Tutorial 13 September 2021

 

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53	COVID and pregnancy. Teresa Kelly.
54	SBA. McCune Albright syndrome
55	EMQ. Montgomery ruling  & consent
56	EMQ. Jacob’s syndrome

 

53.        COVID and pregnancy. Teresa Kelly.

Teresa is a consultant at St. Mary’s hospital in Manchester and, among many other things, the local expert on COVID.

54.         McCune-Albright syndrome.

Abbreviations.

MCA:            McCune Albright syndrome.

PFD:              polyostotic fibrous dysplasia.

PP:                 precocious puberty.

Scenario 1.             Which, if any, of the following are components of the classical triad of MCA?

Option List

A	albinism
B	“cafè Cubano” spots
C	“Coast of California” pigmented areas
D	lentigo
E	osteomalacia
F	polyostotic fibrous dysplasia
G	precocious puberty
H	premature menopause
I	primary amenorrhoea

Scenario 2.             Which, if any, of the following are true in relation to MCA?

Option List

A	it is an example of central primary amenorrhoea
B	it is an example of central secondary amenorrhoea
C	it is an example of central precocious puberty
D	it is an example of peripheral primary amenorrhoea
E	it is an example of peripheral secondary amenorrhoea
F	it is an example of peripheral precocious puberty
G	none of the above

Scenario 3.             Which, if any, of the following are true in relation to MCA?

Option List

A	hyperthyroidism is common
B	hypothyroidism is common
C	thyroid function is similar to those without MCA

Scenario 4.             Which, if any, of the following are true in relation to MCA?

Option List

A	excess growth hormone production  is common
B	inadequate growth hormone production is common
C	growth hormone production is similar to those without MCA

Scenario 5.             Which, if any, of the following is true in relation to MCA?

Option List

A	inheritance is autosomal dominant
B	inheritance is autosomal recessive
C	inheritance is X-linked dominant
D	inheritance is X-linked recessive
E	inheritance is multifactorial
F	it is not a hereditary disorder
G	the aetiology is not genetic
H	none of the above

Scenario 6.             Which, if any, of the following are true in relation to MCA?

Option List

A	renal artery stenosis is more common
B	renal cortex wasting is more common
C	renal phosphate wasting is more common
D	renal waisting is more common
E	none of the above.

Scenario 7.             Approximately what % of children born to women with MCAS will have MCAS?

Option List

A	0
B	1 in 105 - 106
C	1 in 104
D	1 in 100
E	1 in 50
F	1 in 10
G	1 in 2
H	All

 

55.         Montgomery Ruling.

Abbreviations.

BMA:       British Medical Association.

GMC:       General Medical Council.

MR:         Montgomery Ruling.

Question 1.             

Which, if any, of the following statements is most accurate?

Lead-in

A	The MR largely replaces the Bolam ruling
B	The MR largely replaces the Chester ruling
C	The MR largely replaces the Sidaway ruling
D	The MR is being contested in the European Court by the GMC as it infringes doctors’ rights
E	The MR is being contested in the European Court by the BMA as it infringes doctors’ rights

Question 2.             

Which, if any, of the following statements are true? This is not a true EMQ as > 1 of the answers may be correct.

Lead-in

A	the level of risk, however small, must be disclosed if a patient requests it
B	the level of risk of damage from a procedure need not be disclosed if < 1%
C	the level of risk of damage from a procedure need not be disclosed if < 10%
D	a material risk is one that would be reflected in damages > £100,000 if negligence were proved in court
E	a material risk is one that would be reflected in damages > £1,000,000 if negligence were proved in court
F	a material risk is one that involves anatomical damage, not emotional or psychological
G	a material risk is one that a reasonable person in the patient’s situation would be likely to regards as significant

 

56.         Jacob’s syndrome.

Abbreviations.

ADHD:          Attention-Deficit, Hyperactivity Disorder

ASD:              autistic spectrum disorder.

Js:                  Jacob’s syndrome.

Question 1.            What is the approximate incidence of Jacob’s syndrome in newborn females?

Option list. There is none – just give a figure.

Question 2.            What is the approximate incidence of Jacob’s syndrome in newborn males?

Option list. There is none – just give a figure.

Question 3.            What type of disorder is Jacob’s syndrome?

Option list.

A	autosomal dominant
B	autosomal recessive
C	autosomal trisomy
D	sex chromosome trisomy
E	X-linked dominant
F	X-linked recessive
G	trinucleotide repeat

Question 4.            What proportion of cases of Jacob’s syndrome are believed to go undiagnosed?

Question 5.            Which, if any, of the following are true of the Jacob’s phenotype? This is not a true EMQ as there may be > 1 answer.

Option list.

A	ataxia
B	clinodactyly
C	hypertelorism
D	hypotonia
E	macrocephaly
F	microcephaly
G	macroorchidism
H	microorchidism
I	premature ovarian failure
J	short stature
K	tall stature
L	tremor

Question 6.            Which, if any, of the following are more common in Jacob’s syndrome.

Option list.

A	ADHD
B	ASD
C	aggressive behaviour
D	asthma
E	criminal behaviour
F	diabetes
G	epilepsy
H	hypogonadotrophic hypogonadism
I	hypertension
J	infertility
K	low IQ
L	schizophrenia