (CT) scan may show decreased or diffuseattenuation in the liver.
Histologic Findings
Although the criterion standard for diagnosis of AFLP is liver biopsy The histological findings demonstrate pericentral microvesicular fat
infiltration with minimal inflammation or necrosis
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MANAGEMENT
Maternal deaths are reported to be caused by sepsis, hemorrhage,aspiration, renal failure, pancreatitis, and gastrointestinal bleeding. Therapy
is directed toward these complications.
Delivery of the fetus, regardless of gestational age ,if no delivery in 24 hrs.
prior to diagnosis a C-section may be done Mode of delivery is dependent on the following several factors:
Close monitoring of Fetal status
Due to Maternal coagulation status: replacement of their coagulation factors Normal diet and D5 for Management of the severe hypoglycemia .
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MANAGEMENT
Fluid balance should be closely monitored, as patients may
develop pulmonary edema due to low plasma oncotic pressures Maternal hemorrhage: Renal function impairment; acute tubular
necrosis, hepatorenal syndrome
Transfusions: fresh-frozen plasma, cryoprecipitate, whole blood,packed red cells, and platelets are usually necessary if surgery
is performed or if lacerations complicate vaginal delivery
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REFERENCES
Williams Obstetrics23rd Ed.
Current Diagnosis in Obstetrics and Gynecology - 10th Ed.
Current diagnosis and treatment in Gastroenterology 2nd Ed.
Acute Fatty liver of pregnancy. Dey M, Reema K. N Am J Med
Sci. 2012 Nov;4(11):611-2
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Thank you
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