Devo of Kidney and Ureter

Question 1

What are the transcription factors mediating intermediate mesoderm?

Answer 1

Pax2, Pax8, Lim1

Question 2

Where does a mesonephric kidney form?

Answer 2

within teh nephrogenic cord in the thoracolumbar region of embryo

Question 3

What are the 2 components associated with mesonephric kidney?

Answer 3

mesonephric duct

mesonephric tubules

Question 4

What is the cloaca?

Answer 4

dilated caudal end of the primitive hindgut; a transient common outlet for the UG & GI systems

Question 5

What are mesonephric tubules develop into?

Answer 5

they are immature nephrons

Question 6

Where does the metanephric kidney form?

Answer 6

pelvic region of the embryo from teh caudal aspect of nephrogenic cord

Question 7

What two structures interact to form kidneys?

Answer 7

metanepehric diverticulum(ampulla) to metanephrogenic mesenchyme

Question 8

What is potters syndrome?

Answer 8

• renal agenesis
• severe urinary obstruction
• fxs of facial compression, growth retardation, limb deformities

Question 9

What are the inductive interactions between metanephric diverticulum and metanephrogenic mesenchyme result in formation of what?

Answer 9

nephron formation

Question 10

When metanephric diverticulum dissappears what no longer form?

Answer 10

no nephrons after ampulla dissappears

Question 11

What are the signals from MM?

What are the signal from MD?

Answer 11

GDNF, RA

Fgf2, Bmp7

Question 12

What are the early stages of nephron formation?

Answer 12

signals from ampulla cause metanphrogenic mesenchyme to aggregate and form an epithelial vescile

Question 13

What are the nephron derivative of hte proximal part of hte s-shaped tubule?

Answer 13

distal tubule and loop of henle

Question 14

What are hte nephron derivative of the middle part of s-shaped tubule?

Answer 14

proximal tubule

Question 15

What are the nephron derivatives of s-shpaed tubule from distal part?

Answer 15

renal corpuscle

Question 16

Where do the vascular sprouts from for the kidney vasculature?

Answer 16

intersegmental arteries are induced to grow towards the kidney

Question 17

What is multicystic dysplastic kidney?

Answer 17

usually unilateral
non-fucntioning kidny
primitive ductules and cartilage seen; atretic ureter
contralateral kidney hypertrophy

Question 18

What is autosomal recessive polycystic kidney disease?

Answer 18

diagnosed in ultrasound
rare
PKHD1

enlarged kidneys at birth
serious cases incompatible with life
associated with maternal oligohydramnios

smooth kidney with numerous small cysts

Question 19

What is autosomal dominant polycystic kidney disease?

Answer 19

new mutations, family histor 60%
PKD1 gene

cyst formation from abnormal differentiation of epithelial cells
secretion of fluid into cysts
abnormal ECM
interstitial fibrosis

Question 20

What are the extrarenal manifestatioons of ADPKD?

Answer 20

hepatic cysts
intracranial aneurysms (due to HTN)

Question 21

How is ADPKD diagnosed?

Answer 21

symptomatic presentation: typically flank pain+hematuria
screening with U/S
multiple bilateral cysts

Question 22

What is medullary sponge kidney?

Answer 22

no inheritance pattern
dilated medullary and papillary collecting duct
spongy appearance on pyelogram
complicated by:
-recurrent urinary tract infections
-kidney stones
-hematuria

Question 23

What is acquired cystic disease?

Answer 23

develop in 50% of pts on dialysis
usually asymptomatic
clear,fluid filled
uni- or multilocular cysts
cortex
increased incidence of papillary renal cell carcinoma

Question 24

What are the indications for renal biopsy?

Answer 24

pressitant glomerular hematuria
persistent nephrotic range proteinuria
unexplained renal failure
renal transplant rejection

Question 25

What are the contraindications for renal biopsy?

Answer 25

bleeding disorder

anatomic abnormalities

Question 26

What are the complications associated with renal biopsy?

Answer 26

self limited gross hematuria
hematoma
hemorrhage
death (1/8000)

Question 27

What is the multicystic dysplastic kidney treatment?

Answer 27

potential for abnormalities of contralateral kidney
HTN is potential complication
failure of a large MCKD to regress may be indicated for nephrectomy
non-genetic

Question 28

The remodeling of what results in formation of collecting ducts, calyces, pelvis and urter?

Answer 28

branching metanephric diverticulum

Question 29

Where does the metanephric kidney develops?

Answer 29

develops in the pelvis

Question 30

What is nephroblastoma?

Answer 30

wilm’s tumor; a common neoplasm in children; gene located on chromosome 11

Question 31

What is ureteropelvic junction obstruction?

Answer 31

diuresis enhances obstruction of urinary tract

presents in infant as flank mass, uTI, failure to thrive, sepsis

older child or adult: flank pain, colicky pain, UTI, hemturia

Question 32

The division of the cloaca results in what?

Answer 32

seperate outlets for UG and GI systems

Question 33

What divides the cloaca?

Answer 33

the urorectal septum

Question 34

What does the remodeling of posterior wall of bladder result in?

Answer 34

formation of trigone region

associated with entrance of ureters and exit of the urethra

Question 35

What is the extrosphy of hte bladder?

Answer 35

a defect of the vetnral abdominal wall
ining of bladder and urethra is open to surface
associated with UG and skeletal anomalies

Question 36

How is exstrophy treated and how does it turn out?

Answer 36

surgery, incontinence major long term issue, but relatively healthy long term

Question 37

What is the anomalies of the urethra

Answer 37

agenesis and atresia of the urethra

posterior urethral valves

Question 38

What is anemia in CKD?

Answer 38

glycosylated protien of EPO; horomone produced primarily in kidney