Tutorial 22nd. June 2017

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24	EMQ. MBRRACE
25	EMQ. Mode of inheritance

24.   EMQ. MBRRACE. Maternal mortality.

Lead-in.

The following questions relate to MBRRACE and maternal mortality.

Pick one option from the option list.

Each option can be used once, more than once or not at all.

Abbreviations.

CEMD:                Confidential Enquiry into Maternal Death.

CE:                       Confidential Enquiry.

CER:                    Confidential Enquiry Report.

CHD:                   Congenital heart disease.

CMACE:              Centre for Maternal and Child Enquiries (now defunct).

CODAC:              Causes of Death and Associated Conditions. Classification system for perinatal death.

FAST:                  Focused Assessment with Sonography in Trauma.

HQIP:                  Health Quality Improvement Partnership.

ICD-10:               WHO’s International Classification of Diseases. Version 10 from 1990.

ICD-MM:            WHO’s use of ICD-10 for classification of maternal deaths.

ICNARC:             The Intensive Care National Audit and Research Centre.

MBRRACE:         MMBRACE-UK. Mothers and Babies - Reducing Risk through Audits and Confidential Enquiries across the UK.

MBRRACE14:     Saving Lives, Improving Mothers’ Care. 2009-12. Published December 2014.

MBRRACE15:     Saving Lives, Improving Mothers’ Care. 2011-13. Published December 2015.

MBRRACE16:     Saving Lives, Improving Mothers’ Care. 2012-14. Published December 2016.

MMR:                 Maternal mortality rate

MMRat               Maternal mortality ratio.

MMRpt.             Maternal Mortality Report.

MMRpt06-8:     Maternal Mortality Report 2006-8: “Saving Mothers’ Lives. Reviewing maternal deaths to make motherhood safer: 2006-2008

NMNIRP:            National Maternal, Newborn and Infant Review Programme

NPEU:                 National Perinatal Epidemiology Unit at Oxford University.

SADS/MNH:       Sudden adult death syndrome with a morphologically normal heart.

SANDS:               Stillbirth and Neonatal Death Society.

TIMMS:              The Infant Mortality & Morbidity Studies at the University of Leicester.

UKOSS:                UK Obstetric Surveillance System run by the NPEU.

Question 1.    What is the meaning of the acronym MBRRACE-UK”?

Option list.     There is none, to make things more testing.

Question 2.    Which organisation does it replace?

Question 3.    How does it differ structurally from its predecessor?

Question 4.    How will the format of its reports differ from those of its predecessor?

Question 5.    When was MBRRACE’s first Report published?

Question 6.    What was unusual about MBRRACE’s first Report?

A	it covered three years, not two
B	it covered four years, not three
C	it was very amusing
D	it made serious criticisms of the funding of the NHS
E	it made serious criticisms of the hours worked by junior doctors
F	none of the above

Question 7.    What is ICD-MM?

A	ICD-10 as applied to maternal death
B	ICD-11 as applied to maternal death
C	International classification of maternal madness
D	International chocolate delice- Mmmmm!
E	none of the above

Question 8.    When was ICD-MM adopted by MBRRACE?

A	2014
B	2015
C	2016
D	ICD-MM does not exist
E	ICD-MM will be introduced in 2017
F	none of the above

Question 9.    What changes were made to the classification of maternal suicide by MBRRACE?

A	maternal suicide was reclassified as direct death
B	maternal suicide was reclassified as indirect death
C	maternal suicide was reclassified as late death as most occur > 6/52 post-delivery
D	maternal suicide was reclassified as coincidental, as most women were already very ill
E	maternal suicide was reclassified as irrelevant as these women were suicide-likely
F	none of the above

Question 10.  When  were changes made to the classification of maternal suicide by MBRRACE?

A	2014
B	2015
C	2016
D	the changes are planned for 2017
E	no changes have been made and none are planned
F	none of the above

Question 11.  What geographical innovation was included in MBRRACE’s first Report?

Question 12.  What alterations were made to the timings of maternal death to be considered in its Reports?

Question 13.  What was the latest MMR reported by MBRRACE?

Question 14.  How did this compare with the final MMR reported  by CMACE?  

A	MMR was lower, but the difference was not statistically significant
B	MMR was lower and the difference was  statistically significant
C	MMR was higher, but the difference was not statistically significant
D	MMR was higher and the difference was statistically significant
E	MMR was similar

Question 15.  Which, if any, of the following topics were the basis of confidential enquiries in the first MBRRACE Report in December 2014?

Question 16.  Which topics were reviewed in detail in the second Report in 2015?

Question 17.  Which topics were reviewed in detail in the third Report in 2016?

Question 18.  Which topics will be reviewed in detail in the fourth Report in 2017?

Question 19.  What is the definition of a maternal death?

Question 20.  What is the definition of a direct maternal death?

Question 21.  What is the definition of indirect maternal death?

Question 22.  What was the leading direct cause of death in the first MBRRACE Report?

Question 23.  What was the leading indirect cause of death in the first Report?

Question 24.  What were the 5 top causes of direct maternal death in the triennium 2011 – 2013?

Question 25.  What observation was made in the first Report about deaths due to hypertensive diseases?

Question 26.  Which condition was linked to 1 in 11 maternal deaths in the first Report in 2014?

Question 27.  What key messages were singled out in the first MBRRACE Report in 2014?

Question 28.  What key messages were singled out in the second MBRRACE Report in 2015?

Question 29.  What key messages were singled out in the third MBRRACE Report in 2016?

Question 30.  What messages relating to critical care were included in the third MBRRACE Report in 2016?

Question 31.  What is the definition of the maternal mortality rate?

Question 32.  What is the definition of a “maternity”?

Question 33.  What is the definition of a live birth?

Question 34.  What is the definition of a stillbirth?

Question 35.  What is the definition of the maternal mortality ratio?

Question 36.  How many maternal deaths were due to cardiac causes in 2012-14?

Option list.

A	47
B	51
C	56
D	63
E	78
F	82
G	90

Question 37.  How many deaths due to cardiac causes were considered in detail in the Confidential Enquiry into cardiac deaths in the 2012-14 Report?

Option list.

A	35
B	48
C	51
D	78
E	108
F	135
G	153
H	178
I	201

Question 38.  Which day was singled out as the most dangerous for cardiac death?

Option list.

A	the day of onset of labour
B	the 24 hours after the administration of a general anaesthetic in labour
C	the 24 hours after the delivery of a baby by Caesarean section
D	the 24 hours after instrumental delivery of a baby
E	the day of delivery
F	the day of delivery after the birth of the baby
G	the first day at home

Question 39.  What percentage of cardiac deaths took place on the day highlighted as the most dangerous?

Option list.

A	5%
B	10%
C	15%
D	20%
E	25%
F	30%

Question 40.  What were the three most common causes of cardiac death recorded in MBRRACE16?

Option list.

A	Aortic dissection
B	Congenital heart disease (CDH)
C	Hypertension
D	Ischaemic heart disease
E	Myocardial disease / cardiomyopathy
F	Other
G	Rheumatic heart disease.
H	SADS/MNH
I	Valvular heart disease

Question 41.  How many deaths due to congenital heart disease were recorded in MBRRACE16?

Option list.

A	0
B	3
C	5
D	11
E	15
F	24
G	35

Question 42.  What were the main causes of congenital heart disease deaths recorded in MBRRACE16?

Option list.

A	Aortic dissection
B	Aortic rupture
C	Left heart failure
D	Right heart failure
E	Pulmonary artery hypertension
F	Pulmonary vein hypertension
G	Valvular heart disease

Question 43.  Approximately what proportion of the women who died of cardiac disease in MBRRACE16 were known to have cardiac disease before the pregnancy?

Option list.

A	10%
B	20%
C	30%
D	40%
E	50%
F	60%
G	70%
H	80%
I	90%

Question 44.  What other risk factors were noted in MBRRACE16 in relation to the women who died of cardiac causes?

Option list.     There is no option list to make your life harder. But you know the risk factors!

Question 45.  What proportion of the cardiac deaths in MBRRACE16 occurred in ambulances or emergency departments?

Option list.

A	5%
B	10%
C	20%
D	30%
E	40%
F	50%

Question 46.  What “overall messages for future care” in relation to cardiac disease were included in MBRRACE16?

Option list. There is none.

Question 47.  How many deaths occurred due to aortic dissection in 2009-14?

Option list.

A	0
B	3
C	6
D	9
E	15
F	18
G	21
H	24
I	30

Question 48.  Which, if any of the following statements are true in relation to the deaths from aortic dissection in MBRRACE16?

Option list.

A	most occur in late pregnancy / puerperium, the risk being 25 times greater than at other times
B	the most common cause of death is tamponade
C	20 of the deaths involved the descending aorta
D	the classical symptoms are severe chest pain radiating to the back
E	the classical symptoms are severe chest pain radiating to the left arm
F	the classical symptoms are severe chest pain radiating to the neck
G	most cases occurred in women with known aortopathy, especially Marfan’s syndrome
H	surgical repair of congenital, complex coarctation was identified as a risk factor.
I	8 of the 21 women had presented in the days before death but aortic dissection had not been considered
J	42% of the women died at home or before reaching the emergency department.
K	better care might have made a difference to the outcome in almost 60% of cases.

Question 49.  What were the “Key messages” about cardiovascular disease in MBRRACE16?

Option list.     There is none. Write as many as you know.

Question 50.     Acute coronary syndrome. I have written an EMQ about myocardial infarction. It has data from the UKOSS survey. https://www.ncbi.nlm.nih.gov/pubmed/22127355 and https://www.npeu.ox.ac.uk/research/ukoss-myocardial-infarction-136. I’ll add the data from MBRRACE 16 and put it in one of the tutorials.

Question 51.     Approximately how many women died of myocardial disease / cardiomyopathy?

Option list.

A	5
B	10
C	15
D	20
E	25

Question 52.     Approximately how many women died of peripartum cardiomyopathy?

Option list.

A	5
B	10
C	15
D	20
E	25

Question 53.     What type of cardiomyopathy is peripartum cardiomyopathy?

Option list.

A	congenital cardiomyopathy
B	dilated cardiomyopathy
C	hypertrophic cardiomyopathy
D	obesity-related cardiomyopathy
E	restrictive cardiomyopathy

Question 54.     With regard to cardiomyopathy, which symptom is singled out in MBRRACE 16 as particularly needing full investigation?

Option list.

A	angina
B	“drop” attacks
C	dyspnoea
D	nocturnal sweats
E	palpitations

Question 55       Which of the following are especially problematic for women with hypertrophic cardiomyopathy?

Option list.

A	bradycardia
B	epilepsy
C	hyperglycaemia
D	hypertension
E	hypotension
F	tachycardia

Question 56.     MBRRACE 16 records that investigation ceased once a particular diagnosis had been excluded in a number of cases of cardiovascular compromise and the women died later of undiagnosed cardiac disease. What was the diagnosis?

Option list.

A	acute coronary syndrome
B	aortic stenosis
C	atrial fibrillation
D	pulmonary embolism
E	ventricular fibrillation

Question 57.     When are women with peripartum cardiomyopathy most likely to die?

Option list.

A	1st. trimester
B	2nd. trimester
C	3rd. trimester
D	1st. stage of labour
E	2nd. stage of labour
F	3rd. stage of labour
G	1st. 24 hours after delivery
H	in the puerperium
I	from 6 weeks to 1 year after the delivery

Question 58.     Which, if any, of the following statements are true  in relation to obesity-related cardiomyopathy (ORC) ?

Option list.

A	ORC is not a recognised condition
B	MBRRACE16 reported 2 deaths from ORC
C	ORC is associated with cardiac enlargement
D	ORC is associated with fatty infiltration of the ventricular muscle
E	is characterised by myocyte depletion and left ventricular hypoplasia
F	is characterised by myocyte hypertrophy and left ventricular hypertrophy

Question 59.     How many deaths were due to valvular heart disease ?

Option list.

A	1
B	2
C	3
D	4
E	5
F	6
G	7
H	8
I	9
J	10
K	11

Question 60.     Why am I going to write a separate EMQ on valvular heart disease?

Option list.

A	I am now bored with this topic
B	I find it so fascinating that I feel it deserves its own EMQ
C	I don’t know enough about it and need to do some research
D	UKOSS conducted a study from 2013 – 2015 and this needs to be included
E	none of the above.

Question 61.     What were the key messages re hypertensive disease in MBRRACE16?

Option list. There is none. Write as many as you can think of.

Question 62.     How many deaths due to hypertensive disease occurred in 2009-14?

Option list.

A	2
B	5
C	14
D	20
E	23

Question 63.     Which, if any, of the following was the most common cause of death from hypertensive disease in 2009-14?

Option list.

A	acute fatty liver of pregnancy
B	eclampsia / cerebral oedema
C	haemorrhage due to thrombocytopenia
D	HELLP /hepatic necrosis
E	hepatic rupture
F	intracranial haemorrhage
G	left ventricular failure
H	pulmonary oedema

Question 64.     Which, if any, of the following conditions does MBRRACE16 say are usually attributable to poor fluid management?

Option list.

A	acute fatty liver of pregnancy
B	eclampsia / cerebral oedema
C	haemorrhage due to thrombocytopenia
D	HELLP /hepatic necrosis
E	hepatic rupture
F	intracranial haemorrhage
G	left ventricular failure
H	pulmonary oedema

Question 65.     What upper gestational limit was used by MBRRACE16 in the definition of early pregnancy?

Option list.

A	10 weeks
B	12 weeks
C	16 weeks
D	18 weeks
E	20 weeks
F	24 weeks
G	26 weeks

Question 65.     Which of the following ranked top in the causes of death < 24 weeks?

Option list.

A	Cardiac
B	Ectopic
C	Haemorrhage
D	Mental health problems
E	Miscarriage
F	Sepsis
G	Thrombosis & thrombo-embolism
H	TOP

Question 66.     Why did MBRRACE recommend FAST for women presenting to emergency departments with pulmonary embolism in the list of differential diagnoses?

Option list.

A	to exclude aortic dissection before thrombolysis
B	to exclude acute coronary syndrome before thrombolysis
C	to exclude intra-peritoneal bleeding from ectopic pregnancy before thrombolysis
D	to exclude intra-uterine pregnancy before thrombolysis
E	to exclude Bornholm disease before thrombolysis

Question 67.     What were the key messages in relation to early pregnancy deaths?

Option list. There is none. Write as many as you can think of.

Question 68.     What proportion of pregnant / recently delivered women needing critical care survive?

Option list.

A	50%
B	60%
C	70%
D	80%
E	90-94%
F	≥ 95%

Question 69.     MBRRACE16 looked at the cause of death in 144 women admitted to critical care from 2009-14. What was the most common cause of death?

Option list.

A	Amniotic fluid embolism
J	Anaesthetic
I	Cardiac
L	Coincidental
B	Early pregnancy death
D	Haemorrhage
E	Neurological
K	Other indirect
C	PET / eclampsia
H	Psychiatric
G	Sepsis
F	Thrombosis / thrombo-embolism
M	Unascertained

Question 70.     What are the key facts to remember about critical care?

Option list. There is none. Write what you think are the key facts and numbers.

Question 71.     What “red flags” does MBRRACE highlight in relation to maternal sepsis?

Option list. There is none.

Question 72.     What were MBRRACE16’s “key messages” for critical care?

Option list. There is none.

25.   EMQ. Mode of inheritance.

Lead-in.

The following questions relate to the mode of inheritance – some not quite to “mode”, but I am sure you will indulge me!

For each question, write what you think is the mode of inheritance or appropriate answer. There is no option list.

Comment.

You are expected to know a lot of basic genetics and it is hard to remember the details. A list to go over in the days before the exam makes sense. Use this one and add anything else you can think of – and let me know of your additions so I can add them to this list. Don’t add a load of very rare syndromes – you will just end up confused. But add anything that you know has featured in the exam.

List of questions.

1.       achondrogenesis.

2.       achondroplasia.

3.       acute fatty liver of pregnancy (AFLP).

4.       adreno-genital syndrome

5.       adult polycystic kidney disease.

6.       androgen insensitivity syndrome.

7.       albinism.

8.       Angelman syndrome.

9.       Apert syndrome.

10.   Becker muscular dystrophy.

11.   Beckwith-Wiedemann syndrome.

12.   BRCA 1.

13.   BRCA2.

14.   Cavanan syndrome.

15.   Charcot-Marie-Tooth disease.

16.   chondrodystrophy.

17.   Christmas disease.

18.   congenital adrenal hyperplasia.

19.   Cowden syndrome.

20.   cri-du-chat syndrome. 

21.   cystic fibrosis.

22.   Dandy-Walker syndrome.

23.   developmental dysplasia of the hip.

24.   DiGeorge syndrome.

25.   Down’s syndrome.

26.   Duchenne muscular dystrophy

27.   Dwarfism. See isolated growth hormone deficiency.

28.   Edward’s syndrome.

29.   exomphalos.

30.   Ehlers-Danlos syndrome

31.   Fanconi anaemia

32.   Fitz-Hugh-Curtis syndrome.

33.   Fragile X syndrome.

34.   galactosaemia.

35.   gastroschisis.

36.   glucose-6-phosphatase deficiency. G6PD.

37.   glucose-6-phosphate dehydrogenase deficiency. G6PDD.

38.   haemochromatosis.

39.   haemosiderosis.

40.   haemophilia A.

41.   haemophilia B.

42.   Holt-Oram syndrome.

43.   Hunter syndrome.

44.   Huntington’s disease.

45.   ichthyosis.

46.   isolated growth hormone deficiency.

47.   juvenile polycystic kidney disease.

48.   Kallmann’s syndrome.

49.   Klinefelter’s syndrome.

50.   Lesch Nyhan syndrome.

51.   Lynch syndrome (HNPCC).

52.   Malignant hyperthermia.

53.   Maple syrup urine disease. 

54.   Marfan’s syndrome.

55.   Martin-Bell syndrome.

56.   Mayer-Rokitansky-Kuster-Hauser syndrome.

57.   McCune-Albright syndrome.

58.   Meckel-Gruber syndrome.

59.   medium-chain acyl-CoA dehydrogenase deficiency.

60.   mucopolysaccharidosis type I.

61.   myotonic dystrophy.

62.   neurofibromatosis.

63.   Niemann-Pick disease.

64.   Noonan syndrome.

65.   ocular albinism.

66.   osteogenesis imperfecta.

67.   osteoporosis.

68.   Patau’s syndrome.

69.   Perrault syndrome.

70.   phenyketonuria.

71.   polydactyly.

72.   porphyria.

73.   Potter’s syndrome.

74.   Prader-Willi syndrome. 

75.   prune-belly syndrome

76.   pyruvate kinase deficiency.

77.   sickle cell disease.

78.   spherocytosis.

79.   Syndrome X.

80.   Tay-Sach’s disease.

81.   Thalassaemia.

82.   Thrombophilia.

83.   Triple X syndrome.

84.   Turner’s syndrome.

85.   Swyer’s syndrome.

86.   Uniparental disomy.

87.   VACTERL.

88.   vitamin D resistant rickets

89.   von Willebrand’s disease.

90.   A mother has spina bifida. What is the risk of a child being affected? 

91.   A mother has had a child with spina bifida, what is the risk of the next child being affected?   

92.   A mother has had two children with spina bifida. What is the risk of the next child being affected?

93.   A mother has grand-mal epilepsy. What is the risk of her child having epilepsy?

94.   A mother and her partner both have grand-mal epilepsy. What is the risk of their child having epilepsy?

95.   A mother has insulin-dependent diabetes mellitus. What is the risk of a child being affected?

96.   A mother has congenital heart disease. What is the risk of a child being affected? 

97.   A mother takes lithium for bi-polar disorder throughout her pregnancy. What is the risk of the child having congenital heart disease?

98.   A mother has a nuchal translucency scan at 11 weeks. The result is 6 mm. What is the risk of the fetus having congenital heart disease?