Monday, 25 November 2019

Tutorial 25th. November 2019


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13
EMQ. Uterine transplant.
SBA.   Lynch syndrome.
15
EMQ. Maternal Mortality definitions.
16
SBA. Pertussis and pregnancy.
17
EMQ. Phenylketonuria.

13.   EMQ. Uterine transplant.
Uterine transplant.
Abbreviations.
ET:         embryo transfer.
UT:        uterine transplant
Scenario 1.                
When was the 1st. human uterine transplant performed?
Option list.
A
2000
B
2005
C
2010
D
2011
E
2012
F
2013
G
2014
H
2015
I
2016
J
2017
Scenario 2.                
When was the 1st. livebirth after human uterine transplant?
Option list.
Same as Scenario 1.
Scenario 3.                
How many live births had occurred worldwide after UT up to the end of 2018?
Option list
A
< 5
B
5 - 10
C
11 - 20
D
21 - 50
E
51 - 100
F
> 100
Scenario 4.                
For which of the following conditions is UT a possible treatment?
Option list.
A
Androgen Insensitivity syndrome. AIS.
B
Congenital adrenal hyperplasia. CAH.
C
Kallmann’s syndrome. KS.
D
Mayer-Rokitansky-Küster-Hauser syndrome. MRKH.
E
McCune-Albright syndrome. MCAS.
F
Swyer’s syndrome. SS.
G
Turner’s syndrome. TS.
Scenario 5.                
Which, if any, of the following are commonly used for donor selection?
Option list.
A
absence of adenomyosis
B
absence of fibroids
C
age < 65 years
D
good general health
E
negative cervical smear and no high-risk HPV
F
no cancer in past 5 years
G
parous
H
vaginal length > 7 cm.
Scenario 6.                
Has successful transplant occurred using a dead donor?
Option list.
A
No
B
Yes
Scenario 7.                
What is the rate of graft survival at 1 year, failure being the need for hysterectomy?
Option list.
A
< 10%
B
11 – 20%
C
21 – 30%
D
31 – 40%
E
41 – 50%
F
51 – 60%
G
> 60%
H
the figure is unknown
Scenario 8.                
Which of the following statements is correct?
Option list.
A
donor surgery is more extensive than recipient surgery
B
donor surgery is less extensive than recipient surgery
C
donor surgery is as extensive as recipient surgery
Scenario 9.                
What are the main risks for the recipient?
There is no option list to make you think. Write down the main things you can think of.
Scenario 10.            
What are the risks to the donor in addition to the usual ones of bleeding, infection, haematoma and thrombosis? There is no option list.
Scenario 11.            
Which condition has been the reason for recipients needing uterine transplant and which complication is more likely in addition to the usual ones of bleeding, infection, haematoma and thrombosis? There is no option list.
Scenario 12.            
When is IVF and cryopreservation of eggs done?
Option list.
A
before uterine transplantation
B
at the time of uterine transplantation
C
12 months after uterine transplantation to ensure graft rejection does not occur
D
when the recipient chooses
E
none of the above
Scenario 13.            
Which maintenance therapy was used immediately before embryo transfer in the first case resulting in livebirth?
Option list.
A
azathioprine + corticosteroids + tacrolimus
B
azathioprine + ciclosporin + corticosteroids + mycophenolate mofetil
C
azathioprine + corticosteroids + mycophenolate mofetil + tacrolimus
D
azathioprine + corticosteroids + tacrolimus
E
ciclosporin + corticosteroids + mycophenolate mofetil + tacrolimus
F
ciclosporin + mycophenolate mofetil + tacrolimus
G
corticosteroids + mycophenolate mofetil + tacrolimus
H
corticosteroids + tacrolimus

14.   SBA.   Lynch syndrome.
Lynch syndrome.
Abbreviations
CRC:              colorectal cancer.
EC:                 endometrial cancer.
HNPCC:         hereditary non-polyposis colo-rectal cancer.
IBD:               inflammatory bowel disease: Crohn’s & ulcerative colitis.
IDDM:           insulin-dependent diabetes mellitus.
Ls:                  Lynch syndrome.
Question 1.              
Lead-in
What is Lynch syndrome?
Option List
A
auto-immune condition leading to reduced factor X levels in blood
B
hereditary condition which increases the risk of many cancers, particularly breast
C
hereditary condition which increases the risk of many cancers, particularly breast & colorectal
D
hereditary condition which increases the risk of many cancers, particularly colorectal & endometrial
E
none of the above
Question 2.              
Lead-in
How is Lynch syndrome inherited?
Option List
A
it is an autosomal dominant condition
B
it is an autosomal recessive condition
C
it is an X-linked dominant condition
D
it is an X-linked recessive condition
E
none of the above
Question 3.              
Lead-in
Which, if any, of the following genes can cause Lynch syndrome?
Option List
A
MLH1 + MLH2 + MOH1
B
MLH1 + MLH2 + MSH1
C
MLH1 + MLH2 + MSH6
D
MLH1 + MSH2 + MSH6
E
None of the above
Question 4.              
Lead-in
Mutations of which 2 of the following genes cause the majority of cases of Lynch syndrome?
Option List
A
MLH1 + MLH2
B
MLH1 + MSH1
C
MLH1 + MSH2
D
MLH2 + MSH1
E
MLH2 + MSH2
Question 5.              
Lead-in
What is the approximate prevalence of Ls in the UK population?
Option List
A.       
1 in 50
B.       
1 in 100
C.        
1 in 1,000
D.       
3 in 1,000
E.        
none of the above
Question 6.              
Lead-in
Approximately what % of individuals with Ls have had the diagnosis established?
Option List
A.       
< 5%
B.       
5 -10%
C.        
10-20%
D.       
20-30%
E.        
>30%
Question 7.              
Lead-in
Which, if any, of the following conditions are associated with an risk of Lynch syndrome?
Option List
A
acromegaly + Addison’s disease + coeliac disease + IBD + IDDM
B
acromegaly + disease + anosmia + coeliac disease + IBD
C
acromegaly + IBD + IDDM
D
acromegaly + IBD
E
Addison’s disease + anosmia + coeliac disease + IBD + IDDM
F
acromegaly + Addison’s disease + anosmia + coeliac disease + IBD + IDDM
G
none
Question 8.              
Lead-in
Which 2 cancers are most likely in women with Lynch syndrome?
Option List
A
breast + bowel
B
breast + pancreas
C
breast + endometrium
D
bowel + cervix
E
bowel + endometrium
F
bowel + ovary
G
bowel + pancreas
H
endometrium + ovary
Question 9.              
Lead-in
What does NICE recommend about screening for Lynch syndrome for the population with no personal history of colorectal cancer?
Option List
A
offer screening to those aged < 50 years with  ≥ 1 affected 1st.O relative
B
offer screening to those aged < 60 years with ≥ 1 affected 1st.O relative
C
offer screening to those with ≥ 1 affected 1st.O relative aged < 50 years at diagnosis
D
offer screening to those with ≥ 1 affected 1st.O relative aged < 60 years at diagnosis
E
none of the above
Question 10.          
Lead-in
What does NICE recommend in relation to screening for Lynch syndrome in those with a new diagnosis of colorectal cancer?
Option List
A
offer screening to everyone, regardless of age and family history
B
offer screening to those aged < 50 years at diagnosis
C
offer screening to those aged < 60 years at diagnosis
D
offer screening to those aged < 50 years at diagnosis with + ≥ 1 affected 1st.O relative
E
offer screening to those aged < 60 years at diagnosis with + ≥ 1 affected 1st.O relative
Question 11.          
Lead-in
What does NICE recommend about screening for Lynch syndrome for the population with no personal history of thyroid cancer?
Option List
A
offer screening to those aged < 50 years with  ≥ 1 affected 1st.O relative
B
offer screening to those aged < 60 years with ≥ 1 affected 1st.O relative
C
offer screening to those with ≥ 1 affected 1st.O relative aged < 50 years at diagnosis
D
offer screening to those with ≥ 1 affected 1st.O relative aged < 60 years at diagnosis
E
none of the above
Question 12.          
Lead-in
What does NICE recommend in relation to screening for Lynch syndrome in those with a new diagnosis of thyroid cancer?
Option List
A
offer screening to everyone, regardless of age and family history
B
offer screening to those aged < 50 years at diagnosis
C
offer screening to those aged < 60 years at diagnosis
D
offer screening to those aged < 50 years at diagnosis with + ≥ 1 affected 1st.O relative
E
none of the above
Question 13.          
Lead-in
What does NICE recommend about screening for Lynch syndrome for the population with no personal history of endometrial cancer?
Option List
A
offer screening to those aged < 50 years with  ≥ 1 affected 1st.O relative
B
offer screening to those aged < 60 years with ≥ 1 affected 1st.O relative
C
offer screening to those with ≥ 1 affected 1st.O relative aged < 50 years at diagnosis
D
offer screening to those with ≥ 1 affected 1st.O relative aged < 60 years at diagnosis
E
none of the above
Question 14.          
Lead-in
What does NICE recommend in relation to screening for Lynch syndrome in those with a new diagnosis of endometrial cancer?
Option List
A
offer screening to those aged < 50 years with  ≥ 1 affected 1st.O relative
B
offer screening to those aged < 60 years with ≥ 1 affected 1st.O relative
C
offer screening to those with ≥ 1 affected 1st.O relative aged < 50 years at diagnosis
D
offer screening to those with ≥ 1 affected 1st.O relative aged < 60 years at diagnosis
E
none of the above
Question 15.          
Lead-in
What does NICE recommend about screening for Lynch syndrome for the population with no personal history of colorectal cancer?
Option List
A
offer screening to those aged < 50 years with  ≥ 1 affected 1st.O relative
B
offer screening to those aged < 60 years with ≥ 1 affected 1st.O relative
C
offer screening to those with ≥ 1 affected 1st.O relative aged < 50 years at diagnosis
D
offer screening to those with ≥ 1 affected 1st.O relative aged < 60 years at diagnosis
E
none of the above
Question 16.          
Lead-in
What does NICE recommend in relation to screening for Lynch syndrome in those with a new diagnosis of colorectal cancer?
Option List
A
offer screening to everyone, regardless of age and family history
B
offer screening to those aged < 50 years at diagnosis
C
offer screening to those aged < 60 years at diagnosis
D
offer screening to those aged < 50 years at diagnosis with + ≥ 1 affected 1st.O relative
E
offer screening to those aged < 60 years at diagnosis with + ≥ 1 affected 1st.O relative
Question 17.          
Lead-in
What relationship, if any, exists between Ls and acromegaly?
Option List
A
the risk of Ls is in those with acromegaly compared with the general population
B
the risk of Ls is in those with acromegaly compared with the general population
C
the risk of Ls is unchanged in those with acromegaly compared with the general population
D
the risk of Ls in unknown in those with acromegaly
Question 18.          
Lead-in
What is the effect of aspirin consumption on the risk of EC and CRC?
Option List
A
aspirin reduces the risk of EC and  CRC
B
aspirin reduces the risk of EC but not CRC
C
aspirin reduces the risk of CRC but not EC
D
aspirin does not reduce the risk of EC or CRC
E
aspirin reduces the risk of EC and CRC, but the risks outweigh the benefits
Question 19.          
Lead-in
A healthy woman of 35 years is diagnosed with Ls? What are the key elements of the National Screening Programme for people with Ls?
There is no option list – just write down everything you know.
Question 20.       
Lead-in
Which, if any, of the following were recommendations made by Monahan et al, the 30 experts who wrote to the BMJ in 2017?
Option List
A
creation of a national register of people with Ls
B
creation of a post of Consultant in Ls for each NHS Trust
C
creation of a post of Clinical Champion for Ls in each NHS Region.
D
creation of a post of Clinical Champion for Ls in the DOH.
E
none of the above

15.   EMQ. Maternal Mortality definitions.
Lead-in.
Pick the option that best answers the question in each scenario.
Each option can be used once, more than once or not at all.
Option List.
A.    Death of a woman during pregnancy and up to 6 weeks later, including accidental and incidental causes.
B.    Death of a woman during pregnancy and up to 6 weeks later, excluding accidental and incidental causes.
C.    Death of a woman during pregnancy and up to 52 weeks later, including accidental and incidental causes.
D.   Death of a woman during pregnancy and up to 52 weeks later, excluding accidental and incidental causes.
E.    A pregnancy going to 24 weeks or beyond.
F.    A pregnancy going to 24 weeks or beyond + any pregnancy resulting in a live-birth.
G.   Maternal deaths per 100,000 maternities.
H.   Maternal deaths per 100,000 live births.
I.      Direct + indirect deaths per 100,000 maternities.
J.     Direct + indirect deaths per 100,000 live births.
K.    Direct death.
L.     Indirect death.
M.  Early death.
N.   Late death.
O.   Extra-late death.
P.    Fortuitous death.
Q.   Coincidental death.
R.    Accidental death.
S.    Maternal murder.
T.    Not a maternal death.
U.   Yes
V.    No.
W. I have no idea.
X.    None of the above.
Abbreviations.
MMR:      Maternal Mortality Rate.
MMRat:  Maternal Mortality Ratio.
SUDEP:    Sudden Unexplained Death in Epilepsy.            
Scenario 1.
What is a Maternal Death?
Scenario 2.
A woman dies from a ruptured ectopic pregnancy at 10 weeks’ gestation. What kind of death is it?
Scenario 3.
A woman dies from a ruptured appendix at 10 weeks’ gestation. What kind of death is it?
Scenario 4.
A woman dies from suicide at 10 weeks’ gestation. What kind of death is it?
Scenario 5.
A woman with a 10-year-history of coronary artery disease dies of a coronary thrombosis at 36 weeks’ gestation. What kind of death is it?
Scenario 6.
A woman has gestational trophoblastic disease, develops choriocarcinomas and dies from it 24 months after the GTD was diagnosed and the uterus evacuated. What kind of death is it?
Scenario 7
A woman develops puerperal psychosis from which she makes a poor recovery. She kills herself when the baby is 18 months old. What kind of death is it?
Scenario 8
A woman develops puerperal psychosis from which she makes a poor recovery. She kills herself when the baby is 6 months old. What kind of death is it?
Scenario 9
What is a “maternity”.
Scenario 10
What is the definition of the Maternal Mortality Rate?
Scenario 11
What is the Maternal Mortality Ratio?
Scenario 12
A woman is diagnosed with breast cancer. She has missed a period and a pregnancy test is +ve. She decides to continue with the pregnancy. The breast cancer does not respond to treatment and she dies from secondary disease at 38 weeks. What kind of death is it?
Scenario 13
A woman who has been the subject of domestic violence is killed at 12 weeks’ gestation by her partner. What kind of death is it?
Scenario 14
A woman is struck by lightning as she runs across a road. As a result she falls under the wheels of a large lorry which runs over abdomen, rupturing her spleen and provoking placental abruption. She dies of haemorrhage, mostly from the abruption. What kind of death is it?
Scenario 15
A woman is abducted by Martians who are keen to study human pregnancy. She dies as a result of the treatment she receives. As this death could only have occurred because she was pregnant, is it a direct death?
Scenario 16
Could a maternal death from malignancy be classified as “Direct”.
Scenario 17
Could a maternal death from malignancy be classified as “Indirect”.
Scenario 18
Could a maternal death from malignancy be classified as “Coincidental”?
Scenario 19.
A pregnant woman is walking on the beach at 10 weeks when she is struck by lightning and dies. What kind of death is this?
Scenario 20.
A woman is sitting on the beach breastfeeding her 2-month old baby when she is struck by lightning and dies. What kind of death is this.

16.   SBA. Pertussis and pregnancy.
Question  1.       
Lead-in. Why is pertussis of current concern in obstetrics?
Option List
A
Research has linked pertussis in the 1st. trimester with an risk of congenital heart disease
B
A mini-epidemic since 2011 has caused deaths of mothers & of babies < 3 months
C
A mini-epidemic since 2011 has caused deaths of babies < 3 months
D
The infecting organism has become increasingly drug-resistant
E
The infecting organism has become increasingly virulent
Question  2.       
Lead-in
Which organism causes whooping cough?
Option List
A
Bordella pertussis
B
Bacteroides pertussis
C
Rotavirus whoopoe
D
Respiratory syncytiovirus pertussis
E
None of the above
Question  3.       
Lead-in
Which, if any, of the following statements is true about the organism what causes whooping cough? This is not a true SBA as I have condensed several questions into one to save space, there are more than 5 options and there may be more than one correct answer.
Option List
A
the organism is aerobic
B
the organism is anaerobic
C
the organism is capsulated
D
the organism is flagellate
E
the organism is an obligate intra-cellular parasite
F
the organism is a Gram -ve diplococcus
G
the organism is a Gram +ve diplococcus
H
the organism requires special transport media
I
no one is going to ask me any of this stuff










Lead-in
Which of the following statements is true?
Option List
A
Pertussis is no longer a significant threat to infants
B
Pertussis remains a significant threat to infants
C
The risk of death from pertussis is eliminated by timely antibiotic therapy
D
the risk of death from pertussis is eliminated by timely antiviral therapy
E
None of the above
Question  5.       
Lead-in
Which of the following statements is true?
Option List
A
Pertussis is not a notifiable disease
B
Pertussis is a notifiable disease
C
Pertussis is not a notifiable disease, but cases should be reported to the local bacteriologist
D
Pertussis is not a notifiable disease, but cases should be subject to audit
Question  6.       
Lead-in
What is the main mode of spread of the organism that causes pertussis?
Option List
A
contact with contaminated surfaces
B
contaminated food
C
contaminated water
D
respiratory droplets
E
none of the above
Question  7.            
Lead-in
What is the main reservoir of the organism that causes pertussis?
Option List
A
budgerigars
B
cats
C
dogs
D
humans
E
pigeons
F
pigs
G
none of the above
Question  8.       
Lead-in
What is the epidemiology of pertussis?
Option List
A
the condition is endemic
B
the condition is endemic with mini-epidemics every 3-5 years
C
the condition is endemic with mini-epidemics most years in the winter months
D
the condition is epidemic, with outbreaks at roughly three-year intervals
E
the condition is epidemic, with outbreaks at unpredictable intervals
Question  9.            
Lead-in
What is the incubation period for pertussis?
Option List
A
3-6     days
B
7-10   days
C
11-14 days
D
15-18 days
E
none of the above.
Question  10.         
Lead-in
What is the duration of infectivity of someone with pertussis?
Option List
A
2 days from exposure → 5 days after onset of paroxysms of coughing
B
3 days from exposure → 10 days after onset of paroxysms of coughing
C
4 days from exposure → 14 days after onset of paroxysms of coughing
D
6 days from exposure → 21 days after onset of paroxysms of coughing
E
none of the above
Question  11.         
Lead-in
What % of non-immune, close contacts of pertussis will develop the disease?
Option List
A
50%
B
60%
C
70%
D
80%
E
90%
Question  12.   
Lead-in
What practical issues are current for obstetrician in relation to pertussis?
Option List
A
The DOH advises that all pregnant women be immunised to maternal death rates.
B
The DOH advises that all pregnant women be immunised to deaths in babies < 3 months.
C
The DOH advises that all babies be immunised at birth.
D
The DOH advised that “Boostrix- IPV should replace “Repevax” from July 2014.
E
The DOH advises that immunisation of pregnant women be continued until 2019
Question  13.         
Lead-in
Which, if any, of the following statements is true in relation to average annual number of deaths due to pertussis in the years before routing child immunisation was introduced?
Option List
A
the number was 10,000
B
the number was    5,000
C
the number was    4,000
D
the number was    3,500
E
the number was <1,000
Question  14.   
Lead-in
Which, if any, of the following statements are true in relation to pertussis vaccine.
Option List
A
Boostrix- IPV” is a vaccine for pertussis only
B
“Repevax” is a vaccine for pertussis only
C
Boostrix- IPV” & “Repevax” are live, attenuated vaccines
D
Boostrix- IPV” & “Repevax” act against diphtheria, tetanus and polio as well as pertussis
E
Boostrix- IPV” & “Repevax” are acellular
Question  15.   
Lead-in
Which, if any, of the following statements are true in relation to the JCVI’s advice of the best time to administer pertussis vaccine in pregnancy?
Option List
A
20 - 24 weeks
B
25- 28 weeks
C
28 - 32 weeks
D
28 - 34 weeks
E
none of the above
Question  16.         
Lead-in
A woman has suspected pertussis in early pregnancy. Should she still be offered vaccination?
Option List
A
Yes
B
No
C
I don’t know
D
I don’t know
E
I hate this subject now
Question  17.         
Lead-in
A woman has proven pertussis in early pregnancy. Should she still be offered vaccination?
Option List
A
Yes
B
No
C
I don’t know
D
I don’t know
E
I hate this subject now
Question  18.         
Lead-in
A pregnant woman misses out on vaccination as part of the TIPP. Should vaccination still be offered in the puerperium?
Option List
A
Yes
B
No
C
I don’t know
D
I don’t know
E
I am having a breakdown due to this subject now

17.   EMQ. Phenylketonuria.
Some of these are not true EMQs – some have no option list and you have to decide the correct answer for yourself. This is what you are advised to do in the exam – read the question, decide the answer and then look for it on the option list.
Abbreviations.
BH4:         tetrahydrobiopterin.
BH4D:      tetrahydrobiopterin deficiency.
HPA:        hyperphenylalaninaemia.
PA:           phenylalanine.
PAH:        phenylalanine hydroxylase.
PKU:        phenylketonuria.
Tyr:          tyrosine.
Option list.
A.       
autosomal dominant
B.       
autosomal recessive
C.       
X-linked dominant
D.      
X-linked recessive
E.       
1 in 100,000
F.        
1 in 50,000
G.      
1 in 10,000
H.      
1 in 5,000
I.         
deficiency in phenylalanine hydroxylase
J.         
deficiency in phenylalanine oxidase
K.       
deficiency in phenylalanine transferase
L.        
deficiency in phenylketone hydroxylase
M.    
deficiency in phenylketone oxidase
N.      
raised PA levels
O.      
reduced PA levels
P.       
raised tyrosine levels
Q.      
reduced tyrosine levels
R.       
normal tyrosine levels
S.        
No
T.       
Yes
U.      
unknown
What is PKU? Write your answer – there is no option list.
Question 2.           
What is PKU due to? Use the option list.
Question 3.           
What levels of PA and Tyr are typical in PKU? Use the option list. This is not a real EMQ as there are two answers.
Question 4.           
Is PKU subdivided into different categories? If “yes”, what are the categories? Write your answer – there is no option list.
Question 5.           
How is PKU inherited? Use the option list.
Question 6.           
Which chromosome houses the gene related to PKU transmission?
Question 7.           
How many mutations of the gene related to PKU have so far been identified?
Question 8.           
Is a person with PKU likely to have one or two mutations of the relevant gene?
Question 9.           
What are the main consequences of PKU? Write your answer – there is no option list.
Question 10.       
Which, if any, of the following are characteristic of PKU?
Option list.
A.       
alopecia
B.       
angst
C.       
facial dysmorphism
D.      
facial hair in females and pre-pubertal males
E.       
kyphosis
F.        
macroorchidism in post-pubertal males
Question 11.       
Are fetal PKU levels higher or lower than maternal? Write your answer – there is no option list.
Question 12.       
What is the approximate prevalence of PKU in Caucasians?
Question 13.       
The prevalence of PKU varies between ethnic groups.
Match each of the following ethnic groups to the closest prevalence given in the option list.
Option List
1.       
1 in 1,000
2.       
1 in 2,500
3.       
1 in 5,000
4.       
1 in 10,000
5.       
1 in 100,000
6.       
1 in 150,000
7.       
1 in 200,000
8.       
1 in 1,000,000

Ethnic group
Prevalence
Turkish

Irish

Caucasian

East Asian

Japanese

Finnish

Question 14.       
Is screening for PKU a routine part of the neonatal screen in the UK?
Question 15.       
The test for PKU used to be known by the name of its inventor. Who was he and why did he have a particular interest? There is no option list and no one is going to ask you except me!
Lead-in
What is the main treatment of PKU and what problems are associated with it? There is no option list.
Question 17.       
Lead-in
How long should the main treatment of PKU be continued and why? There is no option list.
Question 18.       
Lead-in
A woman with PKU is planning her first pregnancy at the age of 22. She has been off the PKU-restricted diet since the age of 10 and can barely remember being on it. Should she be advised to re-start the diet? If ‘yes’, when should she start and what explanation would you give for the advice?
Question 19.       
Lead-in
Is breast-feeding advisable for women with PKU?
Question 20.       
Lead-in
Are any other therapeutic approaches available? If ‘yes’, what are they and how do they work? If ‘yes’ use the option list for the mode of action.

There is a recent TOG article by Munyame et al.
The linked CPD questions are here:
It is not yet free-access, but the first page is displayed on the internet and happens to have the phenylketonuria questions, so you can access them.


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