21 - Pharm, Peds, PCKD Flashcards
Cockroft Gault formula for estimating GFR (Males and females)
(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
do you need to adjust loading dose, maintenance dose, or both for CKD pts?
maintenance
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
32.5%
what % of people w/ AD PKCD end up w/ renal failure?
50%
which gene mutation of AD PKCD accounts for most cases?
PKD1
AD PKCD is due to mutation in gene for what protein?
polycystin 1 or 2
pathogenesis of PKCD
epithelial dedifferentiation
altered cell polarity
excessive fluid secrection - vasopression via apical AQP channels
diagnostic criteria for AD PKCD
> 4 cysts per kidney if age >60
renal manifestations of AD PKCD
cyst hemorrhage > hematuria and flank pain
HTN
UTIs and kidney stones
extrarenal manifestations of AD PKCD
liver cysts mitral valve prolapse colonic diverticulosis hernias 5% have intra cranial aneurysms **serious one
tx for AD PKCD
HTN control
stronger antimicrobials for UTIs
aggressive hydration (theoretically works)
kidney transplant - wont recur
AR PKCD
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
acquired cystic dz
seen in ESRD pts, inc incidence w/ time on dialysis
no extrarenal manifestations
medullary cystic dz
rare
ESRD in first 3 decades of life
AD
medullary sponge kidney disease
relatively common developmental defect - dilated collecting ducts complicated w/ stones and hematuria and infxn
tuberous sclerosis
inherited
hamartomas in multiple organs
angiomyolipomas in kidney
MC causes (broad) of pediatric ESRD
glomerulonephritis and vasculitis
MCC primary GN causing ESRD in kids
focal segmental glomerulosclerosis
FSGS in kids
presents as nephrotic syndrome, but poorly responsive to steroid therapy
50% reach ESRD in 10 yrs
often recurs in transplants (circulating factor)
Henoch Schonlein Purpura
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
what happens in bilat renal aplasia?
potter’s facies, hypoplastic lungs, oligohydramnios, ESRD
test for posterior urethral valves
VCUG (voiding cysto urethrogram
vesicoureteral reflux (VUR) & reflux nephropathy
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
key hole sign on prenatal ultrasound
lower urinary tract obstruction (LUTO)
