21 - Pharm, Peds, PCKD

Question 1

Cockroft Gault formula for estimating GFR (Males and females)

Answer 1

(140-Age)(lean body mass in kg)/(72*serum creatinine mg/dl)
for girls - mult by 0.85

lean body mass in males - 2.3(height in inch - 60)+50
females - 2.3(height in inch - 60)+45.5

Question 2

do you need to adjust loading dose, maintenance dose, or both for CKD pts?

Answer 2

maintenance

Question 3

what is maintenance dose (% of nl dose) for drug 75% excreted by kidney in pt w/ Cr clearance of 10 ml/min? assume nl cr clear of 100 ml/min

Answer 3

32.5%

Question 4

what % of people w/ AD PKCD end up w/ renal failure?

Answer 4

50%

Question 5

which gene mutation of AD PKCD accounts for most cases?

Answer 5

PKD1

Question 6

AD PKCD is due to mutation in gene for what protein?

Answer 6

polycystin 1 or 2

Question 7

pathogenesis of PKCD

Answer 7

epithelial dedifferentiation
altered cell polarity
excessive fluid secrection - vasopression via apical AQP channels

Question 8

diagnostic criteria for AD PKCD

Answer 8

> 4 cysts per kidney if age >60

Question 9

renal manifestations of AD PKCD

Answer 9

cyst hemorrhage > hematuria and flank pain
HTN
UTIs and kidney stones

Question 10

extrarenal manifestations of AD PKCD

Answer 10

liver cysts
mitral valve prolapse
colonic diverticulosis
hernias
5% have intra cranial aneurysms **serious one

Question 11

tx for AD PKCD

Answer 11

HTN control
stronger antimicrobials for UTIs
aggressive hydration (theoretically works)
kidney transplant - wont recur

Question 12

AR PKCD

Answer 12

presents in childhood (earlier > worse prognosis)
infancy - oligohydramnios, neonatal HTN, potter facies, pulm hypoplasia > death w/in days
kids - HTN, liver cysts > hepatic fibrosis > portal HTN

Question 13

acquired cystic dz

Answer 13

seen in ESRD pts, inc incidence w/ time on dialysis

no extrarenal manifestations

Question 14

medullary cystic dz

Answer 14

rare
ESRD in first 3 decades of life
AD

Question 15

medullary sponge kidney disease

Answer 15

relatively common developmental defect - dilated collecting ducts complicated w/ stones and hematuria and infxn

Question 16

tuberous sclerosis

Answer 16

inherited
hamartomas in multiple organs
angiomyolipomas in kidney

Question 17

MC causes (broad) of pediatric ESRD

Answer 17

glomerulonephritis and vasculitis

Question 18

MCC primary GN causing ESRD in kids

Answer 18

focal segmental glomerulosclerosis

Question 19

FSGS in kids

Answer 19

presents as nephrotic syndrome, but poorly responsive to steroid therapy
50% reach ESRD in 10 yrs
often recurs in transplants (circulating factor)

Question 20

Henoch Schonlein Purpura

Answer 20

young kids
palpable purpura (esp lower extremities)
arthralgia
abd pain, bloody diarrhea, intussusception
nephritis w/ gross or micro hematuria

poor prognosis if nephrotic syndrome or HTN, rarely progresses to ESRD

basically a systemic form of IgA nephropathy

Question 21

what happens in bilat renal aplasia?

Answer 21

potter’s facies, hypoplastic lungs, oligohydramnios, ESRD

Question 22

test for posterior urethral valves

Answer 22

VCUG (voiding cysto urethrogram

Question 23

vesicoureteral reflux (VUR) & reflux nephropathy

Answer 23

mostly in caucasian girls
unilateral or bilateral
may present w/ UTI - abx prophylaxis may preserve fx
may be silent and present w/ HTN, anemia, CKD/ESRD later on
dx w/ VCUG

Question 24

key hole sign on prenatal ultrasound

Answer 24

lower urinary tract obstruction (LUTO)

Question 25

prune belly syndrome

Answer 25

includes LUTO, absent abd muscles, undescended testicles

males

Question 26

congenital nephrotic syndrome

Answer 26

primary - due to mutation in nephrin

secondary - due to congenital infextions (CMV, hep B, rubella, syphilis, etc)

Question 27

alport syndrome

Answer 27

assoc w/ sensorineural deafness and or ocular lesions
mutation in type 4 collagen
x linked

Question 28

wilm’s tumor

Answer 28

most common malignancy of urinary tract in kids
abnormal prolif of metanephric blastema
90% present before age 7, painless abd tumor
overall survival >85%

Question 29

HUS - typical vs atypical

Answer 29

typical - assoc w/ diarrhea (e coli O157:H7, shigella, etc), usually acute renal failure w/ recovery

atypical - no diarrhea. Strep pneumo or cyclosporine, factor H deficiency
multi organ involvement - GI pancreas CNS

Question 30

almost 2/3 of pediatric ESRD caused by ____ and ___

Answer 30

CAKUT (congen anom of kidney and urinary tract)

GN