Week 1 Flashcards
mesoderm derivative that gives rise to kidney and gonads
intermediate mesoderm
mesoderm derivative that gives rise to head and somite
paraxial mesoderm
mesoderm derivative that gives rise to splanchnic, somatic, and extraembryonic
lateral mesoderm
transitory, non-functional kidney
pronephros (cervical nephrotomes)
functional during development of permanent kidney, duct contributes to developing testis, male genital system, and vestigial remnants in female
mesonephros
permanent kidney in 5th week, produces urine at 11-13 weeks
metanephros
ureteric bud forms what structures?
ureter, pelvis, calyces
polyhydramnios
less swallowing, excess amniotic fluid due to esophageal or duodenal atresia
oligohydramnios
less urine output, shortage of amniotic fluid due to failure of kidneys to develop normally
derivative of ureteric bud
collecting tubules
derivative of nephrogenic mesenchyme
nephron and Bowman’s capsule
expressed by mesenchyme, enables tissue to respond to induction by ureteric bud
WT1
receptor that mediates programmed cell death, localized to mesenchyme during ureteric bud branching
Angiotensin II receptor
division of cloaca by urorectal septum into dorsal rectum and ventral urogenital sinus
bladder formation
mesonephric ducts and attached ureteric buds intercalated into posterior wall
exstrophy of the bladder
dysregulation of fetal adrenal gland, hypertrophy of adrenal cortex, overproduction of dehydroepiandrosterone, masculinization of female genitalia
congenital virilizing hyperplasia (CAH)
bilateral renal agenesis, due to oligohydramnios, lung hypoplasia, clubbed feet, bow legs
Potter sequence
nephroblastoma, most common primary renal tumor affecting children, 4th most common pediatric tumor, mutation in WT-1, poorly formed tubules
Wilm’s tumor
pelvic kidney, often pancake shaped or horseshoe kidney where inferior poles fused and ascent blocked by inferior mesenteric artery
ectopic kidneys
tube open at both ends, may cause urine to drain from umbilicus
urachal fistula
exposure and protrusion of posterior wall of bladder through abdominal wall
exstrophy of the bladder
affects laminins, nephrotic syndrome and ocular abnormalities, proteinuria, hypoalbuminemia,
Pierson syndrome
defect in type 4 collagen, males, uremic syndrome to end stage renal failure, treat with dialysis or transplant
Alport disease
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
congenital nephrotic syndrome
normal value for RBF
1.2 L/min, 20-25% of cardiac output
normal value for RPF
660 mL/min
normal value for GFR
125 L/min
hydrostatic pressure, usually 60 mm Hg
Pgc
increases blood flow resistance, decreases renal blood flow, and decreases glomerular capillary pressure
afferent arteriole constriction
increases glomerular capillary pressure, increases renal blood flow, increases peritubular capillary pressure
afferent arteriole dilation
increases blood flow resistance and glomerular capillary pressure, net increase in GFR, decrease RBF
efferent constriction
decreases glomerular capillary pressure, increases RBF
efferent dilation
automatically adjusts resistance of afferent arteriole, contracts in response to increased blood pressure
myogenic response
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
tubuloglomerular feedback mechanism
structural support, modulates glomerular filtration, phagocytic activity, paracrine substances
intraglomerular mesangial cells
near vascular pole and macula densa, produce renin
extraglomerular mesangial cells
- increased sympathetic nerve activity
- decreased renal blood perfusion
- decreased sodium to macula densa
activate release of renin
- arteriolar vasoconstriction
- sodium and water retention
- aldosterone secretion
- negative feedback, inhibits renin secretion
angiotensin II produces these responses
systemic arteriolar dilation, decreased sodium and water reabsorption, decreased aldosterone secretion
ACE inhibitor
maintains plasma calcium by intestinal, renal, and bone reabsorption of calcium
vitamin D
stimulated by hypoxia, anemia, and decreased renal blood flow
EPO production
osmoreceptors signal hypothalamus to secrete this, which stimulates water reabsorption to decrease ECF osmolarity and concentrate urine
ADH (vasopressin)
de novo synthesis of glutathione, stimulates amino acid trasnport into the cell
gamma glutamyl cycle
alpha ketoglutarate –> glutamate –> frees ammonia for urea cycle
transamination
dietary protein, transamination and glutamate dehydrogenase, peroxisome, mitochondria, glutaminase, purine/pyrimidine deamination, glycine cleavage, intestinal bacteria
sources of ammonia
glutamate (glutamate dehydrogenase and transaminases), glutamine (carries 2 amino groups)
precursors of nitrogen in urea
decreased TCA cycle in the brain, depleted glutamate, decreased neurotransmitter synthesis, can lead to coma and brain damage
build up ammonia and depleted alpha ketoglutarate
urea cycle requires how many ATP?
4
ATP + N-acetylglutamate, with ornithine forms citrulline
carbamoyl phosphate synthetase
converts arginine to urea
arginase
regulatory molecule of carbamoyl phosphate synthetase, can be increased by high levels of arginine intake
N-acetylglutamate
lack of carbamoyl phosphate synthetase, newborn lethargic, vomits, fatal due to high ammonia, treat with arginine to activate N-acetylglutamate
type 1 hyperammonemia
lacking ornithine transcarbamoylase, X linked, most common urea cycle disease, treat with low protein diet and ammonia detox
type 2 hyperammonemia
hyperammonemia, coma, acidosis, ataxia, treat with carbamoyl glutamate to activate CPS1
N-acetylglutamate synthetase deficiency
cofactor of carbamoyl phosphate synthetase, represents 15-25% of mitochondria matrix protein
N-acetylglutamate
converts xanthine into uric acid
xanthine oxidase
elevated PRPP –> enhanced purine synthesis –> loss of feedback inhibition –> partial deficiency of HGPRT (salvage path) –> increased levels of uric acid, crystallization in joins
gout
total deficiency of HGPRT, X linked, self mutilation/retardation
Lesch Nyhan syndrome
suicide inhibitor used to treat gout, binds xanthine oxidase
allopurinol
majority asymptomatic or flank pain, males age 62, solid mass with central stellate scar, arises from collecting duct
oncocytoma
asymptomatic or flank pain, dilated vessels, fat deposition, smooth muscle cells; sporadically and hereditary; females, esp during pregnancy and progesterone receptor,
angiomyolipoma
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,
renal cell carcinoma
autosomal dominant, hemangioma of retina, cysts of kidney, 40% get RCC, germline mutation of gene, loss of tumor suppressor gene
VHL
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
Wilm’s tumor
transitional carcinoma from renal pelvis,
urothelial carcinoma
