CAKUT and Pyelonephritis Flashcards
What is important to note about nephrogenesis in the context of congenital anomalies?
- the majority of the development of the kidney occurs in the 3rd trimester
- this means prematurity can affect kidney development
What renal investigations are useful in detecting congenital disease?
- antenatal US
- US
- MCUG (catheterisation and contrast imaging)
- nuclear medicine (DMSA, MAG3)
- CT/MRI
What is renal agenesis?
- congenital absence of renal parenchymal tissue (metanephric stage)
- can be unilateral/bilateral (not compatible with life)
- bilateral is mostly sporadic
What is renal hypodysplasia and its clinical presentation?
- renal hypodysplasia: congenitally small kidneys with dysplasia features
Presentation:
- antenatal: US growth
- neonatal: lung issues, intrauterine growth restriction, acidosis and increased creatinine
- children: failure to thrive, anorexia, vomiting, proteinuria
Describe MCDK and its risks
- multi-cystic dysplastic kidney
- detected by the presence of an abdominal mass in neonates
- risk of involution, hypertension and malignancy
Describe the clinical presentation of ARPKD
- antenatal: antenatal US, oligohydramnios
- infancy: large palpable renal mass, resp distress, renal failure, hypertension, hyponaturaemia
- childhood: renal failure, hypertension
What are the associated anomalies of ARPKD?
- congenital hepatic fibrosis
- portal hypertension
- ascending cholangitis
Describe the pathology of ADPKD
- US shows large echogenic kidneys and macro cysts
- pathology shows cysts originating from tubules
Describe the clinical presentation ADPKD
- antenatal: antenatal US
- childhood: haematuria, hypertension, flank pain, UTIs, renal US - may be unilateral
- adult: majority, renal US, hypertension, haematuria
- family history
What are the associated anomalies and management of ADPKD?
Associated anomalies:
- mitral valve prolapse
- cerebral aneurysm
- AV malformation
- hepatic/pancreatic cysts
- colonic diverticula/hernia
- treatment: supportive, tolvaptan
Describe hydronephrosis
- associated with renal injury and renal impairment
- causes: vesicoureteric reflux
- obstruction of urinary tract
What is PUJ obstruction and how is it diagnosed?
- partial/total blockage of urine at ureter junction with kidneys, bilateral (10%)
- diagnosis: antenatal US, abode mass, UTI, FTT, abdo/flank pain
What is VUJ obstruction and its causes?
- functional/anatomical abnormality at VUJ
- can cause megaureter
- causes: reflux/obstruction (primary), bladder issues (secondary)
What are posterior urethral valves and their clinical presentation?
- most common obstructive uropathy (obstructive membranes that develop in urethra)
- clinical presentation: antenatal detection, bilateral hydronephrosis, UTI
- risk of CKD, bladder dysfunction
What is the investigations and treatment of posterior urethral valves?
- US and MCUG
- treatment: cystoscopy
