Pyelonephritis and Congenital Abnormalities of the Kidney and Urinary Tract

Question 1

At what week of gestation does urine production start?

Answer 1

10 weeks

Question 2

At what week of gestation do no new nephrons develop?

Answer 2

36 weeks

Question 3

What is the GFR of a baby at birth?

Answer 3

40-60mls/min/1.73m^2

Question 4

What renal investigations can be conducted in children?

Answer 4

Anetnatal US
ultrasound
Micturating cystourethrogram
Nuclear medicine
CT
MRI

Question 5

List common congenital abnormalities of the kidneys/urinary tract

Answer 5

Renal dysplasia/hypoplasia
Renal agenesis
MCDK
Renal cystic dysplasia
Genetic cystic disease
Obstructive uropathy
Vesicle-ureteric reflux

Question 6

What is renal genesis?

Answer 6

The congenital absence of renal aprenchumal tissue at the metanephric stage

Question 7

What is the prognosis of unilateral renal agenesis?

Answer 7

Excellent - many people will go through life not knowing the have the condition

Question 8

What is the prognosis of bilateral renal agenesis?

Answer 8

Not compatible with life.

Question 9

Bilateral renal agenesis is usually sporadic. T/F?

Answer 9

True

Question 10

Which sex is usually affected by renal agenesis?

Answer 10

Males

Question 11

What is renal hypopalsia?

Answer 11

A reduction in the number of nephrons but with normal architecture

Question 12

What is renal dysplasia?

Answer 12

Malformed renal tissue

Question 13

What. is renal hypodysplasia?

Answer 13

Congenitally small kidneys with dysplastic features

Question 14

How does renal hypodysplasia present?

Answer 14

Present antenatally as US growth
In the neonate with lung issues, low birth weight, acidosis and raised creatinine
In children as failure to thrive, anorexia, vomiting, proteinuria

Question 15

What are the associated risks of multicystic dysplastic kidney disease?

Answer 15

Hypertension

Malignancy

Question 16

There can be spontaneous involution of multicystic dysplastic kidney disease. T/F?

Answer 16

True

Question 17

What are the signs of ARPKD antenatally?

Answer 17

Seen on ultrasound

Oligohydraminos (less than expected amniotic fluid volume)

Question 18

What are the signs of ARPKD in infancy?

Answer 18

Large palpable renal mass
Respiratory distress
Renal failure
Hypertension
Hyponatraemia

Question 19

What are the associated abnormalities of ARPKD?

Answer 19

Congenital hepatic fibrosis (causes portal hypertension and ascending cholangitis)

Question 20

What is the prognosis fo ARPKD?

Answer 20

20-30% mortality in neonatal. period

Progression to end stage renal failure in 50% of cases over 15 years

Question 21

What are the signs ADPKD in children?

Answer 21

Haemturia
Hypertension
Flank pain
UTIs
Renal US

Question 22

In what age group does ADPKD usually present?

Answer 22

Adults

Question 23

What are the associated conditions of ADPKD?

Answer 23

Mitral valve prolapse
Cerebral aneurysm
AV malformation
Hepatic/pancreatic cysts
Colonic diverticula/hernia

Question 24

What are the associated abnormalities of hydronephrosis?

Answer 24

Renal injury and impairment

Question 25

What signs of hydronehprosis can be seen on antenatal ultrasound?

Answer 25

Transient, increased. AP diameter

Question 26

What signs of hydronehprosis can be seen on post-natal ultrasound?

Answer 26

Renal pelvic diameter >10mm

Question 27

What are the possible causes of hydronephrosis?

Answer 27

Vesicle-ureteric reflux and obstruction of the urinary tract

Question 28

What is a pelvic/ureteric junction obstruction?

Answer 28

A total of partial blockage of urine at the junction of. the ureter with the kidney

Question 29

At what point can pelvic-ureteric junction obstruction be diagnosed?

Answer 29

Antenatally

Question 30

What are the signs/symptoms of pelvic-ureteric junction. obstruction?

Answer 30

Abdominal mass UTI Failure to thirve Abdominal/flank pain

Question 31

A vesicle-ureter junction obstruction will cause megaureter. How is this defined?

Answer 31

Ureteric dilation >7mm

Question 32

What is the most common obstructive uropathy?

Answer 32

Posterior urethral valve

Question 33

How is posterior urethral valve investigated?

Answer 33

Ultrasound | Micturating cystourethrogram

Question 34

How are posterior urethral valves managed?

Answer 34

Cystoscopy

Question 35

What complications result from posterior urethral valves?

Answer 35

Hydronephrosis | UTI

Question 36

What is vesicoureteral reflux?

Answer 36

Retrograde passage of urine from the bladder into the upper urinary tract

Question 37

How is vesicoureteral reflux diagnosed?

Answer 37

Mictruating cystourethrogram

Question 38

What grades of vesicourethral reflux need intervention?

Answer 38

Grades 4 and 5

Question 39

In which sex are UTIs more common?

Answer 39

Females

Question 40

What is the diagnostic criteria for UTI in children?

Answer 40

Significant bacteriuria >10^5 cooling forming units/ml | and a single pathogenic bacteria

Question 41

How can urine collection in suspected UTI be done in children?

Answer 41

Clean catch urine collection Mid-stream sample Catheter specimen Supra-pubic aspirate

Question 42

What bacteria usually causes UTI in children?

Answer 42

E.coli

Question 43

How does upper tract UTI present in children?

Answer 43

Pyrexia Vomiting Systemic upset Abdominal pain

Question 44

How does lower tract UTI present in children?

Answer 44

Dysuria Frequency Haematuria Wetting

Question 45

How can suspected pyelonephritis in children be investigated?

Answer 45

Ultrasound Microrating cystourethorgram nuclear medicine

Question 46

What are the complications of pyelonephritis?

Answer 46

Scarring Hypertension End stage renal failure