Renal Diseases & Blood Vessel Disorders

Question 1

3 major vascular pathologies that can have renal involvement

Answer 1

• HTN
• renal artery stenosis
• thrombotic microangiopathy: HUS and TTP

Question 2

What are some vascular changes in benign hypertensive nephrosclerosis?

Answer 2

• intimal and medial thickening

- hyaline arteriolosclerosis (due to extra action of plasma proteins through the injured endothelium)

Question 3

What are glomerular changes seen in benign hypertensive nephrosclerosis?

Answer 3

• global sclerosis due to ischemic injury leading to nephron loss
• FSGS is an adaptive injury due to compensatory hyperfiltration due to nephron loss

Question 4

Will you see microscopic hematuria in someone with benign hypertensive nephrosclerosis?

Answer 4

NO

Question 5

Bland urine sediment with mild proteinuria is diagnostic of ______

Answer 5

Benign hypertensive nephrosclerosis

Question 6

Fibrosis is a feature of benign/malignant hypertensive nephrosclerosis

Answer 6

Benign

Question 7

HUS/TTP are disorders characterized by __________ leading to ________

Answer 7

Abnormal platelet aggregation; leading to thrombosis in arterioles and capillaries throughout the body → mechanical damage to circulating RBC’s

Question 8

“Young patient has bloody diarrhea and then develops symptoms of acute kidney injury”

What disorder should come to mind?

Answer 8

HUS/TTP

Question 9

What kind of anemia is associated with HUS/TTP?

Answer 9

Microangiopathic hemolytic anemia

Question 10

HUS/TTP has more neurological symptoms such as ______-

Answer 10

TTP;
- headache, confusion, seizure and stroke

HUS: more severe renal failure, less pronounced CNS

Question 11

What other infects is HUS associated with?

Answer 11

EHEC, shigella , salmonella

Question 12

TTP is often associated with (3) _________

Answer 12

• SLE
• HIV
• hematological malignancy

Question 13

What enzyme deficiency is associated with TTP?

Answer 13

ADAMTS 13

Question 14

Deficiency in ADAM TS13 is associated with ___________

Answer 14

TTP

Question 15

Renal artery stenosis is due to occlusion most commonly by __________

Answer 15

Atheromatous plaque

Fibromuscular dysplasia is the cause in 10-25% and is inherited and seen more commonly in women

Question 16

When using a renal artery droplet to diagnose renal artery stenosis, what ratio of blood flow velocities is hemodynamically significant for RAS?

Answer 16

Renal: arotic ratio of blood flow velocities in each vessel should be GREATER than 3.5

Renal: aortic > 3.5

Question 17

ADPKD with a mutation in ________ gene on chromosome ________ has ↓ penetrance and is the milder phenotype

Answer 17

PKD2; chromosome 4

• later onset of cysts, fewer and smaller cysts,

Question 18

Which genetically inherited kidney disease has a gene that is genetically heterogenous?

Answer 18

ADPKD:

• PKD1 on chromosome 16
• PKD2 on chromosome 4

Question 19

Abnormal ___________ is the primary mechanism for the formation of cysts in PKD

Answer 19

Tubular epithelial cilia

Question 20

In ADPDK, the cysts cause compression which leads to _________

Answer 20

Overperfusion of glomeruli because normal adjacent parenchyma can be destroyed by compression

Question 21

Is FSGS associated with ADPKD?

Answer 21

No

Question 22

Cysts that arise from different areas of tubules is seen in ______

Answer 22

ADPKD

Question 23

Hepatic cysts are an extra renal manifestation of PKD, what symptoms would you expect in someone with ADPKD with hepatic cysts?

Answer 23

Usually asymptomatic and synthetic liver function is intact

Pain can be due to distention of the liver on the capsule

Question 24

Which one is genetically homogeneous: ADPKD or ARPDK

Answer 24

ARPDK

Question 25

In childhood polycystic kidney disease, ________ gene which codes for _______ is mutated

Answer 25

PKHD1; fibrocystin protein

Question 26

Cysts arise from the _________ in ARPKD

Answer 26

Collecting ducts

Question 27

Hepatic fibrosis is an extra renal manifestation seen in which PKD?

Answer 27

autosomal recessive

Question 28

Alports sysndome is a defect in collegen _________ synthesis which is an important component of what part of the kidney?

Answer 28

Type IV; important for the glomerular basement membrane → hematuria in children

Question 29

Mutations in _________ and ________ genes cause Alport’s syndrome and is passed on via _______ mode of inheritance

Answer 29

COL4A4 and COL4A5; X linked (more common in males)

Question 30

What other symptoms would you expect in a child with Alport’s Syndrome other than hematuria?

Answer 30

• nerve deafness
• eye disorders
• hematologic disorders such as thrombocytopenia

Question 31

What would you expect to see on EM for Alport’s Syndrome?

Answer 31

Very thin BM, lamination of lamina dense