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Paediatric EMQ 1

Created: 3/12/2007
Updated: 11/1/2008

 

Embryology

A 2 weeks
B 32 days
C 7 weeks
D 42 days
E 36 weeks
F 5 weeks
G 9 weeks
H 3 months
I 21 days
J 6 weeks
K 4 weeks

1 Nephrogenesis complete
2 Pronephros arises from intermediate mesoderm
3 Testosterone production from the Leydig cells commences
4 Ureteric buds arise from the mesonephric ducts
5 Paramesonephric ducts appear
6 Sertoli cells begin to produce anti-Mullerian hormone
7 Formation of the prostate commences

General paediatric urology

A 10%
B 17%
C 20%
D 80%
E 30%
F 33%
G 45%
H 1%

8 Percentage of patients with BXO with glans involvement
9 Percentage of patients with multicystic dysplastic kidney with VUR in the contralateral kidney
10 Proportion of patients with PUJ obstruction who undergo a pyeloplasty
11 Proportion of term children with undescended testes at 1 year
12 Proportion of patients with posterior urethral valves with long-term renal dysfunction
13 Proportion of ureteroceles associated with an upper pole duplex system

Disorders of sexual differentiation/intersex

A 21 hydroxylase deficiency
B Autosomal dominant
C 50%
D Over-virilised female
E Autosomal recessive
F 17-OH progesterone
G Mixed gonadal dysgenesis
H True hermaphrodite
I 95%
J 11B-hydroxylase
K 85%

14 Proportion of patients with disorders of sexual differentiation/intersex in which the underlying disorder is congenital adrenal hyperplasia (CAH)
15 Proportion of CAH-patients with salt-losing crisis
16 Raised serum levels indicate CAH
17 The most common cause of CAH
18 The inheritance pattern of the most common cause of CAH
19 Associated with palpable gonads and an abnormal sex chromosome karyotype

Vesicoureteric reflux

A 10%
B 50%
C 3:1
D Grade 3
E 30%
F 60%
G 5:1
H 2:1
I Grade 2
K Grade 4

20 Proportion of patients with a family history
21 The grade of reflux with contrast passing into ureter, pelvis and calyces with no dilatation
22 Proportion of patients with VUR who have renal scarring on presentation
23 Submucosal tunnel to ureter diameter ratio (Paquin’s law)
24 Grade of VUR with a slightly tortuous ureter, with moderate dilatation of pelvis and blunting of calyces

Answers

1E, 2K, 3G, 4F, 5J, 6C, 7H

8C, 9E
10B This figure comes from Dhillon HK. Great Ormond Street Experience BJU 1998
11H
12F Which is why patients should be followed up into adolescence
13E

14K It is responsible for 85% of intersex disorders overall and 95% of cases of female pseudohermaphroditism/over-virilised female
15C
16F, 17A, 18E 21 hydroxylase deficiency is inherited in an autosomal recessive fashion and is the most common cause of CAH. It catalyses the conversion of 17-OH progesterone to 11-deoxycortisol, its absence therefore results in an increase in 17-OH progesterone levels
19G Mixed gonadal dysgenesis is a spectrum of gonadal and genital abnormalities associated with absence of the 1 of the pair of sex chromosomes

20E, 21I, 22E
23G The Leadbetter-Politano anti-reflux submucosal tunnel should have a tunnel length to ureter diameter ratio of 5:1 (Paquin A. J Urol. 1959; 82: 573)
24K

International Reflux Classification

Grade 1 Contrast into a non-dilated ureter
Grade II – reflux into the renal pelvis and calyces without dilatation
Grade III – mild/moderate dilatation of the ureter, renal pelvis and calyces
Grade IV – dilation of the renal pelvis and calyces with moderate ureteral tortuosity Grade V – gross dilatation and tortuosity of the ureter, pelvis and calyces

 


ArticleDate:20071203
SiteSection: Article
 
   
    
                                            



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