Question 28

Created on Tue, 05/12/2015 - 18:10
Last updated on Mon, 05/01/2017 - 18:37
Pass rate: ?
Highest mark: ?

Other SAQs in this paper

Other SAQs on this topic

A 20-year-old primigravida presents at 37 weeks gestation with jaundice, headache, blurred vision and hypertension (140/90 mmHg). The antenatal period was otherwise unremarkable. She is febrile, drowsy, pale, icteric and has pedal oedema. The uterus is palpated as for a full term pregnancy with a normal CTG trace. Examination is otherwise normal.

The following are her early blood results:

Parameter

Patient Value

Normal Adult Range

Hb

80 G/L*

115 – 160

Platelets

52 x 109/L*

140 – 400

International Normalised Ratio

1.8*

0.9 – 1.3

Activated Partial Thromboplastin Time

55 seconds*

25 – 38

Lactate Dehydrogenase

654 U/L*

110 – 250

Fibrinogen

1.0 G/L*

1.5 – 4.0

Total Bilirubin

51 micromol/L*

< 20

Urea

30 mmol/L*

3 – 8

Creatinine

298 micromol/L*

70 – 120

Potassium

5.1 mmol/L*

3.2 – 4.5

a) List four likely diagnoses for this clinical presentation.

b) For each of your differential diagnoses:

  1. List the important management interventions.
  2. List one additional diagnostic test.

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College Answer

a)

  • Pre-eclampsia
  • HELLP Syndrome
  • Sepsis with DIC
  • HUS-TTP
  • Acute fatty liver of pregnancy

b) 
Pre-eclampsia

  • Deliver baby
  • Control BP
    • Hydralazine, beta blockers
    • SNP/GTN if intravenous agent required.
  • Prevention of seizures
  • Magnesium sulphate
  • Urinalysis – protein, WBCs, RBCs, casts 
  • Evidence of infection or proteinuria (pre-eclampsia) 
  • Renal US

HELLP Syndrome

  • Deliver baby
  • Regular monitoring of platelet count and liver function
  • Supportive measures whilst observing in HDU for dangerous complications
    • hepatic haemorrhage/rupture, progressive renal failure, pulmonary oedema.
  • Peripheral blood film smear 
  • Reticulocyte count, haptoglobins, conjugated/unconjugated bilirubin 
  • Haemolysis screen 
  • Full liver function tests

Sepsis with DIC

  • Timely delivery of baby in consultation with obstetrician.
  • Early broad-spectrum antibiotics.
  • Cardiovascular support – adequate volume resuscitation and establish MAP > 65mmHg.
  • Blood, sputum, urine and vaginal swab for MC&S 
  • Septic screen

HUS-TTP

  • Deliver the baby.
  • Fresh frozen plasma
  • Therapeutic plasma exchange
  • Corticosteroid therapy
  • Monoclonal antibody therapy – Rituximab
  • Evidence of haemolysis or MAHA 
  • Reticulocyte count, haptoglobins, conjugated/unconjugated bilirubin 
  • Haemolysis screen 
  • ADAMTS 13

Acute fatty liver of pregnancy

  • Timely delivery of baby once mother stabilised
  • Correction of DIC
  • Supportive therapy
  • Monitoring and treatment of complications post delivery e.g. pancreatitis
  • Consideration for liver transplantation in with irreversible severe liver failure despite delivery and aggressive supportive care
  • Full set liver function tests 
  • BSL 
  • Liver US
  • Evidence of haemolysis or MAHA 
  • Reticulocyte count, haptoglobins, conjugated/unconjugated bilirubin 
  • Haemolysis screen

Discussion

This question closely resembles Question 6 from the first paper of 2010.

The college has yielded five differentials, each of which deserve a whole chapter dedicated to them.

There is little to add to the sufficently detailed yet concise college answer.

One must remember that in most of these conditions, the key step is to deliver the baby.

The one critical investigations for each differential would be...

  • Pre-eclampsia - urinary protein
  • HELLP Syndrome - hemolytic screen
  • Sepsis with DIC - septic screen
  • HUS-TTP - ADAMTS13 test
  • Acute fatty liver of pregnancy - ultrasound to rule out hepatic rupture 

A more complete list:

Acute Liver Failure in Pregnancy
Cause Diagnostic features Notes and management options
Causes of liver failure which are unrelated to pregnancy
Drug-induced hepatitis
  • Paracetamol level
  • N-acetylcysteine crosses the placenta and has a protective effect in the foetus (Horowitz et al, 1997)
Shock, haemorrhage
  • Ultrasound: structurally normal liver
  • The LFT derangement which follows resuscitation for severe postpartum haemorrhage
  • Should improve after the shock state has resolved
Decompensation of pre-existing liver disease
  • Ultrasound: cirrhosis
  • Risk of preterm delivery and peripartum complications is increased (Aggarwal et al, 1999)
  • Normal management of the cirrhotic patient applies
Causes of liver failure which are exacerbated by pregnancy
Viral hepatitis
  • Hep B and C serology
  • Most common cause of LFT derangement in pregnancy
  • B and C are the most common
  • usually, BP is normal (in contrast with HELLP)
  • Ribavirin is contraindicated (a teratogen)
  • Other antiviral drugs may still be useful to decrease the viral load preior to delivery (to protect the baby from vertical transmission)
Portal vein thrombosis
  • Ultrasound: portal vein occlusion on Doppler
  • Due to hypercoagulable state of pregnancy
  • Heparin infusion is the standard of care 
  • Generally, TIPS procedure is too technically difficult in pregnancy - but that is another option
Hepatic venous thrombosis
  • Ultrasound: hepatic vein occlusion on Doppler
Cholecystitis
  • Ultrasound: thickened gall bladder wall, stones
  • LFTs: cholestatic picture
  • Conservative antibiotic therapy is best
  • Non-emergency surgery has better outcomes, i.e. it pays to delay until the acute flare has passed (Casey et al, 1996)
Pregnancy-related causes of liver failure
Hyperemesis gravidarum
  • LFTs: "transaminitis"
  • Unlike the others, this is a feature mainly of the first trimester
  • It is associated with raised transaminases, as opposed to liver failure per se - synthetic function is preserved (Outlaw et al, 2000)
  • Antiemetics and supportive care are the only options
Intrahepatic cholestasis of pregnancy (icterus gravidarum)
  • History of jaundice and pruritis
  • LFTs: cholestatic picture
  • Ultrasound: gall bladder looks normal
  • Pruritis is usually the patient's greatest concern. Can be managed with ursodeoxycholic acid.
  • Resolves rapidly with delivery
  • Will occur again in the next pregnancy in 60%
Pre-eclampsia
  • LFT derangement is due to fibrin deposition and endothelial dysfunction in the sinusoids.
  • Typically, this is also not "liver failure" but rather an LFt derangement of unclear significance (Munazza et al, 2013)
  • Standard therapy applies: antihypertensives, magnesium sulfate, and urgent delivery
HELLP
  • Thrombocytopenia
  • Low haptoglobin
  • Raised LDH,
  • Uncojugated bilirubin
  • Blood film features of haemolysis
  • See the local chapter.
  • Delivery is the treatment
  • Some authors recommend corticosteroids (Guntupalli et al, 2005) but only as a means of helping foetal lung maturation
  • Thrombocytopenia and LFT derangement will continue for up to 48 hours postpartum
  • Standard therapy applies: antihypertensives, magnesium sulfate, urgent delivery, correction of coagulopathy
Acute fatty liver of pregnancy
  • Presents with abdominal pain, vomiting, hypoglycaemia, coagulopathy
  • Characteristic ultrasound findings of the liver parenchyma
  • 18% maternal mortality, 2% foetal mortality (Guntupalli et al, 2005)
  • Liver failure is present, not just LFT derangement
  • Delivery fixes everything, as in HELLP
  • Fulminant liver failure may be present by then, and liver transplant may be the only option
Acute hepatic rupture
  • Ultrasound: haematoma
  • Haemodynamic instability and haemorrhagic shock
  • Abdominal pain (RUQ)
  • Haemoperitoneum
  • Maternal mortality is around 30% (Manas et al, 1985
  • If it has not ruptured (i.e. only a subcapsular haematoma) then conservative management and urgent dleivery are probably safe
  • Surgical packing and/or angioembolisation may be the only options
Other causes of febrile jaundiced coma with thrombocytopenia
TTP/HUS
  • Pentad: thrombocytopenia, microangiopathic haemolytic anemia, neurologic abnormalities, renal failure, and fever. See local chapter.
  • Low ADAMTS-13 levels are found. 
  • The SAQs often give a picture which could be consistent with TTP. It actually does occur often in pregnancy and the postpartum period (McMinn et al, 2001)
  • "How is this not HELLP?" one might ask. Well:
    • HELLP is never in the first trimester
    • HELLP always resolves following delivery
  • I.e. if after delivery the abnormalities persist, plasmapheresis becomes a serious option.
Sepsis with DIC
  • Bacteraemia
  • Haemodynamic instability, hypotension

References

The UpToDate links for the college's differentials are here: