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Tutorial 3rd. April 2023

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50

EMQ. Kell antibodies 

51

Role-play. Topic revealed at the tutorial.

52

SBA. Fetal origins of adult disease

53

EMQ. G6PDD & G6PD

 50.         EMQ. Kell antibodies.

Kell antibodies & pregnancy.

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 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.         What is the 2nd. most common cause of significant HDFN?

Scenario 3.         What is the 3rd. most common cause of significant HDFN?

Scenario 4.         Which of the following 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.         Can the Kell antigen be detected using cffDNA 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.         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 10.      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 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 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 12.      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 13.      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 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 ∆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 15.      Which, if any, of the following statements are true in relation to the numbers of

reticulocytes in cord blood in moderate to severe HDFN due to anti-K antibodies?

Option list.

A

the numbers are decreased

B

the numbers are increased

C

the numbers are normal

D

none of the above

Scenario 16.      Which, if any, of the following statements are true in relation to the numbers of

erythroblasts in cord blood in moderate to severe HDFN due to anti-K antibodies?

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 level of bilirubin

in cord blood in moderate to severe HDFN due to anti-K antibodies?

Option list.

A

it is decreased

B

it is increased

C

it is greatly increased

D

none of the above

Scenario 18.      Which, if any, of the following 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.

They are open access, which allows me to reproduce them.

Regarding Kell alloimmunisation in pregnancy,

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

2      previous obstetric history does not reliably predict outcome.         True / False

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

4      prophylaxis is available.                                                                                 True / False

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

6      anti-Kell antibodies cause fetal anaemia via the suppression of erythropoiesis rather than red cell destruction.                                                                               True / False

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.                                                                                                         True / False

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.                                                           True / False

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

Transfusion seems to produce less severe disease.

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.                                                         True / False

 

51.         Role-play. Topic revealed at the tutorial.

You do not get forewarning of topics in the exam, so I have done the same here.

 

52.         Fetal origins of adult disease.

Abbreviations.

ADHD:  attention-deficit, hyperactivity disorder

Lead in. These questions relate to disease in adults resulting from events during fetal, infant and child development.

Scenario 1.   What eponymous title is given to the concept that adverse intra-uterine conditions

predispose to the development of disease in adulthood?

Option List                               

A

the Barker hypothesis

B

the Baker’s dozen

C

the Broadbank theory

D

PIPAD: Placental Insufficiency Programmes Adult Disease

E

SIMCARD: Stop In-utero Malnutrition to Conquer Adult-resulting Disease

Scenario 2.   Which other term is used for the concept that adverse intra-uterine conditions

 predispose to the development of disease in adulthood?

Option List

A

FDAD: fetal determination of adult disease

B

FIAD:   fetal influences on adult disease

C

FIDAD: fetal and infancy determinants of adult disease

D

FIGO:   fetal influences on genomic outcomes

E

FP:       fetal programming

Scenario 3.   Which of the following is thought to increase the risk of adult disease?

Option List

A

low birthweight (LBW)

B

LBW followed by poor weight gain in infancy and childhood

C

LBW followed by poor weight gain in infancy but above-average weight gain in childhood

D

above-average birthweight (AABW)

E

AABW followed by poor weight gain in infancy but above-average weight gain in childhood

F

AABW followed by above-average weight gain in infancy and childhood

Scenario 4.   Which adult diseases are generally believed to be more likely in relation to adverse

influences on the fetus, infant and child.

Diseases.

A

asthma

B

chronic bronchitis

C

coronary heart disease

D

diabetes type I

E

diabetes type 2

F

hypertension

G

Mendelson’s syndrome

H

Stroke

Scenario 5.   What adult condition has been linked to raised maternal c-reactive protein levels?

Option List                               

A

asthma

B

ADHD

C

autism

D

inflammatory bowel disease

E

schizophrenia

 

53.         EMQ. G6PDD & G6PD.

Glucose-6-phosphate dehydrogenase deficiency.

Abbreviations.

G6PD:      glucose-6-phosphatase deficiency

G6PDD:   glucose-6-phosphate dehydrogenase deficiency            

Scenario 1.         What is G6PDD? There is no option list.

Scenario 2.         What categories are applied to G6PDD by the WHO? There is no option list.

Scenario 3.         What other names are commonly used for G6PDD? There is no option list.

Scenario 4.         Which, if any, of the following statements are true in relation to G6PDD?

Option list.

A

it is the most common enzyme defect in humans

B

it is the most common RBC enzyme defect in humans

C

it is the most common cause of neonatal jaundice

D

it is the most common cause of sickling crises

E

is a glycogen storage disorder

F

most of those with G6PDD have chronic anaemia

Scenario 5.         Approximately how many people are affected by G6PDD worldwide?

Option list.

A

1,000 million

B

800 million

C

600 million

D

400 million

E

100 million

F

50 million

G

20 million

H

10 million

I

none of the above

Scenario 6.         Which population has the highest prevalence of G6PDD?

Option list.

A

American Amish

B

Asians

C

Ashkenazi Jews

D

Eskimos

E

Irish Travellers

F

Kurdistan Jews

G

Sub-Saharan Africans

H

Turks

I

Uzbekistan albinos

J

None of the above

Scenario 7.         Which, if any, of the following is the mode of inheritance of G6PDD?

Option list.

A

autosomal dominant

B

autosomal recessive

C

mitochondrial pattern

D

X-linked dominant

E

X-linked recessive

F

Y-linked

Scenario 8.         Approximately how many mutations of the G6PDD gene have been identified?

Scenario 9.         Which, if any, of the following is the mode of inheritance of G6PD?

Option list.

A

autosomal dominant

B

autosomal recessive

C

mitochondrial pattern

D

X-linked dominant

E

X-linked recessive

F

Y-linked

Scenario 10.      Which foodstuff can trigger haemolysis in G6PDD and gives us one of the alternative

names for the condition? What is the common name for the foodstuff? Which pest particularly attacks it? There is no option list.

Scenario 11.      Which, if any, of the following drugs may cause haemolysis in those with G6PDD?

Option list.

A

aspirin

B

diphenhydramine

C

nalidixic acid

D

nitrofurantoin

E

paracetamol

F

phenytoin

G

sulphamethoxazole

H

trimethoprim

 

 

 

 

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