Congenital and Vascular Diseases Flashcards
Important congenital anomalis to know…
Renal agenesis
Renal hypoplasia
Ectopic Kidneys
Horseshoe Kidney
What happens in renal agenesis?
Bilateral Agenesis (Potter's syndrome) incompatible with life Unilateral is uncommon. Contralateral side will hypertrophy with progressive sclerosis.
What happens in renal hypoplasia?
True is very rare, usually due to scarring
Bilat. can cause renal failure in childhood
What happens with ectopic kidneys?
Kidneys develop at lower levels of the urinary tract, usually right above or within the pelvis. Can predispose to obstruction/infection
What happens with horseshoe kidney?
Fusion of upper (10%) or lower (90%) poles of the kidneys, typically anterior to great vessels.
Pretty common 1:500
Why are cystic diseases of the kidney important?
Common, can be diagnostically confused with tumors, can cause chronic renal failure
Pathogenic features of cystic diseases?
Compression of the parenchyma –> chronic dynfunction
Associated with dynfxn of other organs (esp. liver)
Describe Cystic Renal dysplasia
- Persistence of kidney abnormal structures such as cartilage/undifferentiated mesenchyme
- Associated with ureteropelvic obstruction, ureteral agenesis
- Large, irregular, cystic kidneys. Lined with flattened epithelium.
Important details about autosomal dominant adult polycystic kidney disease
Hereditary disorder of cell-cell matrix
Presents in 40s, 50s
Cysts expand and destroy parenchyma –> renal failure
Mutations associated with dominant PKD
PKD1 and PKD2
Codes for polycystin, which localizes in cilia for tubular epithelium
How does dominant PKD present?
Abdominal mass, Proteinuria, polyuria, hypertension
Slow progression to azotemia
Asymptomatic cysts in liver, intracranial berry aneurysms w/ hemorrhage, MVP
What is autosomal recessive PKD?
Pediatric Renal Failure
Kidneys enlarged, but smooth exterior
Cylindrical dilation of all tubules
Cysts come from collecting tubules, cuboidal lines
What important non-kidney effects are associated with autosomal recessive PKD?
Patients who survive infancy develop hepatic fibrosis
What is medullary cystic disease?
Medullary sponge kidney. Multiple cystic lesion of the CD in the medulla. Leads to calcification, infection, calculi.
Childhood cysts associated with tubular atrophy + interstitial fibrosis.
Important association to make with Cystic disease secondary to dialysis?
Renal Cell Carcinoma
Cystic disease secondary to dialysis cysts typically contain
Clear fluid
Calcium oxalate crystals
What to know about simple cysts?
Common postmortem finding, not clinically significant
Differentiate from tumors
What is nephrosclerosis
A term which applies to sclerotic changes to small arteries/arterioles seondary to HTN and diabetes. Overall, leads to ischemia of the parenchyma
Pathogenesis of benign nephrosclerosis?
Medial and Intimal Thickening
Hyaline deposition of proteins which extravasate through injured endothelium
Thickening of the BM
Notabe morphology in benign nephrosclerosis
Gross – Fine granularity, Narrowed Cortex
Microscopic – Thickening/Hyalinization of vessel wall
Patchy ischemic atrophy from tubular ischemia/fibrosis and glomerular deposits
Malignant nephrosclerosis is a frequent cause of death in….
Scleroderma patients via uremia
Pathogenesis of Malignant nephrosclerosis
Vascular damage in kidneys, fibrinoid necrosis, hyperplasia of smooth muscle, narrowed lumen.
Activation of RAA –> pos. feedback
Morphology of Malignant nephrosclerosis
Gross - pinpoint petechial hemorrhages
Micro – Fibrinoid necrosis, hyperplstic arteriolitis (onion skin), necrotizing glomeruitis (fills with PMN)
Non-renal Clinical features of Malignant nephrosclerosis?
Diastolic over 130
Papilledema, retinopathy (scomatoma), encephalopathy, CV abnormalities
Hypertensive crisis
