|
29 |
Talk. Neonatal jaundice |
|
30 |
EMQ. Kell antibodies |
|
31 |
MCQ. Coomb’s test |
|
32 |
EMQ. WOMAN trial |
|
33 |
EMQ. Semmelweis, Gordon and Holmes |
|
34 |
EMQ. Jacob’s syndrome |
29. Talk. Neonatal jaundice.
This comes
up occasionally in the exam and I’ll show some slides covering the key issues.
30. 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.
NIPT: non-invasive prenatal testing.
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?
Option list. Use the
option list from Scenario 1.
Scenario 3.
What is the 3rd.
most common cause of significant HDFN?
Option list. Use the
option list from Scenario 1.
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
31. The
Coomb’s Test: This is an MCQ.
Answer
a. the direct test detects maternal IgM
on fetal cells. True / False
b. is used in the investigation of
thrombocytopenia. True / False
c. is positive in the baby with jaundice
due to spherocytosis. True / False
d. who wrote: “A flea hath smaller fleas
that on him prey…..”
e. what was the rest of the verse?
f. what connection has this verse with
the Coomb’s test?
g. the indirect Coomb’s test is used to
detect antibodies in maternal serum. True
/ False
h. the direct test uses anti IgG serum. True / False
32. EMQ. WOMAN trial.
WOMAN Trial. EMQ.
Answer.
Question
1. What does the acronym “WOMAN” mean? There is no option list.
Question
2. Which condition and drug were the subjects of the trial?.
Question
3. What were the main outcomes of the trial?
Question
4. Which, if any, of the following were
in the WHO’s response to the outcomes?
Option list.
|
D |
the drug to be stored at room temperature |
|
A |
the drug to be used for all pregnant women |
|
B |
the drug to be used prophylactically |
|
C |
the drug to be used orally |
|
F |
the drug to be used within 6 hours |
|
E |
drug manufacturers to be asked to reduce the cost to facilitate
use in developing countries |
Question
5. Which, if any, of the following are true about the WOMAN-2
trial?
Option list.
|
D |
the trial does not exist |
|
A |
the drug to be used for all pregnant women |
|
B |
the drug to be used prophylactically |
|
C |
the drug to be used intravenously |
|
F |
the drug to be used within 6 hours |
|
E |
hysterectomy will be included in the outcomes |
33. EMQ.
Semmelweis, Gordon and Holmes.
Gordon, Holmes & Semmelweis.
Scenario
1.
Which, if any, of
the following statements are true in relation to Semmelweis?
Option list.
|
A |
his full name was
Ignác Fülöp Semmelweis, but he was known to friends as "Naci". |
|
B |
he lived from
1818 to 1865 |
|
C |
he revolutionised
understanding of ‘childbed fever’ |
|
D |
he revolutionised
understanding of rheumatic fever |
|
E |
he
revolutionised understanding of tuberculosis |
|
F |
he pioneered
proton beam therapy |
|
G |
his professional
‘Damascene moment’ came after the death of his colleague, Kolletschka, at the
hands of a medical student in 1847 |
|
H |
his work was
vilified by the majority of his professional contemporaries |
|
I |
he died in a
lunatic asylum |
|
J |
he died in a
road traffic accident |
|
K |
he died at home
in bed with his mistress |
Scenario
2.
Which, if any, of
the following statements are true in relation to Gordon?
Option list.
|
A |
his full name was
Hamish Gordon, but he was known to friends as "Hamy". |
|
B |
he lived from
1801 to 1864 |
|
C |
he revolutionised
understanding of ‘childbed fever’ |
|
D |
he revolutionised
understanding of rheumatic fever |
|
E |
he
revolutionised understanding of tuberculosis |
|
F |
he pioneered
proton beam therapy |
|
G |
his professional
‘Damascene moment’ came after epidemics of erysipelas and puerperal fever in
Aberdeen in the late 18th. century |
|
H |
his work was
vilified by the majority of his professional contemporaries |
|
I |
he died in a
lunatic asylum |
|
J |
he died in a
road traffic accident |
|
K |
he died at home
in bed with his mistress |
Scenario 3.
Which, if any, of
the following statements are true in relation to Wendell Holmes?
Option list.
|
A |
his full name was Wendell Holmes, but he was known to
friends as "Wellie". |
|
B |
he lived from 1801 to 1864 |
|
C |
he revolutionised understanding of ‘childbed fever’ |
|
D |
he revolutionised understanding of rheumatic fever |
|
E |
he revolutionised understanding of tuberculosis |
|
F |
he pioneered proton beam therapy |
|
G |
he was a fan of the work of Gordon. |
|
H |
his work on childbed fever was vilified by the majority
of his professional contemporaries |
|
I |
he died in a lunatic asylum |
|
J |
he died in a road traffic accident |
|
K |
he died at home in bed with his mistress |
34. EMQ. 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?
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 |
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