Monday, 8 October 2018

Tutorial 8th. October 2018


30
Role-play. Mechanisms of normal labour & delivery. Explain these to the role-player, who is a medical student and keen to learn how to do a normal delivery. Your consultant has said that she needs a clear understanding of the mechanisms before considering conducting a delivery.
31
Viva. Laboratory results
32
EMQ. G6PDD & G6PD
33
EMQ. Hepatitis B
34
EMQ. Mayer-Rokitansky-KΓΌster-Hauser syndrome

30. Role-play. Normal labour & delivery.
Candidate’s instructions.
You are the SpR on call for the delivery unit. It is unusually quiet. The on-call consultant has asked you to explain normal labour and delivery to a medical student who started with the department yesterday.

31. Viva. Laboratory results.
Candidate’s instructions.
Your consultant is on annual leave. Her secretary has asked you to look through the following results and decide what action should be taken in relation to each.
1.     +ve MSSU at booking. No symptoms.
2.     GTT at 34 weeks. Peak level 11.5.
3.     FBC with ­ MCV at booking.
4.     Thrombocytopenia at booking. 50,000.
5.     Hydatidiform mole after evacuation of suspected miscarriage.
6.     Histology after ERPC for incomplete miscarriage: no trophoblastic tissue.
7.     Endometrial cancer: hysteroscopy: thickened endometrium. Histology: Anaplastic malignancy.
8.     Endometrial cancer: MR scan: reaching serosa and upper endocervical canal.
9.     Consultant does lap drainage of normal looking ovarian cyst. Malignant cells. Nulliparous. Wants children.
10.   HVS: trichomonas.
11.   Clue cells on smear. 12/52 pregnant.
12.   Antenatal discharge: endocervical swab: chlamydia
13.   Actinomyces on smear.
14.   Herpes in pregnancy
15.   Severe dyskaryosis on cervical smear at booking.
16.   Primary infertility: FSH & LH ­ at 25 on day 3 of cycle.
17.   Primary infertility. FSH 3, LH 12 on day 3 of cycle.
18.   Treated with cabergoline for ­ prolactin and pituitary adenoma. +ve beta HCG.
19.   3 cm. ovarian cyst. ­ Ca 125.

32. EMQ. 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
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? There is no option list.
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

33. EMQ. Hepatitis B and pregnancy.
Abbreviations.
CNP:           Handbook of Obstetric Medicine. 5th. Edition. Catherine Nelson-Piercy. CRC Press. 2015. 
HAV:           hepatitis A virus
HBcAg:       hepatitis B core antigen
HBeAg:       hepatitis B e antigen
HBsAg:       hepatitis B surface antigen
HBcAb:       antibody to hepatitis B core antigen
HBeAb:      antibody to hepatitis B e antigen
HBsAb:       antibody to hepatitis B surface antigen
HBIG:         hepatitis B immunoglobulin
HBV:           hepatitis B virus
HBcAg:       hepatitis B core antigen
HBeAg:       hepatitis B e antigen
HBsAg:       hepatitis B surface antigen
HBcAb:       antibody to hepatitis B core antigen
HBeAb:      antibody to hepatitis B e antigen
HBsAb:       antibody to hepatitis B surface antigen
HBIG:         hepatitis B immunoglobulin
HCV:           hepatitis C virus
HEV:           hepatitis E virus
HSV:           herpes simplex virus
VT:              vertical transmission
Option list.
A.       
acyclovir 
B.       
divorce
C.        
HBcAg +ve
D.       
HBeAg +ve
E.        
HbsAg +ve
F.        
HBsAg +ve; HBsAb –ve;  HBcAb –ve; HBeAg +ve
G.       
HBsAg +ve; HBsAb –ve on two tests six months apart
H.       
HBsAg -ve; HBsAb -ve on two tests six months apart
I.          
HBsAg -ve; HBsAb +ve; HBcAb –ve
J.         
HBsAg -ve; HBsAb +ve; HBcAb +ve
K.        
HBsAg -ve; HBsAb +ve
L.        
HBsAg +ve; HBcAg +ve
M.     
HBV vaccine
N.       
HBIG
O.       
HBV vaccine + HBIG
P.        
immune as a result of infection
Q.       
immune as a result of vaccination
R.       
not immune
S.        
chronic carrier of HBV infection
T.        
10%
U.       
30%
V.       
50%
W.     
60%
X.        
70-90%
Y.        
soap and boiling water
Z.        
10% dilution of bleach in water
AA.   
10% dilution of formaldehyde in alcohol
BB.   
ultraviolet irradiation
CC.    
yes
DD.  
no
EE.    
HAV
FF.     
HBV
GG.  
HCV
HH.  
HEV
II.        
HSV
JJ.       
none of the above
Scenario 1.
An asymptomatic primigravida books at 10 weeks. Her partner had an acute HBV infection 4 months ago. What results on routine blood testing would indicate that she has an acute HBV infection?
Scenario 2.
An asymptomatic primigravida books at 10 weeks. Her partner had an acute HBV infection 4 months ago. What results on routine blood testing would indicate that she is immune to the HBV as a result of infection?
Scenario 3.
An asymptomatic primigravida books at 10 weeks. Her partner had an acute HBV infection 4 months ago. What results on routine blood testing would indicate that she is immune to the HBV as a result of HBV vaccine?
Scenario 4.
An asymptomatic primigravida books at 10 weeks. Her partner had an acute HBV infection 9 months ago. What results on routine blood testing would show that she is a chronic carrier of HBV infection?
Scenario 5.
Testing shows that he is positive for HBsAg, positive for HBcAb but negative for IgM HBcAb. What does this mean in relation to his HBV status?
Scenario 6.
Testing shows that he is negative for HBsAg, positive for HBcAb and positive for HBsAb.
What does this mean in relation to his HBV status?
Scenario 7.
How common is chronic HBV carrier status in UK pregnant women?
Scenario 8.
What is the risk of death from chronic HBV carrier status?
Scenario 9.
A primigravid woman at 8 weeks gestation is found to be non-immune to HBV. She has recently married and her husband is a chronic carrier. What should be done to protect her from infection?
Scenario 10.
A woman is a known carrier of HBV. What is the risk of vertical transmission in the first trimester?
Scenario 11.
What is the risk of the neonate who has been infected by vertical transmission becoming a carrier without treatment?
Scenario 12.
Should antiviral maternal therapy in the 3rd. trimester be considered for women with HBeAg or high viral load?
Scenario 13.
How effective is hepatitis B prophylaxis for the neonate in preventing chronic carrier status as a result of vertical transmission?
Scenario 14.
Can a woman who is a chronic HBV carrier breastfeed safely?
Scenario 15.
Hepatitis B infection is the most dangerous of the viral hepatitis infections in pregnancy.
Scenario 16.
A pregnant woman who is not immune to HBV has a partner who is a chronic carrier. Can HBV vaccine be administered safely in pregnancy?
Scenario 17.
A pregnant woman who is not immune has a partner with acute hepatitis due to HBV. He cuts his hand and bleeds onto the kitchen table. How should she clean the surface to ensure that she gets rid of the virus?
Scenario 18.
Is it true that the presence of HBeAg in maternal blood is a particular risk factor for vertical transmission? Not really a scenario, but never mind!
Scenario 19.
Does elective Cs before labour and with the membranes intact reduce the vertical transmission rate?
Scenario 20.
Which hepatitis virus normally produces a mild illness, but represents a major risk to pregnant women, with a mortality rate of up to 5%?
Scenario 21.
A pregnant woman has a history of viral hepatitis and informs the midwife at booking that she is a carrier and that she has a significant risk of cirrhosis and has been advised not to drink alcohol. Which is the most likely hepatitis virus?
Scenario 22.
Which hepatitis virus is an absolute contraindication to breastfeeding after appropriate treatment of the infected mother and prophylaxis for the baby?
Scenario 23.
Which hepatitis virus is linked to an increased risk of obstetric cholestasis?

34. EMQ. Mayer-Rokitansky-KΓΌster-Hauser syndrome (MRKH).
Question 1.              
Lead-in.
What are the main features of MRKH? There is no option list to make life harder.
Question 2.              
Lead-in.
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.              
Lead-in.
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.              
Lead-in.
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.              
Lead-in.
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.              
Lead-in.
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.              
Lead-in.
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.              
Lead-in.
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.              
Lead-in.
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.          
Lead-in.
What is 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.          
Lead-in.
What is 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.          
Lead-in.
What is 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 is 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. Mark the answers true or false.
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.
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.
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.

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