- low PRA - high PAC - high urine free cortisol/cortisone ratio - high ACTH - AD - cortisol-producing zona fasciculata defect d/t promoter fusion causing ACTH dependent activation of aldosterone synthase on cortisol precursors

glucocorticoid-remediable aldosteronism - young female with HTN and hypokalemia - family h/o early onset HTN and hemorrhagic stroke - urinary K+ wasting - increased urinary excretion of aldosterone and 18-OH cortisol during salt loading tratamento =spironolactone and dexamethasone

- low/normal PRA - low/normal PAC - high cortisol - high urine free cortisol/cortisone ratio - mutation of 11-BHSD2

syndrome of apparent mineralocorticoid excess (SAME) causes - can be inherited - can be acquired; - black European licorice (glycyrrhizic acid) - itraconazole - ectopic ACTH - grapefruit

- low/normal PRA - low/normal PAC - low/normal cortisol - low/normal urine free cortisol/cortisone ratio - worse w/ pregnancy and spironolactone

mineralocorticoid receptor gain of function mutation (Geller’s syndrome) - severe HTN with metabolic alkalosis and hypokalemia - young women during the second and third trimester of pregnancy mineralocorticoid corticoid receptor mutation (Geller’s syndrome)

- if metabolic acidosis, compensation should be what?- - calculation

- respiratory alkalosis - ∆CO2 = ∆HCO3 x 1.2

- if metabolic alkalosis, compensation should be what? - calculation

- respiratory acidosis - ∆CO2 = ∆HCO3 x 0.6

ASN QBank Pearls - Potassium, Acid Base, Tubulointerstitial Disorders, and Nephrolithiasis Flashcards by fernanda moraes

low PRA (plasma renin activity)
high PAC (plasma aldosterone concentration)
aldosterone synthesis independent of renin levels

primary aldosteronism

How well did you know this?

Not at all

Perfectly

low PRA
high PAC
high urine free cortisol/cortisone ratio
high ACTH
AD
cortisol-producing zona fasciculata defect d/t promoter fusion causing ACTH dependent activation of aldosterone synthase on cortisol precursors

glucocorticoid-remediable aldosteronism

young female with HTN and hypokalemia
family h/o early onset HTN and hemorrhagic stroke
urinary K+ wasting
increased urinary excretion of aldosterone and 18-OH cortisol during salt loading

tratamento =spironolactone and dexamethasone

How well did you know this?

Not at all

Perfectly

low PRA
high PAC
mass > 5 cm

adrenal Ca

How well did you know this?

Not at all

Perfectly

high PRA
high PAC
unregulated synthesis

renin-secreting tumor

How well did you know this?

Not at all

Perfectly

high PRA
high PAC
atherosclerosis
hypoxia-induced

renovascular HTN

How well did you know this?

Not at all

Perfectly

low/normal PRA
low/normal PAC
high cortisol
high urine free cortisol/cortisone ratio
excess cortisol production

Cushing disease

How well did you know this?

Not at all

Perfectly

low/normal PRA
low/normal PAC
high cortisol
high urine free cortisol/cortisone ratio
mutation of 11-BHSD2

syndrome of apparent mineralocorticoid excess (SAME)

causes

can be inherited
can be acquired;
black European licorice (glycyrrhizic acid)
itraconazole
ectopic ACTH
grapefruit

How well did you know this?

Not at all

Perfectly

low/normal PRA
low/normal PAC
low/normal cortisol
low/normal urine free cortisol/cortisone ratio
hypokalemia
AD
ENaC gain of function mutation

Liddle syndrome

How well did you know this?

Not at all

Perfectly

low/normal PRA
low/normal PAC
low/normal cortisol
low/normal urine free cortisol/cortisone ratio
worse w/ pregnancy and spironolactone

mineralocorticoid receptor gain of function mutation (Geller’s syndrome)

severe HTN with metabolic alkalosis and hypokalemia
young women during the second and third trimester of pregnancy

mineralocorticoid corticoid receptor mutation (Geller’s syndrome)

How well did you know this?

Not at all

Perfectly

MR 810 mutation is activated by what medication, thereby worsening hypertension and hypokalemia

spironolactone

How well did you know this?

Not at all

Perfectly

treatment of mineralocorticoid corticoid receptor mutation (Geller’s syndrome)

often requires delivery
subsequent management includes avoidance of spironolactone

How well did you know this?

Not at all

Perfectly

hyperemic optic disks and putamen swelling are suggestive of

methanol intoxication

How well did you know this?

Not at all

Perfectly

changes in mentation as a result of methanol are present w/i the first 6-24 hours but can be the only abnormality for as long as 72-96 hours if patients have also ingested, what?

ethanol (competes for ADH)

How well did you know this?

Not at all

Perfectly

methanol metabolite

formic acid

How well did you know this?

Not at all

Perfectly

changes in mentation
HAGMA
large osmolal gap
less likely to have cerebral edema

ethylene glycol toxicity

How well did you know this?

Not at all

Perfectly

ketoacidosis (typically after patient stops drinking)
usually small osmolal gap

ethanol toxicity

How well did you know this?

Not at all

Perfectly

solvent used for lorazepam, diazepam, and phenobarbital

propylene glycol

How well did you know this?

Not at all

Perfectly

large osmolal gap
HAGMA
AKI
can progress to multisystem organ failure, if severe
usually a/w dosages above the recommended range of 0.1 mg/kg/hr and/or renal impairment

propylene glycol toxicity

How well did you know this?

Not at all

Perfectly

HTN at young age
CVA at young age
low aldosterone level
hypokalemia
metabolic alkalosis

Liddle’s syndrome

How well did you know this?

Not at all

Perfectly

hypokalemia
normo- or hypotension

Bartter’s or Gitelman’s syndromes

How well did you know this?

Not at all

Perfectly

hyperkalemia
HTN
metabolic acidosis

Gordon’s syndrome (pseudohypoaldosteronism type 2)

How well did you know this?

Not at all

Perfectly

if metabolic acidosis, compensation should be what?- - calculation

respiratory alkalosis
∆CO2 = ∆HCO3 x 1.2

How well did you know this?

Not at all

Perfectly

if metabolic alkalosis, compensation should be what?
calculation

respiratory acidosis
∆CO2 = ∆HCO3 x 0.6

How well did you know this?

Not at all

Perfectly

ACUTE respiratory acidosis, compensation should be what?
calculation

metabolic alkalosis
∆HCO3 = ∆CO2 x 0.1

How well did you know this?

Not at all

Perfectly

- CHRONIC respiratory acidosis, compensation should be what? - calculation

- metabolic alkalosis - ∆HCO3 = ∆CO2 x 0.3

- ACUTE respiratory alkalosis, compensation should be what? - calculation

- metabolic acidosis - ∆HCO3 = ∆CO2 x 0.2

- CHRONIC respiratory alkalosis, compensation should be what? - calculation

- metabolic acidosis - ∆HCO3 = ∆CO2 x 0.5

Winter's formula

PCO2 = 1.5 x [HCO3] +8 +/- 2

- large osmolal gap - normal anion gap (unless hypotension leads to lactic acidosis) - ketonemia - ketonuria

isopropyl alcohol toxicity

major metabolite of isopropyl alcohol

acetone

reason for ketonemia and ketonuria in isopropyl alcohol toxicity

major metabolites of isopropyl alcohol is acetone

when is hemodialysis indicated in isopropyl alcohol toxicity

- severe coma - hypotension, or - serum isopropanol levels \> 200 mg/dL

what is NOT indicated for isopropyl alcohol toxicity?

- ADH inhibition with fomepizole or ethanol

- weight loss drug - hyperoxaluria

orlistat

heavy metal a/w battery plant

cadmium

heavy metals a/w foundry

beryllium and lead

- heavy metal - bone disease - stones - HTN - CKD (chronic tubulointerstitial nephritis with non-nephritic proteinuria and a bland urine sediment)

cadmium

- gout - HTN - CKD (chronic tubulointerstitial nephritis with non-nephritic proteinuria and a bland urine sediment)

lead toxicity

Chinese herb may have this and it is nephrotoxic and leads to CKD (chronic interstitial nephritis)

aristolochic acid

patients exposed to this have a lifelong risk of transitional cell carcinoma especially after transplantation, where it may occur in \> 30%

aristolochic acid

what is recommended to be considered prior to transplant in patients with aristolochic acid nephropathy

bilateral nephrectomy

- recently described rejection episode requiring an increase in immunosuppression - hemorrhagic cystitis - allograft dysfunction

BK viremia

fruit a/w oxalate nephropathy, especially in patients with pre-existing CKD

star fruit

ephedra adverse effects

- HTN - stones

- normal UA - normal renal function - hypokalemia - metabolic alkalosis - HTN - what syndrome does this patient have? - what is the cause?

- apparent mineralocorticoid excess syndrome (AMES) - glycyrrhizic acid from herbal products

noni juice can cause

hyperkalemia

- nephrotic range proteinuria 2/2 MCD - minimal evidence of eosinophilia, rash, or fever

NSAIDs

lead is a cation that affects what part of the nephron

organic cation transport (OCT) system in the proximal tubule

cadmium affects what part of the nephron

proximal tubule

gold causes

nephrotic syndrome from membranous disease

platinum causes

ATN through both apoptosis and necrosis, especially in the proximal tubule

calcium oxalate stone risk factors

- hypercalciuria - increased salt intake - hypocitraturia - high sulfate and low urine - bariatric surgery (enteric hyperoxaluria)

major base in urine

citrate

best marker for dietary acid intake

sulfate excretion

single most important pathogenic factor in uric acid stone formation

low urine pH

ethylene glycol metabolites

glycolaldehyde, which is then oxidized to glycolic acid

ethanol metabolites

acetaldehyde then acetic acid, and acetyl-CoA

risk factors for cysteine stone formation

- low UOP - low urine pH - high dietary protein intake (lower risk factor)

treatment for cystinuria

- oral α-mercaptopropionylglycine   - genetic counseling of the family

what are the finding of allergic interstitial nephritis? - kidney size - skin rash - eosinophilia - nephrotic syndrome - kidney biopsy results

- large - present - present - absent - interstitial T cells

classic presentation of a penicillin- or antibiotic-induced interstitial nephritis

triad - fever - rash - eosinophilia

allergic interstitial nephritis hypersensitivity type

type 4

which syndromes are associated with granulomatous interstitial nephritis?

- tubulointerstitial nephritis and uveitis (TINU)   - sarcoidosis - granulomatosis w/ polyangiitis - Crohn’s disease

what test indicates the presence of AIN and not ATN?

positive gallium scan

initiation of what medication for a diagnosis of AIN, leads to accelerated recovery of renal function and an improved long-term renal function? and in what time period should they be given?

steroids, w/i 2 weeks

complete recovery of renal function occurs in what percentage of AIN cases, with the remaining cases developing CKD?

50%

what stain on kidney biopsy indicates the presence of collagen, and therefore, a positive stain will be indicative of a patient with interstitial fibrosis and CKD?

trichrome stain

Hansel stain is interpreted as positive once urine eosinophils represent what percentage of urine WBCs?

\> 1%

what are some of the many other causes of urine eosinophils other than AIN?

- ATN - cystitis - prostatitis, and - atheroembolic disease (AED)

- refractory hypokalemia - metabolic alkalosis, and - hyperglycemia

increased ACTH production leading to increased cortisol

Cushing disease source of excess cortisol

pituitary gland (makes ACTH)

Cushing syndrome source of excess cortisol

adrenal gland (makes cortisol)

treatment of increased ACTH production by lung cancer (more common w/ SCLC)

- treat tumor - steroid synthesis inhibitors (ketoconazole, metyrapone, and octreotide), and - MR and ENaC blockers

thyroid disease may cause hypokalemia (associated with a K+ cell shift), but not metabolic alkalosis

thyrotoxicosis

renal adverse effects of ifosfamide

- Fanconi syndrome - proximal phosphate wasting - AKI, and - nephrogenic DI

timeline of ifosfamide renal tubular dysfunction

can develop months after completing chemotherapy

tumor-produced FGF-23 will lead to

phosphate wasting and hypophosphatemia

what electrolyte disturbances develop in the setting of obstructive uropathy

hyperkalemia w/ metabolic acidosis

topiramate does what in the kidneys?

inhibits carbonic anhydrase

topiramate is a/w

proximal RTA (and distal RTA), and calcium phosphate stones

atazanavir is a/w

urinary crystals and nephrolithiasis

triamterene is a/w

urinary crystals, but NOT stones

oslistat is a/w

enteric hyperoxaluria and calcium oxalate crystals

most likely cause of urinary stones

atazanavir

why does atazanavir cause urinary stones?

poor solubility in urine

atazanavir is most soluble in what urine pH?

acidic urine, pH \< 4.5

acyclovir is a/w

urinary crystals but has NOT been a/w nephrolithiasis

which medications do NOT form urinary crystals?

- emtricitabine - tenofovir

tenofovir renal adverse effects

- proximal tubular injury w/ AKI - Fanconi syndrome

which medications are a/w hyperkalemia by blocking the epithelial sodium channel (ENaC) in the principal cell in the cortical collecting duct?

- amiloride (K+ sparing diuretic) - pentamidine (PJP ppx) - triamterene (K+ sparing diuretic) - trimethoprim

chronic linezolid treatment a/w

lactic acidosis (disrupts mitochondrial function)

acetaminophen can be associated with what in alcoholics and patients w/ poor nutrition d/t underlying glutathione and deficiencies

oxoproline acidosis

ketorolac is a/w

type 4 RTA (hyporeninemic hypoaldosteronism)

overall prevalence of biopsy-proven acute interstitial nephritis (AIN) in biopsies obtained for any clinical indication?

2-5%

prevalence of biopsy-proven acute interstitial nephritis (AIN) in patients with AKI?

10-15%

MOST common cause of acute interstitial nephritis (AIN) in the United States?

medications

MOST common cause of acute interstitial nephritis (AIN) in developing countries?

50% is d/t drugs and 50% is d/t infection

which drugs are MOST commonly associated with acute interstitial nephritis (AIN)?

antimicrobial agents (antibiotics, antivirals, antifungals, etc)

- fever - weight loss - fatigue - on broad- spectrum antibiotics - diffuse rash - palpable adenopathy - severe eosinophilia - AKI - proteinuria - rbcs on urine - wbc casts

drug rash, eosinophilia, systemic symptoms (DRESS) syndrome with granulomatous acute interstitial nephritis  (AIN)

kidney biopsy diagnostic for drug rash, eosinophilia, systemic symptoms (DRESS) syndrome with granulomatous acute interstitial nephritis  (AIN)

inflammatory infiltrate w/ numerous eosinophils, lymphocytes, and macrophages, as well as granuloma

highest diagnostic utility for drug-induced acute interstitial nephritis (AIN)

kidney biopsy showing an inflammatory interstitial infiltrate

what is a/w an increased likelihood that kidney function will improve following a course of steroid therapy?

steroids are initiated w/i 1 week of diagnosis

are CKD and ESRD complications in patients with drug-induced AIN when the offending agent is removed?

yes, both CKD (up to 50%) and ESRD (\> 5%) are common

- obese patient - doesn't feel well - AKI - health store weight loss regimen - 2-5 wbcs - 1-3 rbcs - 1-2 waxy casts

aristolochic acid (AA)

aristolochic acid (AA) has also been identified as the environmental agent underlying

Balkan-endemic nephropathy

- rapid loss of kidney function - shrunken kidneys - extensive acellular fibrosis on renal histopathology - often develop ESRD and uroepithelial malignancies

Balkan-endemic nephropathy

- pulmonary, eye, skin, and renal involvement - renal biopsy findings of a cellular infiltrate and granuloma

sarcoidosis w/ AIN

eye and kidney involvement w/o granuloma

Sjögren’s syndrome

- uveitis and skin involvement - interstitial nephritis w/ granuloma

TINU

- malaise - fatigue - low-grade fever - pulmonary and renal masses - lymphoplasmacytic infiltrate in lung and kidney tissue

IgG4 disease

IgG4 disease treatment

steroids, highly responsive

- marked plasma cell infiltrate that produces expansile destructive lesions - autoimmune pancreatitis - renal disease w/ deposits in the tubules - classical complement pathway is activated - eosinophilia - low-grade ANA titers

IgG4-related systemic disease

- institution/reinstitution of HAART - pathological inflammatory response in various organs, including kidneys - mononuclear infiltration of renal interstitium; essentially a form of AIN

immune reconstitution inflammatory syndrome (IRIS)

- Fanconi syndrome - AKI - light chain crystals in proximal tubules

light chain-induced acute tubular injury

fractured, glassy casts with associated interstitial reaction/giant cell formation (engulfing casts)

light chain cast nephropathy associated with multiple myeloma

renal histology demonstrates infiltration of the renal interstitium by lymphoma cells

acute infiltrative interstitial nephritis

positive urine anion gap in the setting of nongap metabolic acidosis supports the diagnosis of

renal tubular acidosis

urine pH is often inappropriately alkaline in the face of acidosis and is a time-honored “hallmark” of

DISTAL renal tubular acidosis

with chronic metabolic acidosis, regardless of cause, what increases in response to the systemic acidosis?

ammoniagenesis

- diabetic nephropathy - recent decline in eGFR - evidence of volume overload - hyperkalemia, and - nongap metabolic acidosis

type 4 RTA; hyporeninemic hypoaldosteronism

- seen in patients of Asian or Mexican descent - severe pain during an acute attack d/t sudden shift of K+ from the extracellular fluid space into the intracellular compartment - normal total body potassium content - large transcellular K+ gradient leads to hyperpolarization of the cell

hypokalemic periodic paralysis occurring in a/w hyperthyroidism

- decreased total body potassium d/t renal potassium wasting - both intracellular and extracellular potassium are decreased - transcellular K+ gradient is less steep - cell hyperpolarization is less severe - less symptomatic

- paint thinner abuse - toluene-induced type 1 (distal) RTA

toluene is metabolized to

hippuric acid

nonreabsorbable anion metabolite of toluene that leads to increased distal Na+ delivery, and mild volume contraction

sodium salt, sodium hippurate

explanation of normal serum K+ level w/ depleted total K+ stores in the setting of DKA

they are reduced d/t an osmotic diuresis, but serum levels remain normal because K+ shifts out of cells  d/t insulin deficiency and increased osmolality

expected uric acid level in the setting of high-dose aspirin therapy or aspirin overdose

low d/t increased renal excretion

should you test European American adults with sporadic (nonfamilial) forms of FSGS for causative mutations?

probably

- females with one X-linked mutation in the COL4A5 gene are carriers - typically develop mild disease because they possess another (normal) X chromosome - what renal disease?

Alport syndrome

Alport syndrome mutation and location

COL4A5 mutation on X chromosomes

- single mutation in either COL4A3 or COL4A5 gene - hematuria - preserved kidney function

thin basement nephropathy

progression of kidney disease strongly relates to the presence of

risk variants in the APOL1 with AR inheritance

rare but a dire electrolyte abnormality of high-dose barbiturate coma therapy in order to reduce ICP in traumatic brain injury patients

- hypokalemia - and/or rebound hyperkalemia

- hypokalemia in absence of acidosis - weakness precipitated by carbohydrate load and exercise - low urinary K+

familial periodic paralysis

- YOUNG, usually female patients - very common in ASIANS - signs/symptoms of thyrotoxicosis - often have a NECK MASS - LOW TSH - high T3 or T4

thyrotoxic periodic paralysis

which biochemical abnormality takes place first to maintain normal phosphorus levels the setting of progressively worsening CKD?

FGF-23

ASN QBank Pearls - Potassium, Acid Base, Tubulointerstitial Disorders, and Nephrolithiasis Flashcards

(135 cards)