ACUTE FATTY LIVER OF PREGNANCY (AFLP)

Dr KABERA Ren Family Physician Rwanda

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 acids leads 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 not be used to exclude the diagnosis. Liver ultrasonographic examination may reveal increased echogenicity in severe cases. A computed tomography (CT) scan may show decreased or diffuse attenuation 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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