Liver Disease in pregnancy

Question 1

What is acute fatty liver of pregnancy?

Answer 1

Rare pregnancy associated disorder of fatty infiltration of liver

Question 2

What is the aetiology of acute fatty liver of pregnancy?

Answer 2

Likely due to mitochondrial dysfunction of fatty acid oxidation

Question 3

What are the RFs of acute fatty liver of pregnancy?

Answer 3

Nulliparity, male fetus, multiple pregnancies, obesity, pre-eclampsia

Question 4

What is the epidemiology of acute fatty liver of pregnancy?

Answer 4

5/100k

Question 5

What is in the Hx/Ex of acute fatty liver of pregnancy?

Answer 5

Nil specific. In 3rd trimester, nausea, vomiting, swelling, abdominal pain, jaundice, bleeding.

Liver tenderness, ascites, coagulopathis (petechiae/purpura/bruising). 50% have proteinuric HTN.

Question 6

What is the pathology of acute fatty liver of pregnancy?

Answer 6

Accumulaiton of microvescicular fat in hepatocytes, periportal sparing, small yellow liver.

Question 7

What are the Ix of acute fatty liver of pregnancy?

Answer 7

Blood: FBC (Hb, Pl), clotting (Alb, studies) LFT (high transaminases/BR), glucose.

Question 8

What is the management of acute fatty liver of pregnancy?

Answer 8

Delivery necessary urgent to stop progression

Fluid support, correct hypoglycaemia, blood transfusion, care of preterm neonate.

Correct coagulopathy with FFP/cryoprecipitate if severe.

Liver transplant rarely necessary.

Question 9

What are the complications/ prognosis of acute fatty liver of pregnancy?

Answer 9

Maternal death (20%), hemorrage, renal failure, encephalopathy, sepsis or pancreatitis. Fetal death (30%).

Question 10

What is obstetric cholestasis?

Answer 10

Prutitus in pregnancy, which resolves at delivery, associated with abnormal liver function in the absence of other identifiable pathology.

Question 11

What is the aetiology of obstetric cholestasis?

Answer 11

Complex, likely genetic and hormonal factors.

Due to progesterone and oestrogen in the liver during pregnancy.

There is a link between PBC and OC

Question 12

What are the RFs of obstetric cholestasis?

Answer 12

PMHx, FHx, ethnicity (S. asian), multiple pregnancy, maternal age >35

Question 13

What is the epidemiology of obstetric cholestasis?

Answer 13

UK prevalence 0.7%.

Incidence is higher in winter months.

Question 14

What is in the Hx/Ex of obstetric cholestasis?

Answer 14

T2: generalised pruritus with no rash, worse at night, worse over palms and soles. Rarely dark urine/steathorrea

Excoriations

Question 15

What is the pathology of obstetric cholestasis?

Answer 15

Increased susceptibility to cholestatic effect of oestrogens. Related to impaired sulfation, may be related to defect in membrane phospholipid (inheritable).

Question 16

What are the Ix of obstetric cholestasis?

Answer 16

Bloods: LFT (high transaminases, may have high BR BUT ALP may be within normal for pregnancy!) bile acid (raised) clotting (may be abnormal due to low VitK absorption).

Dx of exclusion: therefore need to PET screen (FBC, UE, LFT, clotting), liver USS, hepatitis serology (A/C/B, E) EBV and CMV serology, liver antibodies (ANA, antiSMA)

Question 17

What is the management of obstetric cholestasis?

Answer 17

Monitor: weekly LFT, clotting, serial USS for fetal or intermittent CTG monitoring

Medication:

1. Chlorpheniramine/ promethazine (control prutirus)
2. Ursodeoxycolic acid (decrease serum bile acids and pruritus, no effect in fetal compromise)
3. VitK (reduce risk of fetal and maternal haemorrhage)
4. Emollients
5. Dexamethasone (if no response to UCDA)

Delivery: induce at 35 (>100)/37-38 (BA>40)/40 (BA<40) with continuous foetal monitoring throughout labour, increased risk of foetal death

Postpartum ensure resolution of abnormal LFTs after 10/7 postpartum.

Question 18

What are the complications/ prognosis of obstetric cholestasis?

Answer 18

Maternal PPH due to low Vit K

Baby HODN due to low Vit K, intracranial haemorrhage IN THE BABY, foetal distress, preterm

Complete recovery postnatally, but 90% recurrence in future pregnancies.

Risk of foetal death 2-3%.