Maternal Medicine Flashcards
What is the prevalence of epilepsy amongst women of childbearing age?
a. 0.1-0.3%
b. 0.5-1%
c. 2-3%
d. 4-5%
e. 6-7%
B - 0.5-1%
Anti-epileptic drugs are known to be taken by women in around 1 in 200 pregnancies overall.
What is the risk of maternal mortality amongst women with epilepsy relative to the general population?
a. Equivalent
b. 2x
c. 3x
d. 10x
e. 20x
D - 10x
The maternal mortality rate for women with epilepsy is of the order of 60 per 100,000 and has not significantly improved over the last decade
You meet a 23 year women with epilepsy in the antenatal clinic. She states that her epilepsy is normally well controlled on lamotrigine with her last seizure over 6 months prior to conception. What proportion of women with epilepsy experience an increase in their seizure frequency during pregnancy?
a. 5%
b. 17%
c. 33%
d. 40%
e. 55%
B - 17%
64% of women with epilepsy remain seizure free in pregnancy.
17% experience an increase in their seizure frequency while 16% experience a decrease.
Around 3.5% of women have a fit in labour.
A 21 year old primigravida with known epilepsy on lamotrigine is seen in the antenatal clinic at booking. Her epilepsy is well controlled and she has been seizure free for 18 months prior to conception. What fetal surveillance is indicated in pregnancy according to RCOG guidelines?
a. SFH measurements with midwife only
b. Uterine artery doppler at 20/40 and serial scans if abnormal
c. Serial scans from 28/40
d. Serial scans from 24/40
e. Single third trimester growth scan at 36/40
C - Serial scans from 28/40
The RCOG recommend that women with epilepsy on anti-epileptic drugs undergo serial growth scans from 28/40.
There is evidence to suggest a 2-fold increased risk of SFGA in such women.
A 38 year old with known epilepsy attends the pre-conceptual counselling clinic. She is currently taking sodium valproate though wishes to conceive in the near future. What is the risk of major congenital malformation amongst women taking sodium valproate in pregnancy?
a. Up to 2%
b. Up to 5%
c. Up to 10%
d. Up to 20%
e. Up to 50%
C - Up to 10%
Valproate has the highest risk of major congenital malformations of any anti-epileptic drug - up to 10%.
Neural tube defects, facial clefts and hypospadias most commonly.
A 24 year old primigravida attends the antenatal clinic at booking. She is known to have poorly controlled epilepsy and is taking lamotrigine, levetiracetam and phenytoin. She wishes to know her risk of a major congenital malformation. What do you advise is the risk of a major congenital malformation in her case?
a. 3-5%
b. 6-8%
c. 9-10%
d. 12-14%
e. 18-20%
B - 6-8%
The risk of a major congenital malformation associated with anti-epileptic drug use is 6-8% for women on polytherapy (10% if includes valproate); comparatively it is 3-5% for women on monotherapy (10% for valproate).
A 27 year old woman is referred to the antenatal clinic at booking on account of a past history of epilepsy. On questioning she reveals that she had a history of seizures in childhood though her last was age 16 and she has been off her anti-epileptic medication for almost 7 years. What schedule of care is most appropriate for this woman in pregnancy?
a. Midwife led care
b. Obstetric led care though no need for additional fetal surveillance
c. Obstetric led care with serial scans from 28 weeks
d. Obstetric led care with fetal echocardiography at 20-24 weeks
e. Obstetric led care with serial scans from 28 weeks and fetal echocardiography at 20-24 weeks
A - Midwife led care
Women who have been seizure free for 10 years or more and off their anti-epileptic drugs for 5 years or more, may be managed as per women without epilepsy.
What is the risk of intrapartum seizures amongst women with epilepsy?
a. 1%
b. 3.5%
c. 10%
d. 15%
e. 33%
B - 3.5%
3.5% of women with epilepsy seize in labour
A woman with known epilepsy is admitted in labour at 5cm. Shortly afterwards she experiences a tonic-clonic seizure. Her blood pressure is normal. Which of the following drugs is considered first line for the management of epileptic seizures in labour?
a. Magnesium Sulphate
b. Phenytoin
c. Lamotrigine
d. Sodium Valproate
e. Lorazepam
E - Lorazepam
Benzodiazepines are first line management of epileptic seizures in labour. Phenytoin may be used in refractory cases.
A 29 year old with known epilepsy is found dead in her home during the second trimester. Following a coroner’s review, sudden unexplained death in pregnancy (SUDEP) is determined to have been the cause. Which of the following anti-epileptic drugs is thought to have the highest association with SUDEP?
a. Sodium valproate
b. Lamotrigine
c. Carbemazepine
d. Diazepam
e. Phenobarbitol
B - Lamotrigine
There are some concerns regarding the incidence of SUDEP amongst women on lamotrigine as rates are higher (2.5 per 1000 patient years) than women on other anti-epileptic drugs (0.5 per 1000 patient years). This may well be related to the higher numbers of women on lamotrigine as well as reduced serum levels with advancing gestation.
What is the normal change in cardiac output seen in pregnancy?
a. Increase by 20%
b. Increase by 40%
c. Increase by 60%
d. Decrease by 20%
e. Decrease by 10%
B - Increase by 40%
This is principally due to an increase in stroke volume though also in part due to a rise in heart rate of approx. 10-20bpm
Source: HB of Obstetric Medicine
What is the normal change in heart rate in pregnancy?
a. Increase by <10bpm
b. Increase by 10-20bpm
c. Increase by 20-30bpm
d. Decrease by <10bpm
e. Decrease by 10-20bpm
B - Increase by 10-20bpm
Source: HB of Obstetric Medicine
What is the most common congenital cardiac defect seen amongst women of reproductive age?
a. Patent ductus arteriosus
b. Coarctation of the aorta
c. Atrial septal defect
d. Hypoplastic left heart syndrome
e. Congenital aortic stenosis
C - Atrial septal defect
These are usually well tolerated in pregnancy. There may be an association with migraine. There is a theoretical risk of paradoxical embolism though this is low.
Blood loss at delivery can increase the left to right shunt leading to a drop in LV output and coronary blood flow.
Source: HB of Obstetric Medicine
A 29 year old woman with Marfan syndrome is reviewed in the antenatal clinic at booking. What is the most significant prognostic consideration amongst patients with Marfan Syndrome in pregnancy?
a. Pectus excavatum
b. Pneumothorax
c. Mitral valve prolapse
d. Mitral regurgutation
e. Aortic root dilatation
E - Aortic root dilatation
Marfan syndrome carries a risk of aortic dissection and rupture. The risk is 10% where the aortic root is >4cm dilated and pregnancy is generally contraindicated above this level.
Women who do continue with pregnancies where the aortic root is >4cm typically require beta-blockade in pregnancy as this has been shown to reduce the rate of complications of further dilatation. Regular echocardiography is required and patients with progressively enlarging or an aortic root >4-4.5cm should be delivered by El. LSCS.
Source: HB of Obstetric Medicine
A woman with a history of a congenital heart defect enquires about the risk of her baby being affected. Overall, what is the risk of congenital heart disease amongst infants born to mothers who themselves have a history of congenital heart defect?
a. No increased risk
b. 1-2%
c. 2-5%
d. 3-8%
e. 9-11%
B - 1-2%
This is well over double the general population risk. The risk is higher for mothers than fathers. Affected women should as such be offered a detailed cardiac scan in the second trimester.
The risk is highest for aortic stenosis (18-20%)
Source: HB of Obstetric Medicine
A 32 year old recent migrant is reviewed in the antenatal clinic at booking. When first seen by her midwife, she disclosed a history of rheumatic fever in infancy. What is the predominant cardiac lesion seen in women with a history of rheumatic fever?
a. Aortic regurgitation
b. Pulmonary atresia
c. Ventriculo-septal defect
d. Mitral stenosis
e. Mitral regurgitation
D - Mitral stenosis
Mitral stenosis accounts for over 90% of rheumatic heart disease seen in women in pregnancy
Source: HB of Obstetric Medicine
A woman with a strong family history of hypertrophic cardiomyopathy attends the pre-conceptual counselling clinic. She has recently undergone echocardiography which is suggestive of mild hypertrophic cardiomyopathy. What class of drugs are the mainstay of therapy for hypertrophic cardiomyopathy in pregnancy?
a. Calcium channel blockers
b. Alpha-blockers
c. Beta-blockers
d. Diuretics
e. ACE-inhibitors
C - Beta-blockers
70% of HCM is familial with autosomal dominant inheritance. It is generally well tolerated in pregnancy owing to an increase in LV cavity size. Women who develop symptoms should be started on beta-blockers in pregnancy.
Source: HB of Obstetric Medicine
A 46 year old Para 4 woman undergoes an echocardiogram in a DCDA twin pregnancy at 34 weeks of gestation for suspected peripartum cardiomyopathy after she presents with worsening shortness of breath, peripheral oedema and palpitations. What threshold of LV ejection fraction is used for the diagnosis of peripartum cardiomyopathy?
a. <90%
b. <75%
c. <60%
d. <45%
e. <30%
D - <45%
Peripartum cardiomyopathy requires an LV ejection fraction of <45% for confirmation of diagnosis.
In addition to cardiac considerations, thromboprophylaxis is important for these women as up to 40% will develop systemic embolisation and 5% will stroke.
Source: HB of Obstetric Medicine
A 35 year old patient with a mechanical heart valve has been anticoagulated with low-molecular weight heparin throughout her pregnancy. She has attended delivery suite with spontaneous labour at 36/40 and her CTG is pathological. She requires urgent delivery. What drug may be used to reverse heparin if required in such a situation?
a. Vitamin K
b. Beriplex (prothrombin complex)
c. Cryoprecipitate
d. Protamine sulphate
e. Fresh frozen plasma
D - Protamine sulphate
Heparin may be reversed with protamine sulphate in an emergency. Warfarin is reversed with FFP and Vitamin K.
Source: HB of Obstetric Medicine
What is the maternal death rate associated with acute myocardial infarction in pregnancy?
a. 1-2%
b. 5-7%
c. 11-15%
d. 18.5-21%
e. 25-30%
B - 5-7%
The incidence of MI in pregnancy is increasing - likely related in part, to advancing maternal age. It occurs most commonly in the third trimester and perpartum.
Management is as per the non-pregnant patient with anti-coagulants, beta blockers and nitrates.
Source: HB of Obstetric Medicine
A 36 year old patient with Ehlers-Danlos syndrome presents at 35 weeks of gestation with acute severe chest pain which radiates to the back and jaw. Blood pressure is noted to be elevated in the right arm at 160/110mmHg though when measured in the left is normal at 110/60mmHg. What is the most likely diagnosis here?
a. Pulmonary embolism
b. Acute myocardial infarction
c. Amniotic fluid embolism
d. Aortic dissection
e. Pulmonary oedema
D - Aortic dissection
Differential blood pressure in each arm is highly suggestive of thoracic aortic dissection which is more common in women with Ehlers-Danlos (type IV - vascular), Turners and Marfan’s syndromes.
A chest radiograph would be expected to show a classic mediastinal widening appearance.
Management is surgical - careful but quick control of blood pressure, delivery by Caesarean section and cardiac surgery to replace the aortic root.
Source: HB of Obstetric Medicine
What is the drug of choice for treating fetal tachyarrhythmias antenatally?
a. Bisoprolol
b. Verapamil
c. Sotalol
d. Flecanide
e. No treatment of known benefit
D - Flecanide
Source: HB of Obstetric Medicine
What is the lifetime prevalence of breast cancer in women in the UK?
a. 1 in 5
b. 1 in 9
c. 1 in 15
d. 1 in 17
e. 1 in 23
B - 1 in 9
Breast cancer is the most common cancer in women with a lifetime risk of 1 in 9 in the UK population. It is the leading cause of death in women aged between 35 and 54.
What percentage of new breast cancer diagnoses are made in women aged <45?
a. 5%
b. 10%
c. 15%
d. 20%
e. 25%
C - 15%
15% of all new breast cancer diagnoses are made in women aged under 45 – equivalent to around 5000 women per year in the UK of reproductive age, 10-20% of which are either associated with pregnancy or occur within 12 months of delivery. Up to 10% of women under 45 who develop breast cancer subsequently fall pregnancy.
What is the preferred mode of screening for metastases of breast cancer diagnosed in pregnancy?
a. PET CT
b. MRI with gadolinium contrast
c. DEXA bone scan
d. USS Liver and CXR
e. CT with contrast
D - USS Liver and CXR
When breast cancer is confirmed, screening for metastases in pregnancy is only indicated if there is a high clinical suspicion and should comprise a chest x-ray and liver ultrasound. Gadolinium contrast MRI is not recommended unless a specific need exists – while data on its safety is limited, no adverse effects of gadolinium have been reported on the fetus. DEXA and CT are avoided owing to the effect of irradiation on the fetus. There is no role for tumours markers in pregnancy.
A patient who is diagnosed with breast cancer in the second trimester requires adjunctive chemotherapy. What is the preferred anti-emetic regimen in chemotherapy- induced sickness?
a. Metoclopramide monotherapy
b. Cyclizine and domperidone
c. Ondansetron and domperidone
d. Ondansetron and dexamethasone
e. Dexamethasone monotherapy
D - Ondansetron and Dexamethasone
Standard chemotherapy anti-emetic regimens should be used in pregnancy – 5HT3-serotonin receptor antagonists (ondansetron) and dexamethasone are most effective.
A woman is restarted on chemotherapy postnatally following a diagnosis of breast cancer in the third trimester. What is the best advice to give mothers on chemotherapy who wish to breastfeed?
a. Breast feeding is safe on chemotherapy – no precautions required
b. Avoid feeding for 24 hours after chemo
c. Avoid feeding for 36 hours after chemo
d. Express and discard 20ml of milk after chemo
e. Avoid breastfeeding entirely while on chemo
E - Avoid breastfeeding entirely while on chemo
Breastfeeding while on chemotherapy is not advised as the drugs cross into milk and may cause neonatal leucopoenia. An interval of 14 days after the last chemotherapy session is advised prior to commencing feeding to enable drug clearance from milk.
In planning elective birth in women on chemotherapy for breast cancer, ideally what interval should have elapsed since the last chemo session prior to delivery?
a. 24-48 hours
b. 6-7 days
c. 1-2 weeks
d. 2-3 weeks
e. >4 weeks
D - 2-3 weeks
Most women affected by breast cancer in pregnancy should deliver at term and aim for normal vaginal delivery. Where possible, birth should be deferred until 2-3 weeks from the last chemotherapy session to allow bone marrow recovery and minimise problems of neutropenia.
You see a 39 year old nulliparous patient in a pre-conceptual counselling clinic with a history of oestrogen-sensitive breast cancer on long term tamoxifen treatment who wishes to discuss her future fertility plans. She has been on tamoxifen for 2 years since initial diagnosis. How long are such patients generally advised to continue on tamoxifen?
a. 3 years
b. 5 years
c. 7 years
d. 10 years
e. Lifelong
B - 5 years
Women with estrogen-receptor positive breast tumours should be advised that tamoxifen treatment is recommended for 5 years. There is a general consensus that women should defer pregnancy for a minimum of 2 years after breast cancer, though this must be weighed against the risk of infertility with the passage of time in certain women. Liaison with the patient’s oncologist and consideration of the specifics of her cancer can be useful in guiding individualised management recommendations.
You see a 39 year old nulliparous patient in a pre-conceptual counselling clinic with a history of oestrogen-sensitive breast cancer on long term tamoxifen treatment who wishes to discuss her future fertility plans. She is anxious however declining rates of fertility with increasing maternal age and states that she is willing to ‘take the risk’ associated with early cessation in tamoxifen therapy to try for a baby. How long would you suggest waiting after stopping tamoxifen before attempting to conceive?
a. No wait necessary
b. 3 months
c. 6 months
d. 9 months
e. 12 months
B - 3 months
Tamoxifen has a relatively long half-life (5-7 days on average – for most drugs half-life is a matter of hours). For this reason, postponing pregnancy for a minimum of 3 months after stopping tamoxifen is advised.
A patient is seen in the antenatal clinic at booking in her first pregnancy. Six years earlier she was diagnosed with breast cancer and was managed with a combination of surgery and doxorubicin chemotherapy. What investigation should be performed routinely in pregnancy in such patients?
a. Spirometry
b. 24 hour ECG
c. Mammography
d. USS liver
e. Echocardiography
E - Echocardiography
There is a dose dependent association between anthracycline chemotherapy agents (doxorubicin) and left-ventricular dysfunction which may be exacerbated by pregnancy. In severe cases, this can even lead to cardiomyopathy. As such, all patients previously treated with anthracyclines should undergo an echocardiography to assess LV function in pregnancy
You review a patient on the post-natal ward with a history of oestrogen sensitive breast cancer, treated by unilateral mastectomy, who wishes to discuss her options for infant feeding. She has been advised by her oncologist to restart her pre-pregnancy tamoxifen following delivery. She would like to feed from the unaffected breast if this was possible. What advice do you give her?
a. Breastfeeding not advised in patients with previous breast ca.
b. Breastfeeding from unaffected breast safe; tamoxifen safe while breastfeeding
c. Breastfeeding from unaffected breast safe; avoid tamoxifen while breastfeeding
d. Breastfeeding from unaffected breast safe; tamoxifen safe though measure levels
e. Breastfeeding from unaffected breast safe, limit dose of tamoxifen to 20mg on alternate days
C - Breastfeeding from unaffected breast safe; avoid tamoxifen while breastfeeding
There is no evidence to suggest breastfeeding increases recurrence risk of breast cancer. While surgery in itself may not inhibit lactation in the affected breast, radiation causes fibrosis which may well do – feeding from the contralateral, unaffected breast is usually possible and safe however. It is unknown whether or not tamoxifen is transmitted in breast milk, thus feeding on tamoxifen is not recommended.
What is the normal daily dose of tamoxifen in women with previously treated oestrogen-sensitive breast cancer?
a. 5mg
b. 10mg
c. 20mg
d. 40mg
e. 100mg
C - 20mg
The standard dose for tamoxifen in women with previous breast cancer is 20mg OD. This is the same dose as when used for most other indications.
A patient who is currently 28 weeks into her first pregnancy is referred to the breast clinic after she notices a lump in her right breast. What is the preferred first line investigation for making a diagnosis in pregnancy?
a. USS
b. Gadolinium-enhanced MRI
c. Mammography with fetal shielding
d. Fine needle aspiration biopsy for cytology
e. CT with contrast
A - USS
Ultrasound is the first line imaging modality in women with suspected breast cancer in pregnancy. If cancer is confirmed, mammography with fetal shielding may be necessary to assess the extent of disease and involvement of the contralateral breast.
What percentage of pregnancies in the UK are complicated by obstetric cholestasis?
a. 0.1%
b. 0.7%
c. 2.5%
d. 3.5%
e. 5%
B - 0.7%
In British obstetric practice, 0.7% of pregnancies (roughly 1 in 140) are affected by obstetric cholestasis though the condition has a varied prevalence amongst ethnic groups – rates in Indian/Pakistani women are approximately twice that of British Caucasians, while rates of 2.4% are seen in Chilean women and up to 5% in Auricanian-Indians of South America.
A patient is seen in the ANC complaining of intense itching of the palms and soles which is worse at night. Blood is collected for LFTs and Bile acids which both fall within normal pregnancy ranges. How often should such testing be repeated assuming there is no improvement in symptoms with conservative measures?
a. 1-2 weekly
b. 3 weekly
c. Monthly
d. No sooner than 8 weeks from initial testing
e. No further testing necessary
A - 1-2 weekly
Pruritus is a common symptom in pregnancy affecting up to 23%. Based on a prevalence of ~0.7% for obstetric cholestasis, this would suggest that only around 1 in 30 women with pruritus could be expected to have OC. Some women may have pruritus of OC for some time (days or weeks) prior to the onset of derangement in their liver function tests. Women with persistent pruritus despite initial normal liver function should have their LFTS repeated every 1-2 weeks.
The majority of women with itching in pregnancy do not have obstetric cholestasis. Approximately what percentage of pregnancy women report itching?
a. 80%
b. 65%
c. 40%
d. 25%
e. 10%
D - 25%
23% of women are thought to report itching at some point in pregnancy, only ~3% of whom have OC.
How often following a diagnosis of obstetric cholestasis should pregnant women have their liver function checked?
a. Fortnightly
b. Monthly
c. Weekly
d. Twice weekly
e. Alternate days
C - Weekly
Following a diagnosis of OC, women should have their LFTs measured weekly until delivery along with a general review, urine and BP measurement. The rise in transaminases may range from just above normal to several hundreds. A rapid rise should prompt consideration of alterative diagnoses as – while such a pattern may occasionally be seen in OC – it is not typical. Similarly if LFTs return to normal antenatally, OC is unlikely to be the root cause.
What is the minimum period of time which should elapse prior to liver function being checked following delivery in women with obstetric cholestasis?
a. 7 days
b. 10 days
c. 2 weeks
d. 6 weeks
e. 12 weeks
B - 10 days
Postnatally, liver function testing in women with obstetric cholestasis should be deferred for at least 10 days - postnatal resolution is essential to confirm the diagnosis. Even in normal pregnancy, a transient rise in LFTs may be seen in the first 10 days. In OC pregnancies LFTs should ideally be performed beyond this time.
Which of the following is not known to be increased in mothers with Obstetric Cholestasis?
a. PPH
b. Caesarean delivery
c. Meconium passage
d. Prematurity
e. IUGR
E - IUGR
There appears to be a small increased risk of both PPH and caesarean section in women with obstetric cholestasis though both may be multifactorial. The risk of stillbirth has yet to be fully determined but any increased risk, if present, if likely to be small. Premature birth is increased in OC pregnancies though a good deal of this is thought to be iatrogenic and the spontaneous preterm rate raised only minimally. There is a well described increased incidence of meconium passage. There is no known association between obstetric cholestasis and fetal growth restriction.
There is debate concerning the optimum timing of delivery in pregnancies affected by obstetric cholestasis with theoretically increased risks of perinatal morbidity balanced against those of early intervention. What percentage of babies delivered by El. CS at 37/40 will require admission to SCBU?
a. 1-2%
b. 4-6%
c. 7-11%
d. 12-15%
e. 18-20%
C - 7-11%
There is a widely adopted practice in obstetric cholestasis of offering elective delivery at 37 weeks though this is not evidence based and as such the potential consequences of iatrogenic early delivery must be considered – the risk of admission to SCBU with elective caesarean is 7-11% at 37/40; 6% at 38/40 and falls to 1.5% at 39/40.
What percentage of infants delivered by El. CS at 38 and 39 weeks will require admission to SCBU respectively?
38/40 39/40
a. 6% 1.5%
b. 3% 0.5%
c. 10% 2.5%
d. 12% 4%
e. 15% 3%
A - 6% at 38/40; 1.5% at 39/40
There is a widely adopted practice in obstetric cholestasis of offering elective delivery at 37 weeks though this is not evidence based and as such the potential consequences of iatrogenic early delivery must be considered – the risk of admission to SCBU with elective caesarean is 7-11% at 37/40; 6% at 38/40 and falls to 1.5% at 39/40.
Which of the following is known to improve neonatal outcomes in pregnancies affected by OC?
a. UCDA
b. Chlorphenamine
c. Cholestyramine
d. A-adenosyl Methionine
e. None of the above
E - None of the above
As it stands, no known drug therapy has been proven to improve fetal nor neonatal outcomes in mothers affected by OC in pregnancy. As such, any proposed therapy should be discussed with this in mind. Ursodeoxycholic acid has been shown to improve pruritus and LFTs though robust data on protection against stillbirth or indeed its safety is lacking.
Which coagulation screen abnormality should prompt use of maternal Vitamin K antenatally?
a. Prolonged PT
b. Prolonged APTT
c. Low platelets
d. Low fibrinogen
e. Reduced APTT
A - Prolned PT
Women with OC should have an informed discussion regarding Vitamin K. Where prothrombin time is prolonged, Vitamin K given at a daily dose of 5-10mg is advised though even where PT is normal there may be a role for its use, providing mothers are counselled regarding the small theoretical risk. Women with OC may be vitamin K deficient as – being a fat soluble vitamin depending on bile for its absorption – uptake from the GI tract can be reduced. There are historical fears regarding a possible risk of haemolytic anaemia (and thus kernicterus and hyperbilirubinaemia) in infants exposed through maternal use. Vitamin K is required for the synthesis of several important clotting factors including II, VII, IX and X thus deficiency carries potential severe consequences. Infants should be offered postnatal Vitamin K in the usual way at birth regardless of maternal use.
What daily dose of Vitamin K (menadiol sodium phosphate) is recommended for at risk mothers in the antenatal period?
a. 5-10mg orally
b. 20-40mg orally
c. 50-100mg orally
d. 5-10micrograms IM
e. 50-100mg IM
A - 5-10mg orally
Women with OC should have an informed discussion regarding Vitamin K. Where prothrombin time is prolonged, Vitamin K given at a daily dose of 5-10mg is advised though even where PT is normal there may be a role for its use, providing mothers are counselled regarding the small theoretical risk. Women with OC may be vitamin K deficient as – being a fat soluble vitamin depending on bile for its absorption – uptake from the GI tract can be reduced. There are historical fears regarding a possible risk of haemolytic anaemia (and thus kernicterus and hyperbilirubinaemia) in infants exposed through maternal use. Vitamin K is required for the synthesis of several important clotting factors including II, VII, IX and X thus deficiency carries potential severe consequences. Infants should be offered postnatal Vitamin K in the usual way at birth regardless of maternal use.
What is the recurrence rate of obstetric cholestasis in future pregnancies?
a. Up to 20%
b. Up to 40%
c. Up to 70%
d. Up to 90%
e. Up to 95%
D - Up to 90%
Women with obstetric cholestasis should be reassured that there are no long term sequelae for mothers and babies though a rather considerable recurrence rate ranging from 45% to as high as 90%.
What contraceptive preparation should generally be avoided in mothers affected by obstetric cholestasis in pregnancy?
a. Injection
b. Combined Pill
c. Implant
d. Levonorgestrel IUS
e. Progesterone-only Pill
B - Combined pill
The RCOG guideline suggests women with a history of OC avoid estrogen-containing contraceptives postnatally. The FSRH guidelines list a history of pregnancy–related cholestasis as a UKMEC 2 for the combined pill, advising that it may increase the risk of cholestasis associated with use of the pill itself (a history of non-obstetric cholestasis is UKMEC 3 for CHC)
Amongst women from which continent is the risk of obstetric cholestasis highest?
a. Europe
b. Oceania
c. Africa
d. Asia
e. South America
E - South America
Obstetric cholestasis has a varied prevalence amongst ethnic groups – rates in Indian/Pakistani women are approximately twice that of British Caucasians, while rates of 2.4% are seen in Chilean women and up to 5% in Auricanian-Indians of South America.
What is the most common strain of malarial infection seen in the UK?
a. Falciparum
b. Vivax
c. Ovale
d. Malariae
e. Knowlesi
A - Falciparum
Falciparum malaria is both the most common strain of the disease seen in UK practice (>75% of cases – mostly from West Africa) as well as that which causes the vast majority of malaria deaths worldwide. In travellers returning from the Indian subcontinent, Vivax is more likely and causes a relapsing type.
What strain of malarial infection is responsible for most deaths worldwide?
a. Falciparum
b. Vivax
c. Ovale
d. Malariae
e. Knowlesi
A - Falciparum
Falciparum malaria is both the most common strain of the disease seen in UK practice (>75% of cases – mostly from West Africa) as well as that which causes the vast majority of malaria deaths worldwide. In travellers returning from the Indian subcontinent, Vivax is more likely and causes a relapsing type.
What is the most common strain of malarial infection seen in returning travellers from the Indian subcontinent to the UK?
a. Falciparum
b. Vivax
c. Ovale
d. Malariae
e. Knowlesi
B - Vivax
Falciparum malaria is both the most common strain of the disease seen in UK practice (>75% of cases – mostly from West Africa) as well as that which causes the vast majority of malaria deaths worldwide. In travellers returning from the Indian subcontinent, Vivax is more likely and causes a relapsing type.
In endemic areas, how much more likely are pregnant women to contract and die from malarial infection compared with non-pregnant women?
a. Equivalent risk to non-pregnant population
b. 2x
c. 3x
d. 5x
e. 10x
B - 2x
Pregnant women in malaria-endemic areas are twice as likely to be bitten, twice as likely to contract and twice as likely to die from malaria than their non-pregnant counterparts. The clinical manifestations of the disease in pregnancy is largely based on ‘premunition’ – the degree of naturally acquired host immunity to the condition.
You see a patient in the antenatal clinic who explains that she is planning a trip to Tanzania to visit family for one month. She wishes to discuss anti-malarial prophylaxis. What is the risk of transmission associated with a one-month stay in Sub-Saharan African without chemoprophylaxis?
a. 1:5
b. 1:10
c. 1:50
d. 1:100
e. 1:500
C - 1 in 50
The uptake of chemoprophylaxis amongst women residing in the UK who later present with malaria is low. The risk of transmission is generally quoted for a one-month stay without chemoprophylaxis and varies enormously from one area to another – highest in endemic parts of Oceania and lowest in America and the Caribbean:
Oceania (Papua New Guinea, Solomon Islands and Vanuatu) 1 in 20 Sub-Saharan Africa 1 in 50 Indian Subcontinent and SE Asia 1 in 500 South America 1 in 2500 Central America/Caribbean 1 in 10,000
You see a patient in the antenatal clinic who explains that she is planning a trip to India to visit family for one month. She wishes to discuss anti-malarial prophylaxis. What is the risk of transmission associated with a one-month stay in the Indian subcontinent without chemoprophylaxis?
a. 1:5
b. 1:10
c. 1:50
d. 1:100
e. 1:500
E - 1 in 500
The uptake of chemoprophylaxis amongst women residing in the UK who later present with malaria is low. The risk of transmission is generally quoted for a one-month stay without chemoprophylaxis and varies enormously from one area to another – highest in endemic parts of Oceania and lowest in America and the Caribbean:
Oceania (Papua New Guinea, Solomon Islands and Vanuatu) 1 in 20 Sub-Saharan Africa 1 in 50 Indian Subcontinent and SE Asia 1 in 500 South America 1 in 2500 Central America/Caribbean 1 in 10,000
A patient is planning a trip to Ghana for a funeral of a close family member during the third trimester of her second pregnancy. Despite awareness of malarial risks, she is unwilling to cancel the trip. Which drug would you recommend for chemoprophylaxis?
a. Chloraquine
b. Mefloquine
c. Atovaquone
d. Artesunate
e. Proguanil
B - Mefloquine
Mefloquine, at a dose of 5mg/kg once weekly, is the recommended drug of choice for malarial chemoprophylaxis in the second and third trimesters. The majority of observational and clinical data suggests the drug is not associated with an increased risk of stillbirth nor congenital malformation at this dose. Available evidence does not support an association with embryotoxicity in the first trimester and thus use may be justified at this time in areas at high risk of falciparum infection. Contraindications to mefloquine include: current or previous depression; neuropsychiatric disorders, epilepsy or hypersensitivity to quinine. The alternative in such patients would be ‘Malarone’ – a combination of atovaquone and proguanil however there is insufficient data on its safety in pregnancy and cases should always be discussed with a malarial specialist prior to prescription in pregnancy.
What is the recommended regimen of emergency ‘standby’ treatment of suspected malaria in patients from the UK?
a. Artesunate
b. Quinine and Clindamycin
c. Quinine
d. Artesunate and Clindamycin
e. Chloraquine and Clindamycin
B - Quinine and clindamycin
The only recommended ‘standby’ treatment for pregnant women from the UK is quinine (600mg and clindamycin 450mg, both TDS, both for 7 days). This should be started in the event of a flu-like illness and temperature >38C in an area with no immediate access to medical care. If there is vomiting within 30 minutes, repeat the full dose; if within 30-60 minutes, repeat a half dose. Mefloquine should then be commenced 1 week after the last treatment dose.
A primigravida at 32/40 develops fever of 39.5C and a flu-like illness while visiting family in Nigeria. Following medical review she is commenced on emergency standby treatment though vomits the first dose 45 minutes after ingestion and is unsure whether or not to repeat the dose. What would you advise in such a scenario?
a. No repeat dose required
b. Repeat half dose and continue next dose at usual time
c. Repeat full dose and continue next dose at usual time
d. Repeat full dose and delay next dose by 12 hours
e. Repeat full dose and omit next dose
B - Repeat half dose and continue next dose at the usual time
The only recommended ‘standby’ treatment for pregnant women from the UK is quinine (600mg and clindamycin 450mg, both TDS, both for 7 days). This should be started in the event of a flu-like illness and temperature >38C in an area with no immediate access to medical care. If there is vomiting within 30 minutes, repeat the full dose; if within 30-60 minutes, repeat a half dose. Mefloquine should then be commenced 1 week after the last treatment dose.
What is the mortality rate associated with malaria infection in the UK?
a. 0.5-1%
b. 2-3%
c. 4-5%
d. 9-10%
e. >10%
A - 0.5-1%
There are approximately 1500 cases of malaria diagnosed in the UK each year with 5-15 deaths (equivalent to a 0.5-1% mortality rate).
- What is the ‘hallmark’ complication of falciparum malaria infection in pregnancy, not seen in vivax, ovale or malariae?
a. IUGR
b. Neonatal jaundice
c. Brachycephaly
d. Placental parasitaemia
e. Thrombocytopenia
D - Placental parasitaemia
The hallmark of falciparum malarial infection in pregnancy is the sequestration of parasites in the placenta which can evade host defence mechanisms such as splenic processing and filtration. This is not known to occur in the ‘benign’ malarias. Parasitaemia itself can lead to fetal growth restriction and low birthweight as well as maternal and fetal anaemia.
Malarial infection is generally confirmed by blood film. How many films are required to effectively exclude malaria in patient’s with suspected infection?
a. One
b. Two, one week apart
c. Two, 24 hours apart
d. Three, 24 hours apart
e. Four, 12 hours apart
D - Three, 24 hours apart
Malaria may be diagnosed both by microscopy and rapid-diagnostic tests. Microscopy – allowing both species identification and estimation of parasitaemia – is considered the gold standard. Rapid detection tests may miss low-parasitaemia which is more likely in pregnancy women. They are also relatively insensitive in Vivax malaria. In any event, a positive rapid detection test should always be followed by microscopy. In a febrile patient with suspected malaria, 3 negative blood smears 12-24 hours apart effectively rule out the diagnosis of malaria.
What is the mortality associated with severe malarial infection in pregnancy?
a. 5%
b. 10%
c. 25%
d. 33%
e. 50%
E - 50%
Mortality rates in severe pregnancy are high – up to 50% (compared with 15-20% in the non-pregnant population). Non-falciparum species are rarely fatal though caution should nonetheless be observed. Mortality in uncomplicated malaria is very low – 0.1%.
A primigravida, recently returned from a trip to visit family in Ghana is admitted with a 3 day history of fever, myalgia, vomiting and diarrhoea. Temperature on admission is elevated at 38C. On clinical examination you note her to be jaundiced and there is splenomegaly evident on abdominal palpation. A blood film confirms falciparum malarial infection with 3% parasitaemia. A full blood count reveals anaemia – Hb 76g/L – and thrombocytopenia – Platelets 45. What is first line treatment for severe falciparum malaria in pregnancy?
a. IV Quinine
b. IV Artesunate
c. Oral Clindamycin
d. Oral Chloraquine
e. Oral Mefloquine
B - IV Artesunate
In patients treated for severe falciparum malaria in pregnancy, what is the recommended ‘step-down’ treatment when well?
a. Oral chloraquine + oral quinine
b. Oral artesunate
c. Oral artesunate and oral clindamycin
d. Oral chloraquine
e. Oral clindamycin
C - Oral artesunate and oral clindamycin
Above what level of parasitaemia should patients be treated per the ‘severe’ malaria protocol irrespective of other clinical signs and/or symptoms?
a. 2%
b. 5%
c. 10%
d. 25%
e. 50%
A - 2%
Regardless of clinical presentation, signs or symptoms, patients with parasitaemia greater than 2% should be treated as ‘severe’ malaria.
A primigravida develops uncomplicated malaria during the second trimester and is commenced on Quinine therapy. What is the most common side effect of quinine therapy?
a. Hypoglycaemia
b. Hypocalcaemia
c. Hypomagnesaemia
d. Hyponatraemia
e. Hypokalaemia
A - Hypoglycaemia
The most common and important side effect of quinine therapy is hypoglycaemia, which is secondary to hyperinsulinaemia. While this is commonly asymptomatic, it may be associated with fetal bradycardia.
What is the most common cause of direct maternal death?
a. Haemorrhage
b. Eclampsia
c. Thromboembolism
d. Amniotic fluid embolism
e. Cardiac disease
C - Thromboembolism
Venous thromboembolism (1.39 per 100,000) and cardiac disease (2.39 per 100,000) were the most common direct and indirect causes of maternal death respectively in the most recent (2014-2016) MBBRACE report, and indeed this has been the case for some time.
What is the most common cause of death overall in pregnancy?
a. Haemorrhage
b. Cardiac disease
c. Thromboembolism
d. Suicide
e. Malignancy
B - Cardiac disease
Venous thromboembolism (1.39 per 100,000) and cardiac disease (2.39 per 100,000) were the most common direct and indirect causes of maternal death respectively in the most recent (2014-2016) MBBRACE report, and indeed this has been the case for some time.
What increase in total plasma volume occurs in pregnancy?
a. 10%
b. 25%
c. 33%
d. 50%
e. 75%
D - 50%
Plasma volume increases by up to 50% in pregnancy, resulting in both dilutional anaemia and a reduced oxygen carrying capacity of the blood. Pulse increases by 15-20 beats per minute though together these changes increase cardiac output by only 40%. Arterial blood pressure drops by 10-15mmHg
By how much (in beats/minute) does heart rate typically change in pregnancy?
a. Fall 5-10 bpm
b. Fall 15-20 bpm
c. No change
d. Rise 15-20 bpm
e. Rise 5-10 bpm
D - Rise 15-20 bpm
Plasma volume increases by up to 50% in pregnancy, resulting in both dilutional anaemia and a reduced oxygen carrying capacity of the blood. Pulse increases by 15-20 beats per minute though together these changes increase cardiac output by only 40%. Arterial blood pressure drops by 10-15mmHg
What proportion of cardiac output does the uterus receive at term?
a. 1/20
b. 1/10
c. 1/5
d. 1/4
e. 1/3
B - 1/10
The uterus receives 10% of total cardiac output at term.
- What is the optimum degree of left-lateral tilt to improve maternal resuscitation in pregnancy?
a. 9 degrees
b. 12 degrees
c. 15 degrees
d. 20 degrees
e. 25 degrees
C - 15 degrees
A left lateral tilt of 15 degrees on a firm surface will relieve aorto-caval compression in most pregnant women and still permit effective chest compressions.
How long after diagnosis of maternal cardiac arrest, should Perimortem caesarean section be undertaken if there is no return of spontaneous circulation?
a. Immediately
b. 2 minutes
c. 4 minutes
d. 5 minutes
e. 6 minutes
C - 4 minutes
If there is no response to correctly performed CPR within 4 minutes of a maternal collapse or if resuscitation is continued beyond this in women >20/40, delivery should be achieved within 5 minutes of the collapse in order to assist resuscitation. Delivery of the fetus/placenta reduces oxygen consumption, improves venous return and cardiac output, facilitates cardiac compressions and renders ventilation easier. Prior to 20/40 there is no proven benefit from delivery of the fetus.
In management of the acutely unwell septic patient in pregnancy, what is the target central venous pressure in patients who are NOT on mechanical ventilation?
a. 6mmHg
b. 8mmHg
c. 12mmHg
d. 15mmHg
e. 20mmHg
B - 8mmHg
In the event of severe hypotension despite initial fluid resuscitation, the aim should be to achieve a CVP >8mmHg with aggressive fluid replacement (or >12mmHg in women on mechanical ventilation).
A patient is admitted in pre-term labour and commenced on Magnesium Sulphate for fetal neuroprotection. Some hours later you are called to review as the midwives are concerned her breathing has become laboured and she is increasingly drowsy. You note the absence of deep tendon reflexes on examination and suspect magnesium toxicity. What is the treatment for magnesium toxicity?
a. 10 units of rapid acting insulin in 500ml of 10% dextrose
b. 20% Intralipid infusion
c. 200mcg Flumazenil
d. 150mg/kg N-acetylcysteine
e. 10ml 10% Calcium Gluconate
E 10ml 10% Calcium Gluconate
The antidote to magnesium sulphate is 10ml of 10% calcium gluconate given slow IV.
What is the recommended dose of 20% Intralipid Emulision in treatment of suspected local anaesthetic toxicity?
a. 1.5ml/kg loading; 0.5ml/kg/hour
b. 1.5ml/kg loading; 0.5ml/kg/minute
c. 1.5ml/kg loading; 0.25ml/kg/minute
d. 2.5ml/kg loading; 0.25ml/kg/minute
e. 2.5ml/kg loading; 0.25ml/kg/hour
C - 1.5ml/kg loading; 0.25ml/kg/minute
So-called ‘lipid-rescue’ is the treatment of choice for suspected local anaesthetic toxicity. This consists of an IV bolus of 20% Intralipid at a dose of 1.5ml/kg over 1 minute (can be repeated a further 2 times at five minute intervals) following by an infusion of 0.25ml/kg/min, increased to 0.5ml/kg/min after 5 minutes.
A patient in early labour is given Benzylpenicillin for intrapartum Group B Strep prophylaxis. Shortly after commencing the first dose, she develops stridor, facial oedema and is struggling to maintain her own airway. What is first line management in suspected anaphylaxis in pregnancy?
a. 200mg Hydrocortisone IV
b. 10mg Chlorphenamine IV
c. 250mcg adrenaline IV
d. 500mcg adrenaline IM
e. 250mcg adrenaline IM
D - 500mcg adrenaline IM
500 micrograms of IM adrenaline (0.5ml of 1:1000) is the definitive treatment for suspected anaphylaxis as described here. This can be repeated after 5 minutes if there is no effect. In experienced hands it may be given IV as a 50 microgram bolus (0.5ml of 1:10,000). Chlorphenamine 10mg and hydrocortisone 200mg (both IM or IV) form adjuvant treatment.
By how much does cardiac output change in pregnancy?
a. Increases 20%
b. Increases 40%
c. Increases 60%
d. Decreases 10%
e. Decreases 20%
B - Increases 40%
Plasma volume increases by up to 50% in pregnancy, resulting in both dilutional anaemia and a reduced oxygen carrying capacity of the blood. Pulse increases by 15-20 beats per minute though together these changes increase cardiac output by only 40%. Arterial blood pressure drops by 10-15mmHg.
A primigravida, originally from Nigeria is referred to the ANC for review as she is known to be have Sickle Cell Trait (HbSA). She asks what implications this may have on her pregnancy. Which of the following is increased in patients with Sickle Cell Trait in pregnancy?
a. Anaemia
b. IUGR
c. VTE
d. UTI
e. Arthralgia
D - UTI
Patients with sickle cell trait – one copy of HbS and one normal HbA – are asymptomatic. They require little, if any, additional attention in pregnancy from a maternal point of view. Interestingly the only complication noted is an increased incidence of microscopic haematuria and UTI.
How many babies are born each year in the UK with sickle cell disease?
a. <50
b. 100-500
c. 500-1000
d. 1000-5000
e. >5000
B - 100-500
Sickle cell disease is the most common inherited condition worldwide around 300,000 affected children born each year. There are thought to be around 12-15,000 people in the UK with sickle cell and around 100-200 pregnancies in affected women each year. 300 babies are born on average in the UK every year with the condition.
What is the average life expectancy of patients born in the UK nowadays with sickle cell disease?
a. Late teens
b. Mid-20s
c. Mid-40s
d. Mid-50s
e. No change in life expectancy
D - Mid-50s
SCD has previously been associated with a high early mortality rate though the majority of children born in the UK today with SCD live past reproductive age and average life expectancy is well into the mid-50s.
Patient’s with sickle cell disease are advised to undergo regular echocardiography. Where not performed in the previous year, an echo is advised pre-conceptually in such patients. What complication does an echo seek to exclude in such patients?
a. Iron overload
b. Pulmonary hypertension
c. Aortic stenosis
d. Infective endocarditis
e. Left ventricular hypertrophy
B - Pulmonary hypertension
Where opportunity for pre-conceptual screening arises, patients with SCD should undergo an echocardiogram to screen for pulmonary hypertension. Incidence of pulmonary hypertension is increased in patients with SCD and there is a significant association with increased mortality. A tricuspid jet velocity of >2.5m/second is associated with a high risk of PH. Where not performed in the last year, this should be done antenatally if possible.
What regimen of folic acid supplementation is advised in women with sickle cell disease both in and out of pregnancy?
Pregnant Non-pregnant
a. 400mcg OD No supplementation required
b. 5mg No supplementation required
c. 400mcg 400mcg
d. 5mg 400mcg
e. 5mg 1mg
E - 5mg in pregnancy; 1mg outwith
Women with SCD are advised to take 1mg folic acid every day outwith pregnancy owing to the increased risk of folate deficiency in haemolytic anaemia. In pregnancy, this should be increased to 5mg to guard against neural tube defects in the first trimester and protect against the increased demands for folate during the second and third.
A patient is seen in the pre-conceptual counselling clinic to discuss her sickle cell disease. She is currently taking hydroxycarbamide (hydroxyurea) to reduce the incidence of painful crises and wishes to know if it is safe to continue this as she plans a pregnancy. What advice do you give?
a. Stop at conception, may restart in 2nd trimester
b. Stop at conception, restart postnatally
c. Stop 6 months pre-conception, restart in 3rd trimester
d. Stop 3 months pre-conception, restart postnatally
e. No need to stop, may use throughout pregnancy
D - Stop 3 months pre-conceptually; restart postnatally
Hydroxyurea (hydroxycarbamide) is a drug known to decrease the incidence of painful crises and acute chest syndrome in patients with SCD. It is known however, to be teratogenic in animal studies – thus advice currently is that women on Hydroxyurea use effective contraception and stop the drug 3 month pre-conceptually. This said there are reports of women continuing through pregnancy on the drug without any adverse effects which may help in counselling women who become pregnant while taking – in such a setting, it should be stopped and detailed ultrasound performed by a fetal medicine specialist looking for abnormality, though is not an indication for TOP in isolation.
Aside from folic acid, what other medical treatment is recommended for all with sickle cell as a matter of routine in pregnancy?
a. Aspirin, Iron and LMWH
b. Aspirin, Iron and Antibiotics
c. Antibiotics and Iron
d. Antibiotics and aspirin
e. Antibiotics, Aspirin and LMWH
D - Antibiotics and aspirin
Patients with SCD are hypo-splenic and at an increased risk of infection, especially from encapsulated bacteria – N. Meningitidis; Strep. Pneumoniae and H. Influenzae. There is clear evidence of benefit to this end from penicillin antibiotic prophylaxis in children (though data is lacking on adults/pregnancy) and guidance suggests this should be given to all patients in and out of pregnancy. Penicillin is first line, erythromycin second if allergic. The flu, hepatitis B and meningococcal vaccines should also be up to date as well as pneumococcal ever 5 years. In addition, women with SCD should be considered for aspirin for PET prophylaxis at the usual dose (consider SCD a mild risk factor). Heparin should be given at prophylactic dose during any inpatient admissions.
Between what weeks of pregnancy can NSAIDs safely be used for pain relief?
a. 12 weeks onwards
b. 12-28 weeks
c. 28 weeks onwards
d. Safe throughout
e. Avoid altogether
B - 12-28 weeks
NSAIDs may be used as effective pain relief for women with a painful sickle cell crisis though are only suitable for use between 12 and 28 weeks of pregnancy.
In addition to routine dating and fetal anomaly scans, what additional scanning schedule is advocated in women with sickle cell disease?
a. Early viability and 4-weekly biometry from 24/40
b. Early viability and 4 weekly biometry from 28/40
c. Early viability and 3 weekly biometry from 28/40
d. One-off scan at 34/40
e. Serial biometry from 24/40
A - early viability and 4-weekly biometry from 24/40
In addition to routine scans, women with SCD should be offered an early viability scan at 7-9 weeks and serial fetal biometry on a 4-weekly basis from 24/40. A number of studies have pointed to an increased risk of fetal growth restriction and pre-eclampsia.
Routine antenatal transfusion is not recommended for all women with sickle cell disease, though certain conditions do typically necessitate transfusion. Which of the following situations is NOT in itself an indication for consideration of routine transfusion?
a. Painful crises
b. Acute chest syndrome
c. Twin pregnancy
d. Acute stroke
e. Hb <60
A - Painful crises
Routine prophylactic transfusion for women with SCD in pregnancy was a historic norm as it was associated with decreased perinatal morbidity and mortality compared with historic controls. It has since been argued however that the appreciable risks of transfusion (alloimmunisation [seen in up to 36%], reactions, iron overload, infection etc.) may outweigh this and as such, routine transfusion is no longer recommended. There are a few instances however in which transfusion IS considered best practice:
• Where Hb <6 or falls > 2 units from baseline
• Acute chest syndrome
• Acute stroke
• Twin pregnancy
• Women with previous serious medical, obstetric or fetal complications
• Women on a pre-pregnancy transfusion regimen for prevention of secondary stroke or severe complications – this should be continued through pregnancy
What proportion of women with sickle cell disease have alloimmunisation?
a. Up to 10%
b. Up to 20%
c. Up to 30%
d. Up to 40%
e. Up to 50%
D - Up to 40%
Routine prophylactic transfusion for women with SCD in pregnancy was a historic norm as it was associated with decreased perinatal morbidity and mortality compared with historic controls. It has since been argued however that the appreciable risks of transfusion (alloimmunisation [seen in up to 36%], reactions, iron overload, infection etc.) may outweigh this and as such, routine transfusion is no longer recommended. There are a few instances however in which transfusion IS considered best practice:
• Where Hb <6 or falls > 2 units from baseline
• Acute chest syndrome
• Acute stroke
• Twin pregnancy
• Women with previous serious medical, obstetric or fetal complications
• Women on a pre-pregnancy transfusion regimen for prevention of secondary stroke or severe complications – this should be continued through pregnancy
You meet a 20 year old primigravida in the ANC at booking. She was born in Uganda though has lived in the UK since age 12. She is known to suffer from sickle cell disease and wishes to know about how the pregnancy may impact upon her disease. What proportion of women with sickle cell disease will experience a painful crisis in pregnancy?
a. <5%
b. 10-20%
c. 25-50%
d. 50-75%
e. >80%
C - 25-50%
25-50% of patients with SCD will experience a painful sickling crises in pregnancy and is the most common cause of hospital admission in this cohort. Avoiding precipitants is highly advisable – excessive exercise, cold, dehydration and stress. Women who do not settle with simple analgesia, are febrile, have atypical pain or chest symptoms should be referred to hospital. Fluid intake should be encouraged with a target of 60ml/kg/24 hours (4.2L for a 70kg woman).
What fluid intake regimen should be the target in women with an acute sickle cell crisis in pregnancy?
a. 25ml/kg/24 hours
b. 10ml/kg/hour
c. 60ml/kg/24 hours
d. 33ml/kg/24 hours
e. 2.5ml/kg/hour
C - 60ml/kg/24 hours
25-50% of patients with SCD will experience a painful sickling crises in pregnancy and is the most common cause of hospital admission in this cohort. Avoiding precipitants is highly advisable – excessive exercise, cold, dehydration and stress. Women who do not settle with simple analgesia, are febrile, have atypical pain or chest symptoms should be referred to hospital. Fluid intake should be encouraged with a target of 60ml/kg/24 hours (4.2L for a 70kg woman).
Acute chest syndrome, a complication of sickle cell disease seen in up 20% of women during pregnancy, closely resembles which other cardio-respiratory condition such that both should typically be treated simultaneously until a diagnosis can be accurately made?
a. Myocarditis
b. Pulmonary oedema/right heart failure
c. Myocardial infarction
d. Interstitial pneumonitis
e. Pneumonia
E - Pneumonia
Acute chest syndrome is the second most common complication of SCD seen in pregnant women, occurring in up to 20% of pregnancies. This condition is characterised by respiratory symptoms – tachypnoea, chest pain, cough, SOB – which may mimic pneumonia. To further confuse matters, the presence of a new infiltrate may be seen on CXR. As such, it is advised that patients be treated for both and given antibiotics in addition to oxygen and top-up blood transfusion. If there is any suspicion of PE – treatment dose heparin until excluded is appropriate.
A Para 1 with sickle cell disease and history of emergency caesarean section for failure to progress in her first pregnancy, comes to the ANC at 36 weeks following a growth scan, wishing to discuss options for delivery. The fetus is normally grown and she is very keen to avoid a caesarean section if possible. What management plan for delivery would you recommend?
a. Await spontaneous onset of labour
b. IOL at 37/40
c. IOL at 38/40
d. LSCS at 39/40
e. LSCS at 38/40
C - IOL at 38/40
SCD is not an indication in itself for caesarean delivery, nor a contraindication in itself to attempting VBAC, though the recommendation is that patients be delivered electively from 38 weeks either via LSCS or IOL. Blood should be cross-matched for delivery in the presence of atypical antibodies – otherwise a group and save will suffice.
A primigravida presents in spontaneous labour at 37/40. She is known to have sickle cell disease. Labour is progressing well though she is struggling increasingly with the pain of contractions and is requesting pain relief. Which of the following analgesic options is NOT an appropriate option for patients with sickle cell disease?
a. Epidural
b. Entonox
c. Pethidine
d. Oramorph
e. Paracetamol
C - Pethidine
Patients with SCD can have almost all means of analgesia available to other women in labour with the exception of pethidine owing to its known association with seizures.
A patient with sickle cell disease is admitted for induction of labour at 37+5/40 following an otherwise uncomplicated pregnancy. Several hours after commencing oxytocin, her oxygen saturations drop abruptly to 92%. What is the most appropriate immediate management?
a. Stop the oxytocin infusion
b. Administer oxygen and check arterial blood gas
c. Administer oxygen and IV antibiotics
d. Cat. 1 caesarean section
e. Cat. 2 caesarean section
B - Administer oxygen and check ABG
The demand for oxygen in sickle cell disease is increased during the intrapartum period and use of pulse oximetry to detect hypoxia in the mother should be employed during labour. Arterial blood gas should be performed and oxygen therapy commenced where oxygen saturations fall to 94% or less.
Which of the following is NOT a common finding in patients with beta-thalassaemia?
a. Cardiac failure
b. Diabetes mellitus
c. Alloimmunisation
d. Respiratory failure
e. Subfertility
D - Respiratory Failure
The basic defect in thalassaemia is reduced globin chain synthesis meaning resultant red cells have inadequate haemoglobin content. Pathophysiology is characterised by extravascular haemolysis due to release into the peripheral circulation of damaged red cells and erythroid precursors due to ineffective erythropoiesis. As a result patients require multiple transfusions per year which may lead to iron overload and resultant organ damage. The condition affects multiple organ systems including the pancreas (diabetes common secondary to insulin resistance and iron-induced islet cell deficiency); thyroid; heart (iron related cardiomyopathy); liver (cirrhosis) and bone (osteoporosis). Patients with thalassaemia tend to suffer from subfertility also. There is no association with lung disease.
A patient with known beta-thalassaemia major and insulin-dependent diabetes attends the pre-conception clinic to discuss implications of her disease on the pregnancy she is planning with her husband who does not have thalassaemia. Which of the following investigations is NOT indicated routinely in patients with beta-thalassaemia prior to embarking on pregnancy?
a. Lung function testing
b. DEXA scan
c. Cardiac MRI
d. Echocardiography
e. Serum frustosamine levels
A - Lung function testing
In view of the multiple co-morbidities which may be found in thalassaemia, pre-conceptual planning and counselling is of the utmost importance. It is recommended that prior to embarking upon a pregnancy, patients undergo screening for end-organ damage including both echocardiography and cardiac MRI as well as bone density scanning, diabetes testing (using serum fructosamine concentrations) and thyroid function testing.
What is the target pre-transfusion Hb in patients with thalassaemia major in pregnancy?
a. 70g/L
b. 85g/L
c. 90g/L
d. 100g/L
e. 110g/L
D - 100g/L
The majority of women with thalassaemia major are already established on a transfusion regimen pre-pregnancy which may continue. Patients with thalassaemia require transfusion on a regular basis and should aim for a pre-transfusion Hb of 100g/L. Initially 2-3 units should be transfused with an additional top up the following week until Hb reaches 120g/L. Hb should be re-checked 2 weeks later and a further 2 units transfused if <100g/L.
A 33 year old primigravida attends the ANC at 12/40 on account of her thalassaemia major. All necessary pre-pregnancy investigations were arranged and returned satisfactory results though platelets were elevated at 678 on a recent full blood count. She wishes to know if there is any medication in additional to her existing 5mg folic acid, which she should be taking during pregnancy. What do you advise?
a. Treatment dose LMWH and 75mg aspirin
b. Prophylactic dose LMWH and 75mg aspirin
c. 75mg aspirin alone
d. Prophylactic dose LMWH alone
e. Treatment dose LMWH and 150mg aspirin
C - 75mg aspirin alone
Women with thalassaemia who have either previously undergone a splenectomy or have a current platelet count >600, should be commenced on low-dose aspirin (75mg OD). Women with both risk factors should commence both aspirin as well as low-molecular weight heparin.
A 21 year old Para 1 attends the ANC at 12/40 on account of her thalassaemia major and history of splenectomy. All necessary pre-pregnancy investigations were arranged and returned satisfactory results. She wishes to know if there is any medication in additional to her existing 5mg folic acid, which she should be taking during pregnancy. What do you advise?
a. Treatment dose LMWH and 75mg aspirin
b. Prophylactic dose LMWH and 75mg aspirin
c. 75mg aspirin alone
d. Prophylactic dose LMWH alone
e. Treatment dose LMWH and 150mg aspirin
C - 75mg aspirin alone
Women with thalassaemia who have either previously undergone a splenectomy or have a current platelet count >600, should be commenced on low-dose aspirin (75mg OD). Women with both risk factors should commence both aspirin as well as low-molecular weight heparin.
A 23 year old primigravida attends the ANC at 13/40 on account of her thalassaemia major and previous splenectomy. All necessary pre-pregnancy investigations were arranged and returned satisfactory results though platelets were elevated at 610 on a recent full blood count. She wishes to know if there is any medication in additional to her existing 5mg folic acid, which she should be taking during pregnancy. What do you advise?
a. Treatment dose LMWH and 75mg aspirin
b. Prophylactic dose LMWH and 75mg aspirin
c. 75mg aspirin alone
d. Prophylactic dose LMWH alone
e. Treatment dose LMWH and 150mg aspirin
B - Prophylactic dose LMWH and 75mg aspirin
Women with thalassaemia who have either previously undergone a splenectomy or have a current platelet count >600, should be commenced on low-dose aspirin (75mg OD). Women with both risk factors should commence both aspirin as well as low-molecular weight heparin.
A primigravida with known thalassaemia major is admitted to delivery suit in early labour at 38/40. She is hoping for a vaginal delivery. A full blood count taken upon admission has returned the following results:
WCC 13.4
Hb 98
Plat 538
Based on these results, what management do you advise?
a. Delivery by LSCS Cat 2, administer 2 grams desferrioxamine
b. Delivery by LSCS Cat 2, administer 2 grams desferrioxamine and transfuse 2 units RBCs
c. Vaginal delivery, transfuse 2 units RBCs
d. Vaginal delivery, administer 2 grams desferrioxamine
e. Vaginal delivery, administer 2 grams desferrioxamine, cross-match 2 units RBCs
E - Vaginal delivery, administer 2 grams desferrioxamine, cross-match 2 units RBCs
Thalassaemia is not in itself an indication for delivery by caesarean section though patients should be adequately prepared for labour. High levels of circulating toxic non-transferrin bound iron may pre-dispose to cardiac dysrhythmias during labour. As such, chelation with desferrioxamine 2g over 24 hours should be given for the duration of labour. Patients in whom Hb is found to be <100g/L upon admission to labour ward should have 2 units of RBCs cross-matched.
Amongst patients with beta-thalassaemia major, who are transfusion dependent but not on a chelating agent, what is the risk associated with high serum levels of non-transferrin bound iron in labour?
a. VTE
b. Respiratory failure
c. Cardiac Arrhythmia
d. Hepatic failure
e. Impaired blood glucose control
C - Cardiac arrhythmias
Thalassaemia is not in itself an indication for delivery by caesarean section though patients should be adequately prepared for labour. High levels of circulating toxic non-transferrin bound iron may pre-dispose to cardiac dysrhythmias during labour. As such, chelation with desferrioxamine 2g over 24 hours should be given for the duration of labour. Patients in whom Hb is found to be <100g/L upon admission to labour ward should have 2 units of RBCs cross-matched.
How many transfusions per year distinguishes beta-thalassaemia major from intermedia?
a. 3
b. 7
c. 10
d. 15
e. 18
B - 7
Thalassaemia major describes women who require in excess of 7 transfusions per year, while intermedia refers to women requiring less than 7. Women who are heterozygotes - i.e. minor – do not require transfusion though may suffer from some degree of mild-moderate anaemia.
Measurement of HbA1C in women with thalassaemia is unreliable owing to the dilutional effect of multiple transfusions and fructosamine should be used instead. What level is advised in women with established DM prior to falling pregnant?
a. <50
b. <100
c. <200
d. <300
e. <500
D - <300
HbA1C is unreliable in women with thalassaemia as repeated transfusions dilute levels resulting in underestimation. Serum fructosamine is preferred for monitoring. The pre-pregnancy target is <300nmol/L for 3 months which is equivalent to an HbA1C of <43.
What is the recommended target weight of liver iron pre-pregnancy?
a. <5mg/g
b. <7mg/g
c. <11mg/g
d. <23mg/g
e. <35mg/g
B - <7mg/g
Women embarking on pregnancy should be assessed for liver iron concentration – ideally liver iron should be <7mg/g (dry weight).
What proportion of women with beta-thalassaemia are affected by alloimmunity?
a. 1/10
b. 1/8
c. 1/6
d. 1/2
e. 3/4
C - 1/6
16.5% (or 1/6) of women with thalassaemia have some degree of alloimmunisation pre-pregnancy owing to recurrent transfusions.
A women known to be a carrier for beta-thalassaemia presents for counselling pre-conceptually. Her partner has been tested and does not carry the gene for beta-thalassaemia itself, though does appear to carry another haemoglobin variant. Which of the following, when combined with beta-thalassaemia does NOT risk a serious haemoglobinopathy in the offspring?
a. HbS
b. Hb Lepore
c. HbO Arab
d. Hb Constant Spring
e. HbC
E - HbC
Other haemoglobinopathies in the partner of a women affected by beta-thalassaemia may carry significant implications for her offspring as follows:
Risk of serious haemoglobinopathy: HbS HbE Delat-beta thalassaemia Hb Lepore HbO Arab Hb Constant Spring
Risk of mild-moderate disorder only:
HbC
Other variant Hb
