Nephrology Pearls of Wisdom Flashcards
tuberous sclerosis genetics
AD
medication class that slows tumor growth in tuberous sclerosis
mTOR inhibitors
what are the mTOR inhibitors?
- sirolimus (aka rapamycin)
- everolimus
- temsirolimus
renal manifestation of tuberous sclerosis
- renal angiomyolipomas
- RCC (very rare)
Fabry disease genetics
XL
Fabry disease mutation
GLA mutation
skin manifestation seen in Fabry disease
angiokeratomas
enzyme defect causing Fabry disease
a-galactosidase A
finding seen on EM in Fabry disease
Zebra bodies
Zebra bodies can also be seen on patients taking what medication?
hydroxychloroquine
treatment for Fabry disease
ERT (enzyme replacement therapy)
MCD age distribution
children and elderly (bimodal distribution)
what should be ruled out in patients with MCD?
- NSAID use
- lymphoma
- lithium use
if AIN and AKI think of
NSAID use
rate of relapse in MCD
50%
what other renal manifestation is also commonly seen in patients with MCD?
ATN
ADPKD1 compared to ADPKD2
occurs earlier and more ESRD
PKD1, which chromosome?
16
PKD2, which chromosome?
4
polycystin proteins affect what?
cilium
extrarenal manifestations of ADPKD
- brain aneurysms
- diverticula
- abdominal wall hernias
- MVP
preferred bp medication for PKD
ACEI
PKD medication to slow cyst growth
vaptans
West African Sub-Saharan gene that protects against trypanosomes
APOL1
2 variants of APOL1
G1 and G2
2 high-risk alleles of APOL1 increase risk of
- HIV nephropathy
- sickle cell nephropathy
- ESRD from FSGS
- hypertensive nephrosclerosis
2 high-risk alleles of APOL1 increase what in ESRD patients?
survival
what % of African Americans carry 2 high risk alleles
14%
MCC of AIN
abx, namely B-lactams and sulfa-based antibiotics
AIN from abx have what w/i infiltrate?
eosinophils
AIN from NSAIDs does NOT have what?
eosinophils
what improves short- and long-term outcome if administered early in AIN?
steroids
rash is seen in AIN when caused by
abx
in AIN, which medication class do you NOT see rash?
PPIs
aside from AIN, other manifestations seen in IgG4-related disease
- pancreatitis
- retroperitoneal fibrosis
- masses in other organs
aside from AIN, other manifestations seen in sarcoidosis
- hypercalcemia
- hypercalciuria
monoclonal gammopathy of renal significance (MGRS)
- AL amyloidosis
- is usually what type?
lambda
monoclonal gammopathy of renal significance (MGRS)
- AL amyloidosis
- what size fibrils?
10 nm
monoclonal gammopathy of renal significance (MGRS)
- light-chain deposition disease (LCDD)
- is usually what type?
kappa > lambda
monoclonal gammopathy of renal significance (MGRS)
- light-chain deposition disease (LCDD)
- staining?
tubular light chain staining
monoclonal gammopathy of renal significance (MGRS)
- light-chain deposition disease (LCDD)
- appearance on biopsy?
nodular, looks like diabetic KW disease
monoclonal gammopathy of renal significance (MGRS)
- cast nephropathy
- usually what type?
kappa = lambda
monoclonal gammopathy of renal significance (MGRS)
- cast nephropathy
- what’s seen on LM?
fractured casts
monoclonal gammopathy of renal significance (MGRS) can present w/?
Fanconi syndrome (proximal tubulopathy)
in monoclonal gammopathy of renal significance (MGRS), urine dipstick doesn’t detect what?
light chains (Bence Jones proteins)
serum [Na+] ~
(TBNa+ + TBK+)/TBW
- hyponatremia
- hypotonic
- hypERvolemic
- urine Na+ < 20 mmol/L
- heart failure
- liver failure
- hyponatremia
- hypotonic
- hypERvolemic
- urine Na+ > 20 mmol/L
- renal failure
- hyponatremia
- hypotonic
- EUvolemic
- urine Na+ > 20 mmol/L
- urine Osm > 100
- SIADH
- adrenal insufficiency
- hypothyroidism
- stress
- drug use
- hyponatremia
- hypotonic
- EUvolemic
- urine Na+ > 20 mmol/L
- urine Osm < 100
- primary polydipsia
- low solute intake
- hyponatremia
- hypotonic
- EUvolemic
- urine Na+ > 20 mmol/L
- variable urine Osm
reset osmostat
- hyponatremia
- hypotonic
- hypovolemia
- urine Na+ > 20 mmol/L
- salt wasting nephropathy
- hypoaldosteronism
- active diuretic use
- hyponatremia
- hypotonic
- hypovolemia
- urine Na+ < 20 mmol/L
- extrarenal sodium loss (GI)
- remote diuretic use
- hyponatremia
- hypertonic
- hyperglycemia
- mannitol
- hyponatremia
- normo-osmolar
- hyperlipidemia
- HLD can be 2/2 lipoprotein X a/w cholestatic or obstructive jaundice
presentation of D-lactic acidosis
- AMS
- ataxia
lactic acid level in D-lactic acidosis
normal, because they only measure L-lactate
cause for D-lactic acidosis
colonic bacteria exposed to glucose, usually from blind loop
tx for D-lactic acidosis
complex carbohydrate diet and antibiotics
vbg vs abg
- pH in vbg compared to abg
- pCO2 in vbg compared to abg
- HCO3 in vbg compared to abg
- pO2 in vbg compared to abg
- lower by 0.04
- higher by 4
- higher by 1
- lower
MCC of serum osmolal gap
ethanol
osmolal gap and AG in isopropyl alcohol
- increased osmolal gap
- NO AG (unless shock develops leading to lactic acidosis)
in ICU patient w/ AG consider what possible cause
propylene glycol from iv gtts
in ER patient w/ AG consider what possible causes
- salicylate
- EG
- methanol
- metformin intoxication
sglt2 inhibitor w/ AG, think of
euglycemic DKA
what elements are mismeasured as Cl-?
- bromide
- iodide
high unmeasured cation
lithium
high unmeasured cation in MM
gammaglobulins (severe hypergammaglobulinemia)
elements that are unmeasured cations
- Ca++
- Mg++
mechanism of PPI causing hypomagnesemia
decreased intestinal Mg absorption
urine FeMg in PPI use
low
hypomagnesemia causes
- hypoparathyroidism –> hypocalcemia
- K+ wasting
medications that cause renal wasting of hypomagnesemia
- aminoglycosides
- cisplatin
- cetuximab
- loop diuretics
- thiazide diuretics (except amiloride)
syndrome that leads to hypomagnesemia
Gitelman syndrome
causes of Fanconi syndrome
- tenofovir (+/- nephrogenic DI)
- ifosfamide (+/- nephrogenic DI)
- cisplatin
- MM
adults need w/u for what when Fanconi syndrome is diagnosed?
paraproteinemia
what do you see on labs w/ Fanconi syndrome?
- renal K+, PO4, and urate wasting
- aminoaciduria
what other renal abnormality can be seen w/ Fanconi syndrome?
proximal, type 2, RTA
magnesium in Fanconi syndrome
normal
HAGMA in elderly woman
5-oxoproline (pyroglutamic acid) d/t acetaminophen use
HAGMA from sglt2 inhibitor
severe ketoacidosis w/ euglycemia
HAGMA from metformin
D lactic acidosis
HAGMA from linezolid
lactic acidosis
HAGMA from diethylene glycol
- brake fluid
- cranial nerve abnormalities
hypomagnesemia causes
- UMg < 20
- FeMg < 2.5
- PPI
- diarrhea
hypomagnesemia
- UMg > 20
- FeMg > 2.5
- what do you check next?
check 24-hour urine Ca++
hypomagnesemia causes
- UMg > 20
- FeMg > 2.5
- if 24-hour urine Ca++ < 150
- thiazide diuretics
- Gitelman syndrome
hypomagnesemia causes
- UMg > 20
- FeMg > 2.5
- if 24-hour urine Ca++ > 250
- loop diuretics
- Bartter syndrome
- cisplatin
hypokalemia causes
- low/normal volume state
- low bicarbonate
- diarrhea
- laxative use
- toluene
- RTA
- NAGMA
hypokalemia causes
- low/normal volume state
- low magnesium
- familial hypomagnesemia
hypokalemia causes
- low/normal volume state
- urine Cl- low
- vomiting
hypokalemia causes
- low/normal volume state
- urine Cl- high
- urine Ca++ LOW
- thiazide diuretics
- Gitelman syndrome
hypokalemia causes
- low/normal volume state
- urine Cl- high
- urine Ca++ HIGH
- loop diuretics
- Bartter syndrome
hypokalemia causes
- high volume state
- LOW renin and LOW aldosterone
- Liddle syndrome
- apparent mineralocorticoid excess (AME)
- licorice
hypokalemia causes
- high volume state
- LOW renin and HIGH aldosterone
- primary hyperaldosteronism
- Cushing
- glucocorticoid remediable aldosteronism (GRA)
hypokalemia causes
- high volume state
- HIGH renin and HIGH aldosterone
- malignant HTN
- renal artery stenosis (RAS)
- renin secreting tumor
tolvaptan for hyponatremia
- start only in hospital
- do NOT fluid restrict
- avoid in liver disease
- need HIGHER doses in CHF
tolvaptan effect in ADPKD
- slows cyst growth
- slows GFR loss
tolvaptan dose in ADPKD
2-4 times that for hyponatremia
adverse effects from hypophosphatemia
- rhabdomyolysis
- respiratory failure
- heart failure
pseudohypophosphatemia cause
- when labs drawn from catheter w/ heparin
distal, type 1, RTA
- what disease should you think of?
Sjogren
distal, type 1, RTA
- urine pH
> 5.5 (inappropriately high)
distal, type 1, RTA
- urine AG
POSITIVE
distal, type 1, RTA
- kidney stone type
CaPO4 stones
distal, type 1, RTA
- treatment
NaHCO3 1 meq/kg/day po
incomplete distal, type 1, RTA is seen in what cystic kidney disease?
medullary sponge kidney
what should be treated first in distal, type 1, RTA?
- hypokalemia
- giving bicarb first will further lower K+ can lead to respiratory failure
hypophosphatemia causes
- LOW UPhos
- < 100 mg/day
- FePO4 < 5%
cellular shifts
- refeeding syndrome
- treatment of DKA
- hyperventilation
GI loss
- alcoholism
- chronic diarrhea
- milk-alkali (calcium-alkali) syndrome (Ca2+ binding to PO4- in GIT)
# sudden drop in PTH - s/p parathyroidectomy (hungry bone syndrome)
hypophosphatemia causes
- HIGH UPhos
- > 100 mg/day
- FePO4 > 5%
- serum Ca++ LOW
- vitamin D deficiency
hypophosphatemia causes
- HIGH UPhos
- > 100 mg/day
- FePO4 > 5%
- serum Ca++ HIGH
PHPT
hypophosphatemia causes
- HIGH UPhos
- > 100 mg/day
- FePO4 > 5%
- serum Ca++ NORMAL
- FGF-23-MEDIATED
- XLH
- ADHR
- ARHR
- TIO
- FD
- linear sebaceous nevus syndrome
- postrenal transplantation hypophosphatemia
- iron polymaltose infusions
hypophosphatemia causes
- HIGH UPhos
- > 100 mg/day
- FePO4 > 5%
- serum Ca++ NORMAL
- NON-FGF-23-mediated
- diuretics
- drug-induced
- Fanconi syndrome
- genetic causes
- hyperparathyroidism
- HHRH (hereditary hypophosphatemic rickets with hypercalciuria; NPT2c)
- XLH name
- associated gene
- XL hypophosphatemia
- PHEX
- ADHR name
- associated gene
- AD hypophosphatemic rickets
- FGF-23
- ARHR name
- associated gene
- AR hypophosphatemic rickets
- DMP1, ENPP1
TIO name
tumor-induced osteomalacia
FD name
fibrous dysplasia
- HHRH name
- associated gene
- hereditary hypophosphatemic rickets w/ hypercalciuria
- NPT2c
GENETIC causes of hypophosphatemia
- HIGH UPhos
- > 100 mg/day
- FePO4 > 5%
- serum Ca++ NORMAL
- NON-FGF-23-mediated
- HHRH (hereditary hypophosphatemic rickets with hypercalciuria; NPT2c)
- Dent’s disease
- cystinosis
- NaPi2a mutations
- others
DRUG-INDUCED hypophosphatemia
- HIGH UPhos
- > 100 mg/day
- FePO4 > 5%
- serum Ca++ NORMAL
- NON-FGF-23-mediated
- adefovir
- aminoglycosides
- cisplatin
- ifosfamide
- imatinib
- streptozocin
- tenofovir
- tetracyclines
- toluene
Andrew And Carol, If I’m Supposed To, Ten Tents
Alport’s disease genetics
- XL
- young men
Alport disease mutation
alpha 5 chain of type 4 collagen
clinical manifestations of Alport disease
- renal disease
- deafness
- anterior lenticonus
clinical manifestations in WOMEN w/ Alport disease
- hematuria ONLY
- rarely CKD
can be seen in Alport disease patients POST-renal transplant
crescentic anti-GBM GN, but NO pulmonary hemorrhage
EM findings in crescentic anti-GBM GN in Alport disease
basement membrane looks layered
TBMD genetics
- AD
- family history w/o ESRD or deafness
TBMD clinical manifestations
- microscopic hematuria
- usually minimal proteinuria
- usually clinically benign
hypocomplementemic GNs
- SLE
- classical pathway
- usually BOTH C3 and C4
hypocomplementemic GNs
- cryoglobulinemia
- classical pathway
- VERY LOW C4; can be undetectable
- sometimes low C3
hypocomplementemic GNs
- postinfectious GN
- alternate pathway
- C3 ONLY
hypocomplementemic GNs
- MPGN
- alternate pathway
- C3 ONLY
- especially in C3 nephropathy
hypocomplementemic GNs can also be seen in what instances?
- chronic infection
- subacute bacterial endocarditis
- HCV
- AED
hypocomplementemic GNs
- C3 GN
- C3 ONLY
hypocomplementemic GNs
- DDD
- C3 ONLY
primary membranous GN lab and histological finding
PLA2R Ab in serum and on biopsy stain
primary membranous GN is a/w what Ab?
IgG4
membranous GN w/ “full-house” IF
think of SLE
lupus nephritis - class 1
minimal mesangial LN
lupus nephritis
- class 1
- LM findings
normal
lupus nephritis
- class 1
- IF findings
mesangial immune deposits
lupus nephritis
- class 1
- clinical manifestations
mild proteinuria
lupus nephritis - class 2
mesangial proliferative LN
lupus nephritis
- class 2
- LM findings
- mesangial hypercellularity
- mesangial matrix expansion
- mesangial immune deposits
lupus nephritis
- class 2
- IF and EM findings
- mesangial immune deposits
- few subepithelial or subendothelial immune deposits
lupus nephritis
- class 2
- clinical manifestations
- mild renal disease such as hematuria or proteinuria
- usually no specific treatment needed
lupus nephritis - class 3 A
active lesions
- focal proliferative LN
lupus nephritis - class 3 A/C
active and chronic lesions
- focal proliferative and sclerosing LN
lupus nephritis - class 3 C
chronic inactive lesions
- focal sclerosing LN
lupus nephritis
- class 3
- LM findings
- active or inactive, focal, segmental, or global GN
- < 50% of all glomeruli
lupus nephritis
- class 3
- IF and EM findings
subendothelial and mesangial immune deposits
lupus nephritis
- class 3
- clinical manifestations
- mild to moderate renal disease
- hematuria
- moderate proteinuria
- worsening renal function in significant minority
lupus nephritis - class 4-S (A)
active lesions
- diffuse segmental proliferative LN
lupus nephritis - class 4-G (A)
active lesions
- diffuse global proliferative LN
lupus nephritis - class 4-S (A/C)
active and chronic lesions
- diffuse segmental proliferative and sclerosing LN
lupus nephritis - class 4-G (A/C)
active and chronic lesions
- diffuse global proliferative and sclerosing LN
lupus nephritis - class 4-S (C)
chronic inactive lesions w/ scars
- diffuse segmental sclerosing LN
lupus nephritis - class 4-G (C)
chronic inactive lesions w/ scars
- diffuse global sclerosing LN
lupus nephritis
- class 4
- LM findings
- active or inactive, segmental or global GN > 50% glomeruli
- diffuse segmental (class 4-S) when 50% glomeruli have SEGMENTAL lesions
- global (class 4-G) when 50% have GLOBAL lesions
lupus nephritis
- class 4
- IF and EM findings
subendothelial immune deposits
lupus nephritis
- class 4
- clinical manifestations
- HTN
- edema
- active urinary sediment
- worsening renal function
- nephrotic range proteinuria
- active extrarenal SLE in many patients
lupus nephritis - class 5
membranous LN
lupus nephritis
- class 5
- LM findings
- diffuse GBM thickening w/o inflammatory infiltrate
- subepithelial deposits and BM spikes on silver and trichrome stains
- may occur in combination w/ class 2 or 4
- may show advanced sclerosis
lupus nephritis
- class 5
- IF and EM findings
- subepithelial and intramembranous immune deposits
- subendothelial deposits present ONLY when proliferative component present
lupus nephritis
- class 5
- clinical manifestations
- nephrotic syndrome
- usually w/o manifestations of active SLE
lupus nephritis - class 6
advanced sclerosis LN
lupus nephritis
- class 6
- LM findings
- advanced glomerular sclerosis = 90% of glomeruli
- severe IFTA
- manifestations of irreversible renal injury
lupus nephritis
- class 6
- clinical manifestations
- significant CKD or ESRD in most cases
- unlikely to respond to medical therapy
if PLA2R negative, think of
- cancer
- SLE
- medications
- viral infections
- syphilis
- parasites
membranous GN treatment
- RAAS blockade x 6 months
- if NO response;
- steroids w/ CYA,
- steroids w/ tacrolimus,
- steroids alternating w/ chlorambucil (Ponticelli protocol),
- steroids alternating w/ CP (Ponticelli protocol),
OR - rituximab
anti-GBM disease age distribution
- bimodal
- young men and elderly
- anti-GBM Abs can be seen in what disease?
- how does it affect prognosis?
- ANCA vasculitis
- worsens prognosis
anti-GBM disease, if anuric
rarely recovers
does anti-GBM recur in patients or transplant?
usually no
anti-GBM a/w
- smoking
- hydrocarbon exposure
HIVAN is typically what on histology?
collapsing FSGS
HIVAN RUS
large kidneys
HIVAN clinical manifestations
- no HTN
- little to no edema
HIVAN pathology
- microcysts
- tubular reticular inclusions (TRIs)
common in HIVAN
immune complex disease lesions
increased risk of HIVAN in patients w/ what?
APOL-1 high-risk alleles
IgA nephropathy pathogenesis
abnormal polymeric IgA, missing galactose on side chain (glycosylation)
IgA nephropathy epidemiology
- Asians
- Hispanics
- rare in African Americans
IgA nephropathy commonly seen in
liver disease, but usually not clinically important
IgA nephropathy in transplant
high recurrence?
IgA dominant PIGN seen w/?
- Staphylococcus aureus infections, especially in DM
- bad prognosis
scleroderma renal crisis clinical manifestations
- malignant HTN
- TMA
- MAHA (microangiopathic hemolytic anemia)
- AKI
scleroderma renal crisis more common w/
severe skin involvement
scleroderma renal crisis lifesaving treatment
RAAS blockade
scleroderma renal crisis can be caused by
steroids
sickle cell nephropathy renal manifestations
microscopic hematuria to papillary necrosis
urine in sickle cell nephropathy
isosthenuria early in disease
RTA in sickle cell nephropathy
type 4 RTA
can cause proteinuria and ESRD in sickle cell disease patients
FSGS
hematuria in SCT
can be microscopic or gross hematuria
what cancer can be seen in SCT?
rarely, medullary cell Ca (aggressive)
primary FSGS acts like
MCD
if FSGS is steroid non-responsive think of
podocin mutation
FSGS variant w/ best steroid response
tip lesion
more common in secondary FSGS
perihilar lesions
causes of FSGS
- primary (idiopathic)
- secondary;
- familial/genetic
- virus-associated
- drug-induced
- adaptive
collapsing FSGS a/w
- HIV-1
- parvovirus B19
- pamidronate
TMA results from
- alternate complement pathway abnormalities
- factor H, factor 1, C3, factor B, thrombomodulin
TMA can p/w
- AKI
- HTN
- MAHA
recurrence rate of TMA in transplant patients
high
causes of NAGMA
- GI (diarrhea)
- renal (RTAs)
- hyperkalemia
- aldosterone deficiency
causes of NAGMA w/ POSITIVE urine anion gap
- distal, type 1, RTA
- proximal, type 2, RTA, prior to reaching steady state
- toluene
causes of NAGMA w/ NEGATIVE urine anion gap
- diarrhea
- proximal, type 2, RTA, at steady state
causes of HAGMA
- high acid input
- ingestion
- EtOH
- methanol
- ethylene glycol
- ASA
causes of HAGMA
- high acid input
- production
- shock
- lactic acidosis
- ketoacidosis
- starvation
- DM
- EtOH
causes of HAGMA
- low acid input
- AKI
- CKD
NAGMA HARDUP
- Hyperalimentation/HCl
- Acetazolamide
- RTA
- Diarrhea
- Ureteroenteric fistula (ileal conduit)
- Post-hypOcapnic state
HAGMA GOLDMARK
- Glycols
- Oxoproline (pyroglutamic acid)
- L-lactate
- D-lactate
- Methanol
- Aspirin
- Renal failure
- Ketones
mortality in HD decreased in
- blacks
- larger BMI
- longer tx time
mortality in HD increased in
- hyperphosphatemia
- hypoalbuminemia
mortality in HD a/w
- low K+ baths
- higher UF rates
mortality in HD a/w what conditions
- restless leg syndrome
- sleep d/o’s
- pruritus
- ESA resistance
clinical manifestations of lead nephropathy
- HTN
- gout
- CKD
- AKI w/ acute Pb intoxication
lead nephropathy histopathology
chronic tubulointerstitial nephritis
causes of lead nephropathy
- battery factory work
- moonshine consumption
diagnose lead toxicity
EDTA test
lead exposure a/w
CKD
lithium nephropathy clinical manifestations
- nephrogenic DI
- CKD in chronic exposure
- ATN in acute toxicity
may prevent toxicity w/ chronic lithium use
amiloride
a/w lithium nephropathy
- distal, type 1, RTA
- MCD
- drug-induced FSGS (rare)
treatment of acute lithium overdose
- dialyze (beware of rebound, HD x 4 hours, CRRT as adjuvant)
IgG4-related disease histopathology
AIN w/ “storiform” (swirly) pattern
IgG4-related disease clinical manifestations
- kidney mass
- AI pancreatitis +/- pancreatic mass
- retroperitoneal fibrosis
- aortitis
IgG4-related disease serum IgG4 level
high
IgG4-related disease treatment
- steroids, long course
- high relapse rate
mechanism of hypercalcemia in sarcoidosis
granulomatous production of 1-25 vitamin D (1-hydroxylation)
most common renal manifestation of sarcoidosis
hypercalciuria
if tubulointerstitial nephritis and UVEITIS think
TINU (tubulointerstitial nephritis and uveitis)
histopathology of sarcoidosis
renal granulomas, but not always
test which may help in diagnosis of idiopathic AIN
ACE level
sarcoidosis treatment
- steroids acutely
- methotrexate, MMF, or azathioprine chronically
clinical manifestations in pregnancy
- cardiac output increases
- plasma volume increases
- GFR increases
serum Cr in pregnancy
should be low
physiology of low bp in pregnancy
SVR decreases more than CO increases
acid base in pregnancy
respiratory alkalosis (d/t progesterone) w/ compensatory NAGMA
hyponatremia in pregnancy is d/t?
reset osmostat
treatment for primary hyperoxaluria
OLT
secondary (enteric) hyperoxaluria seen in
intestinal bypass (bariatric surgeries)
secondary (enteric) hyperoxaluria can cause
- CaOxalate stones
- CKD
- ESRD
other causes of secondary (enteric) hyperoxaluria
- excessive vitamin C
- cashews
- starfruit (carambola) juice
- Orlistat
secondary (enteric) hyperoxaluria treatment
- oral calcium to bind ingested oxalate and prevent absorption
- low-oxalate diet
metabolic alkalosis
- urine Cl- < 15 mmol/l
- chloride RESPONSIVE
- GI loss
- renal loss
metabolic alkalosis
- urine Cl- < 15 mmol/l
- chloride RESPONSIVE
- causes of GI loss
- gastric fluid losses
- Cl- losing diarrhea
- laxative abuse
metabolic alkalosis
- urine Cl- < 15 mmol/l
- chloride RESPONSIVE
- causes of renal loss
- diuretic use
- severe hypokalemia
- post-hypercapnic alkalosis
treatment of chloride RESPONSIVE metabolic alkalosis
- volume expansion (NaCl)
- K+ replacement (KCl)
metabolic alkalosis
- urine Cl- > 20 mmol/l
- chloride RESISTANT
- causes
- mineralocorticoid excess
- ACTIVE diuretic use
- Bartter syndrome
- Gitelman syndrome
- Cushing syndrome
- hypokalemia
treatment of chloride RESISTANT metabolic alkalosis
- treat cause
- NaCl INeffective
medications that cause CaPO4 stones
- topiramate
- zonisamide
- acetazolamide
cause of indinavir stones
indinavir
cause of atazanavir stones
atazanavir
medications that cause CaOxalate stones
- Orlistat
- vitamin C
laxative abuse causes what kind of stones
ammonium acid urate stones
cause of sulfa stones
sulfa medications
Gitelman syndrome is like taking which diuretic
thiazide diuretics
Bartter syndrome is like taking which diuretic
loop diuretics
Gitelman syndrome abnormal transporter
NaCl cotransporter
Bartter syndrome abnormal transporter
NaK2Cl cotransporter
Bartter age group
childhood
Gitelman age group
adulthood
Bartter genetics
AR
Gitelman genetics
AR
Bartter and Gitelman both require what to be ruled out first?
diuretic abuse
Gitelman is a/w
- hypomagnesemia (renal Mg2+ wasting)
- hypocalciuria
Bartter is a/w
hypERcalciuria
Bartter and Gitelman treatment
amiloride or spironolactone
other treatment for Bartter and why
- NSAIDs
- PGs elevated in Bartter
AA amyloidosis histopathology
- Congo red stain positive
- no light chain restriction
- amyloid A protein positive
AA amyloidosis seen in
- chronic skin infection; IVDA skin poppers
- chonic OM
- TB
AA amyloidosis seen in which conditions
- chronic inflammatory diseases
- Crohn’s disease
- RA
- familial Mediterranean fever (FMF); renal amyloidosis
AA amyloidosis can be seen in which hereditary condition?
hereditary fibrinogen A
what conditions and medications should you think of in proximal RTA?
- MM
- amyloidosis
- Fanconi syndrome
- tenofovir
- ifosfamide
- cisplatin
K+ level in proximal RTA
low
what treatment should initially be avoided in proximal RTA as to not worsen hypokalemia?
bicarbonate
proximal RTA is benign, except in
children - stunts growth
urine pH in proximal RTA once steady state is reached
< 5.5 (appropriately low to maintain homeostasis)
treatment for proximal RTA
10-15 meq/kg/day bicarb and K+ citrate
fibromuscular dysplasia epidemiology
- usually young women
- rare in blacks
what is icodextrin, and what is it metabolized into?
- glucose polymer
- metabolized to maltose
how does UF occur w/ icodextrin?
from colloid oncotic osmotic pressure
can occur in up to 10% of patients on icodextrin
rash
what is a rare complication of icodextrin?
sterile peritonitis
icodextrin can be useful in what situation?
type 1 UF failure for high solute transporters
through what pores does UF occur w/ icodextrin use?
medium sized, 4-6 nm, interendothelial cell pores
recirculation in HD equation
% = P-A/P-V x 100
- P = peripheral blood urea concentration
- A = PRE-dialyzer arterial access
- V = POST-dialyzer venous access
what are the 2 systemic causes that can lead to a decreased Kt/V?
- access recirculation
- cardiopulmonary recirculation, especially at lower CO
cardiopulmonary recirculation in HD can occur w/?
CVC
MCC of recirculation in AVF or AVG
outflow stenosis
Kt/V not affected by
- pre-dialysis BUN
- diet compliance
- frequency of HD
how is Kt/V achieved?
diffusive clearance AND convective clearance from UF removal
Kt/V increased by what factors?
- increase in BFR
- increase in DFR
- time on HD
- mass transfer of dialyzer (size)
Kt/V treatment goal which ONLY applies to 3x/week HD
1.2
Kt/V decreased by what factors?
- access recirculation
- dialyzer fiber clotting
often preceded by a period of type 1 (high effective peritoneal surface area) UF failure
encapsulating peritoneal sclerosis
can present early w/ bloody PD fluid
encapsulating peritoneal sclerosis
can present later w/ peritoneal calcification or intestinal obstruction w/ complete cocooning of peritoneal membrane
encapsulating peritoneal sclerosis
treatment for encapsulating peritoneal sclerosis
- switch to HD and start steroids
- tamoxifen if diagnosed early
- surgery for bowel obstruction
can occur in PD patients even after transplant
encapsulating peritoneal sclerosis
complication of PD a/w high mortality rate
encapsulating peritoneal sclerosis
calcific uremic arteriolopathy (calciphylaxis) usually occurs in what tissue type?
fatty tissues
calcific uremic arteriolopathy (calciphylaxis) sex distribution
women > men
calcific uremic arteriolopathy (calciphylaxis) signs/symptoms
- painful
- very tender
- indurated nodules
- subcutaneous plaques that eventually become necrotic
calcific uremic arteriolopathy (calciphylaxis) diagnosis
- bone scan which shows soft tissue calcium deposition
- biopsy should be avoided d/t poor healing
risk factors for calcific uremic arteriolopathy (calciphylaxis)
- warfarin
- corticosteroids
- vitamin D analogs
- possibly severe hyperparathyroidism
calcific uremic arteriolopathy (calciphylaxis) treatment
- remove risk factors
- sodium thiosulfate (12.5-25 g IV) after HD
- hyperbaric O2 may help wound healing
least common cause of all infectious peritonitis
fungal peritonitis (3-6%)
major risk factor for fungal peritonitis
antibiotic use to treat bacterial peritonitis
fungal peritonitis can be avoided during treatment of bacterial peritonitis by doing what?
antifungal ppx
fungal peritonitis treatment
- prolonged antifungal tx
- prompt PD catheter removal
fungal peritonitis is more common after what?
gram negative peritonitis
fungal peritonitis commonly results in
PD dropout and switch to HD
decrease in Kt/V in a new start HD patient is usually d/t what?
loss of residual renal function (RRF) over time
what can slow down loss of residual renal function (RRF) in PD patients?
ACEI
should be used for V in PD patients
ideal body weight
not affected by PET transport group types
Kt/V
survival in PD patients is related to what?
renal-Kt/V»_space; PD Kt/V (RRF enhances survival)
polyuria definition
UOP > 3 L/24 hours
polyuria
- > 1000 mOsm/day
- osmotic diuresis
- causes
- glucose
- mannitol
- urea
- resolving ATN
- diuretics
- propylene glycol
polyuria
- < 800 mOsm/day
- water diuresis
- next step
water deprivation test
polyuria
- < 800 mOsm/day
- water diuresis
- Na+ > 140 meq/l
- water deprivation test shows NO change in UOsm
give DDAVP
polyuria
- < 800 mOsm/day
- water diuresis
- Na+ > 140 meq/l
- water deprivation test shows NO change in UOsm
- if INCREASE in UOsm after DDAVP
central DI
polyuria
- < 800 mOsm/day
- water diuresis
- Na+ > 140 meq/l
- water deprivation test shows NO change in UOsm
- if still NO CHANGE in UOsm after DDAVP
nephrogenic DI
polyuria
- < 800 mOsm/day
- water diuresis
- Na+ LESS THAN 140 meq/l
- water deprivation test shows INCREASE in UOsm
primary polydipsia
PD noninfectious complications
- pleural effusions, right > left
- chylous ascites
- ventral hernias
- transient green effluent s/p cholecystectomy
- bloody effluent in premenopausal women w/ menses or ovarian cyst rupture
transplant medication adverse effects;
- cyclosporine
- HTN
- hyperkalemia
- HLD
- renal dysfunction
- gingival hyperplasia
- hirsutism
transplant medication drug-drug interactions;
- cyclosporine
- diltiazem
- verapamil
- increases CSA level
transplant medication adverse effects;
- tacrolimus
- HTN (Na+ retention)
- hyperkalemia
- tremor
- renal dysfunction
- hyperglycemia
- alopecia
transplant medication drug-drug interactions;
- tacrolimus
- diltiazem
- verapamil
- increases FK level
transplant medication adverse effects;
- sirolimus
- proteinuria
- HLD
- cytopenia
- slow wound healing
- pulmonary infiltrates
transplant medication adverse effects;
- mycophenolate
- GI upset
- myelosuppression
- hepatotoxic
- teratogenic
transplant medication adverse effects;
- azathioprine
- skin cancer
- myelosuppression w/ allopurinol or febuxostat use d/t decreased xanthine oxidase
- myelosuppression in patients w/ TMPT (thiopurine S-methyltransferase) deficiency
AMR often occurs when?
after decreasing IS therapy
AMR diagnosis
- peritubular capillaritis
- +C4d staining
- +DSA
AMR treatment
- steroids
- plasmapheresis
AND - IVIG
acute cellular (T-cell) mediated rejection pathogenesis
T-cell reaction to donor histocompatibility Ags
acute cellular (T-cell) mediated rejection grading criteria
Banff criteria
Banff criteria
grades
- interstitium
- tubules
- arteries
acute cellular (T-cell) mediated rejection treatment
- steroids
- increase IS
and/or - anti-thymocyte globulin OR alemtuzumab
immunosuppression medications mechanism of action
- belatacept
binds CD80 and CD86 which blocks co-stimulation of T-cells
immunosuppression medications mechanism of action
- mTOR inhibitors (sirolimus, temsirolimus, and everolimus)
inhibits mammalian target of rapamycin (mTOR) kinase activity which inhibits T and B lymphocyte activation and proliferation
immunosuppression medications mechanism of action
- basiliximab
IL-2 receptor blocker
immunosuppression medications mechanism of action
- azathioprine
disrupts purine synthesis
immunosuppression medications mechanism of action
- mycophenolate mofetil (MMF)
disrupts purine synthesis
causes of SIADH
- malignancy
- pulmonary issues
- CNS issues
- nausea
- pain
- opiates
- ecstasy
- SSRIs
should be ruled out in evaluation of SIADH
- adrenal insufficiency
- hypothyroidism
volume state in SIADH
euvolemic
serum Osm in SIADH
hypoosmolar, < 275 mOsm/kg
urine Osm and UNa+ in SIADH
> 100 mOsm/kg and > 40 mmol/l
SIADH treatment
- fluid restriction
- oral NaCl or urea
- 3% saline
- V2 antagonists (not acutely)
- demeclocycline
distal, type 1, RTA
- K+ level
LOW
distal, type 1, RTA
- bicarb level
NAGMA, usually < 10
distal, type 1, RTA
- urine pH
> 5.5 (inappropriately HIGH)
distal, type 1, RTA
- urine AG (INDIRECT way to measure urine NH4+)
POSITIVE; corresponds to LACK of urine NH4+
distal, type 1, RTA
- causes
- Sjogren
- SLE
- amphotericin
- topiramate
abused substance that mimics distal, type 1, RTA
toluene (found in paints, paint thinners, fingernail polish, lacquers, adhesives, and rubber)
distal, type 1, RTA
- a/w
- nephrolithiasis
- nephrocalcinosis
- CKD
distal, type 1, RTA
- treatment
1-2 meq/kg/d of base, usually K+ citrate
proximal, type 2, RTA
- K+ level
LOW
proximal, type 2, RTA
- bicarb level
NAGMA, usually 12-20
distal, type 1, RTA
- pathophysiology
unable to secrete NH4+
proximal, type 2, RTA
- pathophysiology
decreased proximal HCO3 reabsorption (lower threshold for reabsorption)
proximal, type 2, RTA
- urine pH once steady state is reached
5 (appropriately LOW)
proximal, type 2, RTA
- urine AG (INDIRECT way to measure urine NH4+)
NEGATIVE; corresponds to PRESENCE of urine NH4+
proximal, type 2, RTA
- a/w
Fanconi syndrome
proximal, type 2, RTA
- effects in adults
benign
proximal, type 2, RTA
- effects in children
stunts growth
proximal, type 2, RTA
- treatment
- 5-10 meq/kg/day of base
- potassium-sparing diuretic (amiloride, or spironolactone)
RTA type 4 (hypoaldosteronism)
- K+ level
HIGH
RTA type 4 (hypoaldosteronism)
- bicarb level
usually > 17
RTA type 4 (hypoaldosteronism)
- pathophysiology
low aldosterone release and/or response
RTA type 4 (hypoaldosteronism)
- urine AG
variable
RTA type 4 (hypoaldosteronism)
- causes
- DM
- NSAIDs
- cyclosporine
- old age
RTA type 4 (hypoaldosteronism)
- treatment
- loop diuretic
- bicarb
- mineralocorticoid
AND - K+ binding resin
causes of osmotic nephropathy
- sucrose containing IVIG
- hydroxyethyl starch (HES)
- dextran
osmotic nephropathy LM on kidney biopsy
swollen tubular cells packed w/ numerous cytoplasmic vacuoles
osmotic nephropathy EM on kidney biopsy
vacuoles with phagolysosomes containing the offending agent (sucrose, HES, etc)
medications that decrease calcineurin inhibitor level
- rifampin
- barbiturates
- phenytoin
- carbamazepine
- nafcillin
- trimethoprim
- imipenem
- cephalosporins
- St. John wort
- INH
medications that increase calcineurin inhibitor level
- verapamil
- diltiazem
- ketoconazole
- fluconazole
- erythromycin
- grapefruit
- amiodarone
- carvedilol
- dapsone
medications and condition that increase calcineurin inhibitor toxicity
- amphotericin
- aminoglycosides
- NSAIDs
- lovastatin
- rhabdomyolysis**
Liddle syndrome pathophysiology
- GOF mutation
- ENaC overactivity
apparent mineralocorticoid excess pathophysiology
11β-HSD2 deficiency
glucocorticoid remediable aldosteronism pathophysiology
- chimeric gene fusion (CYP11B1 and CYP11B2)
- zona fasciculata defect
- ACTH receptor overstimulated by cortisol
renal artery stenosis renin/aldosterone pathophysiology
renal parenchymal ischemia
- causes of hypercalcemia
- LOW PTH
- normal 25-OH vitamin D
- LOW 1,25-(OH)2 vitamin D
- hyperthyroidism
- malignancy
- immobilization
- Paget’s disease
- milk alkali syndrome
pregnancy-associated TMA
- > 24 weeks
- HTN, edema
- effect of delivering fetus
- preeclampsia
- improved
pregnancy-associated TMA
- > 27 weeks
- liver dysfunction and coagulopathy
- effect of delivering fetus
- acute fatty liver (AFL)
- improved
pregnancy-associated TMA
- > 34 weeks
- elevated LFTs (not bili), and severe MAHA
- effect of delivering fetus
- HELLP
- improved
pregnancy-associated TMA
- postpartum
- renal failure
- effect of delivering fetus
- HUS
- none
pregnancy-associated TMA
- < 24 weeks
- neurological symptoms
- effect of delivering fetus
- TTP
- none
calculator for estimating likelihood of severe ADAMTS13 deficiency in adults w/ suspected TTP
PLASMIC score
treatment for suspected TTP
plasmapheresis
treatment for drug-induced TMA
- quinine-containing beverage
- IVDU
- chemotherapy
- CNI
- d/c medication
- may be able to avoid plasmapheresis
treatment for postpartum complement-mediated TMA
prompt anti-complement treatment (eculizumab)
alternative treatment for TMA if plasmapheresis not available
plasma infusion until transfer to another hospital
alternative treatment for TMA if anaphylactic reaction to plasma
factor VIII concentrate (contains ADAMTS13) and apheresis (plasma removal)
alternative treatment for TMA if can’t give any blood products for religious reasons
intensive IS and apheresis
how is adequate response to plasmapheresis assessed?
normalization of platelet count
eculizumab is removed by
plasmapheresis
what ppx must be given prior to eculizumab administration?
- abx x 2 weeks before vaccine is effective
- meningococcal vaccine
cause for primary FSGS
- specific cause unknown
- ? circulating permeability factors
cause for familial/genetic FSGS
mutation in podocyte genes (eg podocin, nephrin)
causes for virus-associated FSGS
- HIV-1
- parvovirus B19
- SM 40
- CMV
- EBV
causes for drug-induced FSGS
- heroin
- interferon alfa, beta, and gamma
- lithium
- pamidronate
- sirolimus
- CNIs
- anabolic steroids
causes for adaptive FSGS
- conditions w/ reduced renal mass
- very low birth weight
- u/l renal agenesis
- reflux nephropathy
- advanced renal disease w/ reduced functioning nephrons
causes for adaptive FSGS
- conditions w/ initially normal renal mass
- HTN
- AED
- TMA
- RAS
- cyanotic congenital heart disease
- sickle cell anemia
the adaptive form of FSGS is mediated by adaptive structural-functional responses to glomerular hypertension caused by
elevated glomerular capillary pressures and flows
initial urine pH in proximal, type 2, RTA
alkaline, 8
urine NH4+ level in proximal, type 2, RTA once steady state is reached
HIGH
some studies have indicated that magnesium wasting in Gitelman syndrome may be d/t down-regulation of what?
TRPM6 in DCT
expected acid-base disturbance in aspirin OD
respiratory alkalosis w/ compensatory metabolic acidosis
best initial test for a patient suspected of having pheochromocytoma
24-hour URINARY fractionated metanephrines
- 98% sensitivity
- 98% specificity
best test for a patient at HIGH RISK for pheochromocytoma (high risk familial syndrome such as MEN2, or VHL syndrome)
plasma catecholamines
- 96-100% sensitivity
- only 85-89% specificity
what treatment should a patient with pheochromocytoma receive prior to surgery?
- alpha and beta blockers
- HIGH SALT diet (to mitigate volume contraction and orthostasis)
generally, if problem on PD is inadequate solute clearance, what is the next best step?
increase volume in each exchange
ACCOMPLISH trial showed combination of which antihypertensives reduced cardiovascular morbidity and mortality, and slowed CKD progression?
ACEI and CCB
according to NHANES, which modifiable risk factor in patients with CKD has the highest overall survival rate compared to other modifiable risk factors?
smoking cessation
renal and GU abnormalities and associated cause
- Turner’s syndrome
horseshoe kidney
renal and GU abnormalities and associated cause
- epispadias
bladder extrophy
renal and GU abnormalities and associated cause
- undescended testes
- inguinal hernias
- CHARGE syndrome
hypospadias
renal and GU abnormalities and associated cause
- WAGR
- aniridia
- GU malformation
- intellectual disability
Wilm’s tumor
reabsorption (or secretion) rate formula
filtration rate - excretion rate
- FR = GFR x Px
- ER = V x Ux
- GFR = U(inulin)/P(inulin) x V (urine flow rate)
inulin clearance (GFR) formula
- inulin is neither secreted NOR reabsorbed
U(inulin)/P(inulin) x V (urine flow rate)
PAH clearance (ERPF) formula
- PAH is filtered by glomeruli and actively secreted by proximal tubutes
CPAH = ERPF = U(PAH)/P(PAH) x V (urine excretion rate)
RBF = RPF/(1 - Hct)
respiratory alkalosis
- for every decrease in PCO2 below 40, bicarb decreases by
2-3
respiratory acidosis
- for every increase in PCO2 above 40, bicarb increases by
2-3
