Common signs and symptoms of AFLP
Common signs and symptoms
(%)
Nausea, vomiting, jaundice
70
Abdominal pain
60–70
Nervous system (altered sensorium, confusion, disorientation, psychosis, restlessness, seizures, coma)
60–80
Disseminated intravascular coagulation
55–80
Gastrointestinal bleeding
20–60
Acute renal failure
50
Oliguria
40–60
Tachycardia
50
Late-onset pyrexia
50
A.
Lab findings in AFLP [4–9]
1.
Hematology
Hemoglobin | Normal (unless hemorrhage/hemolysis) |
Hematocrit | Normal (unless hemorrhage/hemolysis) |
White blood cells | Mildly elevated |
Platelets | Normal to mild decrease |
2.
Liver function
Aspartate aminotransferase | Moderate to marked elevation |
Alanine aminotransferase | Moderate to marked elevation |
Gamma-glutamyltransferase | Mild elevation |
Alkaline phosphatase | Moderate to marked elevation |
Lactate dehydrogenase | Normal and then mild decrease |
Bilirubin, total | Moderate to marked elevation |
Bilirubin, direct | Moderate to marked elevation |
Ammonia | Mild elevation |
Lactate | Mild elevation |
Glucose | Moderate-marked decrease |
Cholesterol | Mild decrease |
Triglycerides | Mild decrease |
3.
Coagulation tests
International normalized ratio | Moderately to markedly elevated |
Prothrombin time | Mildly elevated |
Partial thromboplastin time | Mildly elevated |
Fibrinogen | Moderately to markedly decreased |
Fibrin split products | Present |
Antithrombin III | Moderately to markedly decreased |
4.
Renal
Uric acid | Moderately to markedly elevated |
Blood urea nitrogen | Mildly elevated |
Creatinine | Moderately to markedly elevated |
B.
Imaging: Ultrasound and computed tomography may show fatty infiltration of the liver; however, the findings are not sensitive or specific enough to make a definitive diagnosis of AFLP. False-negative results are common.
C.
Histopathology: Liver biopsy is usually not necessary for diagnosis. History, clinical findings, and lab and imaging results are sufficient to make the diagnosis in most cases. Liver biopsy should not be performed to confirm a diagnosis of AFLP or to distinguish AFLP from severe pre-eclampsia, because management of both conditions are the same. Liver biopsy may be justified rarely in cases when liver function does not return to normal postpartum. Histologically, microvesicular steatosis with sparing of zone 1 is the characteristic feature. There may be patchy hepatocellular necrosis. Widespread necrosis or inflammation is absent.
Diagnosis
1.
Diagnosis of AFLP is challenging because the initial clinical presentation may be nonspecific.
2.
Among other causes of pathological hepatic dysfunction, acute fatty liver of pregnancy (AFLP) is uncommon compared to pre-eclampsia and HELLP syndrome.
3.
History, clinical features, and biochemical abnormalities may mimic acute viral hepatitis, pre-eclampsia, HELLP syndrome, obstetric cholestasis, or other causes of hepatic dysfunction.
4.
AFLP is uncommon. Therefore, the best approach to any pregnant women with liver dysfunction is to quickly rule out other, more likely causes.
Differentiating AFLP from Other Causes of Pathological Hepatic Dysfunction in Pregnancy [3–9]
1.
Pre-eclampsia and eclampsia:
(a)
Onset: 2nd or 3rd trimester.
(b)
Incidence: 5–10 %.
(c)
Features: Nausea, vomiting, epigastric pain, edema, hypertension, mental status changes, and jaundice (late feature).
(d)
Labs: ALT <500 U/L, proteinuria, and DIC (7 %).
(e)
Maternal complications: Hypertensive crisis, renal impairment, hepatic rupture/infarct, and neurological (seizures, cerebrovascular accidents).
(f)
Fetal complications: Abruption and prematurity; IUGR and perinatal morbidity and mortality.
(g)
One of the most common multiorgan diseases of late pregnancy.
(h)
Women with AFLP can also have pre-eclampsia; however women with pre-eclampsia alone do not usually have jaundice or hypoglycemia which are characteristic of AFLP.
(i)
AFLP often presents more acutely whereas pre-eclampsia develops over several days or weeks.
(j)
Pre-eclampsia rarely presents with severe coagulopathy.
2.
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HELLP syndrome:
(a)
Onset: 3rd trimester
(b)
Incidence: 0.10 % (4–12 % of women with pre-eclampsia)
(c)
Features: Symptoms of pre-eclampsia (hypertension, headache, blurred vision), epigastric or right upper quadrant pain, nausea, vomiting, hematuria, and jaundice (late feature)
(d)
Labs: Hemolysis, ALT <500 U/L, platelets <100 × 109/L, elevated LDH, and DIC (20–40 %)