Pyelonephritis and CAKUT Flashcards
Explain the embryology of the kidney
- Pronephros
- Mesonephros
- Metanephros
- At 10 weeks urine production
When does nephrogenesis commence?
Week 10
What are the different renal investigations?
- Antenatal ultrasound scan
- Ultrasound
- MCUG- micturating cystourethrogram
- Nuclear medicine: DMSA, MAG3
- CT
- MRI
What does CAKUT stand for?
Congenital abnormalities of kidney and urinary tract
What are the types of anomalies under CAKUT?
- Renal dysplasia/hypoplasia
- Renal agenesis
- MCDK
- Renal cystic dysplasia
- Genetic cystic disease
- Obstructive uropathy
- Vesicle-ureteric reflux
What is renal agenesis?
Congenital absence of renal parenchymal tissue
What is renal hypoplasia?
Reduction in the number of nephrons but a normal architecture
What is renal dysplasia?
Malformed renal tissue
What is renal hypodysplasia?
congenitally small kidneys with dysplastic features
What is the presentation of renal hypo dysplasia in foetuses?
Abnormal growth seen on the prenatal US scan
What is the presentation of renal hypo dysplasia in neonates?
- lung tissue
- IUGR - intrauterine growth restriction
- acidosis
- Increased creatinine
What is the presentation of renal hypodysplasia in children?
- Failure to thrive
- Anorexia
- vomiting
- Proteinuria
What are the risks of multi cystic dysplastic kidney?
- hypertension
* Malignancy
What is the antenatal presentation of autosomal recessive polycystic kidney disease?
- Seen on antenatal ultrasound
* Oligohydramnios (deficiency of amniotic fluid)
What is the infancy presentation of autosomal recessive polycystic kidney disease (ARPKD)
- Large palpable renal mass
- Respiratory distress
- Renal failure
- Hypertension
- Hyponatremia: urinary concentrating defect
What is the childhood presentation of autosomal recessive polycystic kidney disease?
- renal failure
* hypertension
What is associated with ARPKD?
Congenital hepatic fibrosis: subclinical to liver disease (portal hypertension and ascending cholangitis)
what is the prognosis of ARPKD?
- 20-30% mortality in the neonatal period
- 5 year survival 70-88%
- Progression to ESRF is over 50%, often within 15 years
- 30-50% progress to ESRF within 10 years
State the genetic mutations associated with autosomal dominant polycystic kidney disease
- PKD1 mutation - polycystin 1
* PKD2 mutation - polycystin 2
What is the classical ultrasound appearance in ADPKD?
- Large echogenic kidneys
* Macrocysts: occasionally in infancy, multiple in older children
What is the pathogenesis of ADPKD?
Cysts originate from the renal tubules
What is the childhood presentation of ADPKD?
- Haematuria
- Hypertension
- Flank pain
- UTIs
- Renal ultrasound: may be unilateral
What is the adult presentation of ADPKD?
- majority of presentations
- Renal ultrasound
- Hypertension
- haematuria
What are the associations with ADPKD?
- Mitral valve prolapse
- Cerebral aneurysm
- AV malformation
- Hepatic /pancreatic cysts
- Colonic diverticula/hernia
