Electrolytes Flashcards
HypoK alkalosis causes?
If high BP: - high aldo low renin: Conn's, GRA - low aldo low renin: Liddle, AME, Licorice If normal BP: - If FECl < 0.5%: extrarenal hypoVol - If FECl > 0.5%: Bartter / gitelman
HyperK acidosis causes?
if high BP: PHA2 if normal BP: - high aldo high renin: PHA1 - low aldo high renin: adrenal insufficiency, aldo biosynthesis deficiency - low aldo low renin: DM, CNI, NSAID
bartter types and features?
BS1: NKCC; hypercalciuria, pre-natal onset
BS2: ROMK; hypercalciuria, prenatal onset
BS3: ClCKNB; hypoMg, first decade
BS4: Barttin; SNHL
BS5: CaSR overactivation cause FHH
All: reduce Cl resorption -> TGR reduce -> increase PGE2 -> increase RAAS
bartter MOI?
AR
bartter Tx?
NSAID (indomethacine 1-3mg/kg/d)
KCl
spirolactone but watch out for hypoVol
gitelman MOI?
AR
bartter symptoms?
hypoK, alkalosis, hypoVol, polyuria, hypercalciuria and nephrocalcinosis if BS1-2
gitelman cause?
SLC12A3 inactivation mutation -> loss of NCCT function
gitelman features?
hypoK, alkalosis, hypoMg, polyuria, chondrocalcinosis, sclerochoroidal calcification
gitelman ddx?
BS3, HNF1B, EAST, Sjogren, thiazide use
gitelman Tx?
KCl and Mg supp, spirolactone if severe
features of EAST?
Epilepsy, Ataxia, SNHL, tubulopathy (same as gitelman)
Liddle MOI?
AD
Liddle cause?
mutation of overactive ENaC
Liddle ddx?
GRA, AME, Licorice
AME cause?
11 beta hydroxyvitamin dehydrogenase deficiency
reduce cortisol conversion to cortisone thus activate MR
AME MOI?
AR
AME feature?
hypoK alkalosis high BP low aldo low renin
AME Ix?
increase tetrahydrocortisol (THF) or allo-THF to tetrahydrocortisone (THE) level genetic test
AME Tx?
spirolactone / amiloride
GRA MOI?
AD
GRA feature?
Early HT, hypoK, alkalosis, high BP, low renin, high aldo
prone to ICH
GRA cause?
CYP11B1 (11beta hydroxylase for aldo) promoter fuse with CYP11B2 (aldo synthatase) on Ch8 to cause ACTH induced Aldo production
GRA Ix?
increased hybrid steroid (18 hydroxycortisol and 18 oxocortisol)
GRA Tx?
Pred 2.5 - 5mg daily +/- spirolactone / amiloride
PHA1 MOI?
AR or AD
PHA1 cause?
ENaC defect, acquired form from obstructive uropathy +/- pyelonephritis
PHA1 features?
hyperK, acidosis, normal BP, high renin high aldo, miliary rash from high Na sweet, CF like high salt lung secretion
PHA1 Tx?
NS IVF, NaCl and NaHCO3 replacement, Na resin
PHA2 MOI?
AD
PHA2 cause?
WNK4 inactivating mutation, disinhibit NCCT and ROMK function
WNK1 activating mutation, inhibit WNK4
acquired form by CNI
PHA2 features?
hyperK from birth or later, NAGMA, low renin and aldo
PHA2 Tx?
Thiazide
NDI MOI?
XR, female carrier variable severity
rarely AR / AD
NDI cause?
CKD hypoK, hyperCa SCD malnutrition demeclocycline, Li use (block by amiloride) pregnancy AVPR2 mutation AR / AD AQP2 final effector protein
NDI features?
first week of month polyuria, FTT, vomiting
dystrophic calcification in BG
ADHD due to drinking?
NDI Ix?
low urine osmo with hyperNa
DDAVP test (limit intake Vol to UO to avoid rapid hypoNa), 0.3mcg/kg IV observe 2 hrs
Uosmo > 800 mOsm/kg normal, remains < 200 diagnostic
infant may < 800 but should > 300
AR / AD still has AVPR2 thus will have BP drop (vasodilatation) and release vWF
NDI ddx?
Central DI / habitual polydipsia (ddx by DDAVP normal response)
secondary NDI: Bartter (with polyhydramnios), AME (HT)
NDI Tx?
minimize osmotic load by low protein and caloric intake
thiazide (HCT 1-2mg/kgQ12)reduce salt reabsorption -> mild hypoVol -> increase fluid reabsorpton in PT
+/- amiloride to control hypoK
COXi reduce UO
NSIAD cause?
gain of function mutation of AVPR2