Obstetric Cholestasis/AFLP Flashcards

1
Q

Prevalence of OC?

A

0.7% of all pregnancies

1.2%-1.5% women of Indian-Asian or Pakistani-Asian descent

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Aetiology of OC?

A

The cause is multifactorial and believed to have a genetic and environmental component.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Definition of OC?

A

OC refers to pruritus in the absence of a primary skin condition with abnormal maternal bile acid concentrations.

Pruritus and raised bile acid concentrations should return to normal after birth.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

In patients with OC/ICP, which tests are associated with the risk of stillbirth?

A

Only the maternal bile acid concentrations are associated with the risk of stillbirth.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Are maternal bile salts associated with the intensity of pruritus?

A

No

There is no correlation between maternal pruritus and biochemical derangement.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

True/False. Pregnant women with normal bile acid concentrations, raised transaminases and pruritus can be diagnosed with OC?

A

False. The current consensus, according to the greentop guidelines, is that the diagnosis of ICP requires elevated maternal bile acid concentrations.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What’s the diagnosis: pruritus in pregnancy with peak bile acid conc <19micromol/L?

A

Gestational pruritis

Basically a normal bile acid conc + pruritus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What’s the diagnosis: pruritus in pregnancy with peak bile acid conc 19-39 micromol/L?

A

Mild ICP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What’s the diagnosis: pruritus in pregnancy with peak bile acid conc 40-99 micromol/L?

A

Moderate ICP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What’s the diagnosis: pruritus in pregnancy with peak bile acid conc same/greater than 100 micromol/L?

A

Severe ICP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What’s the upper limit of normal for bile acid conc in pregnancy?

A

18

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the clinical concerns/issues with ICP?

A
  1. The mother being able to cope with the itching, which can range from mild to unbearable, localized to widespread and can interfere with sleep. This can have a negative impact on her mental health.
  2. Options available to control the maternal symptoms e.g. pruritus
  3. Options for monitoring
  4. Reducing the fetal risks
  5. Preterm birth
  6. Anxiety and difficulty sleeping
  7. The optimal timing of birth
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Diagnosis of ICP?

A

Pruritus in pregnant women with normal appearing skin and a raised peak bile salt conc of 19 micromol/L and above.

  • The diagnosis is more likely if confirmed by resolution of the pruritus and elevated bile salts postpartum.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

True/False: ICP may resolve in pregnancy

A

False. Resolution usu occurs post delivery. If resolution occurs during pregnancy, then a diagnosis of ICP is unlikely.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Workup of a patient with suspected ICP?

A

As ICP is a diagnosis of exclusion it is important to rule out other differentials, especially if pt has an atypical/ uncertain picture of ICP.

  1. Serum bile acid concs - most sensitive and specific.
    2.LFTs - deranged transaminases
    between 2-10 x ULN, elevated bilirubin (Unconj + conj)
  2. PT/PTT- coagulopathy present w/
    preeclampsia and liver disease
    4.Viral Hepatitis screen (A, B, C)
    - CMV and EBV
  3. Autoimmune testing - r/o
    autoimmune hepatitis; ANA, anti-smooth muscle antibodies, anti-mitochondrial antibodies (r/o intrahepatic cholestasis).
  4. Liver ultrasound - cirrhosis, structural causes of cholestasis - bile duct dilation, cholelithiasis/choledocholithiasis

*2022 GTG suggests that viral, autoimmune and liver ultrasound are not indicated because the likelihood of identifying viral/autoimmune/structural causes is very low, and did not result in new diagnoses.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

When to consult a hepatologist?

A
  • If she has severe, very early (T1/T2) or atypical presentation of ICP, because these patients are more likely to have an underlying genetic predisposition or an alternative/additional diagnosis.
  • Refer postpartum if the pruritus and biochemical abnormalities fail to resolve.
17
Q

When does pruritus stop?

A

Within the first few hours or days for the majority of women.

18
Q

Post delivery, when are bile acids and LFTs repeated?

A

4 weeks postpartum.

Lfts may become abnormal during birth and may be elevated in the immediate postpartum period. Testing 4weeks after allows time for the results to normalize.

19
Q

What are the maternal risks associated with ICP?

A

1.Pruritus- sleep disruption; negative
impacts on emotional wellbeing and mental health.
2. Preeclampsia- OR:3.7. Ensure ongoing BP assessment and urinalysis at each ANC review.
3. GDM- OR:2.4, however screening follows the recommended guidelines
4. Hepatobiliary disease later in life
5. Immune mediated diseases (DM, thyroid, crowd’s disease + inflamm polyarthritis)
6. Hep C

20
Q

For an uncomplicated singleton gestation, what are the risk of stillbirth for the following peak bile salt concs?
A. 19-39 micromol/L
B. 40-99 micromo/L
C. 100 micromol/L or more

A

A. Risk similar to background risk
(0.13%)
B. Risk similar to background risk
until 38-39wks (0.28%) ~0.3%
C. Increased risk (3.44%) ~3%

21
Q

What are the perinatal risks?

A

Patients with mod/severe ICP are at higher risks of:
1. Preterm birth (spontaneous/
iatrogenic)
2. MSL during birth and delivery
3. Nursery admission
4. Perinatal morbidity
5. Stillbirth

22
Q

How often to repeat maternal peak bile acids after diagnosis?

A

To decide of the timing of delivery, repeat testing is done weekly for both mild ICP (19-39) and mod ICP (40-99).

Severe ICP: future testing may not impact management and MAY not be routinely offered.

23
Q

What fetal monitoring do you recommend?

A

As these patients are at increased risk of adverse perinatal outcomes, evaluation includes cardiotocography/non-stress test and obstetric ultrasound for BPP, EFW and dopplers.
- Isolated ICP does not cause FGR, however monitoring for placental insufficiency can be considered in patients who develop preeclampsia or GDM.
- It should be explained to the mother that close fetal monitoring does not predict stillbirth.
- She should be advised to monitor the quality and quantity of her fetal movements and she should present immediately to the maternity unit if any reduction or change in fetal movements is noted.

24
Q

What medical treatment will you offer?

A
  1. Topical emollients such as calamine lotion or aqueous cream with or without 1% menthol.
  2. Antihistamines (chlorphenamine, diphenhydramine) act as nighttime sedatives.
    -Loratidine and cetrizine have no sedative effect and may also be used.
  3. Ursodeoxycholic acid may reduce pruritus in some patients.
  4. Use of Rifampicin is currently being researched to evaluate use in patients with ICP.
25
Q

Should ursodeoxycholic acid be used and why?

A

-Ursodeoxycholic acid decreases pruritus by decreasing circulating bile salts concentration.
-It may be beneficial in reducing pruritus in some patients so it may be offered.
- UDCA may offer some benefit in reducing late preterm birth in women with bile acid concs 40 micromol/L and above, who are 34-36 weeks pregnant. However this does not prevent stillbirth.
-UCDA it is not recommended to reduce the risk of adverse perinatal outcomes.

26
Q

Should routine vit k be given?

A

No, only for the minority of patients with:
1. Reduced absorption of dietary fats e.g. steatorrhea or
2. abnormal coagulation studies.

27
Q

At what gestational age would you deliver a pt with ICP?

A

Mild - by 40 weeks
Moderate - planned delivery by
38-39 weeks
Severe- planned delivery at 35-36
weeks. Patient would be advised that her risk of stillbirth is higher than the background risk at 3.44%

Patients with comorbodities such as preeclampsia, GDM, multifetal pregnancy should be advised that they have an increased risk of stillbirth and that influences the timing of planned birth.

28
Q

What determines the mode of birth?

A

The presence or absence of obstetric or medical indications.
- Planned deliveries may be induced.

29
Q

Outline your intrapartum management of this patient?

A

Offer continuous ctg monitoring for women with a severe icp/peak bile acids of 100 or more because of the increased risk of adverse perinatal outcomes/stillbirth in these patients.
Patients with moderate/severe icp are at increased risk of MSL and this
influences the need for continuous ctg monitoring.

30
Q

True/False: Women with ICP have an increased risk of PPH?

A

False

31
Q

What is your contraceptive advice for a postpartum pt?

A

LNG-IUS, copper T IUD, POP, POIM and medroxyprogesterone actetate can be used without any restriction (UKMEC of 1).

She may also be advised to avoid oestrogen containing contraception because it increases the risk of intrahepatic cholestasis. Of note, the 2022 GTG notes that this is unlikely to happen in the majority of women and patients with a h/o ocp-related cholestasis should should advised to avoid estrogen containing contraception. Otherwise, the advantages of outweigh the potential risks (UKMECS -2).
– These patients are advised to return if recurrence of symptoms as this could indicate a diagnosis of ocp-related cholestasis. Alternative contraception options would be discussed (LNG-IUS, copper T IUD, POP, POIM).

32
Q

Advise for future pregnancies

A

1.High Risk of recurrence - up to 90%
2.Risk of occurrence in sibling/sister
3.Benign condition no long term material sequelae.
4.Increased risk of childhood obesity and dyslipidaemia by age 16

*At booking for subsequent pregnancies, perforom baseline LFTs and bile salts to record her baseline levels, since the risk of recurrence is high.

33
Q

How’s Acute fatty liver of pregnancy diagnosed?

A

Using the Swansea criteria, 6 or more of the following should be present:
1. Encephalopathy
2. Diabetes insipidus
3. Vomiting
4. Abd pain
5. Hypoglycaemia 70%
6. Hyperuricaemia
7. Raised ammonia
8. AKI
9. Leucocytosis
10. Coagulopathy 90%, usu w/o thrombocytopenia
11. DIC

34
Q

How does AFLP differ from HELLP syndrome?

A

ALFP:

  1. Has hypoglycaemia
  2. Significantly high hyperuricaemia
  3. Coagulopathy but normal platelets
  4. Elevated wbc
  5. More elevated liver enzymes
35
Q

Maternal symptoms associated with OCP?

A
  • pruritus ( an be very intense at night and prevent sleep).
  • steatorrhea
  • pale stools
  • dark urine
36
Q

What is the percentage increase in foetal death for every 1mmol/L rise in bile acid?

A

1-2% increase

37
Q

What class of drug is cyclophoshamide? What are the recommendations for use in pregnancy?

A
  • Class D drug
  • Known to be fetotoxic & teratogenic
  • Stop 3 months prior to contraception
38
Q

Incidence of AFLP?

A

1 I 7000 to 1 in 20,000