Tutorial 4 August 2014

Website.
Contact us.

	EMQ. AIS, MRKH and Swyer’s syndrome
59	You have been asked to write guidance for the unit in relation to labour and delivery in water. 1. Outline how you will go about the task.                             8 marks. 2. Discuss the key issues to be included in the guidance. 12 marks
60	A 20 year-old woman with a known history of drug abuse attends the hospital antenatal booking clinic in her first pregnancy. a. outline the factors determining her level of risk  4 marks. b. outline the risks to the mother                                 6 marks. c. outline the risks to the fetus                                      4 marks. d. outline the risks to the neonate & infant                4 marks. e. outline the risk to others                                           2 marks.
61	Discuss the key issues relating to the second trimester fetal anomaly ultrasound scan.
62	With regard to female genital mutilation (FGM). 1.   what are the key aspects of the law in the UK relating to FGM.                    2 marks. 2.   what are the responsibilities of the doctor who suspects that a child may be subjected to FGM?                                                                                                                                           2 marks. 3.   how is FGM graded?                                                                                               3 marks. 4.  outline the management of woman found at booking to have had FGM.  13 marks.

AIS, MRKH and Swyer’s syndrome

Lead-in.

The following scenarios relate to disorders of sexual development.

Pick the option from the option list that best fits each scenario.

Each option can be used once, more than once or not at all.

Abbreviations.

AIS:        androgen insensitivity syndrome.

AMH:    anti-Mullerian hormone.

CAH:      congenital adrenal hyperplasia.

CAI:       complete androgen insensitivity syndrome.

DSD:      disorder of sexual differentiation.

KS:         Kallmann’s syndrome.

LMB:     Laurence-Moon-Biedl syndrome.

MRKH:  Mayer-Rokitansky- Küster-Hauser syndrome.

PAI:       partial androgen insensitivity syndrome.

PW:       Prader-Willi syndrome.

SW:        Swyer’s syndrome.

TU:         Turner’s syndrome.

UPD:     uni-parental disomy.

Option list 1.

A.        has a uterus of normal size for her age.

B.        has a uterus that is hypoplastic for her age.

C.        has a vestigial uterus (anlagen).

D.        has no uterus.

E.         commonly has esthiomene

F.         I don’t know and I don’t care.

G.       the question makes no sense.

H.        none of the above.

Scenario 1.

a.      a girl with congenital adrenal hyperplasia at the start of puberty.

b.     a girl with complete androgen insensitivity syndrome at the start of puberty.

c.      a girl with a disorder of sexual differentiation at the start of puberty.

d.     a girl with Kallmann’s syndrome at the start of puberty.

 

e.     a girl with Laurence-Moon-Biedl syndrome at the start of puberty.

f.      a girl with Mayer-Rokitansky-Kuster-Hauser syndrome at the start of puberty.

g.      a girl with partial androgen insensitivity syndrome at the start of puberty.

h.     a girl with Prader-Willi syndrome at the start of puberty.

i.       a girl with Swyer’s syndrome at the start of puberty.

j.       a girl with Turner’s syndrome at the start of puberty.