Nephrology Pearls of Wisdom Flashcards
(414 cards)
1
Q
tuberous sclerosis genetics
A
AD
medication class that slows tumor growth in tuberous sclerosis inibidor da mtor
renal manifestation of tuberous sclerosis
- renal angiomyolipomas
- RCC (very rare)
2
Q
what are the mTOR inhibitors?
A
- sirolimus (aka rapamycin)
- everolimus
- temsirolimus
3
Q
Fabry disease genetics
A
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
treatment for Fabry disease ERT (enzyme replacement therapy)
4
Q
Zebra bodies can also be seen on patients taking what medication?
A
hydroxychloroquine
5
Q
what should be ruled out in patients with MCD?
A
- NSAID use
- lymphoma
- lithium use
6
Q
if AIN and AKI think of
A
NSAID use
7
Q
rate of relapse in MCD
A
50%
8
Q
what other renal manifestation is also commonly seen in patients with MCD?
A
ATN
9
Q
ADPKD1 compared to ADPKD2
A
occurs earlier and more ESRD
10
Q
PKD1, which chromosome?
A
16
11
Q
PKD2, which chromosome?
A
4
12
Q
polycystin proteins affect what?
A
cilium
13
Q
extrarenal manifestations of ADPKD
A
- brain aneurysms
- diverticula
- abdominal wall hernias
- MVP
14
Q
preferred bp medication for PKD
A
ACEI
15
Q
PKD medication to slow cyst growth
A
vaptans
16
Q
West African Sub-Saharan gene that protects against trypanosomes
A
APOL1
17
Q
2 variants of APOL1
A
G1 and G2
18
Q
2 high-risk alleles of APOL1 increase risk of
A
- HIV nephropathy
- sickle cell nephropathy
- ESRD from FSGS
- hypertensive nephrosclerosis
19
Q
2 high-risk alleles of APOL1 increase what in ESRD patients?
A
survival
20
Q
what % of African Americans carry 2 high risk alleles
A
14%
21
Q
MCC of AIN
A
abx, namely B-lactams and sulfa-based antibiotics
22
Q
AIN from abx have what w/i infiltrate?
A
eosinophils
23
Q
AIN from NSAIDs does NOT have what?
A
eosinophils
24
Q
what improves short- and long-term outcome if administered early in AIN?
A
steroids
25
rash is seen in AIN when caused by
abx
26
in AIN, which medication class do you NOT see rash?
PPIs
27
aside from AIN, other manifestations seen in IgG4-related disease
- pancreatitis
- retroperitoneal fibrosis
- masses in other organs
28
aside from AIN, other manifestations seen in sarcoidosis
- hypercalcemia
- hypercalciuria
29
monoclonal gammopathy of renal significance (MGRS)
- AL amyloidosis
- is usually what type?
lambda
30
monoclonal gammopathy of renal significance (MGRS)
- AL amyloidosis
- what size fibrils?
10 nm
31
monoclonal gammopathy of renal significance (MGRS)
- light-chain deposition disease (LCDD)
- is usually what type?
kappa \> lambda
32
monoclonal gammopathy of renal significance (MGRS)
- light-chain deposition disease (LCDD)
- staining?
tubular light chain staining
33
monoclonal gammopathy of renal significance (MGRS)
- light-chain deposition disease (LCDD)
- appearance on biopsy?
nodular, looks like diabetic KW disease
34
monoclonal gammopathy of renal significance (MGRS)
- cast nephropathy
- usually what type?
kappa = lambda
35
monoclonal gammopathy of renal significance (MGRS)
- cast nephropathy
- what's seen on LM?
fractured casts
36
monoclonal gammopathy of renal significance (MGRS) can present w/?
Fanconi syndrome (proximal tubulopathy)
37
in monoclonal gammopathy of renal significance (MGRS), urine dipstick doesn't detect what?
light chains (Bence Jones proteins)
38
serum [Na+] ~
(TBNa+ + TBK+)/TBW
39
- hyponatremia
- hypotonic
- hypERvolemic
- urine Na+ \< 20 mmol/L
- heart failure
- liver failure
40
- hyponatremia
- hypotonic
- hypERvolemic
- urine Na+ \> 20 mmol/L
- renal failure
41
- hyponatremia
- hypotonic
- EUvolemic
- urine Na+ \> 20 mmol/L
- urine Osm \> 100
- SIADH
- adrenal insufficiency
- hypothyroidism
- stress
- drug use
42
- hyponatremia
- hypotonic
- EUvolemic
- urine Na+ \> 20 mmol/L
- urine Osm \< 100
- primary polydipsia
- low solute intake
43
- hyponatremia
- hypotonic
- EUvolemic
- urine Na+ \> 20 mmol/L
- variable urine Osm
reset osmostat
44
- hyponatremia
- hypotonic
- hypovolemia
- urine Na+ \> 20 mmol/L
- salt wasting nephropathy
- hypoaldosteronism
- active diuretic use
45
- hyponatremia
- hypotonic
- hypovolemia
- urine Na+ \< 20 mmol/L
- extrarenal sodium loss (GI)
- remote diuretic use
46
- hyponatremia
- hypertonic
- hyperglycemia
- mannitol
47
- hyponatremia
- normo-osmolar
- hyperlipidemia
- HLD can be 2/2 lipoprotein X a/w cholestatic or obstructive jaundice
48
presentation of D-lactic acidosis
- AMS
- ataxia
49
lactic acid level in D-lactic acidosis
normal, because they only measure L-lactate
50
cause for D-lactic acidosis
colonic bacteria exposed to glucose, usually from blind loop
51
tx for D-lactic acidosis
complex carbohydrate diet and antibiotics
52
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
53
MCC of serum osmolal gap
ethanol
54
osmolal gap and AG in isopropyl alcohol
- increased osmolal gap
- NO AG (unless shock develops leading to lactic acidosis)
55
in ICU patient w/ AG consider what possible cause
propylene glycol from iv gtts
56
in ER patient w/ AG consider what possible causes
- salicylate
- EG
- methanol
- metformin intoxication
57
sglt2 inhibitor w/ AG, think of
euglycemic DKA
58
what elements are mismeasured as Cl-?
- bromide
- iodide
59
high unmeasured cation
lithium
60
high unmeasured cation in MM
gammaglobulins (severe hypergammaglobulinemia)
61
elements that are unmeasured cations
- Ca++
- Mg++
62
mechanism of PPI causing hypomagnesemia
decreased intestinal Mg absorption
63
urine FeMg in PPI use
low
64
hypomagnesemia causes
- hypoparathyroidism --\> hypocalcemia
- K+ wasting
65
medications that cause renal wasting of hypomagnesemia
- aminoglycosides
- cisplatin
- cetuximab
- loop diuretics
- thiazide diuretics (except amiloride)
66
syndrome that leads to hypomagnesemia
Gitelman syndrome
67
causes of Fanconi syndrome
- tenofovir (+/- nephrogenic DI)
- ifosfamide (+/- nephrogenic DI)
- cisplatin
- MM
68
adults need w/u for what when Fanconi syndrome is diagnosed?
paraproteinemia
69
what do you see on labs w/ Fanconi syndrome?
- renal K+, PO4, and urate wasting
- aminoaciduria
70
what other renal abnormality can be seen w/ Fanconi syndrome?
proximal, type 2, RTA
71
magnesium in Fanconi syndrome
normal
72
HAGMA in elderly woman
5-oxoproline (pyroglutamic acid) d/t acetaminophen use
73
HAGMA from sglt2 inhibitor
severe ketoacidosis w/ euglycemia
74
HAGMA from metformin
D lactic acidosis
75
HAGMA from linezolid
lactic acidosis
76
HAGMA from diethylene glycol
- brake fluid
- cranial nerve abnormalities
77
hypomagnesemia causes
- UMg \< 20
- FeMg \< 2.5
- PPI
- diarrhea
78
hypomagnesemia
- UMg \> 20
- FeMg \> 2.5
- what do you check next?
check 24-hour urine Ca++
79
hypomagnesemia causes
- UMg \> 20
- FeMg \> 2.5
- if 24-hour urine Ca++ \< 150
- thiazide diuretics
- Gitelman syndrome
80
hypomagnesemia causes
- UMg \> 20
- FeMg \> 2.5
- if 24-hour urine Ca++ \> 250
- loop diuretics
- Bartter syndrome
- cisplatin
81
hypokalemia causes
- low/normal volume state
- low bicarbonate
- diarrhea
- laxative use
- toluene
- RTA
- NAGMA
82
hypokalemia causes
- low/normal volume state
- low magnesium
- familial hypomagnesemia
83
hypokalemia causes
- low/normal volume state
- urine Cl- low
- vomiting
84
hypokalemia causes
- low/normal volume state
- urine Cl- high
- urine Ca++ LOW
- thiazide diuretics
- Gitelman syndrome
85
hypokalemia causes
- low/normal volume state
- urine Cl- high
- urine Ca++ HIGH
- loop diuretics
- Bartter syndrome
86
hypokalemia causes
- high volume state
- LOW renin and LOW aldosterone
- Liddle syndrome
- apparent mineralocorticoid excess (AME)
- licorice
87
hypokalemia causes
- high volume state
- LOW renin and HIGH aldosterone
- primary hyperaldosteronism
- Cushing
- glucocorticoid remediable aldosteronism (GRA)
88
hypokalemia causes
- high volume state
- HIGH renin and HIGH aldosterone
- malignant HTN
- renal artery stenosis (RAS)
- renin secreting tumor
89
tolvaptan for hyponatremia
- start only in hospital
- do NOT fluid restrict
- avoid in liver disease
- need HIGHER doses in CHF
90
tolvaptan effect in ADPKD
- slows cyst growth
- slows GFR loss
91
tolvaptan dose in ADPKD
2-4 times that for hyponatremia
92
adverse effects from hypophosphatemia
- rhabdomyolysis
- respiratory failure
- heart failure
93
pseudohypophosphatemia cause
- when labs drawn from catheter w/ heparin
94
distal, type 1, RTA
- what disease should you think of?
Sjogren
stones -foSfato de calcio
\> 5.5 (inappropriately high) acima de 5,5 a"SS"ima de cinco,5
ag urinario poSitivo (todas as atrs
sem citrato
NaHCO3 1 meq/kg/day po
95
incomplete distal, type 1, RTA is seen in what cystic kidney disease?
medullary sponge kidney
96
what should be treated first in distal, type 1, RTA?
- hypokalemia
- giving bicarb first will further lower K+ can lead to respiratory failure
97
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)
98
hypophosphatemia causes
- HIGH UPhos
- \> 100 mg/day
- FePO4 \> 5%
- serum Ca++ LOW
- vitamin D deficiency
99
hypophosphatemia causes
- HIGH UPhos
- \> 100 mg/day
- FePO4 \> 5%
- serum Ca++ HIGH
PHPT
100
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
101
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)
102
- XLH name
- associated gene
- XL hypophosphatemia
- PHEX
103
- ADHR name
- associated gene
- AD hypophosphatemic rickets
- FGF-23
104
- ARHR name
- associated gene
- AR hypophosphatemic rickets
- DMP1, ENPP1
105
TIO name
tumor-induced osteomalacia
106
FD name
fibrous dysplasia
107
- HHRH name
- associated gene
- hereditary hypophosphatemic rickets w/ hypercalciuria
- NPT2c
108
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
109
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
110
Alport's disease genetics
- XL
- young men
111
Alport disease mutation
alpha 5 chain of type 4 collagen
112
clinical manifestations of Alport disease
- renal disease
- deafness
- anterior lenticonus
113
clinical manifestations in WOMEN w/ Alport disease
- hematuria ONLY
- rarely CKD
114
can be seen in Alport disease patients POST-renal transplant
crescentic anti-GBM GN, but NO pulmonary hemorrhage
115
EM findings in crescentic anti-GBM GN in Alport disease
basement membrane looks layered
116
TBMD genetics
- AD
- family history w/o ESRD or deafness
117
TBMD clinical manifestations
- microscopic hematuria
- usually minimal proteinuria
- usually clinically benign
118
hypocomplementemic GNs
- SLE
- classical pathway
- usually BOTH C3 and C4
119
hypocomplementemic GNs
- cryoglobulinemia
- classical pathway
- VERY LOW C4; can be undetectable
- sometimes low C3
120
hypocomplementemic GNs
- postinfectious GN
- alternate pathway
- C3 ONLY
121
hypocomplementemic GNs
- MPGN
- alternate pathway
- C3 ONLY
- especially in C3 nephropathy
122
hypocomplementemic GNs can also be seen in what instances?
- chronic infection
- subacute bacterial endocarditis
- HCV
- AED
123
hypocomplementemic GNs
- C3 GN
- C3 ONLY
124
hypocomplementemic GNs
- DDD
- C3 ONLY
125
primary membranous GN lab and histological finding
PLA2R Ab in serum and on biopsy stain
126
primary membranous GN is a/w what Ab?
IgG4
127
membranous GN w/ "full-house" IF
think of SLE
128
```
lupus nephritis
- class 1
```
minimal mesangial LN
129
lupus nephritis
- class 1
- LM findings
normal
130
lupus nephritis
- class 1
- IF findings
mesangial immune deposits
131
lupus nephritis
- class 1
- clinical manifestations
mild proteinuria
132
```
lupus nephritis
- class 2
```
mesangial proliferative LN
133
lupus nephritis
- class 2
- LM findings
- mesangial hypercellularity
- mesangial matrix expansion
- mesangial immune deposits
134
lupus nephritis
- class 2
- IF and EM findings
- mesangial immune deposits
- few subepithelial or subendothelial immune deposits
135
lupus nephritis
- class 2
- clinical manifestations
- mild renal disease such as hematuria or proteinuria
- usually no specific treatment needed
136
```
lupus nephritis
- class 3 A
```
active lesions
- focal proliferative LN
137
```
lupus nephritis
- class 3 A/C
```
active and chronic lesions
- focal proliferative and sclerosing LN
138
```
lupus nephritis
- class 3 C
```
chronic inactive lesions
- focal sclerosing LN
139
lupus nephritis
- class 3
- LM findings
- active or inactive, focal, segmental, or global GN
- \< 50% of all glomeruli
140
lupus nephritis
- class 3
- IF and EM findings
subendothelial and mesangial immune deposits
141
lupus nephritis
- class 3
- clinical manifestations
- mild to moderate renal disease
- hematuria
- moderate proteinuria
- worsening renal function in significant minority
142
```
lupus nephritis
- class 4-S (A)
```
active lesions
- diffuse segmental proliferative LN
143
```
lupus nephritis
- class 4-G (A)
```
active lesions
- diffuse global proliferative LN
144
```
lupus nephritis
- class 4-S (A/C)
```
active and chronic lesions
- diffuse segmental proliferative and sclerosing LN
145
```
lupus nephritis
- class 4-G (A/C)
```
active and chronic lesions
- diffuse global proliferative and sclerosing LN
146
```
lupus nephritis
- class 4-S (C)
```
chronic inactive lesions w/ scars
- diffuse segmental sclerosing LN
147
```
lupus nephritis
- class 4-G (C)
```
chronic inactive lesions w/ scars
- diffuse global sclerosing LN
148
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
149
lupus nephritis
- class 4
- IF and EM findings
subendothelial immune deposits
150
lupus nephritis
- class 4
- clinical manifestations
- HTN
- edema
- active urinary sediment
- worsening renal function
- nephrotic range proteinuria
- active extrarenal SLE in many patients
151
```
lupus nephritis
- class 5
```
membranous LN
152
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
153
lupus nephritis
- class 5
- IF and EM findings
- subepithelial and intramembranous immune deposits
- subendothelial deposits present ONLY when proliferative component present
154
lupus nephritis
- class 5
- clinical manifestations
- nephrotic syndrome
- usually w/o manifestations of active SLE
155
```
lupus nephritis
- class 6
```
advanced sclerosis LN
156
lupus nephritis
- class 6
- LM findings
- advanced glomerular sclerosis = 90% of glomeruli
- severe IFTA
- manifestations of irreversible renal injury
157
lupus nephritis
- class 6
- clinical manifestations
- significant CKD or ESRD in most cases
- unlikely to respond to medical therapy
158
if PLA2R negative, think of
- cancer
- SLE
- medications
- viral infections
- syphilis
- parasites
159
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
160
anti-GBM disease age distribution
- bimodal
- young men and elderly
161
- anti-GBM Abs can be seen in what disease?
- how does it affect prognosis?
- ANCA vasculitis
- worsens prognosis
162
anti-GBM disease, if anuric
rarely recovers
163
does anti-GBM recur in patients or transplant?
usually no
164
anti-GBM a/w
- smoking
- hydrocarbon exposure
165
HIVAN is typically what on histology?
collapsing FSGS
166
HIVAN RUS
large kidneys
167
HIVAN clinical manifestations
- no HTN
- little to no edema
168
HIVAN pathology
- microcysts
- tubular reticular inclusions (TRIs)
169
common in HIVAN
immune complex disease lesions
170
increased risk of HIVAN in patients w/ what?
APOL-1 high-risk alleles
171
IgA nephropathy pathogenesis
abnormal polymeric IgA, missing galactose on side chain (glycosylation)
172
IgA nephropathy epidemiology
- Asians
- Hispanics
- rare in African Americans
173
IgA nephropathy commonly seen in
liver disease, but usually not clinically important
174
IgA nephropathy in transplant
high recurrence?
175
IgA dominant PIGN seen w/?
- Staphylococcus aureus infections, especially in DM
- bad prognosis
176
scleroderma renal crisis clinical manifestations
- malignant HTN
- TMA
- MAHA (microangiopathic hemolytic anemia)
- AKI
177
scleroderma renal crisis more common w/
severe skin involvement
178
scleroderma renal crisis lifesaving treatment
RAAS blockade
179
scleroderma renal crisis can be caused by
steroids
180
sickle cell nephropathy renal manifestations
microscopic hematuria to papillary necrosis
isosthenuria early in disease
type 4 RTA
181
RTA in sickle cell nephropathy
type 4 RTA
182
can cause proteinuria and ESRD in sickle cell disease patients
FSGS
183
hematuria in SCT
can be microscopic or gross hematuria
184
what cancer can be seen in SCT?
rarely, medullary cell Ca (aggressive)
185
primary FSGS acts like
MCD
186
if FSGS is steroid non-responsive think of
podocin mutation
187
FSGS variant w/ best steroid response
tip lesion
188
more common in secondary FSGS
perihilar lesions
189
causes of FSGS
- primary (idiopathic)
- secondary;
- familial/genetic
- virus-associated
- drug-induced
- adaptive
190
collapsing FSGS a/w
- HIV-1
- parvovirus B19
- pamidronate
191
TMA results from
- alternate complement pathway abnormalities
- factor H, factor 1, C3, factor B, thrombomodulin
192
TMA can p/w
- AKI
- HTN
- MAHA
193
recurrence rate of TMA in transplant patients
high
194
causes of NAGMA
- GI (diarrhea)
- renal (RTAs)
- hyperkalemia
- aldosterone deficiency
195
causes of NAGMA w/ POSITIVE urine anion gap
- distal, type 1, RTA
- proximal, type 2, RTA, prior to reaching steady state
- toluene
196
causes of NAGMA w/ NEGATIVE urine anion gap
- diarrhea
- proximal, type 2, RTA, at steady state
197
causes of HAGMA
- high acid input
- ingestion
- EtOH
- methanol
- ethylene glycol
- ASA
198
causes of HAGMA
- high acid input
- production
- shock
- lactic acidosis
- ketoacidosis
- starvation
- DM
- EtOH
199
causes of HAGMA
- low acid input
- AKI
- CKD
200
NAGMA HARDUP
- Hyperalimentation/HCl
- Acetazolamide
- RTA
- Diarrhea
- Ureteroenteric fistula (ileal conduit)
- Post-hypOcapnic state
201
HAGMA GOLDMARK
- Glycols
- Oxoproline (pyroglutamic acid)
- L-lactate
- D-lactate
- Methanol
- Aspirin
- Renal failure
- Ketones
202
mortality in HD decreased in
- blacks
- larger BMI
- longer tx time
203
mortality in HD increased in
- hyperphosphatemia
- hypoalbuminemia
204
mortality in HD a/w
- low K+ baths
- higher UF rates
205
mortality in HD a/w what conditions
- restless leg syndrome
- sleep d/o's
- pruritus
- ESA resistance
206
clinical manifestations of lead nephropathy
- HTN
- gout
- CKD
- AKI w/ acute Pb intoxication
207
lead nephropathy histopathology
chronic tubulointerstitial nephritis
208
causes of lead nephropathy
- battery factory work
- moonshine consumption
209
diagnose lead toxicity
EDTA test
210
lead exposure a/w
CKD
211
lithium nephropathy clinical manifestations
- nephrogenic DI
- CKD in chronic exposure
- ATN in acute toxicity
212
may prevent toxicity w/ chronic lithium use
amiloride
213
a/w lithium nephropathy
- distal, type 1, RTA
- MCD
- drug-induced FSGS (rare)
214
treatment of acute lithium overdose
- dialyze (beware of rebound, HD x 4 hours, CRRT as adjuvant)
215
IgG4-related disease histopathology
AIN w/ "storiform" (swirly) pattern
216
IgG4-related disease clinical manifestations
- kidney mass
- AI pancreatitis +/- pancreatic mass
- retroperitoneal fibrosis
- aortitis
217
IgG4-related disease serum IgG4 level
high
218
IgG4-related disease treatment
- steroids, long course
- high relapse rate
219
mechanism of hypercalcemia in sarcoidosis
granulomatous production of 1-25 vitamin D (1-hydroxylation)
220
most common renal manifestation of sarcoidosis
hypercalciuria
221
if tubulointerstitial nephritis and UVEITIS think
TINU (tubulointerstitial nephritis and uveitis)
222
histopathology of sarcoidosis
renal granulomas, but not always
223
test which may help in diagnosis of idiopathic AIN
ACE level
224
sarcoidosis treatment
- steroids acutely
- methotrexate, MMF, or azathioprine chronically
225
clinical manifestations in pregnancy
- cardiac output increases
- plasma volume increases
- GFR increases
226
serum Cr in pregnancy
should be low
227
physiology of low bp in pregnancy
SVR decreases more than CO increases
228
acid base in pregnancy
respiratory alkalosis (d/t progesterone) w/ compensatory NAGMA
229
hyponatremia in pregnancy is d/t?
reset osmostat
230
treatment for primary hyperoxaluria
OLT
231
secondary (enteric) hyperoxaluria seen in
intestinal bypass (bariatric surgeries)
232
secondary (enteric) hyperoxaluria can cause
- CaOxalate stones
- CKD
- ESRD
233
other causes of secondary (enteric) hyperoxaluria
- excessive vitamin C
- cashews
- starfruit (carambola) juice
- Orlistat
234
secondary (enteric) hyperoxaluria treatment
- oral calcium to bind ingested oxalate and prevent absorption
- low-oxalate diet
235
metabolic alkalosis
- urine Cl- \< 15 mmol/l
- chloride RESPONSIVE
- GI loss
- renal loss
236
metabolic alkalosis
- urine Cl- \< 15 mmol/l
- chloride RESPONSIVE
- causes of GI loss
- gastric fluid losses
- Cl- losing diarrhea
- laxative abuse
237
metabolic alkalosis
- urine Cl- \< 15 mmol/l
- chloride RESPONSIVE
- causes of renal loss
- diuretic use
- severe hypokalemia
- post-hypercapnic alkalosis
238
treatment of chloride RESPONSIVE metabolic alkalosis
- volume expansion (NaCl)
- K+ replacement (KCl)
239
metabolic alkalosis
- urine Cl- \> 20 mmol/l
- chloride RESISTANT
- causes
- mineralocorticoid excess
- ACTIVE diuretic use
- Bartter syndrome
- Gitelman syndrome
- Cushing syndrome
- hypokalemia
240
treatment of chloride RESISTANT metabolic alkalosis
- treat cause
- NaCl INeffective
241
medications that cause CaPO4 stones
- topiramate
- zonisamide
- acetazolamide
242
cause of indinavir stones
indinavir
243
cause of atazanavir stones
atazanavir
244
medications that cause CaOxalate stones
- Orlistat
- vitamin C
245
laxative abuse causes what kind of stones
ammonium acid urate stones
246
cause of sulfa stones
sulfa medications
247
Gitelman syndrome is like taking which diuretic
thiazide diuretics
248
Bartter syndrome is like taking which diuretic
loop diuretics
249
Gitelman syndrome abnormal transporter
NaCl cotransporter
250
Bartter syndrome abnormal transporter
NaK2Cl cotransporter
251
Bartter age group
childhood
252
Gitelman age group
adulthood
253
Bartter genetics
AR
254
Gitelman genetics
AR
255
Bartter and Gitelman both require what to be ruled out first?
diuretic abuse
256
Gitelman is a/w
- hypomagnesemia (renal Mg2+ wasting)
- hypocalciuria
257
Bartter is a/w
hypERcalciuria
258
Bartter and Gitelman treatment
amiloride or spironolactone
259
other treatment for Bartter and why
- NSAIDs
- PGs elevated in Bartter
260
AA amyloidosis histopathology
- Congo red stain positive
- no light chain restriction
- amyloid A protein positive
261
AA amyloidosis seen in
- chronic skin infection; IVDA skin poppers
- chonic OM
- TB
262
AA amyloidosis seen in which conditions
- chronic inflammatory diseases
- Crohn's disease
- RA
- familial Mediterranean fever (FMF); renal amyloidosis
263
AA amyloidosis can be seen in which hereditary condition?
hereditary fibrinogen A
264
what conditions and medications should you think of in proximal RTA?
- MM
- amyloidosis
- Fanconi syndrome
- tenofovir
- ifosfamide
- cisplatin
265
K+ level in proximal RTA
low
266
what treatment should initially be avoided in proximal RTA as to not worsen hypokalemia?
bicarbonate
267
proximal RTA is benign, except in
children - stunts growth
268
urine pH in proximal RTA once steady state is reached
\< 5.5 (appropriately low to maintain homeostasis)
269
treatment for proximal RTA
10-15 meq/kg/day bicarb and K+ citrate
270
fibromuscular dysplasia epidemiology
- usually young women
- rare in blacks
271
what is icodextrin, and what is it metabolized into?
- glucose polymer
- metabolized to maltose
272
how does UF occur w/ icodextrin?
from colloid oncotic osmotic pressure
273
can occur in up to 10% of patients on icodextrin
rash
274
what is a rare complication of icodextrin?
sterile peritonitis
275
icodextrin can be useful in what situation?
type 1 UF failure for high solute transporters
276
through what pores does UF occur w/ icodextrin use?
medium sized, 4-6 nm, interendothelial cell pores
277
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
278
what are the 2 systemic causes that can lead to a decreased Kt/V?
- access recirculation
- cardiopulmonary recirculation, especially at lower CO
279
cardiopulmonary recirculation in HD can occur w/?
CVC
280
MCC of recirculation in AVF or AVG
outflow stenosis
281
Kt/V not affected by
- pre-dialysis BUN
- diet compliance
- frequency of HD
282
how is Kt/V achieved?
diffusive clearance AND convective clearance from UF removal
283
Kt/V increased by what factors?
- increase in BFR
- increase in DFR
- time on HD
- mass transfer of dialyzer (size)
284
Kt/V treatment goal which ONLY applies to 3x/week HD
1.2
285
Kt/V decreased by what factors?
- access recirculation
- dialyzer fiber clotting
286
often preceded by a period of type 1 (high effective peritoneal surface area) UF failure
encapsulating peritoneal sclerosis
287
can present early w/ bloody PD fluid
encapsulating peritoneal sclerosis
288
can present later w/ peritoneal calcification or intestinal obstruction w/ complete cocooning of peritoneal membrane
encapsulating peritoneal sclerosis
289
treatment for encapsulating peritoneal sclerosis
- switch to HD and start steroids
- tamoxifen if diagnosed early
- surgery for bowel obstruction
290
can occur in PD patients even after transplant
encapsulating peritoneal sclerosis
291
complication of PD a/w high mortality rate
encapsulating peritoneal sclerosis
292
calcific uremic arteriolopathy (calciphylaxis) usually occurs in what tissue type?
fatty tissues
293
calcific uremic arteriolopathy (calciphylaxis) sex distribution
women \> men
294
calcific uremic arteriolopathy (calciphylaxis) signs/symptoms
- painful
- very tender
- indurated nodules
- subcutaneous plaques that eventually become necrotic
295
calcific uremic arteriolopathy (calciphylaxis) diagnosis
- bone scan which shows soft tissue calcium deposition
- biopsy should be avoided d/t poor healing
296
risk factors for calcific uremic arteriolopathy (calciphylaxis)
- warfarin
- corticosteroids
- vitamin D analogs
- possibly severe hyperparathyroidism
297
calcific uremic arteriolopathy (calciphylaxis) treatment
- remove risk factors
- sodium thiosulfate (12.5-25 g IV) after HD
- hyperbaric O2 may help wound healing
298
least common cause of all infectious peritonitis
fungal peritonitis (3-6%)
299
major risk factor for fungal peritonitis
antibiotic use to treat bacterial peritonitis
300
fungal peritonitis can be avoided during treatment of bacterial peritonitis by doing what?
antifungal ppx
301
fungal peritonitis treatment
- prolonged antifungal tx
- prompt PD catheter removal
302
fungal peritonitis is more common after what?
gram negative peritonitis
303
fungal peritonitis commonly results in
PD dropout and switch to HD
304
decrease in Kt/V in a new start HD patient is usually d/t what?
loss of residual renal function (RRF) over time
305
what can slow down loss of residual renal function (RRF) in PD patients?
ACEI
306
should be used for V in PD patients
ideal body weight
307
not affected by PET transport group types
Kt/V
308
survival in PD patients is related to what?
renal-Kt/V \>\> PD Kt/V (RRF enhances survival)
309
polyuria definition
UOP \> 3 L/24 hours
310
polyuria
- \> 1000 mOsm/day
- osmotic diuresis
- causes
- glucose
- mannitol
- urea
- resolving ATN
- diuretics
- propylene glycol
311
polyuria
- \< 800 mOsm/day
- water diuresis
- next step
water deprivation test
312
polyuria
- \< 800 mOsm/day
- water diuresis
- Na+ \> 140 meq/l
- water deprivation test shows NO change in UOsm
give DDAVP
313
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
314
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
315
polyuria
- \< 800 mOsm/day
- water diuresis
- Na+ LESS THAN 140 meq/l
- water deprivation test shows INCREASE in UOsm
primary polydipsia
316
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
317
transplant medication adverse effects;
- cyclosporine
- HTN
- hyperkalemia
- HLD
- renal dysfunction
- gingival hyperplasia
- hirsutism
transplant medication drug-drug interactions;
- cyclosporine
diltiazem
- verapamil
- increases CSA level
318
transplant medication adverse effects;
- tacrolimus
- HTN (Na+ retention)
- hyperkalemia
- tremor
- renal dysfunction
- hyperglycemia
- alopecia
- diltiazem
- verapamil
- increases FK level
319
transplant medication adverse effects;
- sirolimus
- proteinuria
- HLD
- cytopenia
- slow wound healing
- pulmonary infiltrates
320
transplant medication adverse effects;
- mycophenolate
- GI upset
- myelosuppression
- hepatotoxic
- teratogenic
321
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
322
AMR often occurs when?
after decreasing IS therapy
AMR diagnosis
- peritubular capillaritis
- +C4d staining
- +DSA
323
AMR treatment
- steroids
- plasmapheresis
AND
- IVIG
324
acute cellular (T-cell) mediated rejection pathogenesis
T-cell reaction to donor histocompatibility Ags
325
acute cellular (T-cell) mediated rejection grading criteria
Banff criteria
326
Banff criteria
grades
- interstitium
- tubules
- arteries
327
acute cellular (T-cell) mediated rejection treatment
- steroids
- increase IS
and/or
- anti-thymocyte globulin OR alemtuzumab
328
immunosuppression medications mechanism of action
- belatacept
binds CD80 and CD86 which blocks co-stimulation of T-cells
329
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
330
immunosuppression medications mechanism of action
- basiliximab
IL-2 receptor blocker
331
immunosuppression medications mechanism of action
- azathioprine
disrupts purine synthesis
332
immunosuppression medications mechanism of action
- mycophenolate mofetil (MMF)
disrupts purine synthesis
333
causes of SIADH
- malignancy
- pulmonary issues
- CNS issues
- nausea
- pain
- opiates
- ecstasy
- SSRIs
334
should be ruled out in evaluation of SIADH
- adrenal insufficiency
- hypothyroidism
335
volume state in SIADH
euvolemic
336
serum Osm in SIADH
hypoosmolar, \< 275 mOsm/kg
337
urine Osm and UNa+ in SIADH
\> 100 mOsm/kg and \> 40 mmol/l
338
SIADH treatment
- fluid restriction
- oral NaCl or urea
- 3% saline
- V2 antagonists (not acutely)
- demeclocycline
339
distal, type 1, RTA
- K+ level
LOW
340
distal, type 1, RTA
- bicarb level
NAGMA, usually \< 10
341
distal, type 1, RTA
- urine pH
\> 5.5 (inappropriately HIGH)
342
distal, type 1, RTA
- urine AG (INDIRECT way to measure urine NH4+)
POSITIVE; corresponds to LACK of urine NH4+
343
distal, type 1, RTA
- causes
- Sjogren
- SLE
- amphotericin
- topiramate
344
abused substance that mimics distal, type 1, RTA
toluene (found in paints, paint thinners, fingernail polish, lacquers, adhesives, and rubber)
345
distal, type 1, RTA
- a/w
- nephrolithiasis
- nephrocalcinosis
- CKD
346
distal, type 1, RTA
- treatment
1-2 meq/kg/d of base, usually K+ citrate
347
proximal, type 2, RTA
- K+ level
LOW
348
proximal, type 2, RTA
- bicarb level
NAGMA, usually 12-20
349
distal, type 1, RTA
- pathophysiology
unable to secrete NH4+
350
proximal, type 2, RTA
- pathophysiology
decreased proximal HCO3 reabsorption (lower threshold for reabsorption)
351
proximal, type 2, RTA
- urine pH once steady state is reached
5 (appropriately LOW)
352
proximal, type 2, RTA
- urine AG (INDIRECT way to measure urine NH4+)
NEGATIVE; corresponds to PRESENCE of urine NH4+
353
proximal, type 2, RTA
- a/w
Fanconi syndrome
354
proximal, type 2, RTA
- effects in adults
benign
355
proximal, type 2, RTA
- effects in children
stunts growth
356
proximal, type 2, RTA
- treatment
- 5-10 meq/kg/day of base
- potassium-sparing diuretic (amiloride, or spironolactone)
357
RTA type 4 (hypoaldosteronism)
- K+ level
HIGH
358
RTA type 4 (hypoaldosteronism)
- bicarb level
usually \> 17
359
RTA type 4 (hypoaldosteronism)
- pathophysiology
low aldosterone release and/or response
360
RTA type 4 (hypoaldosteronism)
- urine AG
variable
361
RTA type 4 (hypoaldosteronism)
- causes
- DM
- NSAIDs
- cyclosporine
- old age
362
RTA type 4 (hypoaldosteronism)
- treatment
- loop diuretic
- bicarb
- mineralocorticoid
AND
- K+ binding resin
363
causes of osmotic nephropathy
- sucrose containing IVIG
- hydroxyethyl starch (HES)
- dextran
364
osmotic nephropathy LM on kidney biopsy
swollen tubular cells packed w/ numerous cytoplasmic vacuoles
365
osmotic nephropathy EM on kidney biopsy
vacuoles with phagolysosomes containing the offending agent (sucrose, HES, etc)
366
medications that decrease calcineurin inhibitor level
- rifampin
- barbiturates
- phenytoin
- carbamazepine
- nafcillin
- trimethoprim
- imipenem
- cephalosporins
- St. John wort
- INH
367
medications that increase calcineurin inhibitor level
- verapamil
- diltiazem
- ketoconazole
- fluconazole
- erythromycin
- grapefruit
- amiodarone
- carvedilol
- dapsone
368
medications and condition that increase calcineurin inhibitor toxicity
- amphotericin
- aminoglycosides
- NSAIDs
- lovastatin
- rhabdomyolysis\*\*
369
Liddle syndrome pathophysiology
- GOF mutation
- ENaC overactivity
370
apparent mineralocorticoid excess pathophysiology
11β-HSD2 deficiency
371
glucocorticoid remediable aldosteronism pathophysiology
- chimeric gene fusion (CYP11B1 and CYP11B2)
- zona fasciculata defect
- ACTH receptor overstimulated by cortisol
372
renal artery stenosis renin/aldosterone pathophysiology
renal parenchymal ischemia
373
- 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
374
pregnancy-associated TMA
- \> 24 weeks
- HTN, edema
- effect of delivering fetus
- preeclampsia
- improved
375
pregnancy-associated TMA
- \> 27 weeks
- liver dysfunction and coagulopathy
- effect of delivering fetus
- acute fatty liver (AFL)
- improved
376
pregnancy-associated TMA
- \> 34 weeks
- elevated LFTs (not bili), and severe MAHA
- effect of delivering fetus
- HELLP
- improved
377
pregnancy-associated TMA
- postpartum
- renal failure
- effect of delivering fetus
- HUS
- none
378
pregnancy-associated TMA
- \< 24 weeks
- neurological symptoms
- effect of delivering fetus
- TTP
- none
379
calculator for estimating likelihood of severe ADAMTS13 deficiency in adults w/ suspected TTP
PLASMIC score
380
treatment for suspected TTP
plasmapheresis
381
treatment for drug-induced TMA
- quinine-containing beverage
- IVDU
- chemotherapy
- CNI
- d/c medication
- may be able to avoid plasmapheresis
382
treatment for postpartum complement-mediated TMA
prompt anti-complement treatment (eculizumab)
383
alternative treatment for TMA if plasmapheresis not available
plasma infusion until transfer to another hospital
384
alternative treatment for TMA if anaphylactic reaction to plasma
factor VIII concentrate (contains ADAMTS13) and apheresis (plasma removal)
385
alternative treatment for TMA if can't give any blood products for religious reasons
intensive IS and apheresis
386
how is adequate response to plasmapheresis assessed?
normalization of platelet count
387
eculizumab is removed by
plasmapheresis
388
what ppx must be given prior to eculizumab administration?
- abx x 2 weeks before vaccine is effective
- meningococcal vaccine
389
cause for primary FSGS
- specific cause unknown
- ? circulating permeability factors
390
cause for familial/genetic FSGS
mutation in podocyte genes (eg podocin, nephrin)
391
causes for virus-associated FSGS
- HIV-1
- parvovirus B19
- SM 40
- CMV
- EBV
392
causes for drug-induced FSGS
- heroin
- interferon alfa, beta, and gamma
- lithium
- pamidronate
- sirolimus
- CNIs
- anabolic steroids
393
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
394
causes for adaptive FSGS
- conditions w/ initially normal renal mass
- HTN
- AED
- TMA
- RAS
- cyanotic congenital heart disease
- sickle cell anemia
395
the adaptive form of FSGS is mediated by adaptive structural-functional responses to glomerular hypertension caused by
elevated glomerular capillary pressures and flows
396
initial urine pH in proximal, type 2, RTA
alkaline, 8
397
urine NH4+ level in proximal, type 2, RTA once steady state is reached
HIGH
398
some studies have indicated that magnesium wasting in Gitelman syndrome may be d/t down-regulation of what?
TRPM6 in DCT
399
expected acid-base disturbance in aspirin OD
respiratory alkalosis w/ compensatory metabolic acidosis
400
best initial test for a patient suspected of having pheochromocytoma
24-hour URINARY fractionated metanephrines
- 98% sensitivity
- 98% specificity
401
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
402
what treatment should a patient with pheochromocytoma receive prior to surgery?
- alpha and beta blockers
- HIGH SALT diet (to mitigate volume contraction and orthostasis)
403
generally, if problem on PD is inadequate solute clearance, what is the next best step?
increase volume in each exchange
404
ACCOMPLISH trial showed combination of which antihypertensives reduced cardiovascular morbidity and mortality, and slowed CKD progression?
ACEI and CCB
405
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
406
renal and GU abnormalities and associated cause
- Turner's syndrome
horseshoe kidney
407
renal and GU abnormalities and associated cause
- epispadias
bladder extrophy
408
renal and GU abnormalities and associated cause
- undescended testes
- inguinal hernias
- CHARGE syndrome
hypospadias
409
renal and GU abnormalities and associated cause
- WAGR
- aniridia
- GU malformation
- intellectual disability
Wilm's tumor
410
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)
411
inulin clearance (GFR) formula
- inulin is neither secreted NOR reabsorbed
U(inulin)/P(inulin) x V (urine flow rate)
412
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)
413
respiratory alkalosis
- for every decrease in PCO2 below 40, bicarb decreases by
2-3
414
respiratory acidosis
- for every increase in PCO2 above 40, bicarb increases by
2-3
