Week 1

Question 1

mesoderm derivative that gives rise to kidney and gonads

Answer 1

intermediate mesoderm

Question 2

mesoderm derivative that gives rise to head and somite

Answer 2

paraxial mesoderm

Question 3

mesoderm derivative that gives rise to splanchnic, somatic, and extraembryonic

Answer 3

lateral mesoderm

Question 4

transitory, non-functional kidney

Answer 4

pronephros (cervical nephrotomes)

Question 5

functional during development of permanent kidney, duct contributes to developing testis, male genital system, and vestigial remnants in female

Answer 5

mesonephros

Question 6

permanent kidney in 5th week, produces urine at 11-13 weeks

Answer 6

metanephros

Question 7

ureteric bud forms what structures?

Answer 7

ureter, pelvis, calyces

Question 8

polyhydramnios

Answer 8

less swallowing, excess amniotic fluid due to esophageal or duodenal atresia

Question 9

oligohydramnios

Answer 9

less urine output, shortage of amniotic fluid due to failure of kidneys to develop normally

Question 10

derivative of ureteric bud

Answer 10

collecting tubules

Question 11

derivative of nephrogenic mesenchyme

Answer 11

nephron and Bowman’s capsule

Question 12

expressed by mesenchyme, enables tissue to respond to induction by ureteric bud

Answer 12

WT1

Question 13

receptor that mediates programmed cell death, localized to mesenchyme during ureteric bud branching

Answer 13

Angiotensin II receptor

Question 14

division of cloaca by urorectal septum into dorsal rectum and ventral urogenital sinus

Answer 14

bladder formation

Question 15

mesonephric ducts and attached ureteric buds intercalated into posterior wall

Answer 15

exstrophy of the bladder

Question 16

dysregulation of fetal adrenal gland, hypertrophy of adrenal cortex, overproduction of dehydroepiandrosterone, masculinization of female genitalia

Answer 16

congenital virilizing hyperplasia (CAH)

Question 17

bilateral renal agenesis, due to oligohydramnios, lung hypoplasia, clubbed feet, bow legs

Answer 17

Potter sequence

Question 18

nephroblastoma, most common primary renal tumor affecting children, 4th most common pediatric tumor, mutation in WT-1, poorly formed tubules

Answer 18

Wilm’s tumor

Question 19

pelvic kidney, often pancake shaped or horseshoe kidney where inferior poles fused and ascent blocked by inferior mesenteric artery

Answer 19

ectopic kidneys

Question 20

tube open at both ends, may cause urine to drain from umbilicus

Answer 20

urachal fistula

Question 21

exposure and protrusion of posterior wall of bladder through abdominal wall

Answer 21

exstrophy of the bladder

Question 22

affects laminins, nephrotic syndrome and ocular abnormalities, proteinuria, hypoalbuminemia,

Answer 22

Pierson syndrome

Question 23

defect in type 4 collagen, males, uremic syndrome to end stage renal failure, treat with dialysis or transplant

Answer 23

Alport disease

Question 24

defect in nephrin compromising slit diaphragm, massive proteinuria and albuminemia (causing edema), treat w/ albumin and ACE inhibitors, requires dialysis/transplant, leaking protein into urine

Answer 24

congenital nephrotic syndrome

Question 25

normal value for RBF

Answer 25

1.2 L/min, 20-25% of cardiac output

Question 26

normal value for RPF

Answer 26

660 mL/min

Question 27

normal value for GFR

Answer 27

125 L/min

Question 28

hydrostatic pressure, usually 60 mm Hg

Answer 28

Pgc

Question 29

increases blood flow resistance, decreases renal blood flow, and decreases glomerular capillary pressure

Answer 29

afferent arteriole constriction

Question 30

increases glomerular capillary pressure, increases renal blood flow, increases peritubular capillary pressure

Answer 30

afferent arteriole dilation

Question 31

increases blood flow resistance and glomerular capillary pressure, net increase in GFR, decrease RBF

Answer 31

efferent constriction

Question 32

decreases glomerular capillary pressure, increases RBF

Answer 32

efferent dilation

Question 33

automatically adjusts resistance of afferent arteriole, contracts in response to increased blood pressure

Answer 33

myogenic response

Question 34

mediated by macula densa at end of ascending limb of loop of Henle, senses change in NaCl through Na/K/Cl, releases adenosine for constriction

Answer 34

tubuloglomerular feedback mechanism

Question 35

structural support, modulates glomerular filtration, phagocytic activity, paracrine substances

Answer 35

intraglomerular mesangial cells

Question 36

near vascular pole and macula densa, produce renin

Answer 36

extraglomerular mesangial cells

Question 37

1. increased sympathetic nerve activity 2. decreased renal blood perfusion 3. decreased sodium to macula densa

Answer 37

activate release of renin

Question 38

1. arteriolar vasoconstriction 2. sodium and water retention 3. aldosterone secretion 4. negative feedback, inhibits renin secretion

Answer 38

angiotensin II produces these responses

Question 39

systemic arteriolar dilation, decreased sodium and water reabsorption, decreased aldosterone secretion

Answer 39

ACE inhibitor

Question 40

maintains plasma calcium by intestinal, renal, and bone reabsorption of calcium

Answer 40

vitamin D

Question 41

stimulated by hypoxia, anemia, and decreased renal blood flow

Answer 41

EPO production

Question 42

osmoreceptors signal hypothalamus to secrete this, which stimulates water reabsorption to decrease ECF osmolarity and concentrate urine

Answer 42

ADH (vasopressin)

Question 43

de novo synthesis of glutathione, stimulates amino acid trasnport into the cell

Answer 43

gamma glutamyl cycle

Question 44

alpha ketoglutarate --> glutamate --> frees ammonia for urea cycle

Answer 44

transamination

Question 45

dietary protein, transamination and glutamate dehydrogenase, peroxisome, mitochondria, glutaminase, purine/pyrimidine deamination, glycine cleavage, intestinal bacteria

Answer 45

sources of ammonia

Question 46

glutamate (glutamate dehydrogenase and transaminases), glutamine (carries 2 amino groups)

Answer 46

precursors of nitrogen in urea

Question 47

decreased TCA cycle in the brain, depleted glutamate, decreased neurotransmitter synthesis, can lead to coma and brain damage

Answer 47

build up ammonia and depleted alpha ketoglutarate

Question 48

urea cycle requires how many ATP?

Answer 48

4

Question 49

ATP + N-acetylglutamate, with ornithine forms citrulline

Answer 49

carbamoyl phosphate synthetase

Question 50

converts arginine to urea

Answer 50

arginase

Question 51

regulatory molecule of carbamoyl phosphate synthetase, can be increased by high levels of arginine intake

Answer 51

N-acetylglutamate

Question 52

lack of carbamoyl phosphate synthetase, newborn lethargic, vomits, fatal due to high ammonia, treat with arginine to activate N-acetylglutamate

Answer 52

type 1 hyperammonemia

Question 53

lacking ornithine transcarbamoylase, X linked, most common urea cycle disease, treat with low protein diet and ammonia detox

Answer 53

type 2 hyperammonemia

Question 54

hyperammonemia, coma, acidosis, ataxia, treat with carbamoyl glutamate to activate CPS1

Answer 54

N-acetylglutamate synthetase deficiency

Question 55

cofactor of carbamoyl phosphate synthetase, represents 15-25% of mitochondria matrix protein

Answer 55

N-acetylglutamate

Question 56

converts xanthine into uric acid

Answer 56

xanthine oxidase

Question 57

elevated PRPP --> enhanced purine synthesis --> loss of feedback inhibition --> partial deficiency of HGPRT (salvage path) --> increased levels of uric acid, crystallization in joins

Answer 57

gout

Question 58

total deficiency of HGPRT, X linked, self mutilation/retardation

Answer 58

Lesch Nyhan syndrome

Question 59

suicide inhibitor used to treat gout, binds xanthine oxidase

Answer 59

allopurinol

Question 60

majority asymptomatic or flank pain, males age 62, solid mass with central stellate scar, arises from collecting duct

Answer 60

oncocytoma

Question 61

asymptomatic or flank pain, dilated vessels, fat deposition, smooth muscle cells; sporadically and hereditary; females, esp during pregnancy and progesterone receptor,

Answer 61

angiomyolipoma

Question 62

most common renal tumor, peak 60 yo, smoking doubles risk, VHL in 90%, triad of hematuria, flank pain and mass; yellow orange solid, encapsulated, proximal tubule,

Answer 62

renal cell carcinoma

Question 63

autosomal dominant, hemangioma of retina, cysts of kidney, 40% get RCC, germline mutation of gene, loss of tumor suppressor gene

Answer 63

VHL

Question 64

nephroblastoma, most common renal tumor in kids, assoc'd with WAGR, Deny's Drash, and Beckwith-Weideman syndromes, encapsulated lobulated tumor mass, triphasic pattern, prognosis good with chemo and surgery

Answer 64

Wilm's tumor

Question 65

transitional carcinoma from renal pelvis,

Answer 65

urothelial carcinoma