Question 6

Created on Mon, 05/18/2015 - 17:28
Last updated on Fri, 06/08/2018 - 18:34
Pass rate: 63%
Highest mark: ?

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A  20  year  old  primi-gravida   presents  at  37  weeks  gestation  with  jaundice, headache,  blurred vision and hypertension  (140/90mmHg).  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:

Hb*

80 G/L

(115-160)

Platelets*

52 x 109/L

(140-400)

INR*

1.8

(0.9-1.3)

APTT*

55 seconds

(25-38)

LDH*

654 U/L

(110-250)

Fibrinogen*

1.0 G/L

(1.5-4.0)

Total Bilirubin*

51µmol/L

(<20)

Urea*

30 mmol/L

(3-8)

Creatinine*

298 µmol/L

(70-120)

Potassium*

5.1 mmol/L

(3.2-4.5)

(a) List 4 likely differential diagnoses for this clinical presentation.

(b) What other investigations would you order for this patient and why?

(c) List the important management interventions for each of your differential diagnoses.

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College Answer

(a) List 4 likely differential diagnoses for this clinical presentation.

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

(b) What other investigations would you order for this patient and why?

•  Transaminases (full liver function tests) Assessment of HELLP

•  Peripheral blood film smear
Evidence of haemolysis or MAHA

•  Reticulocyte count, haptoglobins, conjugated/unconjugated bilirubin
Haemolysis screen

•  Blood, sputum, urine and vaginal swab for MC&S Septic screen

•  Urinalysis – protein, WBCs, RBCs, casts
Evidence of infection or proteinuria (pre-eclampsia)

•  Renal tract ultrasound
Rule out obstruction

(c) List the important management interventions for each of your differential diagnoses.

a.  Pre-eclampsia
i.  Deliver baby

ii.  Control BP
iii.  Hydralazine, beta blockers
iv.  SNP/GTN if intravenous agent required.

v.  Prevention of seizures
vi.  Magnesium sulphate

b.  HELLP Syndrome
i.  Deliver baby
ii.  Regular monitoring of platelet count and liver function
iii. Supportive measures whilst observing in HDU for dangerous complications  – hepatic haemorrhage/rupture,  progressive  renal failure, pulmonary oedema.

c.  Sepsis with DIC
i.  Timely delivery of baby in consultation with obstetrician.

ii.  Early broad spectrum antibiotics.
iii.  Cardiovascular support – adequate volume resuscitation and establish a MAP > 65mmHg.

d.  HUS-TTP
i.  Deliver the baby.
ii.  Fresh frozen plasma
iii.  Therapeutic plasma exchange

iv.  Corticosteroid therapy
v.  Monoclonal antibody  therapy – Rituximab

e.  Acute fatty liver of pregnancy
i.       Timely delivery of baby once mother stabilised

ii.      Correction of DIC
iii.       Supportive therapy
iv.      Monitoring      and     treatment     of     complications     post     delivery     eg pancreatitis
v.      Consideration for liver transplantation in with irreversible severe liver
failure despite delivery and aggressive supportive care

Discussion

One cannot add very much to this question.

One also cannot help but notice that it closely resembles Question 28 from the second paper of 2013.The question has been slightly altered, but otherwise it is exactly the same.

(a) List 4 likely differential diagnoses for this clinical presentation.

Pre-eclampsia is present - on the basis of the hypertension

  • HELLP (grade 1)- because of the combination of thrombocytopenia and deranged LFTs
  • TTP-HUS - on the basis of thrombocytopenia and renal failure
  • Sepsis and DIC - on the basis of fever, thrombocytopenia and low fibrinogen
  • Acute fatty liver of pregnancy - on the basis of LFT derangement and coagulopathy

(b) What other investigations would you order for this patient and why?

  • blood film (for HELLP, TTP, HUS)
  • Septic screen (for sepsis)
  • Quantitiative D-dimer (for DIC)
  • formal LFTs (for HELLP)
  • ADAMTS13 (for TTP)
  • Urinary protein (for pre-eclampsia)
  • Ammonia level (for encephalopathy of acute fatty liver of pregnancy)
  • Liver ultrasound (for fatty liver of pregnancy, as well as to exclude liver rupture)

(c) List the important management interventions for each of your differential diagnoses.

For all of them, expeditious delivery of the baby.

For all of them, correction of coagulopathy

For all of them, supportive measures and observation in critical care environment

For pre-eclampsia, standard management as routine

For HELLP, no specific management

For sepsis, IV antibiotics

For TTP, plasma exchange

For acute fatty liver of pregnancy, nothing specific until they need a transplant.

References

Brief summaries with references:

Pre-eclampsia and eclampsia

HELLP syndrome

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

Oh's Intensive Care manual: Chapter 63   (pp. 677) Preeclampsia  and  eclampsia by Wai  Ka  Ming  and  Tony  Gin

RCOG Guidelines for the management of severe pre-eclampsia/eclampsia (2006)

Haram, Kjell, Einar Svendsen, and Ulrich Abildgaard. "The HELLP syndrome: clinical issues and management. A review." BMC pregnancy and childbirth 9.1 (2009): 8.

Geary, Michael. "The HELLP syndrome." BJOG: An International Journal of Obstetrics & Gynaecology 104.8 (1997): 887-891.