A 30-year-old female who is 34 weeks pregnant (G1PO) has presented with nausea and vomiting for 3 days with right upper quadrant pain. On examination she is confused, jaundiced with a blood pressure of 120/70 mmHg.
The following are results from a venous blood sample taken on admission:
Parameter |
Patient Value |
Normal Adult Range |
Sodium |
138 mmol/L |
135 - 145 |
Potassium |
3.8 mmol/L |
3.5 - 5.0 |
Urea |
15.0 mmol/L* |
3.0 - 8.0 |
Creatinine |
245 µmol/L* |
45 - 90 |
Albumin |
30 q/L* |
33 - 40 |
Glucose |
2.5 mmol/L* |
3.0 - 7.8 |
Bilirubin (total) |
142 umol/L* |
< 20 |
Alkaline phosphatase |
293 U/L* |
30 - 110 |
Aspartate aminotransferase |
99 U/L* |
< 31 |
Alanine aminotransferase |
88 U/L* |
< 34 |
y-Glutamyl transferase |
67 U/L* |
< 40 |
Lactate dehydrogenase |
180 U/L |
110 - 250 |
Uric acid |
0.72 mmol/L* |
0.15 - 0.50 |
Activated partial thromboplastin time |
45 sec* |
36 - 38 |
International normalised ratio |
2.8* |
0.9 - 1.3 |
Platelets |
123 x 109/L* |
150 - 450 |
List three likely differential diagnoses for the above clinical picture. (30% marks)
College answer
- Acute fatty liver of pregnancy (AFLP):
- HELLP (Haemolysis, elevated liver enzymes and low platelets) Syndrome:
- Pre-eclampsia with hepatic involvement
- Viral hepatitis – commonest cause of jaundice in pregnancy
- ALT and AST would be expected to be greatly elevated (>500 – 1000 U/L) and DIC is rare
- Intrahepatic Cholestasis of Pregnancy
Additional Examiners‟'Comments:
Many candidates did not complete part 2.
Discussion
The abnormalities are:
- Confusion
- RUQ pain
- Renal failure
- Hypoglycaemia
- Raised bilirubin
- Raised LFTs - no specific pattern
- Raised urate
- Coagulopathy (both APTT and INR)
- Thrombocytopenia, which is mild
Thus, this is pre-eclampsia, or something along the spectrum between pre-eclampsia and HELLP syndrome. A distant third possibility is TTP-HUS, but the LDH is within the normal range, which means that there is probably no haemolysis.
Thus, the differentials might include
- Pre-eclampsia
- HELLP
- TTP/HUS
- Acute fatty liver of pregnancy
Such things are usually best described in the form of a table. Good articles inform the discussion, most notably the article by Guntupalli et al from the 2005 special pregnancy issue of Critical Care Medicine.
Cause | Diagnostic features | Notes and management options |
Causes of liver failure which are unrelated to pregnancy | ||
Drug-induced hepatitis |
|
|
Shock, haemorrhage |
|
|
Decompensation of pre-existing liver disease |
|
|
Causes of liver failure which are exacerbated by pregnancy | ||
Viral hepatitis |
|
|
Portal vein thrombosis |
|
|
Hepatic venous thrombosis |
|
|
Cholecystitis |
|
|
Pregnancy-related causes of liver failure | ||
Hyperemesis gravidarum |
|
|
Intrahepatic cholestasis of pregnancy (icterus gravidarum) |
|
|
Pre-eclampsia |
|
|
HELLP |
|
|
Acute fatty liver of pregnancy |
|
|
Acute hepatic rupture |
|
|
Other causes of febrile jaundiced coma with thrombocytopenia | ||
TTP/HUS |
|
|
Sepsis with DIC |
|
References
Chapter 64 (pp. 684) General obstetric emergencies by Winnie TP Wan and Tony Gin
Chapter 65 (pp. 692) Severe pre-existing disease in pregnancy by Jeremy P Campbell and Steve M Yentis
Pandey, Chandra Kant, et al. "Acute liver failure in pregnancy: Challenges and management." Indian journal of anaesthesia 59.3 (2015): 144.
Sahai, Shweta, and Ravi Kiran. "Acute liver failure in pregnancy: Causative and prognostic factors." Saudi journal of gastroenterology: official journal of the Saudi Gastroenterology Association 21.1 (2015): 30.
Guntupalli, Saketh R., and Jay Steingrub. "Hepatic disease and pregnancy: an overview of diagnosis and management." Critical care medicine 33.10 (2005): S332-S339.
Aggarwal, Neelam, et al. "Pregnancy and cirrhosis of the liver." Australian and New Zealand journal of obstetrics and gynaecology 39.4 (1999): 503-506.
Horowitz, Rivka S., et al. "Placental transfer of N-acetylcysteine following human maternal acetaminophen toxicity." Journal of Toxicology: Clinical Toxicology 35.5 (1997): 447-451.
Outlaw, William M., Jamal A. Ibdah, and Kenneth L. Koch. "Hyperemesis Gravidarum and Maternal Liver Disease." (2000).
Munazza, Bibi, et al. "Liver function tests in preeclampsia." J Ayub Med Coll Abbottabad 23.4 (2011): 3-5.
Manas, Kenneth J., et al. "Hepatic hemorrhage without rupture in preeclampsia." New England Journal of Medicine 312.7 (1985): 424-426.
Kang, Yun Dan. "Portal Vein Thrombosis during Pregnancy." Korean Journal of Perinatology 26.3 (2015): 245-249.
Casey, Brian M., and Susan M. Cox. "Cholecystitis in pregnancy." Infectious diseases in obstetrics and gynecology 4.5 (1996): 303-309.
McMinn, Johnny R., and James N. George. "Evaluation of women with clinically suspected thrombotic thrombocytopenic purpura‐hemolytic uremic syndrome during pregnancy." Journal of clinical apheresis 16.4 (2001): 202-209.