Case Study
A 31-year-old primigravida with a twin pregnancy presented at 34 weeks with a 2-week history of malaise, nausea, and vomiting. There was no relevant past medical history, and physical examination was unremarkable. The patient was initially presumed to have a nonspecific viral infection. Later she developed abdominal pain and began to develop some of the clinical features of preeclampsia (i.e., hypertension and proteinuria) as well as mild polydipsia and polyuria. She was admitted to hospital for observation, and blood results revealed a marked rise in transaminases and urate with a thrombocytopenia. The rest of her blood results, including serum bilirubin, coagulation, and renal function, were normal.
With 24 hours, she became confused, with deterioration in her hepatic and renal function. Her clotting screen was abnormal (international normalized ratio [INR] was elevated to 1.5) and was associated with some mild jaundice and intermittent hypoglycemia. Urgent imaging of the abdomen showed ascites. Over the course of the day, the patient became encephalopathic and developed the features of worsening renal failure and disseminated intravascular coagulation (DIC).
She required a dextrose infusion to prevent hypoglycemia and fresh frozen plasma to normalize her coagulopathy before undergoing cesarean section under general anesthesia, resulting in the birth of twin males. The mother required organ support on intensive care. Although her renal function had improved by 48 hours, her liver function remained deranged for nearly a week. By day 8, she was much improved, and other than some persistently elevated transaminases, she returned to the postnatal ward. The babies required oxygen and treatment of hypoglycemia but otherwise had an uneventful course. However, they required follow-up by pediatricians with a specialist interests in genetic screening and dietary management of fatty acid oxygenation disorders. A diagnosis of acute fatty liver of pregnancy was made
Key Points
Acute fatty liver of pregnancy usually presents in the second trimester, often with nonspecific symptoms.
An acute hepatitis-like picture develops, with a marked transaminase rise but with jaundice not a prominent feature.
There is often coexisting preeclampsia and sometimes some of the features of HELLP (hemolysis, elevated liver enzymes, low platelets)syndrome.
The condition has a significant morbidity and mortality for both mother and fetus, and liver function may continue to deteriorate into the puerperium.
Management of these patients requires delivery of the fetus and recognition and treatment of the complications of liver failure.
Imaging may be unremarkable, but liver biopsy showing microvesicular fatty infiltration may aid diagnosis. However, in practice, coagulopathy may preclude this.
Discussion
Liver disease in pregnancy covers a wide spectrum of clinical entities. First, women may suffer from liver disease unrelated to pregnancy or as direct result of the pregnancy, with occasionally preexisting liver disease altered by the pregnancy itself. Second, liver disease may present in several ways, including a hepatitis-like picture, jaundice, abdominal pain, malaise, encephalopathy, variceal bleeding, and sepsis, possibly complicated by other organ failures such as renal or cardiorespiratory.
Another area to consider is that the normal ranges for many biochemical tests are different in pregnancy (Table 19.1), which needs to be borne in mind in their interpretation.1
Table 19.1 Changes to Liver Function in Pregnancy
| Test | Prepregnancy range | Pregnancy range | Comments |
|---|---|---|---|
| Transaminases (IU/liter) | 0–40 | 0–30 | – |
| (alanine and aspartate) | |||
| Bilirubin (μmol/liter) | 0–17 | 3–15 | – |
| Gamma glutamyl transferase (IU/liter) | 10–50 | 5–40 | – |
| Alkaline phosphatase | 30–130 | 30–130 | First and second trimesters |
| (IU/liter) | 130–400 | Third trimester | |
| (produced by placenta) | |||
| Albumin (g/liter) | 35–45 | 28–37 | Secondary to hemodilution |
| Bile salts (μmol/liter) | 0–14 | 0–14 | – |
Unrelated or Coincidental Liver Disease
Young woman may suffer with ongoing liver disease that predates the pregnancy or is acquired coincidentally with the pregnancy. Commonly, this may be due to viral hepatitis or gallstones. Hepatitis may result from hepatitis A, B, C, D, and E, seronegative hepatitis, and other infections such as chickenpox, herpes simplex, and cytomegalovirus (which are much more commonly in immunocompromised patients). Gallstones may present with abdominal pain and jaundice. Other causes include drugs and toxins such as acetaminophen, recreational drugs, drug reactions, and mushroom poisoning from Amanita phalloides. Vascular events such as ischemia, veno-occlusive disease, and Budd Chiari syndrome (hepatic vein thrombosis), Wilson’s disease, and autoimmune diseases also may occur (Table 19.2). Finally, pregnancy in patients with alcoholic cirrhosis, while once very rare, is now becoming more common, as is pregnancy in patients after liver transplantation. Patients require consideration of the transmission of any infective agent to the fetus, and the impact of any ongoing medication is paramount.
Table 19.2 Liver Disease Unrelated to Pregnancy
| Disease | Examples | Comments |
|---|---|---|
| Hepatitis |
| Hepatitis E associated with increased perinatal mortality |
| Gallstones | ||
| Drug induced |
| May be idiosyncratic or toxicity related |
| Autoimmune disease |
| May be overlapping features; many patients will require ongoing treatment throughout pregnancy |
| Vascular events |
| |
| Others |
| From Amanita phalloides |
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