Jaundice in pregnancy, whilst relatively rare, has potentially serious consequences for maternal and fetal health. It can be caused by pregnancy or occur intercurrently. Causes of jaundice specific to pregnancy include:
Pre-eclampsia associated with HELLP syndrome (= h aemolysis, e levated l iver enzymes and l ow p latelet count).
- Acute fatty liver of pregnancy.
- Hyperemesis gravidarum.
- Intrahepatic cholestasis of pregnancy.
Approximately 3-5% of pregnant women may have abnormal liver function tests.
Cause of Jaundice in Pregnancy
Hepatic disorders in pregnancy may be:
- Unique to pregnancy
- Coincident with pregnancy and possibly exacerbated by pregnancy
Acute fatty liver of pregnancy
- It is a rare condition with an incidence of five in 100,000 pregnancies.
- Risk factors include first pregnancies, pre-eclampsia, twin pregnancies and male fetuses.
- It may be associated with a mutant gene producing a defect in mitochondrial fatty acid oxidation and infants born to mothers with acute fatty liver of pregnancy should be screened for defects in this system.
This usually presents acutely with nausea, vomiting and abdominal pain, fevers, headache and pruritus, beginning typically at about 35 weeks of gestation but can occur much earlier. It may also appear immediately after delivery.
Jaundice appears soon after onset of symptoms and can become intense in a large proportion of patients. Fulminant liver failure may follow.
- The white cell count is often elevated. There may also be neutrophilia and thrombocytopenia.
- Liver transaminases are moderately high.
- Raised serum bilirubin.
- Abnormal clotting with coagulopathy (prolongation of prothrombin and partial thromboplastin times with depression of fibrinogen levels).
- Biopsy would be diagnostic but coagulation problems often preclude it. CT/MRI scanning may show reduced attenuation in the liver.
Consider early delivery as the condition usually resolves afterwards with complete recovery. Supportive ITU care is frequently required.
Acute fatty liver of pregnancy is a life-threatening condition with an 18% maternal and 23% fetal mortality rate (up to 85% if associated with pre-eclampsia).
Serious complications include:
- Disseminated intravascular coagulation (DIC) and gastrointestinal bleeding.
- Hepatic coma.
- Renal failure.
- Intrahepatic cholestasis of pregnancy
- Intrahepatic cholestasis is defined as pruritus with elevated serum bile acids occurring in the second half of pregnancy, which resolves after delivery.
The incidence in Europe ranges from 0.1% to 1.5% of pregnancies but there is increased prevalence in South America and Scandinavia.
Pathogenesis remains unclear but is related to abnormal biliary transport across the canalicular membrane. Direct effects of female sex hormones induce cholestasis and inhibit the bile salt export pump.
This is supported by the fact that women with a history of intrahepatic cholestasis of pregnancy (ICP) are prone to cholestasis induced by oral contraceptives and vice versa.
The main symptom is pruritus, especially of the palms and soles, which is followed by generalised symptoms. This usually occurs from week 25 of gestation.
Jaundice is uncommon, but when present, arises 2-4 weeks after the onset of pruritus.
Aminotransferase activity can be increased by 20 times the normal level.
Raised gamma-glutamyltransferase activity is unusual but is indicative of MDR3 mutation or underlying liver disease unrelated to pregnancy.
The key diagnostic test is a fasting serum bile acid concentration of greater than 10 mmol/L.
Ursodeoxycholic acid (10-15 mg/kg bodyweight) provides relief against pruritus, improved liver function tests, and is well tolerated both by mother and fetus.
Maternal morbidity is low. The importance of this disorder is the effects on the fetus. It can lead to chronic placental insufficiency which may result in anoxia, prematurity, perinatal death, fetal distress, and stillbirth.