Thursday 25 June 2020

Tutorial 25th. June 2020





Role-play. Pre-pregnancy counselling.
40
Structured discussion. WHO criteria for a screening test.
41
EMQ. Diabetes insipidus
42
EMQ. G6PDD & G6PD.
43
EMQ. Peutz-Jeghers syndrome.


39. Role-play. Pre-pregnancy counselling.
Candidate’s instructions.
You are an SpR5. Your consultant has asked you to see this patient as she thinks it will be good for your preparation to become a consultant. She has said that she wishes to discuss the station with you afterwards, but that you are to use your initiative and knowledge to deal with the patient entirely on your own without asking the consultant for advice.
Your task is to see the patient and deal with her in the way that a good consultant would.

40. Structured discussion. WHO criteria for a screening test.
Candidate’s instructions.
This is a structured discussion about the WHO criteria for a screening test.
When you have finished a question, you will not be allowed to return to it as later questions may indicate the answer. If you return, no marks will be awarded, even for correct answers.

41. EMQ. Diabetes insipidus.
You might think this so ‘small print’ that you should ignore it. There was a TOG article in 2018, so it is a hot topic and you need a grasp of the basics.
Abbreviations.
ADH:        antidiuretic hormone.
Scenario 1.              
What is the origin of the word ‘diabetes’?
Option list.
A.
the Greek word for ‘bucket’
B.
the Greek word for ‘halitosis’
C.
the Greek word for ‘siphon’
D.
the Greek word for ‘stream’
E.
the Greek word for ‘waterfall’
Scenario 2.              
What is the origin of the word ‘mellitus’?
Option list.
A.
the Latin word for ‘darling’
B.
the Latin word for ‘honeyed’
C.
the Latin word for ‘honey-pie’
D.
the Latin word for ‘sugar’
E.
the Latin word for ‘sweet’
Scenario 3.              
What is the origin of the word ‘insipidus’?
Option list.
A.
the Latin word for ‘dull’
B.
the Latin word for ‘pale grey’
C.
the Latin word for ‘savoury’
D.
the Latin word for ‘spicy’
E.
the Latin word ‘to have taste’
Scenario 4.              
What is the origin of the word ‘insulin’?
Option list.
A.
the Latin noun ‘insula’, ‘island’ to reflect its production in the islets of Langerhans
B.
the Latin verb ‘insultare’ ‘to insult’ as this is an affront to your intelligence
C.
the Latin verb ‘insultare’, ‘to assault’; reflecting its ability to deal with DM
D.
the Latin verb ‘insulare’, ‘to insulate’; reflecting its ability to protect from DM
E.
none of the above
What is the definition of DI?
Option list.

A.
diabetes mellitus with low or normal blood glucose levels
B.
diabetes mellitus with low serum osmolality
C.
diabetes mellitus with polyuria despite low or normal blood glucose levels
D.
failure of the renal glomeruli to conserve water
E.
failure of the renal tubules to conserve water
F.
polyuria with urine output > 3 litres per 24 hours
G.
polyuria with urine output > 5 litres per 24 hours
H.
polyuria and polydipsia due to low or absent ADH production
J.
polyuria and polydipsia due to excess ADH production
K.
polyuria and polydipsia due to low or absent vasopressinase production
L.
polyuria and polydipsia due to excess ADH vasopressinase production
M.
none of the above.

Scenario 6.              
What is the definition of polyuria?
Option list.
A.
urine volume 2 l. per 24 hours
B.
urine volume 3 l. per 24 hours
C.
urine volume 4 l. per 24 hours
D.
urine volume 5 l. per 24 hours
E.
urine volume 10 l. per 24 hours
F.
urine volume > fluid intake
G.
urine volume > fluid intake minus estimated ‘imperceptible’ fluid loss
H.
none of the above
Scenario 7.              
What is the approximate incidence of DI in pregnancy?
Option list.
A.
2-4 per million
B.
2-4 per 100,000
C.
2-4 per   10,000
D.
2-4 per     1,000
E.
none of the above
Scenario 8.              
Which, if any, of the following are included in the classification of DI?
Option list.
A.
central
B.
dipsogenic
C.
dipsomaniacal
D.
gestational
E.
neurotic
F.
nephrogenic
G.
neurogenic
H.
peripheral
I.
primary
J
psychogenic
K.
renal
Scenario 9.              
Which, if any, of the following are the most common type of DI in the non-pregnant? There may be more than one correct answer as some of the terms are used synonymously.
Option list.
Use the option list from question 8.
Scenario 10.           
Which, if any, of the following are the most common type of DI in pregnancy? There may be more than one correct answer as some of the terms are used synonymously.
Option list.
Use the option list from question 8.
Scenario 11.           
Which, if any, of the following apply to ADH?
Option list.
A.
it is an octapeptide
B.
it is a nonapeptide
C.
it is a decapeptide
D.
it is a glycoprotein
E.
none of the above
Scenario 12.           
Which, if any, of the following are true in relation to the mechanisms controlling release of ADH?
Option list.
A.
the main factor controlling ADH release is plasma albumin
B.
the main factor controlling ADH release is plasma albumin
C.
the main factor controlling ADH release is plasma osmolality
D.
the main factor controlling ADH release is plasma sodium
E.
the main factor controlling ADH release is plasma viscosity
F.
the main factor controlling ADH release is plasma volume
Scenario 13.           
Which, if any, of the following is true about the effect of ADH?
Option list.
A.
it the creation of aquaporins
B.
it the creation of aquapourins
C.
it the creation of aquasporrins
D.
it the creation of aquaporins
E.
it the creation of aquapourins
F.
it the creation of aquasporrins
G.
none of the above
Scenario 14.           
Which, if any, of the following statements are true about the V1 receptors?
Option list.
A.
they are stimulated by ADH
B.
high ADH levels cause vasoconstriction
C.
high ADH levels cause vasodilatation
D.
high ADH levels reabsorption of water
E.
high ADH levels reabsorption of water
Scenario 15.           
Which, if any, of the following statements are true about the V2 receptors?
Option list.
A.
they are stimulated by ADH
B.
high ADH levels cause vasoconstriction
C.
high ADH levels cause vasodilatation
D.
high ADH levels reabsorption of water
E.
high ADH levels reabsorption of water
Scenario 16.           
Which, if any of the following statements is true about ADH?
Option list.
A.
it is produced by the hypothalamus
B.
it is produced by the anterior pituitary
C.
it is produced by the posterior pituitary
D.
shares all but two of its peptides with oxytocin
E.
acts on the V1 renal receptors to reabsorption of water
F.
acts on the V1 renal receptors to vasoconstriction
G.
acts on the V2 renal receptors to reabsorption of water
H.
acts on the V2 renal receptors to reabsorption of water
I.
ADH secretion increases x 4 by the 3rd. trimester
J.
has got me bored out of my head
Scenario 17.           
Which, if any, of the following statements best fits the changes in systemic vascular resistance?
Option list.
A.
it falls from the 1st. to the 3rd. trimester
B.
it falls from the 1st. to the mid-2nd. trimester
C.
it falls from the 2nd. to the 3rd. trimester
D.
it rises from the 1st. to the 3rd. trimester
E.
it rises from the 1st. to the mid-2nd. trimester
F.
it rises from the 2nd. to the 3rd. trimester
G.
none of the above
Scenario 18.           
Which, if any, of the following are correct in relation to vasopressinase?
Option list.
A.
it is mainly produced in the fetal liver
B.
it is mainly produced by trophoblasts
C.
vasopressinase activity is directly proportionate to the liquor volume
D.
vasopressinase activity is directly proportionate to the weight of the fetus
E.
vasopressinase activity is directly proportionate to the weight of the placenta
F.
vasopressinase activity is similar with ADH and DDAVP
Scenario 19.           
Which, if any, of the following investigations may be appropriate in DI in pregnancy?
Option list.
A.
urea & electrolytes
B.
blood glucose
C.
urine glucose
D.
blood calcium
E.
plasma osmolality
F.
urine osmolality
G.
urine specific gravity
H.
MRI scan
Which, if any, of the following statements are true about DDAVP?
Option list.

A.
It is also known vasopressin
B.
it is also known as dismopressin
C
is resistant to vasopressinase
D.
it is believed to be safe in pregnancy
E.
it may cause delayed onset of labour
F.
it may cause dysfunctional labour
G.
it may cause hypernatraemia
H.
it may cause precipitate labour
I.
it may increase the risk of retention of the placenta and PPH
J.
it may interfere with the initiation of lactation
K.
it is usually administered nasally
L.
is the treatment of choice for central DI
M.
is the treatment of choice for gestational DI
N.
is the treatment of choice for nephrogenic DI
O.
is the treatment of choice for psychogenic DI


CPD question from TOG 2018. Vol 20; 1. These are open access, so reproduced here.
Diabetes insipidus (DI) in pregnancy,
1.     is the failure of water conservation by the renal tubules.                                                 True / False
2.     occurs in approximately 2–4 per 100 000 pregnancies.                                                 True / False
3.     is associated with a mortality rate of approximately 50%.                                                 True / False
4.     usually arises in the first trimester.                                                                             True / False
5.     presents with oliguria.                                                                                                   True / False
Antidiuretic hormone (ADH),
6.     acts at the kidney by stimulating V2 receptors.                                                         True / False
7.     has a similar structure to oxytocin.                                                                              True / False
Vasopressinase,
8.     is released from the fetal renal vasculature.                                                              True / False
9.     undergoes hepatic metabolism.                                                                                   True / False
10.   secretion is increased in multiple pregnancies.                                                         True / False
Regarding the pathophysiology of DI,
11.   the neurogenic (central) type is caused by either inadequate production or release of vADH from the posterior pituitary.                                                                                           True / False
12.   in the gestational type ADH synthesis is unaffected.                                                 True / False
13.   primary polydipsia occurs as a result of excess fluid intake with normal pituitary and renal function.                                                                                                                   True / False
Desmopressin (1-deamino-8-D-arginine vasopressin, DDAVP),
14.   is an ADH analogue.                                                                                                       True / False
15.   is a second-line treatment in pregnancy for the non-renal type of DI.                True / False
16.   can be administered rectally.                                                                                       True / False
17.   is administered intravenously.                                                                                     True / False
Pregnant women with DI,
18.   can be managed solely with obstetric input.                                                              True / False
19.   have a good prognosis if its uncomplicated.                                                              True / False
20.   will require senior clinician (obstetrics and anaesthetics) review in < 5% of cases.       True / False

42. 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

43.  EMQ. Peutz-Jeghers syndrome.
Abbreviations.
PJS:       Peutz-Jeghers syndrome.
Scenario 12.           
Which, if any, of the following are characteristics of PJS?
Option list.
A.       
buccal pigmentation
B.       
gastro-intestinal hamartomas
C.       
perianal pigmentation
D.      
increased risk of breast cancer
E.       
increased risk of cervical adenoma malignum
F.       
increased risk of colo-rectal cancer
G.      
increased risk of endometrial cancer
H.      
increased risk of ovarian cancer
I.         
increased risk of pancreatic cancer
J.        
increased risk of prostate cancer
K.       
increased risk of stomach cancer
Scenario 13.           
What is the approximate prevalence of PJS?
Option list.
A.       
< 1 in 1,000
B.       
1 in 1,000 to 1 in 10,000
C.       
1 in 10,000 to 1 in 100,000
D.      
1 in 25,000 to 1 in 100,000
E.       
1 in 25,000 to 1 in 200,000
F.       
1 in 25,000 to 1 in 300,000
G.      
1 in 300,000 to 1 in 500,000
H.      
< 1 in 500,000
Scenario 14.           
What is the mode of inheritance in PJS?
Option list.
A
autosomal dominant
B
autosomal recessive
C
X-linked dominant
D
X-linked recessive
E
Y-linked dominant
F
Y-linked recessive
G
triplet repeat
Scenario 15.           
Which, if any, of the following statements are true of PJS?
Option list.
A
PJS only occurs in families with other affected members
B
PJS mainly occurs in families with other affected members
C
PJS may arise de-novo in families with no other affected members
D
PJS may arise de-novo in families with other affected members
E
PJS does not arise de-novo in families with no other affected members
Scenario 16.           
What is the approximate lifetime risk of developing cancer in PJS?
Option list.
A.       
10%
B.       
20%
C.       
30%
D.      
40%
E.       
50%
F.       
60%
G.      
70%
H.      
80%
I.         
90%
J.        
>90%
Scenario 17.           
What is the relevance of STK11 to PJS?
Option list.
A.       
It is part of the postcode of the Peutz-Jeghers Society
B.       
It is the name of the gene most commonly associated with PJS
C.       
It is the Ornithological Society’s code for the Orkney Skua
D.      
Somatic mutations have been found in cervical cancer
E.       
None of the above