Monday 30 November 2020

Tutorial 30th. November 2020

 


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16

EMQ. Kell antibodies

17

EMQ. Mayer-Rokitansky-KΓΌster-Hauser syndrome

18

SBA. Quinolone antibiotics

19

EMQ. Parvovirus

20

EMQ. Galactosaemia

 

16.   EMQ. Kell antibodies.

Abbreviations.

∆OD450:      spectrophotometric measurement of deviation in optical density at wavelength 450 nm.

FMM:         feto-maternal medicine

HDFN:        haemolytic disease of the fetus and newborn.

MCAPSV:   middle cerebral artery peak systolic velocity.

RBC:           red blood cell.

Scenario 1.              

Which, if any, of the following alloantibodies is the most common cause of significant HDFN?

Option list.

A

anti-D

B

anti-C

C

anti-c

D

anti-e

E

Duffy: Fya

F

Duffy: Fyb

G

Kell

H

Kidd: Jka

I

Kidd: Jkb

Scenario 2.              

Which, if any, of the following alloantibodies is the 2nd. most common cause of significant HDFN?

Option list. Use the option list from Scenario 1.

Scenario 3.              

Which, if any, of the following alloantibodies is the 3rd. most common cause of significant HDFN?

Option list. Use the option list from Scenario 1.

Scenario 4.              

Which of the following statements is true in relation to the Kell antigen?

Option list.

A

it is named after Mrs. Kelleher who was found to have antibodies to it in 1946

B

it is named after Gene Kelly, the American actor, dancer and singer as the research group who found the antigen were big fans

C

there are > 50 significant variants of the Kell antigen

D

Kell antibodies are mainly IgA

E

Kell antibodies are mainly IgM

F

none of the above

Scenario 5.              

What proportion of the Caucasian population is K +ve?

Option list.

A

1%

B

5%

C

9%

D

15%

E

25%

F

33%

G

57%

H

none of the above

Scenario 6.              

The Kell antigen can be detected using cell-free fetal DNA in maternal serum.  True / False.

Scenario 7.              

Anti-K is thought to occur mainly as a result of feto-maternal transfusion of Kell +ve cells during pregnancy and delivery. True / False.

Scenario 8.              

Kell HDFN resulting from transfusion of Kell +ve blood is thought to produce more severe HDFN than that resulting from feto-maternal transfusion. True / False.

Scenario 9.              

The Kell antigen can be detected using cell-free fetal DNA in maternal serum.  True / False.

Scenario 10.           

Which of the following statements is true in relation to anti-Kell antibodies in a Kell-negative mother with a Kell +ve pregnancy?

Option list.

A

HDND is mainly due to haemolysis of fetal RBC

B

HDND is mainly due to haemolysis of fetal & neonatal RBC

C

HDND is mainly due to haemolysis of neonatal RBC

D

HDND is mainly due to sequestration of fetal RBC

E

HDND is mainly due to sequestration of fetal & neonatal RBC

F

HDND is mainly due to sequestration of neonatal RBC

G

HDND is mainly due to suppression of fetal erythroid progenitor cells

H

HDND is mainly due to suppression of neonatal erythroid progenitor cells

I

none of the above

Scenario 11.           

Which of the following statements is true in relation to antenatal detection of HDFN due to anti-K antibodies?

Option list.

A

the threshold for significant HDFN is a titre of 1 in 4

B

the threshold for significant HDFN is a titre of 1 in 8

C

the threshold for significant HDFN is a titre of 1 in 16

D

the threshold for significant HDFN is a titre of 1 in 32

E

the threshold for significant HDFN is a titre of 1 in 64

F

the threshold for significant HDFN is a titre of 1 in 128

G

the threshold for significant HDFN is a titre of 1 in 256

H

none of the above

Scenario 12.           

Which of the following statements is true in relation to antenatal detection of HDFN due to anti-K antibodies?

Option list.

A

the threshold for significant HDFN is a level > 2 iu/L.

B

the threshold for significant HDFN is a level > 4 iu/L.

C

the threshold for significant HDFN is a level > 7.5 iu/L.

D

the threshold for significant HDFN is a level > 10 iu/L.

E

the threshold for significant HDFN is a level > 15 iu/L.

F

the threshold for significant HDFN is a level > 25 iu/L.

G

the threshold for significant HDFN is any level if anti-E is also present.

H

none of the above

Scenario 13.           

Which, if any, of the following statements are true in relation to referral to a FMM expert when Kell antibodies are detected?

Option list.

A

the threshold for referral is a level of anti-K > 2 iu/L.

B

the threshold for referral is a level of anti-K > 4 iu/L.

C

the threshold for referral is a level of anti-K > 7.5 iu/L.

D

the threshold for referral is a level of anti-K > 10 iu/L.

E

the threshold for referral is a level of anti-K > 15 iu/L.

F

the threshold for referral is a level of anti-K > 25 iu/L.

G

the threshold for referral is any level of anti-K.

H

the threshold for referral is any level of anti-K if anti-E is also present.

I

none of the above

Scenario 14.           

Which of the following statements is true in relation to the threshold for antenatal diagnosis of significant HDFN due to anti-K when using measurement of MCAPSV?

Option list.

A

MoM > 1.25

B

MoM > 1.50

C

MoM > 1.75

D

MoM > 2.00

E

MoM > 2.50

F

MoM > 3.00

G

none of the above

Scenario 15.           

Which of the following statements is true in relation to the threshold for antenatal diagnosis of significant HDFN due to anti-K when using measurement of ∆OD450?

Option list.

A

MoM > 1.25

B

MoM > 1.50

C

MoM > 1.75

D

MoM > 2.00

E

MoM > 2.50

F

MoM > 3.00

G

none of the above

Scenario 16.           

Which, if any, of the following statements are true in relation to the numbers of reticulocytes in cord blood in moderate to severe HDFN?

Option list.

A

the numbers are decreased

B

the numbers are increased

C

the numbers are normal

D

none of the above

Scenario 17.           

Which, if any, of the following statements are true in relation to the numbers of erythroblasts in cord blood in moderate to severe HDFN?

Option list.

A

the numbers are decreased

B

the numbers are increased

C

the numbers are normal

D

none of the above

Scenario 18.           

Which, if any, of the following statements are true in relation to the level of bilirubin in cord blood in moderate to severe HDFN?

Option list.

A

it is decreased

B

it is increased

C

it is greatly increased

D

none of the above

Scenario 19.           

Which, if any, of the following statements are true in relation to King Henry VIII and Kell?

Option list.

A

Kell may have been the cause of his subfertility

B

He may have had the McLeod syndrome

C

He may have inherited the Kell antigen from Jacquetta Woodville

D

The Kell antigen may have explained his passion for jousting

E

The Kell antigen may have explained his passion for extramarital dalliance

 

The TOG questions for the Gajjar article can be found  here. These are open access, so reproduced here. These are obviously MCQs with True or False options.

Regarding Kell alloimmunisation in pregnancy,

1      the amniotic fluid bilirubin level correlates well with the degree of fetal anaemia.

2      previous obstetric history does not reliably predict outcome.

3      the incidence in the obstetric population is approximately 1–2 per 1000.

4      prophylaxis is available.

5      the relationship between fetal middle cerebral artery peak systolic velocity (MCA-PSV) and haemoglobin concentration is poor.

6      anti-Kell antibodies cause fetal anaemia via the suppression of erythropoesis rather than red cell destruction.

With regard to maternal anti-Kell antibody screening,

7      if the father of the fetus is Kell antigen positive, the fetus is likely to be affected with severe HDFN.

8      where the father is heterozygous for Kell, there is a 50% chance of the fetus carrying the Kell antigen on its fetal red cells.

9      anti-Kell antibodies stimulated by transfusion are known to affect the fetus to the same degree as those stimulated from a previous pregnancy.

10    where the critical titre of anti-Kell antibodies has been reached in the maternal serum, amniocentesis for spectral analysis of amniotic fluid is a reliable means of establishing the degree and severity of fetal anaemia.

 

17.   EMQ. Mayer-Rokitansky-KΓΌster-Hauser syndrome.

Some of the questions are not true EMQs as there may be more than one correct answer – this is me being lazy and saving typing.

Mayer–Rokitansky–K

¨

uster–Hauser

syndrome: diagnosis and management

With regard to the MRKH syndrome,

61. there is failure of development of the

mesonephric ducts. T F

62. the phenotype and genotype are female. T F

63. studies have established a link between the

syndrome and the use of diethylstilbestrol in

pregnancy. T F

With regard to the anatomical abnormalities seen in

MRKH syndrome,

64. symmetrical uterovaginal aplasia is found in

type I disorders. T F

65. renal abnormalities are seen in more than

half of cases. T F

66. skeletal abnormalities are reported in up to

one-fifth of cases. T F

67. up to one-quarter of women have a

malformed ear or auditory canal. T F

68. the close proximity of the m

¨

ullerian and

wolffian duct derivatives to the metanephric

duct in the developing embryo explains the

higher association of malformations of the

kidneys with this condition. T F

69. vaginal agenesis is caused by failure of the

caudal part of the m

¨

ullerian duct system to

develop. T F

Regarding the diagnosis of MRKH syndrome,

70. magnetic resonance imaging is the gold

standard tool. T F

71. two-dimensional ultrasound scanning is not

useful for associated renal tract

abnormalities. T F

72. complete androgen insensitivity syndrome is

an important differential diagnosis. T F

73. the presence of cyclical abdominal pain will

rule out the diagnosis, as it indicates the

presence of functioning endometrium. T F

With regard to the creation of a neovagina,

74. it is recommended that treatment is initiated

as soon as the diagnosis is made. T F

75. psychological support to women undergoing

this procedure is of the utmost importance. T F

76. vaginal dilators are acceptable as an option

for first-line therapy. T F

77. Ingram’s modified Frank’s technique involves

the use of vaginal dilators. T F

With regard to the surgical creation of a neovagina,

78. in the Davydov procedure the neovagina is

lined with peritoneum. T F

With regard to fertility in women with the MRKH

syndrome,

79. transvaginal egg retrieval is recognised to be

difficult during in vitro fertilisation. T F

80. the condition has been shown to be

transmissible to the offspring. T F

Abbreviations.

AIS:          androgen insensitivity syndrome

AMH:       anti- MΓΌllerian hormone

MRKH:    Mayer-Rokitansky-KΓΌster-Hauser syndrome

MURCS:  MΓΌllerian duct aplasia, renal dysplasia and cervical somite anomaly syndrome.

 

Question 1.             

What are the main features of MRKH? There is no option list to make life harder.

Question 2.             

Which, if any, are the main secondary features associated with MRKH?

Option list.

A

anosmia

B

attention-deficit-hyperactivity syndrome

C

auditory anomalies

D

neural tube defects

E

renal anomalies

F

skeletal anomalies

Question 3.             

How does MRKH syndrome usually present?

Option list.

A

cyclical pain due to haematometra

B

delayed puberty

C

precocious puberty

D

premature menopause

E

primary amenorrhoea

F

recurrent otitis media

G

recurrent urinary tract infection

H

secondary amenorrhoea

Question 4.             

Which of the following chromosome patterns are typical of MRKH?

Option list.

A

45XO

B

45YO

C

46XX

D

46XY

E

47XXX

F

47XXY

Question 5.             

What is the approximate incidence of MRKH in newborn girls?

Option list.

A

~ 1 in 1,000

B

~ 1 in 2,000

C

~ 1 in 4,000

D

~ 1 in 6.000

E

~ 1 in 8,000

F

~ 1 in 10,000

G

~ 1 in 100,000

H

the figure is unknown

I

it does not occur

Question 6.             

What is the approximate incidence of MRKH in newborn boys?

Option list.

A

~ 1 in 1,000

B

~ 1 in 2,000

C

~ 1 in 4,000

D

~ 1 in 6.000

E

~ 1 in 8,000

F

~ 1 in 10,000

G

~ 1 in 100,000

H

the figure is unknown

I

it does not occur

Question 7.             

Which of the following statements are correct in relation to urinary tract anomalies associated with MRKH?

Option list.

A

absent bladder

B

absent kidney

C

ectopic ureter

D

horseface kidney

E

hypospadias

F

urinary tract anomalies are not part of the syndrome

Question 8.             

Which of the following statements are correct in relation to skeletal anomalies associated with MRKH?

Option list.

A

absent thumb

B

absent big toe

C

developmental dysplasia of the hip

D

Klippel-Feil anomaly

E

ulnar hypoplasia

F

vertebral fusion

G

skeletal anomalies are not part of the syndrome

Question 9.             

Which of the following statements are correct in relation to auditory anomalies associated with MRKH?

Option list.

A

absent ear

B

absent stapes

C

acoustic neuroma

D

conductive deafness

E

inductive deafness

F

stapedial ankylosis

G

auditory anomalies are not part of the syndrome

Question 10.         

Lead-in.

What is the recommended first-line management for creation of a neovagina.

Option list.

A

digital dilatation

B

marriage to a virile husband

C

vaginal balloons

D

vaginal dilators

E

vaginoplasty

F

there is no recommended 1st. line management

Question 11.         

What are the key features of Davydov vaginoplasty?

Option list.

A

horseshoe perineal incision with labial flaps used to create a pouch

B

creation of space between bladder and rectum and lining it with amnion

C

creation of space between bladder and rectum and lining it with skin graft

D

creation of space between bladder and rectum and lining it with sigmoid colon

E

creation of space between bladder and rectum and lining it with peritoneum

F

traction via threads running to the abdomen from a vaginal bead

Question 12.         

What  are the key features of McIndoe vaginoplasty?

Option list.

A

horseshoe perineal incision with labial flaps used to create a pouch

B

creation of space between bladder and rectum and lining it with amnion

C

creation of space between bladder and rectum and lining it with skin graft

D

creation of space between bladder and rectum and lining it with sigmoid colon

E

creation of space between bladder and rectum and lining it with peritoneum

F

traction via threads running to the abdomen from a vaginal bead

Question 13.         

What  are the key features of Vecchietti vaginoplasty?

Option list.

A

horseshoe perineal incision with labial flaps used to create a pouch

B

creation of space between bladder and rectum and lining it with amnion

C

creation of space between bladder and rectum and lining it with skin graft

D

creation of space between bladder and rectum and lining it with sigmoid colon

E

creation of space between bladder and rectum and lining it with peritoneum

F

traction via threads running to the abdomen from a vaginal bead

Question 14.         

Lead-in.

What are the key features of Williams vaginoplasty?

Option list.

A

horseshoe perineal incision with labial flaps used to create a pouch

B

creation of space between bladder and rectum and lining it with amnion

C

creation of space between bladder and rectum and lining it with skin graft

D

creation of space between bladder and rectum and lining it with sigmoid colon

E

creation of space between bladder and rectum and lining it with peritoneum

F

traction via threads running to the abdomen from a vaginal bead

TOG CPD questions.

With regard to the MRKH syndrome.

1.     there is failure of development of the mesonephric ducts.

2.     the phenotype and genotype are female

3.     studies have established a link between the syndrome and the use of diethylstilboestrol in pregnancy.

With regard to the anatomical abnormalities seen in MRKH syndrome.

4.     symmetrical uterovaginal aplasia is found in type I disorders

5.     renal abnormalities are seen in more than half of cases.

6.     skeletal abnormalities are reported in up to one-fifth of cases.

7.     up to one-quarter of women have a malformed ear or auditory canal.

8.     the close proximity of the MΓΌllerian and Wolffian duct derivatives to the duct in the developing embryo explains the higher association of malformations of the kidneys with this condition.

9.     vaginal agenesis is caused by failure of the caudal part of the MΓΌllerian duct system to develop.

Regarding the diagnosis of MRKH syndrome,

10.   magnetic resonance imaging is the gold standard tool.

11.   two-dimensional ultrasound scanning is not useful for associated renal tract abnormalities.

12.   complete androgen insensitivity syndrome is an important differential diagnosis.

13.   the presence of cyclical abdominal pain will rule out the diagnosis, as it indicates the presence of functioning endometrium.

With regard to the creation of a neovagina,

14.   it is recommended that treatment is initiated as soon as the diagnosis is made.

15.   psychological support to women undergoing this procedure is of the utmost importance.

16.   vaginal dilators are acceptable as an option for first-line therapy.

17.   Ingram’s modified Frank’s technique involves the use of vaginal dilators.

With regard to the surgical creation of a neovagina,

18.   in the Davydov procedure the neovagina is lined with peritoneum.

With regard to fertility in women with the MRKH syndrome,

19.   transvaginal egg retrieval is recognised to be difficult during in vitro fertilisation.

20.   the condition has been shown to be transmissible to the offspring.

 

18.   SBA. Quinolone antibiotics.

Quinolone & fluoroquinolone antibacterial drugs

Not all of the questions are true SBAs as some have more than one answer. The true SBAs have ‘is’, not ‘are’ in the wording.

Abbreviations.

FQ:             fluoroquinolone.

QUI:           quinolone.

Question  1.           

Which, if any, of the following drugs are QUIs or FQs? 

Drugs

A.       

cimetidine

B.       

ciprofloxacin

C.       

nalidixic acid

D.      

neomycin

E.       

nitrofurantoin

Question  2.           

Which, if any, of the following statements are true in relation to QUIs & FQs? This is not a true SBA as there may be more than one answer.

Statements

A.       

nalidixic acid is an older quinolone and is mainly excreted in the urine

B.       

ciprofloxacin is effective against most Gram +ve and –ve bacteria and 1st- line treatment for pneumococcal pneumonia.

C.       

ciprofloxacin is contraindicated in pregnancy due to the ↑ risk of neonatal haemolysis

D.      

many staphylococci are resistant to quinolones

E.       

quinolones are particularly useful in the treatment of MRSA

Question  3.           

Which was the first QUI antibiotic?

Option List

A

acetylsalicylic acid

B

nalidixic acid

C

oxalic acid

D

pipemidic acid

E

none of the above

Question  4.           

How do QUI and FQ antibiotics work? There is only one correct answer.

Option List

A

impair bacterial DNA coiling

B

impair bacterial DNA binding

C

impair bacterial RNA action

D

impair bacterial mitochondrial action

E

none of the above.

Question  5.           

Which, if any, of the following QUIs & FQs is not available for prescription in the UK.

Option List

A

ciprofloxacin

B

levofloxacin

C

nalidixic acid

D

moxifloxacin

E

ofloxacin

Question  6.           

Which, if any, of the following statements are true in relation to the quinolones and fluoroquinolones and pregnancy?

Option list.

A.       

FQs are newer than QUIs with better systemic spread and efficacy

B.       

QUIs concentrate in urine but have a special affinity for cartilage

C.       

consumption of a FQ in the 1st. trimester is grounds for TOP

D.      

if an FQ is used, norfloxacin and ciprofloxacin should be considered 1st.

E.       

FQs are linked to a risk of discolouration of the teeth of offspring

Question  7.           

Which of the following is true about the warning issued by the FDA in 2008 in relation to QUIs?

Option List

A

they may cause congenital cartilage defects

B

they may cause congenital deafness

C

they may cause tendonitis and tendon rupture

D

they may cause prolongation of the Q-T interval

E

none of the above

Question  8.           

Which of the following is true about the warning issued by the FDA in 2011 in relation to QUIs?

Option List

A

they may cause exacerbation of eczema

B

they may cause exacerbation of hypertension

C

they may cause exacerbation of multiple sclerosis

D

they may cause exacerbation of myasthenia gravis

E

they may cause exacerbation of SLE

Question  9.           

Which of the following is true about the warning emphasised by the FDA in 2013 in relation to QUIs?

Option List

A

they may cause aortic dissection

B

they may cause mitral stenosis

C

they may cause pancreatitis

D

they may cause peripheral neuropathy

E

they may cause flare of SLE

Question  10.        

FDA issued a warning in July 2016. Which, if any, of the following were included?

Option List

A

the risks generally outweigh the benefits

B

QUIs & FQs should not be used for acute sinusitis,

C

QUIs & FQs should not be used for exacerbation of chronic bronchitis

D

QUIs & FQs should not be used for uncomplicated UTI

E

QUIs & FQs may be useful for anthrax and plague

Question  11.        

FDA issued a warning in July 2018 about the use of FQs in pregnancy. Which, if any, of the following were included in the reasons for its publication?

Option List

A

to strengthen previous warnings about hyperglycaemia and mental health risks

B

to strengthen previous warnings about hypoglycaemia and mental health risks

C

to strengthen previous warnings about the risk of ASD in the offspring

D

to strengthen previous warnings about the risk of acute pancreatitis

E

to strengthen previous warnings about the risk of PET

Question  12.        

The FDA issued a warning in December 2018 about the use of FQs in pregnancy. Which, if any, of the following was included? This is an SBA with only one correct answer.

Option List

A

↑ risk of atrial fibrillation

B

↑ risk of aortic aneurysm and rupture

C

↑ risk of mitral stenosis

D

↑ risk of pulmonary hypertension

E

↑ risk of ulcerative colitis

 

19.   EMQ. Parvovirus.

Parvovirus and pregnancy. EMQ. Question.

Abbreviations.

PvB19:          parvovirus B19

PvIgG:           parvovirus B19 IgG

PvIgM:          parvovirus B19 IgM

Option list.

There are no option lists apart from the last few questions. Make up your own answers! In the exam it is best if you decide the answer without reference to the option list and then identify it on the list.

Scenario 1.              

What type of virus is parvovirus?

Scenario 2.              

Is the title B19 something to do with the American B19 bomber, its potentially devastating bomb load and the comparably devastating consequences of the parvovirus on human erythroid cell precursors?

Scenario 3.              

PVB19 in the UK occurs in mini-epidemics at 3 to 4-year intervals, usually during the summer.

Scenario 4.              

Which animal acts as the main reservoir for infection?

Scenario 5.              

What is the approximate incidence of maternal parvovirus infection in the UK?

Scenario 6.              

What percentage of UK adults are immune to parvovirus infection?

Scenario 7.              

What names are given to acute infection in the human?

Scenario 8.              

What is the incubation period for parvovirus infection?

Answer: 14-21 days according to GOVRIP.

Scenario 9.              

What is the duration of infectivity for parvovirus infection?

Scenario 10.           

What are the usual symptoms of parvovirus infection in the adult?

Scenario 11.           

What is the incidence of parvovirus infection in pregnancy?

Scenario 12.           

How is recent infection diagnosed?

Scenario 13.           

How long does PvIgM persist and why is this important?

Scenario 14.           

What is the rate of vertical transmission of parvovirus infection?

Scenario 15.           

Are women with parvovirus infection who are asymptomatic less likely to pass the virus to their fetuses?

Scenario 16.           

To what degree is parvovirus infection teratogenic?

Scenario 17.           

What proportion of pregnancies infected with parvovirus are lost?

Scenario 18.           

What is the timescale for the onset of hydrops?

Scenario 19.           

Laboratories are advised to retain bloods obtained at booking for at least 2 years for possible future reference. True or false?

Scenario 20.           

What ultrasound features would trigger consideration of cordocentesis?

Scenario 21.           

Must suspected parvovirus infection be notified to the authorities?

Scenario 22.           

Possible parvovirus infection does not need to be investigated after 20 week’s gestation. True or false?

Scenario 23.           

If serum is sent to the laboratory from a woman with a rash in pregnancy for screening for rubella, the laboratory should automatically test for parvovirus infection too?

Scenario 24.           

A woman attends the pre-pregnancy counselling clinic as she is planning her first pregnancy.

She wants to know what screening for parvovirus is recommended.

Scenario 25.           

A pregnant woman has had a significant contact with a child with PARV infection. She has had urgent tests for PvIgG and PvIgM. Both results were -ve. Which of the options best fits the advice she should be given?

Option list.

1.       

the tests show acute parvovirus infection

2.       

the tests show chronic parvovirus infection

3.       

the tests show that she has not had PARV infection and is susceptible to it

4.       

the tests show no evidence of PARV infection but she should have repeat tests in 1 month

5.       

the tests show old PARV infection and immunity

6.       

the tests show recent PARV infection

7.       

none of the above

Scenario 26.           

A pregnant woman has had a significant contact with a child with PARV infection. She has had urgent tests for PvIgG and PvIgM. Both results were +ve. Which of the options best fits the advice she should be given?

Option list.

1.       

the tests show acute parvovirus infection

2.       

the tests show chronic parvovirus infection

3.       

the tests show that she has not had PARV infection and is susceptible to it

4.       

the tests show no evidence of PARV infection but she should have repeat tests in 1 month

5.       

the tests show old PARV infection and immunity

6.       

the tests show recent PARV infection

7.       

none of the above

Scenario 27.           

A pregnant woman has had a significant contact with a child with PARV infection. She has had urgent tests for PvIgG and PvIgM. The results were PvIgG +ve and PvIgM -ve. Which of the options best fits the advice she should be given?

Option list.

1.       

the tests show acute parvovirus infection

2.       

the tests show chronic parvovirus infection

3.       

the tests show that she has not had PARV infection and is susceptible to it

4.       

the tests show no evidence of PARV infection but she should have repeat tests in 1 month

5.       

the tests show old PARV infection and immunity

6.       

the tests show recent PARV infection

7.       

none of the above

Scenario 28.           

A pregnant woman has had a significant contact with a child with PARV infection. She has had urgent tests for PvIgG and PvIgM. The results were PvIgG -ve and PvIgM +ve. Which of the options best fits the advice she should be given?

Option list.

1.       

the tests show acute parvovirus infection

2.       

the tests show chronic parvovirus infection

3.       

the tests show that she has not had PARV infection and is susceptible to it

4.       

the tests show no evidence of PARV infection but she should have repeat tests in 1 month

5.       

the tests show old PARV infection and immunity

6.       

the tests show recent PARV infection

7.       

none of the above

Scenario 29.           

A pregnant woman has had a significant contact with a child with PARV infection. What prophylaxis should be offered?

Option list.

1.       

acyclovir orally

1.       

acyclovir i.m.

2.       

acyclovir i.v.

3.       

hand-washing and avoiding small children

4.       

i.v. hyperimmune globulin

5.       

PVV vaccine

6.       

there is no proven prophylaxis

 

20.         EMQ. Galactosaemia.

Galactosaemia.

Some of the questions have no option list. The technique for the exam is to decide your answer before you read the option list. The absence of an option list forces this behaviour!

Abbreviations.

GA:             galactose

GAA:           galactosaemia

Scenario 1.              

What is galactosemia? There is no option list.

Scenario 2.              

What is the mode of inheritance? There is no option list.

Scenario 3.              

Which of the following is the most common cause of galactosemia in Caucasians?

Option list.

A

mutation of the GALE gene

B

mutation of the GALF gene

C

mutation of the GALK gene

D

mutation of the GALk1 gene

E

mutation of the GALT gene

Scenario 4.              

What is the mutation which causes Classical Galactosaemia?

Option list.

A

Q188L

B

Q188M

C

Q188R

D

R188L

E

R188M

F

R188R

G

None of the above

Scenario 5.              

What is the Duarte mutation? There is no option list.

Scenario 6.              

What are the main sources of galactose? There is no option list.

Scenario 7.              

What is the approximate prevalence of galactosemia? There is no option list.

Scenario 8.              

Which of the following groups has the highest prevalence of galactosaemia?

Option list.

A

Armenians

B

Ashkenazi Jews

C

French absinthe drinkers

D

Irish campers

E

Irish travellers

F

Masai

G

Scottish campers

H

None of the above

Scenario 9.              

Which is the most common mutation in the group with the highest incidence of galactosemia? There is no option list.

Scenario 10.           

Which, if any, of the following are linked to untreated GAA in the newborn?

Option list.

A

­ risk of coagulation problems

B

­ risk of congenital hypothyroidism

C

­ risk of diabetes

D

­ risk of diarrhoea

E

­ risk of failure to thrive

F

­ risk of liver failure

G

­ risk of renal failure

H

­ risk of staphylococcal infection

Scenario 11.           

What are the main problems associated with non-treatment of galactosaemia in adults? There is no option list.

Scenario 12.           

Which, if any, of the following statements are true in relation to the effects of a galactose-reduced diet (GRD) on long-term complications (LTCs)?

Option list.

A

a GRD has a major protective effect on LTCs, but only if started within 2 weeks of birth

B

a GRD has a major protective effect on LTCs, but only if started within 12 weeks of birth

C

a GRD has a major protective effect on LTCs, but only if followed meticulously

D

a GRD has a major protective effect on LTCs, but only if started within 2 weeks of birth and continued for life

E

a GRD has a major protective effect on LTCs, but only if started within 2 weeks of birth and continued for life

F

none of the above

Scenario 13.           

Is screening for galactosaemia included in the UK neonatal screening programme? If not, why not?