renal

Question 1

multicystic dysplastic kidney

Answer 1

hydronephrosis, palpable kidney
usually unilateral with normal function from other kidney but need vcug/renal scan to rule out contralateral anomalies. affected kidney usually atrophies. no treatment, just monitor with renal us and creatinine. no risk of htn or tumor. Will see large cysts by 20w

Question 2

wilms tumor

Answer 2

most common nephroblastoma, abdominal/flank mass with hematuria, sometimes htn. rarely presents in neonate

Question 3

renal vein thrombosis

Answer 3

most common blood clot
hematuria, flank mass, thrombocytopenia
some have htn

Question 4

prune belly syndrome (Eagle Barrett Syndrome)

Answer 4

Usually caused by urethral obstruction, most common in boys.
Increased bladder pressure causes triad of #1 cryptorchidism, #2 poor adbdominal musculature, #3 dilated urinary tract
Can include renal dysplasia, hydroureter. +/- malro, cardiac anomalies, compression of vessels to lower extremities.
oligohydramnios –> pulm insufficiency
should be on uti ppx for poorly functioning bladder

Question 5

puv

Answer 5

most still progress to ESRD in first 10y. abnormal bladder function –> persistent UTIs –> renal scarring. Dx: VCUG with spinning top deformity (proximal urethra dilated, distal constricted), VU reflux, thickened bladder

Question 6

autosomal recessive polycystic kidney disease

Answer 6

ARPKD, mc presents in neonatal period. Assc with congenital hepatic fibrosis. Enlarged kidneys and oligo in utero. Urine concentrating defect. B/l flank mass. +/- renal fx impaired. cysts not usually present in neonatal period. may have hptn, hypo Na. 50% develop renal failure in first decade.

Question 7

vasopressin

Answer 7

released from post pit. Binds to receptors in kidney –> camp –> aquaporins. In premature babies, poor production of camp.
x-linked: v2r mutation, receptor unable to bind AVP
secretion most impacted by intravascular status, then osmolarity
v1 and v2 receptors. v1 in vessels and platelets, v2 only in kidneys
acts on distal tubule and collecting ducts

Question 8

renal embryology

Answer 8

can see kidney at 9 wks, bladder at 15th wk

Question 9

loop diuretics

Answer 9

bumetadine and furosemide

act on thick ascending LOH on Na K 2Cl transporter

Question 10

thiazide diuretics

Answer 10

hydrochlorothiazide
act on distal tubule on Na Cl cotransporter
lead to increased calcium reabsorption

Question 11

aldosterone

Answer 11

spironolactone

weak diuretic pootassium sparing agent

Question 12

fena

Answer 12

S(Cr)xU(Na) / S(Na) X U(Cr)

Question 13

RTA

Answer 13

all non anion gap metabolic acidosis
I: distal: poor secretion of H+, urine is basic around 6-7. Associated with nephrocalcInosis
II: proximal: poor reabsorption of HCO3, can compensate with distal secretion of H+. Urine is normal around 5.5. Assc w fanconi syndrome and many electroylyte abnormaltiies
IV: aldosterone: poor response to aldosterone or decreased production. Hyperkalemia and acidosis.

Question 14

bladder extrophy

Answer 14

assc with inguinal hernia

Question 15

cloacal extrophy

Answer 15

anal atresia, omphalocele,

Question 16

what is a normal urine osmolality? What is pt vs t vs adult concentration ability?

Answer 16

75-300 mOsm/k
Pt: 500
T: 600
Adult: 1500

Question 17

What is normal urine spec gravity?

Answer 17

1.003 - 1.102

Question 18

what is osmotic reflection coefficient

Answer 18

(s), a measure of endothelial permeability. The higher ‘s’, the less permeable.s062020

Question 19

What is the most common cause of congenital hydronephrosis?

Answer 19

#1: Ureteropelvic Junction obstruction (44%) - can be associated with other syndromes
#2: Ureterovesical junction obstruction (21%) - dilation of ureter
#3: PUV (9%) - thickened bladder wall

Question 20

most common cause of hypertension

Answer 20

renovascular disease, specifically renal artery thrombosis

Question 21

what do patients with APCKD usually die of?

Answer 21

respiratory failure from pulm hypoplasia. in utero, little urine production –> oligo. on US, large, sometimes non-dysplastic kidneys. associated with congenital hepatic fibrosis. (VS multicystic dysplastic kidney, usually unilateral and can see large cysts on US)

Question 22

what is normal renal development?

Answer 22

~9w: first nephron is formed, start making urine
~ 20 w: 5ml/hr of urine, 95% of AF is urine, 1/3 of enphrons present
~36 w: all nephrons are present
~ 40w: 50ml/hr of urine

Question 23

what is lowe’s syndrome

Answer 23

‘the OCRR is beLOWE the bridge” oculo, cerebral renal syndrome:
cataracts, glaucoma
hypotonia, areflexia, MR
renal tubular dysfunction, proteinuria, aminoacidemia, nephrotic syndrome
cryptoorchidism
dx: elevateed AFP

Question 24

What is Bartter syndrome

Answer 24

loop of henle abnormality, inability to reabsorb so hypo natremia, hypocalcemia, hypokalemia with dysmorphic features including triangular face. alkalotic.

Question 25

compare APCKD to MCDK

Answer 25

apckd: don’t see cysts as early on US, usually bilateral, have htn (maybe intrinisic disease or increased ADH), hyponatremic, assc with hepatic fibrosis (65%). 25% die, 25% have poor growth.
mcdk: usually can see multiple large cysts by 20w, unilateral, no assc with malignancy, hepatic fibrosis or htn. Usually doesn’t require intervention

Question 26

what is urine osm and Na concentration and FENA score in prerenal vs renal failure?

Answer 26

pre-renal: urine osm high, Na low (b/c kidney tries to reabsorb to help with volume status), FeNa <2%
renal: urine osm low (cannot concentrate), Na high, FeNA >2%

Question 27

What is the formula for free water deficit vs Na deficit?

Answer 27

Free water: change desired x wt x 4ml

Na deficit: change desires x at x 0.6

Question 28

Where do the different diuretics and adh act on the nephron?

Answer 28

Acetazolamide : PCT
Loop diuretics : ascending loop of henle 
Thiazide : DCT
Spironolactone : distal DCT 
adh: CD

Question 29

when does nephrogenesis happen?

Answer 29

5-35 weeks.

CO to kidneys drops during birth (think only need to pee once on dol1) and then picks up in the next two months