4.1 Renal system Flashcards
Renal Embryology
Kidneys form from pro-, meso- and metanephros; first two regress and only latter persists
Ureteric bud from outgrowth of mesonephric duct
Form in sacrum and migrate caudally by 8 weeks gestation; maturation continues to 5 y/o
US Renal cortex and medulla
Renal medulla hypoechoic to cortex
Renal Mass CT protocol
Noncontrast Corticomedullary phase (25-30 seconds) Nephrographic phase (80 seconds)
Horseshoe kidney - formation, risks
Abnormal axis - lower calyx more medial than upper
Bilateral malrotation with pelvises in anterior position
Isthmus fibrous or parenchymal, between IMA and aorta
Obstruction, infection, calculus formation in 30%, particularly PUJ
Increased risk with trauma and malignancies including Wilms tumour
Association with Cardiovascular, MSK, anorectal anomalies
Septum of Bertin - location, association
90% upper pole, 60% bilateral
Associated with bifid renal pelvis
Congenital megacalyces - definition, consequences
Excess of enlarged calyces (20-25 vs normal 10-14), causing hypoplastic pyramids
No obstruction, normal renal parenchyma and function
Pyelocalcyceal Diverticulum - definition, types
Outpouching of calyx into corticomedullary region; usually asymptomatic but may cause calculi
Type 1: from minor calyx
Type 2: from infundibulum
Type 3: from renal pelvis
ADPKD - definition, prevalance, association, criteria
Cystic dilatation of collecting tubules and nephrons; 0.1% population, 10% dialysis patients, strong penetrance
Hepatic cysts (70%), intracranial berry aneurysms (20%), pancreatic and splenic cysts less than 5%
Presents with flank pain and haematuria, can get cyst calcification and haemorrhage
Criteria: 2 or more cysts bilaterally on US in at risk patient at less than 30 y/o or any pt 30-60 y/o, or 4 or more cysts bilaterally if > 60 y/o
Acquired cystic kidney disease - definition
Multiple renal cysts developed in CRF; occur in 90% patients on dialysis
3-5 cysts in each kidney, CRF and no history of inherited cystic disease
Hemorrhagic cysts occur in 50%, can rupture
RCC develops in 7%
Bosniak criteria
- Benign simple cyst, don’t communicate with collecting system
- Minimally complicated, benign but with concerning features
Thin septations (less than 1 mm)
Fine calcifications
Hyperattenuating (>20)
2F. Multiple septa with or without perceptible but not measurable enhancement; can have thick or nodular calcification, also included if hyperattenuating and >3 cm - Complicated, multiloculated but with malignant features.
- Clearly malignant with large cystic component
Miscellaneous cysts
Milk of calcium: calcium carbonate granules, not true cyst but calyceal diverticulum
Parapelvic: originates in renal parenchyma but expands into renal sinus
Peripelvic: from sinus structures, likely lymphatic, may look like hydronephrosis on US
Perinephric: not true cyst, are extravasated urine trapped beneath capsule (urinoma)
Medullary cystic disease - cause
Due to tubulointerstitial fibrosis
Presents with anaemia and renal failure
Small kidneys with multiple small medullary cysts, often merely causes medullary increased echogenicity, thinned cortex
Types of medullary cystic disease
Familial nephronophthisis (70%): AR, juvenile onset at 3-5 years but also adult type
Adult medullary cystic disease (15%): AD inheritance
Renal retinal dysplasia (15%): recessive, associated with retinitis pigmentosa
Causes of Nephrocalcinosis
Metastatic (normal kidneys), dystrophic (injured tissue) or urine stasis
Hyperparathyroidism (primary and secondary)
Metastatic carcinoma to bone and hypercalcaemia of malignancy
Prolonged immobilisation
Sarcoidosis
Milk alkali syndrome
Hypervitaminosis D
RTA (often dense medullary calcification)
Medullary sponge kidney (urine stasis)
Hyperoxaluria
Bartter’s syndrome
Prolonged frusemide (usually premature infants)
Nephrotoxic drugs
Papillary necrosis
Medullary sponge kidney - AKA, associations, IVP
AKA Benign renal tubular ectasia, Cacchi-Ricci disease
Dysplastic cystic dilatation of papillary / medullary portions of collecting ducts, usually 20-40 y/o
75% bilateral, often asymptomatic; 10% develop progressive renal failure
IVP - striated nephrogram (brushlike), ‘bunch of flowers’ appearance
Associations: Hemihypertrophy, Beckwith-Wiedemann, Pyloric stenosis, Ehlers Danlos, RTA, any form of renal cystic disease / ectopia
Renal cell carcinoma - presentation, association
Haematuria, pain, mass, weight loss
Paraneoplastic: HTN - renin, erythrocytosis - EPO, hypercalcaemia - PTH, gynaecomastia - gonadotropin, Cushing - ACTH.
Associations: tobacco, vHL (bilateral), TS, dialysis, family history, petroleum, acquired cystic renal disease
Usually recurs at less than 2 years, but can have late recurrence - 10% recur 10 years after nephrectomy
RCC Types and imaging
Clear cell (65%) - proximal tubule
Papillary (15%) - chromophil, proximal tubule
Chromophobe (10%) - intercalated cell of cortical collecting duct
Oncocytoma (5%)
Unclassified (5%)
Best evaluated in nephrographic phase, venous invasion best characterised by MRI
US: 70% hyperechoic if > 3 cm, 30% if less than 3 cm
95% hypervascular, 10-30% calcified
Oncocytoma - origin, CT
From epithelial cells of proximal tubule, usually well-differentiated and benign but need resection
Central stellate scar (25-33% on CT) and spoke wheel (angiography), usually homogeneous enhancement, can’t differentiate from RCC on imaging, but FNA can discriminate
Juxtaglomerular tumour - associations
Rare. Secrete renin, cause HTN, hypernatraemia, hypokalaemia