A 37-year-old woman, gravida 1 para 0, at 34 weeks gestation comes to the emergency department due to nausea, vomiting, and severe epigastric pain. The patient first noticed the pain after breakfast yesterday. The pain continued throughout the day and now involves the right upper quadrant. She has been taking acetaminophen with no pain relief. The patient developed nausea and recurrent vomiting 4 hours ago. The patient has not had routine prenatal care. Temperature is 37.2 C (99 F), blood pressure is 130/80 mm Hg, and pulse is 106/min. Fetal heart rate monitoring shows a baseline of 170/min with minimal variability. There are no contractions on tocodynamometer. There is tenderness to palpation over the epigastrium and right upper quadrant, with no rebound or guarding. The uterus is nontender. Laboratory results are as follows: Which of the following is the most likely diagnosis in this patient?

Question

A 37-year-old woman, gravida 1 para 0, at 34 weeks gestation comes to the emergency department due to nausea, vomiting, and severe epigastric pain.  The patient first noticed the pain after breakfast yesterday.  The pain continued throughout the day and now involves the right upper quadrant.  She has been taking acetaminophen with no pain relief.  The patient developed nausea and recurrent vomiting 4 hours ago.  The patient has not had routine prenatal care.  Temperature is 37.2 C (99 F), blood pressure is 130/80 mm Hg, and pulse is 106/min.  Fetal heart rate monitoring shows a baseline of 170/min with minimal variability.  There are no contractions on tocodynamometer.  There is tenderness to palpation over the epigastrium and right upper quadrant, with no rebound or guarding.  The uterus is nontender.  Laboratory results are as follows:  Which of the following is the most likely diagnosis in this patient?

Quizplus · Accepted Answer

11ec25c8_d9fc_736f_bc81_1b3cd28c80d0_MD0003_00 This patient has acute fatty liver of pregnancy (AFLP), a rare but life-threatening condition that typically develops in the third trimester. Like other disorders that affect the liver during pregnancy (eg, preeclampsia, HELLP [Hemolysis, Elevated Liver enzymes, and Low Platelet count] syndrome), AFLP causes hepatic inflammation that leads to right upper quadrant pain, leukocytosis, and mildly elevated aminotransferases. However, AFLP is unique because it is an intrahepatic process due to microvesicular fatty infiltration of hepatocytes secondary to abnormal maternal-fetal fatty acid metabolism. This massive intrahepatic injury results in acute-onset fulminant liver failure that manifests as profound hypoglycemia (from inability to convert glycogen to glucose), hyperbilirubinemia, thrombocytopenia (<100,000/mm³), and possible disseminated intravascular coagulopathy. Patients develop rapid decompensation and multiorgan failure (eg, hemolytic anemia, renal failure); this in turn can cause placental hypoperfusion and fetal compromise (eg, fetal tachycardia, minimal variability). Due to high maternal and fetal mortality rates, management is immediate delivery. (Choice A) Acetaminophen toxicity can also cause fulminant liver failure; however, it typically causes markedly elevated aminotransferases (eg, >3,000 U/L), which are not seen in this patient. (Choice B) Pregnancy is a risk factor for acute cholecystitis, which can present with postprandial right upper quadrant pain and elevated aminotransferases. However, acute cholecystitis does not cause hyperbilirubinemia or fulminant liver failure (eg, hypoglycemia, thrombocytopenia). (Choice D) Acute pancreatitis can present with persistent vomiting, severe epigastric pain, leukocytosis, and elevated amylase and lipase levels. This patient has an elevated amylase (but normal lipase) level, likely from vomiting. A normal lipase and the right upper quadrant pain are not consistent with pancreatitis. (Choice E) Preeclampsia with severe features can present in the third trimester with right upper quadrant pain, elevated aminotransferases, and thrombocytopenia due to end-organ damage from hypertension (systolic ≥140 mm Hg or diastolic ≥90 mm Hg). This patient is normotensive, making this diagnosis less likely. Educational objective: Acute fatty liver of pregnancy typically occurs in the third trimester due to microvesicular fatty infiltration of hepatocytes, leading to liver inflammation (eg, right upper quadrant pain, elevated aminotransferases) and fulminant liver failure (eg, profound hypoglycemia, thrombocytopenia). Management is immediate delivery. References: Acute fatty liver disease of pregnancy: updates in pathogenesis, diagnosis, and management. (http://www.ncbi.nlm.nih.gov/pubmed/28291236) Pregnancy and liver disease. (http://www.ncbi.nlm.nih.gov/pubmed/26658682)

A 37-Year-Old Woman, Gravida 1 Para 0, at 34 Weeks