Pyelonephritis & Congenital Abnormalities

Question 1

What is renal agenesis? [1]

Answer 1

congenital absence of kidney due to failure of formation at the metanephric stage - can be unilateral or bilateral

Question 2

Renal Hypodysplasia:

1. define renal hypoplasia? [1]
2. define renal dysplasia? [1]
3. define renal hypodysplasia? [1]
4. presentation antenatally? [1]
5. presentation in neonate? [4]
6. presentation in children? [4]
7. management? [1]

Answer 2

1. Reduction in number of nephrons but normal architecture
2. Malformed renal tissue:
3. Congenitally small kidneys with dysplastic features
4. Antenatal Presentation:
   
   • abnormal growth detected on ultrasound
5. Presentation in Neonates:
   
   • lung issues
   • intrauterine growth restriction (IUGR)
   • acidosis
   • raised creatinine
6. Presentation in Children:
   
   • failure to thrive
   • anorexia
   • vomiting
   • proteinuria
7. Supportive treatment

Question 3

Describe the pathogenesis of multicystic dysplastic kidney (MCDK) [2]

Answer 3

1. MCDK is a non-inherited congenital disease where the ureteric bud fails to develop properly
2. this results in urine buildup in the kidney leading to the formation of multiple large cysts

Question 4

Autosomal Recessive Polycystic Kidney Disease (ARPKD):

1. antenatal presentation? [2]
2. presentation in infancy? [5]
3. presentation in childhood? [2]
4. associated anomalies? [3]

Answer 4

1. antenatal presentation:
   
   • detected on ultrasound
   • oligohydramnios
     
     • decreased amniotic fluid surrounding the fetus, which results in restricted fetal growth
2. presentation in infancy:
   
   • large palpable renal mass
   • respiratory distress
   • renal failure
   • hypertension
   • hyponatremia: urinary concentrating defect
3. presentation in childhood:
   
   • renal failure
   • hypertension
4. associated anomalies:
   
   • congenital hepatic fibrosis: subclinical to liver disease
   • portal hypertension
   • ascending cholangitis

Question 5

Autosomal Dominant Polycystic Kidney Disease (ADPKD):

1. features on ultrasound? [2]
2. pathology? [1]
3. antenatal presentation? [1]
4. presentation in childhood? [5]
5. presentation in adults? [2]
6. associated anomalies? [5]
7. management? [2]

Answer 5

1. features on ultrasound:
   
   • large echogenic kidneys
   • macrocysts (multiple in older child)
2. pathology = cysts originating from tubules
3. detected on ultrasound antenatally
4. presentation in childhood:
   
   • haematuria
   • hypertension
   • flank pain
   • UTIs
   • detected on renal ultrasound - may be unilateral
5. presentation in adults:
   
   • most commonly presents in adults
   • hypertension
   • haematuria
6. associated anomalies:
   
   • mitral valve prolapse
   • cerebral aneurysm
   • AV malformation
   • hepatic/pancreatic cysts
   • colonic diverticula/hernia
7. management:
   
   • supportive
   • directed → tolvaptan

Question 6

Hydronephrosis

1. features on ultrasound? [1]
2. causes? [2]

Answer 6

1. increased renal pelvis diameter - dilation >10mm
2. causes:
   
   • vescio-ureteric reflux
   • obstruction of urinary tract

Question 7

Pelvis/Ureter Junction (PUJ) Obstruction:

1. definition? [1]
2. more common in which sex? [1]
3. presentation? [4]

Answer 7

1. partial or total blockage of urine at ureter junction with kidney
2. more common in males
3. presentation:
   
   • abdominal mass
   • UTI
   • failure to thrive
   • abdominal/flank pain

Question 8

Vesico-Ureteric Junction (VUJ) Obstruction

1. definition? [1]
2. what is megaureter? [1]
3. primary causes of megaureter? [2]
4. secondary causes of megaureter? [1]

Answer 8

1. functional/anatomical abnormality at vesico-ureteric junction
2. megaureter is gross dilatation of the ureter >7mm
3. primary causes:
   
   • vesicoureteric reflux → reflux of urine from bladder into ureters
   • obstruction
4. secondary causes:
   
   • bladder issues

Question 9

Posterior Urethral Valves

1. definition? [1]
2. presentation? [3]
3. investigations? [2]
4. management? [1]
5. PUV puts you at increased risk of? [2]

Answer 9

1. most common obstructive uropathy
2. presentation:
   
   • can be detected antenatally
   • bilateral hydronephrosis
   • UTI
3. investigations:
   
   • ultrasound
   • micturating cystourethrogram (MCUG) → scan that shows how well a child’s bladder is working
4. management:
   
   • cystoscopy → endoscopy of bladder via the urethra
5. PUV puts you at increased risk of:
   
   • chronic kidney disease
   • bladder dysfunction

Question 10

Vesico-Ureteric Reflux (VUR)

1. definition? [1]
2. complications? [4]
3. how is it diagnosed? [1]
4. describe the grading? [4]

Answer 10

1. retrograde passage of urine from the bladder into the upper urinary tract
2. complications:
   
   • recurrent UTIs which leads to:
     
     • scarring
     • hypertension
     • end-stage renal failure
3. diagnosed by micturating cystourethrogram (MCUG)
4. grading of VUR:
   
   • Grade I → reflux into a non-dilated ureter
   • Grade II → reflux into the pelvis and calyces without dilation
   • Grade III → mild to moderate dilation of the ureter, renal pelvis and calyces with minimal blunting of the fornices
   • Grade IV → moderate ureteral tortuosity and dilation of the pelvis and calyces
   • Grade V → gross dilation of ureter, pelvis and calyces; loss of papillary impressions and ureteral tortuosity

Question 11

Urinary Tract Infection (UTI):

1. microbiological causes? [3]
2. host factors associated with UTI? [7]
3. presentation of UTI in the upper urinary tract? [4]
4. presentation of UTI in the lower urinary tract? [4]
5. investigations? [3]
6. complications? [3]

Answer 11

1. microbiological causes:
   
   • E.coli (most common)
   • Others such as Klebsiella/Pseudomonas
2. host factors:
   
   • age
   • circumcision
   • urinary obstruction
   • vesico-ureteric reflux
   • bladder/bowel dysfunction
   • catheterisation
   • sexual activity
3. presentation of UTI in the upper urinary tract:
   
   • pyrexia - rigors
   • vomiting
   • systemic upset
   • abdominal pain
4. presentation of UTI in the lower urinary tract:
   
   • dysuria
   • frequency
   • haematuria
   • wetting
5. investigations:
   
   • ultrasound
   • micturating cystourethrogram (MCUG)
   • nuclear medicine
6. complications:
   
   • scarring
   • hypertension
   • end-stage renal failure