Renal Diseases & Blood Vessel Disorders Flashcards
3 major vascular pathologies that can have renal involvement
- HTN
- renal artery stenosis
- thrombotic microangiopathy: HUS and TTP
What are some vascular changes in benign hypertensive nephrosclerosis?
- intimal and medial thickening
- hyaline arteriolosclerosis (due to extra action of plasma proteins through the injured endothelium)
What are glomerular changes seen in benign hypertensive nephrosclerosis?
- global sclerosis due to ischemic injury leading to nephron loss
- FSGS is an adaptive injury due to compensatory hyperfiltration due to nephron loss
Will you see microscopic hematuria in someone with benign hypertensive nephrosclerosis?
NO
Bland urine sediment with mild proteinuria is diagnostic of ______
Benign hypertensive nephrosclerosis
Fibrosis is a feature of benign/malignant hypertensive nephrosclerosis
Benign
HUS/TTP are disorders characterized by __________ leading to ________
Abnormal platelet aggregation; leading to thrombosis in arterioles and capillaries throughout the body → mechanical damage to circulating RBC’s
“Young patient has bloody diarrhea and then develops symptoms of acute kidney injury”
What disorder should come to mind?
HUS/TTP
What kind of anemia is associated with HUS/TTP?
Microangiopathic hemolytic anemia
HUS/TTP has more neurological symptoms such as ______-
TTP;
- headache, confusion, seizure and stroke
HUS: more severe renal failure, less pronounced CNS
What other infects is HUS associated with?
EHEC, shigella , salmonella
TTP is often associated with (3) _________
- SLE
- HIV
- hematological malignancy
What enzyme deficiency is associated with TTP?
ADAMTS 13
Deficiency in ADAM TS13 is associated with ___________
TTP
Renal artery stenosis is due to occlusion most commonly by __________
Atheromatous plaque
Fibromuscular dysplasia is the cause in 10-25% and is inherited and seen more commonly in women
When using a renal artery droplet to diagnose renal artery stenosis, what ratio of blood flow velocities is hemodynamically significant for RAS?
Renal: arotic ratio of blood flow velocities in each vessel should be GREATER than 3.5
Renal: aortic > 3.5
ADPKD with a mutation in ________ gene on chromosome ________ has ↓ penetrance and is the milder phenotype
PKD2; chromosome 4
- later onset of cysts, fewer and smaller cysts,
Which genetically inherited kidney disease has a gene that is genetically heterogenous?
ADPKD:
- PKD1 on chromosome 16
- PKD2 on chromosome 4
Abnormal ___________ is the primary mechanism for the formation of cysts in PKD
Tubular epithelial cilia
In ADPDK, the cysts cause compression which leads to _________
Overperfusion of glomeruli because normal adjacent parenchyma can be destroyed by compression
Is FSGS associated with ADPKD?
No
Cysts that arise from different areas of tubules is seen in ______
ADPKD
Hepatic cysts are an extra renal manifestation of PKD, what symptoms would you expect in someone with ADPKD with hepatic cysts?
Usually asymptomatic and synthetic liver function is intact
Pain can be due to distention of the liver on the capsule
Which one is genetically homogeneous: ADPKD or ARPDK
ARPDK
In childhood polycystic kidney disease, ________ gene which codes for _______ is mutated
PKHD1; fibrocystin protein
Cysts arise from the _________ in ARPKD
Collecting ducts
Hepatic fibrosis is an extra renal manifestation seen in which PKD?
autosomal recessive
Alports sysndome is a defect in collegen _________ synthesis which is an important component of what part of the kidney?
Type IV; important for the glomerular basement membrane → hematuria in children
Mutations in _________ and ________ genes cause Alport’s syndrome and is passed on via _______ mode of inheritance
COL4A4 and COL4A5; X linked (more common in males)
What other symptoms would you expect in a child with Alport’s Syndrome other than hematuria?
- nerve deafness
- eye disorders
- hematologic disorders such as thrombocytopenia
What would you expect to see on EM for Alport’s Syndrome?
Very thin BM, lamination of lamina dense
