Antenatal Care: Cholestasis of Pregnancy & Acute Fatty Liver of Pregnancy Flashcards
What is obstetric cholestasis?
Obstetric cholestasis is characterised by the reduced outflow of bile acids from the liver during pregnancy. The condition resolves after delivery of the baby.
Relatively common complication of pregnancy (1%).
When does obstetric cholestasis typically occur?
It usually develops later in pregnancy (i.e. after 28 weeks)
What is obstetric cholestasis thought to be the result of?
Increased oestrogen & progesterone
What ethnicity is obstetric cholestasis more common in?
South Asian
Describe journey of bile from liver to intestines
1) Bile acids are produced in the liver from the breakdown of cholesterol.
2) Bile acids flow from liver to the hepatic ducts, past the gallbladder
3) Bile acids flow out of the bile duct to the intestines
What are bile acids made from?
Breakdown of cholesterol
What happens in obstetric cholestasis?
In obstetric cholestasis, the outflow of bile acids is reduced, causing them to build up in the blood, resulting in the classic symptoms of itching (pruritis).
What complication is obstetric cholestasis associated with?
an increased risk of stillbirth.
What is the key symptom of obstetric cholestasis?
Itching (pruritus) - particularly affecting the palms of the hands and soles of the feet.
Symptoms of obstetric cholestasis?
1) Pruritus
2) Fatigue
3) Dark urine
4) Pale, greasy stools
5) Jaundice
Is there a rash associated with obstetric cholestasis?
No - if a rash is present, an alternative diagnosis should be considered, such as polymorphic eruption of pregnancy or pemphigoid gestationis.
Classic LFT feature in most obstetric cholestasis cases?
raised bilirubin is seen in > 90% of cases
Where is itching tyically worse in obstetric cholestasis?
palms, soles and abdomen
Differentials of obstetric cholestasis (i.e. other causes of pruritus and deranged LFTs)?
1) Gallstones
2) Acute fatty liver
3) Autoimmune hepatitis
4) Viral hepatitis
What 2 investigation should be done in women presenting with pruritus?
1) LFTs: abnormal liver function tests (LFTs), mainly ALT, AST and GGT
2) Bile acids: raised
What is a normal LFT change in pregnancy?
It is normal for alkaline phosphatase (ALP) to increase in pregnancy.
A rise in ALP without other abnormal LFT results is usually due to placental production of ALP, rather than liver pathology.
Why does ALP rise in pregnancy?
Placenta produces ALP
1st line treatment of obstetric cholestasis?
Ursodeoxycholic acid: it improves LFTs, bile acids and symptoms.
How can symptoms of itching be managed in obstetric cholestasis?
1) Emollients (i.e. calamine lotion) to soothe the skin
2) Antihistamines (e.g. chlorphenamine) can help sleeping (but does not improve itching)
What can be given in clotting (prothrombin) time is deranged in obstetric cholestasis?
Water-soluble vitamin K
How can a lack of bile salts in obstetric cholestasis lead to vitamin K deficiency?
What can this lead to?
1) Bile acids are important in the absorption of fat-soluble vitamins in the intestines.
2) A lack of bile acids can lead to vitamin K deficiency.
3) Vitamin K is an important part of the clotting system, and deficiency can lead to impaired clotting of blood.
Monitoring of obstetric cholestasis?
Monitor of LFTs is required during pregnancy (weekly) and after delivery (after at least ten days).
When does sobstetric cholestasis typically resolve?
After birth
Potential management of obstetric cholestasis when LFTs and bile acids are severely deranged.?
Planned delivery after 37 weeks may be considered
Overview of management of obsetric cholestasis:
1) ursodeoxycholic acid
2) vitamin K supplementation
3) induction of labour at 37 weeks is common practice
What is acute fatty liver of pregnancy?
Acute fatty liver of pregnancy is rare complication which may occur in the third trimester or the period immediately following delivery.
There is a rapid accumulation of fat within the liver cells (hepatocytes), causing acute hepatitis.
When does acute fatty liver of pregnancy typically occur?
3rd trimester or period immediately following delivery.
Complications of acute fatty liver of pregnancy?
There is a high risk of liver failure and mortality, for both the mother and fetus.
Pathophysiology of acute fatty liver of pregnancy?
1) Acute fatty liver of pregnancy results from impaired processing of fatty acids in the placenta.
2) This is the result of a genetic condition in the fetus that impairs fatty acid metabolism: most common cause is long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency in the fetus.
3) This is an autosomal recessive condition: this mode of inheritance means the mother will also have one defective copy of the gene.
4) The LCHAD enzyme is important in fatty acid oxidation, breaking down fatty acids to be used as fuel.
5) The fetus and placenta are unable to break down fatty acids.
6) These fatty acids enter the maternal circulation, and accumulate in the liver.
7) The accumulation of fatty acids in the mother’s liver leads to inflammation and liver failure.
What is most common genetic condition in the foetus that leads to acute fatty liver in pregnancy?
Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency in the fetus
Role of the LCHAD enzyme?
Fatty cid oxidation: breaks down fatty acids to be used as fuel
inheritance pattern of LCHAD deficiency?
Autosomal recessive (means the mother will also have one defective copy of the gene)
Presentation of acute fatty liver of pregnancy?
The presentation is with vague symptoms associated with hepatitis :
- General malaise and fatigue
- Nausea and vomiting
- Jaundice
- Abdominal pain
- Anorexia (lack of appetite)
- Ascites
- Hypoglycaemia
- Severe disease may result in pre-eclampsia
What can severe acute fatty liver of pregnancy result in?
Pre-eclampsia
Main investigation in acute fatty liver of pregnacy?
LFTs
What will LFTs show in acute fatty liver of pregnancy?
Elevated liver enzymes (ALT and AST) –> ALT is typically elevated e.g. 500 u/l
What other bloods may be needed in acute fatty liver of pregnancy?
1) Bilirubin: raised
2) WBC: raised
3) Clotting: raised prothrombin and INR
4) Platelets: low
In you exams, what should elevated liver enzymes and low platelets in a pregnant woman make you think of?
HELLP syndrome: a type of preeclampsia that causes elevated liver enzymes and low platelet count.
This is much more common than acute fatty liver of pregnancy but keep this in mind as differential.
Management of acute fatty liver of pregnancy?
Obstetric emergency: prompt admission and delivery of the baby.
Name 4 pregnancy-related skin changes and rashes
1) Polymorphic Eruption of Pregnancy
2) Atopic Eruption of Pregnancy
3) Melasma
4) Pyogenic Granuloma
5) Pemphigoid Gestationis
What is polymorphic eruption of pregnancy?
AKA: pruritic and urticarial papules and plaques of pregnancy.
It is an itchy rash that tends to start in the third trimester.
When does polymorphic eruption of pregnancy tend to appear?
3rd trimester
Presentation of polymorphic eruption of pregnancy?
- Itchy rash: usually begins on the abdomen
- Particularly associated with stretch marks (striae).
- Urticarial papules (raised itchy lumps)
- Wheals (raised itchy areas of skin)
- Plaques (larger inflamed areas of skin)
Google photos on passmed!
Management of polymorphic eruption of pregnancy?
The condition will get better towards the end of pregnancy and after delivery. Management is to control the symptoms depending on severity, with:
1) Topical emollients
2) Topical steroids
3) Oral antihistamines
4) Oral steroids may be used in severe cases
What is the commonest skin disorder found in pregnancy?
Atopic eruption of pregnancy
How do lesions of polymorphic eruption of pregnancy often first appear?
lesions often first appear in abdominal striae
What does atopic eruption of pregnancy essentially refers to?
Eczema flare ups during pregnancy: this includes both women that have never suffered with eczema and those with pre-existing eczema.
When does atopic eruption of pregnancy present?
first and second trimester of pregnancy.
What are the 2 types of atopic eruption of pregnancy?
1) E-type, or eczema-type
2) P-type, or prurigo-type
Describe E type vs P type of atopic eruption of pregnancy
E-type, or eczema-type: eczematous, inflamed, red and itchy skin, typically affecting the insides of the elbows, back of knees, neck, face and chest.
P-type, or prurigo-type: intensely itchy papules (spots) typically affecting the abdomen, back and limbs.
Which type of atopic eruption of pregnancy has intensely itchy papules (spots) typically affecting the abdomen, back and limbs?
P-type, or prurigo-type
Which type of atopic eruption of pregnancy has eczematous, inflamed, red and itchy skin, typically affecting the insides of the elbows, back of knees, neck, face and chest?
E-type, or eczema-type
Which type of atopic eruption of pregnancy affects abdomen, back and limbs?
P-type, or prurigo-type
Which type of atopic eruption of pregnancy affects insides of the elbows, back of knees, neck, face and chest?
E-type, or eczema-type
Management of atopic eruption of pregnancy?
The condition will usually get better after delivery. Management is with:
1) Topical emollients
2) Topical steroids
3) Phototherapy with ultraviolet light (UVB) may be used in severe cases
4) Oral steroids may be used in severe cases
What is melasma?
Melasma is also known as mask of pregnancy.
It is characterised by increased pigmentation to patches of the skin on the face.
How does melasma present?
It is characterised by increased pigmentation to patches of the skin on the face. This is usually symmetrical and flat, affecting sun-exposed areas.
Describe increased pigmentation seen in melasma
This is usually symmetrical and flat, affecting sun-exposed areas.
What areas does melasma affect?
Sun-exposed areas
What is melasma thought to be partly related to in pregnancy?
Increased female sex hormones
What else is melasmaa associated with (outside pregnancy)?
1) COCP
2) HRT
3) Sun exposure
4) Thyroid disease
5) FH
Management of melasma?
No active treatment is required if the appearance is acceptable to the woman. Management is with:
1) Avoiding sun exposure and using suncream
2) Makeup (camouflage)
3) Skin lightening cream (e.g. hydroquinone or retinoid creams), although not in pregnancy and only under specialist care
4) Procedures such as chemical peels or laser treatment (not usually on the NHS)
What is pyogenic granuloma?
Pyogenic granuloma is also known as lobular capillary haemangioma. This is a benign, rapidly growing tumour of capillaries.
How does pyogenic granuloma present?
It present as a discrete lump with a red or dark appearance - it is a benign, rapidly growing tumour of capillaries.
- A rapidly growing lump that develops over days up to 1-2 cm in size
- Often occur on fingers, or on the upper chest, back, neck or head
- May cause profuse bleeding and ulceration if injured
What 3 factors are linked to pyogenic granuloma?
1) trauma
2) pregnancy
3) more common in women and young adults
Most common sites of pyogenic granuloma?
- head/neck, upper trunk and hands
- oral mucosa in pregnancy
Describe progression of pyogenic granuloma
1) initially small red/brown spot
2) rapidly progress within days to weeks forming raised, red/brown lesions which are often spherical in shape
What needs to be excluded in pyogenic granuloma?
Other differentials, such as malignancy, need to be excluded (particularly nodular melanoma).
Management of pyogenic granuloma?
When they occur in pregnancy, they usually resolve without treatment after delivery.
Treatment is with surgical removal with histology to confirm the diagnosis.
What is pemphigoid gestationis?
A rare autoimmune skin condition that occurs in pregnancy.
What happens in pemphigoid gestationis?
1) Autoantibodies are created that damage the connection between the epidermis and the dermis.
2) The pregnant woman’s immune system may produce these antibodies in response to placental tissue.
3) This causes the epidermis and dermis to separate, creating a space that can fill with fluid, resulting in large fluid-filled blisters (bullae).
What may cause a pregnant woman’s immune system to produce these antibodies in pemphigoid gestationis?
Response to placental tissue
when does pemphigoid gestationis usually occur?
2nd or 3rd trimester
Typical presentation of pemphigoid gestationis?
An itchy red papular or blistering rash around the umbilicus, that then spreads to other parts of the body. Over several weeks, large fluid-filled blisters form.
Management of pemphigoid gestationis?
The rash usually resolves without treatment after delivery. It may go through stages of improvement and worsening during pregnancy and after birth. The blisters heal without scarring.
Treatment is with:
1) Topical emollients
2) Topical steroids
3) Oral steroids may be required in severe cases
4) Immunosuppressants may be required where steroids are inadequate
5) Antibiotics may be necessary if infection occurs
Risks to baby in pemphigoid gestationis?
1) Fetal growth restriction
2) Preterm delivery
3) Blistering rash after delivery (as the maternal antibodies pass to the baby)
