Kidney: Anatomy/ Congenital Disorders/ Acquired Cystic Disease

Question 1

“fenestrated epithelial cells”

Answer 1

podocytes

Question 2

“modified smooth muscle cells”

Answer 2

mesangial cells

Question 3

What molecules make up the GBM?

Answer 3

Type IV collagen and GAGs

Question 4

What cells are important in glomerular inflammation and thrombosis?

Answer 4

Glomerular epithelial cells

Question 5

Where are podocytes located?

Answer 5

rest on the outer aspect of the GMB

Question 6

What are foot processes?

Answer 6

cytoplasmic projections from podocytes onto the lamina rara externa of the GMB

Question 7

What are the functions of the mesangium?

5 functions

Answer 7

1- mechanical support

2- endocytosis and processing of plasma protein

3- maintenance of GMB and matrix

4- modulation of glomerular filtration

5- generation of molecular mediators

Question 8

What comprises the JG apparatus?

Answer 8

Macula densa, mesangial cells, terminal afferent and proximal efferent arterioles

Question 9

What do cortical interstitial cells secrete?

Answer 9

EPO

Question 10

“insufficient amniotic fluid, compressed fetus, low ears, small chin, beak node, pilmonary hypoplasia”

Answer 10

Potter sequence

Question 11

“complete absence of renal tissue”

Answer 11

renal agenesis

Question 12

Difference between unilateral and bilateral renal agenesis?

Answer 12

unilateral–> asymptomatic and existing kidney undergoes hypertrophy

bilateral–> still born, link with potter sequence

Question 13

Unilateral renal agenesis is at increased risk for….

Answer 13

Progressive glomerular sclerosis

Question 14

“few than 6 renal lobes”

Answer 14

Renal hypoplasia

Question 15

“enlargement of renal lobules when there are too few lobules”

Answer 15

Oligomeganephronia

Question 16

Most common location of an ectopic kidney?

Answer 16

pelvis

Question 17

“conjoined kidneys usually in the lower abdomen”

Answer 17

Horseshoe kidney

Question 18

Most common renal congenital anomaly?

Answer 18

Horseshoe kidney

Question 19

“undifferentiated tubules and primitive mesenchyme, cysts may be present”

Answer 19

renal dysplasia

Question 20

What is the most common abd mass in newborns?

Answer 20

Multicystic renal dysplasia

Question 21

“inherited defect leading to bilateral enlarged kidneys with cysts in the renal cortex and medulla”

Answer 21

PKD

Question 22

Who gets the AR form of PKD?

Answer 22

infants

Question 23

How does AR PKD present?

Answer 23

worsening renal failure ad HTN, potter sequence possible

Hepatic cysts/ fibrosis are also possible

Question 24

Who gets the AD form of PKD?

Answer 24

Adults

Question 25

How does AD PKD present?

Answer 25

Young adults with HTN, hematuria, worsening renal failure

Berry aneurysms, hepatic cysts, mitral valve prolapse

Question 26

PKD1/2 gene is involved in…

Answer 26

primary cilia or tubular epithela cell cell adhesion complexes

so without tubules are abnormal and undifferentiated

Question 27

Arrangement of cysts in AR PKD?

Answer 27

Radial arrangement

Question 28

“AD inherited defect leading to cysts in the medullary Collecting Ducts”

Answer 28

medullary cystic kidney diseases

Question 29

Gene in medullary cystic kidney diseases?

Answer 29

MCKD 1/2 mutation

Question 30

Symptoms of nephrothisis and medullary cystic disease?

Answer 30

polyuria, polydipsia, enuresis, azotemia, renal failure/ ESRD, atrophic tubules thickened and laminated BM, sclerosis fibrosis, and inflammation

Question 31

“multiple small cysts in the papillae that arise from CDs

Answer 31

medullary sponge disease

Question 32

How does a person get acquired cystic disease?

Answer 32

long term dialysis

Question 33

Acquired cystic disease leads to an increased risk of…

Answer 33

Renal cell carcinoma