Nephrology Pearls of Wisdom Flashcards
1
Q
tuberous sclerosis genetics
A
AD
2
Q
medication class that slows tumor growth in tuberous sclerosis
A
mTOR inhibitors
3
Q
what are the mTOR inhibitors?
A
- sirolimus (aka rapamycin)
- everolimus
- temsirolimus
4
Q
renal manifestation of tuberous sclerosis
A
- renal angiomyolipomas
- RCC (very rare)
5
Q
Fabry disease genetics
A
XL
6
Q
Fabry disease mutation
A
GLA mutation
7
Q
skin manifestation seen in Fabry disease
A
angiokeratomas
8
Q
enzyme defect causing Fabry disease
A
a-galactosidase A
9
Q
finding seen on EM in Fabry disease
A
Zebra bodies
10
Q
Zebra bodies can also be seen on patients taking what medication?
A
hydroxychloroquine
11
Q
treatment for Fabry disease
A
ERT (enzyme replacement therapy)
12
Q
MCD age distribution
A
children and elderly (bimodal distribution)
13
Q
what should be ruled out in patients with MCD?
A
- NSAID use
- lymphoma
- lithium use
14
Q
if AIN and AKI think of
A
NSAID use
15
Q
rate of relapse in MCD
A
50%
16
Q
what other renal manifestation is also commonly seen in patients with MCD?
A
ATN
17
Q
ADPKD1 compared to ADPKD2
A
occurs earlier and more ESRD
18
Q
PKD1, which chromosome?
A
16
19
Q
PKD2, which chromosome?
A
4
20
Q
polycystin proteins affect what?
A
cilium
21
Q
extrarenal manifestations of ADPKD
A
- brain aneurysms
- diverticula
- abdominal wall hernias
- MVP
22
Q
preferred bp medication for PKD
A
ACEI
23
Q
PKD medication to slow cyst growth
A
vaptans
24
Q
West African Sub-Saharan gene that protects against trypanosomes
A
APOL1
25
2 variants of APOL1
G1 and G2
26
2 high-risk alleles of APOL1 increase risk of
- HIV nephropathy
- sickle cell nephropathy
- ESRD from FSGS
- hypertensive nephrosclerosis
27
2 high-risk alleles of APOL1 increase what in ESRD patients?
survival
28
what % of African Americans carry 2 high risk alleles
14%
29
MCC of AIN
abx, namely B-lactams and sulfa-based antibiotics
30
AIN from abx have what w/i infiltrate?
eosinophils
31
AIN from NSAIDs does NOT have what?
eosinophils
32
what improves short- and long-term outcome if administered early in AIN?
steroids
33
rash is seen in AIN when caused by
abx
34
in AIN, which medication class do you NOT see rash?
PPIs
35
aside from AIN, other manifestations seen in IgG4-related disease
- pancreatitis
- retroperitoneal fibrosis
- masses in other organs
36
aside from AIN, other manifestations seen in sarcoidosis
- hypercalcemia
| - hypercalciuria
37
monoclonal gammopathy of renal significance (MGRS)
- AL amyloidosis
- is usually what type?
lambda
38
monoclonal gammopathy of renal significance (MGRS)
- AL amyloidosis
- what size fibrils?
10 nm
39
monoclonal gammopathy of renal significance (MGRS)
- light-chain deposition disease (LCDD)
- is usually what type?
kappa > lambda
40
monoclonal gammopathy of renal significance (MGRS)
- light-chain deposition disease (LCDD)
- staining?
tubular light chain staining
41
monoclonal gammopathy of renal significance (MGRS)
- light-chain deposition disease (LCDD)
- appearance on biopsy?
nodular, looks like diabetic KW disease
42
monoclonal gammopathy of renal significance (MGRS)
- cast nephropathy
- usually what type?
kappa = lambda
43
monoclonal gammopathy of renal significance (MGRS)
- cast nephropathy
- what's seen on LM?
fractured casts
44
monoclonal gammopathy of renal significance (MGRS) can present w/?
Fanconi syndrome (proximal tubulopathy)
45
in monoclonal gammopathy of renal significance (MGRS), urine dipstick doesn't detect what?
light chains (Bence Jones proteins)
46
serum [Na+] ~
(TBNa+ + TBK+)/TBW
47
- hyponatremia
- hypotonic
- hypERvolemic
- urine Na+ < 20 mmol/L
- heart failure
| - liver failure
48
- hyponatremia
- hypotonic
- hypERvolemic
- urine Na+ > 20 mmol/L
- renal failure
49
- hyponatremia
- hypotonic
- EUvolemic
- urine Na+ > 20 mmol/L
- urine Osm > 100
- SIADH
- adrenal insufficiency
- hypothyroidism
- stress
- drug use
50
- hyponatremia
- hypotonic
- EUvolemic
- urine Na+ > 20 mmol/L
- urine Osm < 100
- primary polydipsia
| - low solute intake
51
- hyponatremia
- hypotonic
- EUvolemic
- urine Na+ > 20 mmol/L
- variable urine Osm
reset osmostat
52
- hyponatremia
- hypotonic
- hypovolemia
- urine Na+ > 20 mmol/L
- salt wasting nephropathy
- hypoaldosteronism
- active diuretic use
53
- hyponatremia
- hypotonic
- hypovolemia
- urine Na+ < 20 mmol/L
- extrarenal sodium loss (GI)
| - remote diuretic use
54
- hyponatremia
| - hypertonic
- hyperglycemia
| - mannitol
55
- hyponatremia
| - normo-osmolar
- hyperlipidemia
| - HLD can be 2/2 lipoprotein X a/w cholestatic or obstructive jaundice
56
presentation of D-lactic acidosis
- AMS
| - ataxia
57
lactic acid level in D-lactic acidosis
normal, because they only measure L-lactate
58
cause for D-lactic acidosis
colonic bacteria exposed to glucose, usually from blind loop
59
tx for D-lactic acidosis
complex carbohydrate diet and antibiotics
60
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
61
MCC of serum osmolal gap
ethanol
62
osmolal gap and AG in isopropyl alcohol
- increased osmolal gap
| - NO AG (unless shock develops leading to lactic acidosis)
63
in ICU patient w/ AG consider what possible cause
propylene glycol from iv gtts
64
in ER patient w/ AG consider what possible causes
- salicylate
- EG
- methanol
- metformin intoxication
65
sglt2 inhibitor w/ AG, think of
euglycemic DKA
66
what elements are mismeasured as Cl-?
- bromide
| - iodide
67
high unmeasured cation
lithium
68
high unmeasured cation in MM
gammaglobulins (severe hypergammaglobulinemia)
69
elements that are unmeasured cations
- Ca++
| - Mg++
70
mechanism of PPI causing hypomagnesemia
decreased intestinal Mg absorption
71
urine FeMg in PPI use
low
72
hypomagnesemia causes
- hypoparathyroidism --> hypocalcemia
| - K+ wasting
73
medications that cause renal wasting of hypomagnesemia
- aminoglycosides
- cisplatin
- cetuximab
- loop diuretics
- thiazide diuretics (except amiloride)
74
syndrome that leads to hypomagnesemia
Gitelman syndrome
75
causes of Fanconi syndrome
- tenofovir (+/- nephrogenic DI)
- ifosfamide (+/- nephrogenic DI)
- cisplatin
- MM
76
adults need w/u for what when Fanconi syndrome is diagnosed?
paraproteinemia
77
what do you see on labs w/ Fanconi syndrome?
- renal K+, PO4, and urate wasting
| - aminoaciduria
78
what other renal abnormality can be seen w/ Fanconi syndrome?
proximal, type 2, RTA
79
magnesium in Fanconi syndrome
normal
80
HAGMA in elderly woman
5-oxoproline (pyroglutamic acid) d/t acetaminophen use
81
HAGMA from sglt2 inhibitor
severe ketoacidosis w/ euglycemia
82
HAGMA from metformin
D lactic acidosis
83
HAGMA from linezolid
lactic acidosis
84
HAGMA from diethylene glycol
- brake fluid
| - cranial nerve abnormalities
85
hypomagnesemia causes
- UMg < 20
- FeMg < 2.5
- PPI
| - diarrhea
86
hypomagnesemia
- UMg > 20
- FeMg > 2.5
- what do you check next?
check 24-hour urine Ca++
87
hypomagnesemia causes
- UMg > 20
- FeMg > 2.5
- if 24-hour urine Ca++ < 150
- thiazide diuretics
| - Gitelman syndrome
88
hypomagnesemia causes
- UMg > 20
- FeMg > 2.5
- if 24-hour urine Ca++ > 250
- loop diuretics
- Bartter syndrome
- cisplatin
89
hypokalemia causes
- low/normal volume state
- low bicarbonate
- diarrhea
- laxative use
- toluene
- RTA
- NAGMA
90
hypokalemia causes
- low/normal volume state
- low magnesium
- familial hypomagnesemia
91
hypokalemia causes
- low/normal volume state
- urine Cl- low
- vomiting
92
hypokalemia causes
- low/normal volume state
- urine Cl- high
- urine Ca++ LOW
- thiazide diuretics
| - Gitelman syndrome
93
hypokalemia causes
- low/normal volume state
- urine Cl- high
- urine Ca++ HIGH
- loop diuretics
| - Bartter syndrome
94
hypokalemia causes
- high volume state
- LOW renin and LOW aldosterone
- Liddle syndrome
- apparent mineralocorticoid excess (AME)
- licorice
95
hypokalemia causes
- high volume state
- LOW renin and HIGH aldosterone
- primary hyperaldosteronism
- Cushing
- glucocorticoid remediable aldosteronism (GRA)
96
hypokalemia causes
- high volume state
- HIGH renin and HIGH aldosterone
- malignant HTN
- renal artery stenosis (RAS)
- renin secreting tumor
97
tolvaptan for hyponatremia
- start only in hospital
- do NOT fluid restrict
- avoid in liver disease
- need HIGHER doses in CHF
98
tolvaptan effect in ADPKD
- slows cyst growth
| - slows GFR loss
99
tolvaptan dose in ADPKD
2-4 times that for hyponatremia
100
adverse effects from hypophosphatemia
- rhabdomyolysis
- respiratory failure
- heart failure
101
pseudohypophosphatemia cause
- when labs drawn from catheter w/ heparin
102
distal, type 1, RTA
| - what disease should you think of?
Sjogren
103
distal, type 1, RTA
| - urine pH
> 5.5 (inappropriately high)
104
distal, type 1, RTA
| - urine AG
POSITIVE
105
distal, type 1, RTA
| - kidney stone type
CaPO4 stones
106
distal, type 1, RTA
| - treatment
NaHCO3 1 meq/kg/day po
107
incomplete distal, type 1, RTA is seen in what cystic kidney disease?
medullary sponge kidney
108
what should be treated first in distal, type 1, RTA?
- hypokalemia
| - giving bicarb first will further lower K+ can lead to respiratory failure
109
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)
```
110
hypophosphatemia causes
- HIGH UPhos
- > 100 mg/day
- FePO4 > 5%
- serum Ca++ LOW
- vitamin D deficiency
111
hypophosphatemia causes
- HIGH UPhos
- > 100 mg/day
- FePO4 > 5%
- serum Ca++ HIGH
PHPT
112
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
113
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)
114
- XLH name
| - associated gene
- XL hypophosphatemia
| - PHEX
115
- ADHR name
| - associated gene
- AD hypophosphatemic rickets
| - FGF-23
116
- ARHR name
| - associated gene
- AR hypophosphatemic rickets
| - DMP1, ENPP1
117
TIO name
tumor-induced osteomalacia
118
FD name
fibrous dysplasia
119
- HHRH name
| - associated gene
- hereditary hypophosphatemic rickets w/ hypercalciuria
| - NPT2c
120
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
121
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
122
Alport's disease genetics
- XL
| - young men
123
Alport disease mutation
alpha 5 chain of type 4 collagen
124
clinical manifestations of Alport disease
- renal disease
- deafness
- anterior lenticonus
125
clinical manifestations in WOMEN w/ Alport disease
- hematuria ONLY
| - rarely CKD
126
can be seen in Alport disease patients POST-renal transplant
crescentic anti-GBM GN, but NO pulmonary hemorrhage
127
EM findings in crescentic anti-GBM GN in Alport disease
basement membrane looks layered
128
TBMD genetics
- AD
| - family history w/o ESRD or deafness
129
TBMD clinical manifestations
- microscopic hematuria
- usually minimal proteinuria
- usually clinically benign
130
hypocomplementemic GNs
| - SLE
- classical pathway
| - usually BOTH C3 and C4
131
hypocomplementemic GNs
| - cryoglobulinemia
- classical pathway
- VERY LOW C4; can be undetectable
- sometimes low C3
132
hypocomplementemic GNs
| - postinfectious GN
- alternate pathway
| - C3 ONLY
133
hypocomplementemic GNs
| - MPGN
- alternate pathway
- C3 ONLY
- especially in C3 nephropathy
134
hypocomplementemic GNs can also be seen in what instances?
- chronic infection
- subacute bacterial endocarditis
- HCV
- AED
135
hypocomplementemic GNs
| - C3 GN
- C3 ONLY
136
hypocomplementemic GNs
| - DDD
- C3 ONLY
137
primary membranous GN lab and histological finding
PLA2R Ab in serum and on biopsy stain
138
primary membranous GN is a/w what Ab?
IgG4
139
membranous GN w/ "full-house" IF
think of SLE
140
```
lupus nephritis
- class 1
```
minimal mesangial LN
141
lupus nephritis
- class 1
- LM findings
normal
142
lupus nephritis
- class 1
- IF findings
mesangial immune deposits
143
lupus nephritis
- class 1
- clinical manifestations
mild proteinuria
144
```
lupus nephritis
- class 2
```
mesangial proliferative LN
145
lupus nephritis
- class 2
- LM findings
- mesangial hypercellularity
- mesangial matrix expansion
- mesangial immune deposits
146
lupus nephritis
- class 2
- IF and EM findings
- mesangial immune deposits
| - few subepithelial or subendothelial immune deposits
147
lupus nephritis
- class 2
- clinical manifestations
- mild renal disease such as hematuria or proteinuria
| - usually no specific treatment needed
148
```
lupus nephritis
- class 3 A
```
active lesions
| - focal proliferative LN
149
```
lupus nephritis
- class 3 A/C
```
active and chronic lesions
| - focal proliferative and sclerosing LN
150
```
lupus nephritis
- class 3 C
```
chronic inactive lesions
| - focal sclerosing LN
151
lupus nephritis
- class 3
- LM findings
- active or inactive, focal, segmental, or global GN
| - < 50% of all glomeruli
152
lupus nephritis
- class 3
- IF and EM findings
subendothelial and mesangial immune deposits
153
lupus nephritis
- class 3
- clinical manifestations
- mild to moderate renal disease
- hematuria
- moderate proteinuria
- worsening renal function in significant minority
154
```
lupus nephritis
- class 4-S (A)
```
active lesions
| - diffuse segmental proliferative LN
155
```
lupus nephritis
- class 4-G (A)
```
active lesions
| - diffuse global proliferative LN
156
```
lupus nephritis
- class 4-S (A/C)
```
active and chronic lesions
| - diffuse segmental proliferative and sclerosing LN
157
```
lupus nephritis
- class 4-G (A/C)
```
active and chronic lesions
| - diffuse global proliferative and sclerosing LN
158
```
lupus nephritis
- class 4-S (C)
```
chronic inactive lesions w/ scars
| - diffuse segmental sclerosing LN
159
```
lupus nephritis
- class 4-G (C)
```
chronic inactive lesions w/ scars
| - diffuse global sclerosing LN
160
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
161
lupus nephritis
- class 4
- IF and EM findings
subendothelial immune deposits
162
lupus nephritis
- class 4
- clinical manifestations
- HTN
- edema
- active urinary sediment
- worsening renal function
- nephrotic range proteinuria
- active extrarenal SLE in many patients
163
```
lupus nephritis
- class 5
```
membranous LN
164
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
165
lupus nephritis
- class 5
- IF and EM findings
- subepithelial and intramembranous immune deposits
| - subendothelial deposits present ONLY when proliferative component present
166
lupus nephritis
- class 5
- clinical manifestations
- nephrotic syndrome
| - usually w/o manifestations of active SLE
167
```
lupus nephritis
- class 6
```
advanced sclerosis LN
168
lupus nephritis
- class 6
- LM findings
- advanced glomerular sclerosis = 90% of glomeruli
- severe IFTA
- manifestations of irreversible renal injury
169
lupus nephritis
- class 6
- clinical manifestations
- significant CKD or ESRD in most cases
| - unlikely to respond to medical therapy
170
if PLA2R negative, think of
- cancer
- SLE
- medications
- viral infections
- syphilis
- parasites
171
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
172
anti-GBM disease age distribution
- bimodal
| - young men and elderly
173
- anti-GBM Abs can be seen in what disease?
| - how does it affect prognosis?
- ANCA vasculitis
| - worsens prognosis
174
anti-GBM disease, if anuric
rarely recovers
175
does anti-GBM recur in patients or transplant?
usually no
176
anti-GBM a/w
- smoking
| - hydrocarbon exposure
177
HIVAN is typically what on histology?
collapsing FSGS
178
HIVAN RUS
large kidneys
179
HIVAN clinical manifestations
- no HTN
| - little to no edema
180
HIVAN pathology
- microcysts
| - tubular reticular inclusions (TRIs)
181
common in HIVAN
immune complex disease lesions
182
increased risk of HIVAN in patients w/ what?
APOL-1 high-risk alleles
183
IgA nephropathy pathogenesis
abnormal polymeric IgA, missing galactose on side chain (glycosylation)
184
IgA nephropathy epidemiology
- Asians
- Hispanics
- rare in African Americans
185
IgA nephropathy commonly seen in
liver disease, but usually not clinically important
186
IgA nephropathy in transplant
high recurrence?
187
IgA dominant PIGN seen w/?
- Staphylococcus aureus infections, especially in DM
| - bad prognosis
188
scleroderma renal crisis clinical manifestations
- malignant HTN
- TMA
- MAHA (microangiopathic hemolytic anemia)
- AKI
189
scleroderma renal crisis more common w/
severe skin involvement
190
scleroderma renal crisis lifesaving treatment
RAAS blockade
191
scleroderma renal crisis can be caused by
steroids
192
sickle cell nephropathy renal manifestations
microscopic hematuria to papillary necrosis
193
urine in sickle cell nephropathy
isosthenuria early in disease
194
RTA in sickle cell nephropathy
type 4 RTA
195
can cause proteinuria and ESRD in sickle cell disease patients
FSGS
196
hematuria in SCT
can be microscopic or gross hematuria
197
what cancer can be seen in SCT?
rarely, medullary cell Ca (aggressive)
198
primary FSGS acts like
MCD
199
if FSGS is steroid non-responsive think of
podocin mutation
200
FSGS variant w/ best steroid response
tip lesion
201
more common in secondary FSGS
perihilar lesions
202
causes of FSGS
- primary (idiopathic)
- secondary;
- familial/genetic
- virus-associated
- drug-induced
- adaptive
203
collapsing FSGS a/w
- HIV-1
- parvovirus B19
- pamidronate
204
TMA results from
- alternate complement pathway abnormalities
| - factor H, factor 1, C3, factor B, thrombomodulin
205
TMA can p/w
- AKI
- HTN
- MAHA
206
recurrence rate of TMA in transplant patients
high
207
causes of NAGMA
- GI (diarrhea)
- renal (RTAs)
- hyperkalemia
- aldosterone deficiency
208
causes of NAGMA w/ POSITIVE urine anion gap
- distal, type 1, RTA
- proximal, type 2, RTA, prior to reaching steady state
- toluene
209
causes of NAGMA w/ NEGATIVE urine anion gap
- diarrhea
| - proximal, type 2, RTA, at steady state
210
causes of HAGMA
- high acid input
- ingestion
- EtOH
- methanol
- ethylene glycol
- ASA
211
causes of HAGMA
- high acid input
- production
- shock
- lactic acidosis
- ketoacidosis
- starvation
- DM
- EtOH
212
causes of HAGMA
| - low acid input
- AKI
| - CKD
213
NAGMA HARDUP
- Hyperalimentation/HCl
- Acetazolamide
- RTA
- Diarrhea
- Ureteroenteric fistula (ileal conduit)
- Post-hypOcapnic state
214
HAGMA GOLDMARK
- Glycols
- Oxoproline (pyroglutamic acid)
- L-lactate
- D-lactate
- Methanol
- Aspirin
- Renal failure
- Ketones
215
mortality in HD decreased in
- blacks
- larger BMI
- longer tx time
216
mortality in HD increased in
- hyperphosphatemia
| - hypoalbuminemia
217
mortality in HD a/w
- low K+ baths
| - higher UF rates
218
mortality in HD a/w what conditions
- restless leg syndrome
- sleep d/o's
- pruritus
- ESA resistance
219
clinical manifestations of lead nephropathy
- HTN
- gout
- CKD
- AKI w/ acute Pb intoxication
220
lead nephropathy histopathology
chronic tubulointerstitial nephritis
221
causes of lead nephropathy
- battery factory work
| - moonshine consumption
222
diagnose lead toxicity
EDTA test
223
lead exposure a/w
CKD
224
lithium nephropathy clinical manifestations
- nephrogenic DI
- CKD in chronic exposure
- ATN in acute toxicity
225
may prevent toxicity w/ chronic lithium use
amiloride
226
a/w lithium nephropathy
- distal, type 1, RTA
- MCD
- drug-induced FSGS (rare)
227
treatment of acute lithium overdose
- dialyze (beware of rebound, HD x 4 hours, CRRT as adjuvant)
228
IgG4-related disease histopathology
AIN w/ "storiform" (swirly) pattern
229
IgG4-related disease clinical manifestations
- kidney mass
- AI pancreatitis +/- pancreatic mass
- retroperitoneal fibrosis
- aortitis
230
IgG4-related disease serum IgG4 level
high
231
IgG4-related disease treatment
- steroids, long course
| - high relapse rate
232
mechanism of hypercalcemia in sarcoidosis
granulomatous production of 1-25 vitamin D (1-hydroxylation)
233
most common renal manifestation of sarcoidosis
hypercalciuria
234
if tubulointerstitial nephritis and UVEITIS think
TINU (tubulointerstitial nephritis and uveitis)
235
histopathology of sarcoidosis
renal granulomas, but not always
236
test which may help in diagnosis of idiopathic AIN
ACE level
237
sarcoidosis treatment
- steroids acutely
| - methotrexate, MMF, or azathioprine chronically
238
clinical manifestations in pregnancy
- cardiac output increases
- plasma volume increases
- GFR increases
239
serum Cr in pregnancy
should be low
240
physiology of low bp in pregnancy
SVR decreases more than CO increases
241
acid base in pregnancy
respiratory alkalosis (d/t progesterone) w/ compensatory NAGMA
242
hyponatremia in pregnancy is d/t?
reset osmostat
243
treatment for primary hyperoxaluria
OLT
244
secondary (enteric) hyperoxaluria seen in
intestinal bypass (bariatric surgeries)
245
secondary (enteric) hyperoxaluria can cause
- CaOxalate stones
- CKD
- ESRD
246
other causes of secondary (enteric) hyperoxaluria
- excessive vitamin C
- cashews
- starfruit (carambola) juice
- Orlistat
247
secondary (enteric) hyperoxaluria treatment
- oral calcium to bind ingested oxalate and prevent absorption
- low-oxalate diet
248
metabolic alkalosis
- urine Cl- < 15 mmol/l
- chloride RESPONSIVE
- GI loss
| - renal loss
249
metabolic alkalosis
- urine Cl- < 15 mmol/l
- chloride RESPONSIVE
- causes of GI loss
- gastric fluid losses
- Cl- losing diarrhea
- laxative abuse
250
metabolic alkalosis
- urine Cl- < 15 mmol/l
- chloride RESPONSIVE
- causes of renal loss
- diuretic use
- severe hypokalemia
- post-hypercapnic alkalosis
251
treatment of chloride RESPONSIVE metabolic alkalosis
- volume expansion (NaCl)
| - K+ replacement (KCl)
252
metabolic alkalosis
- urine Cl- > 20 mmol/l
- chloride RESISTANT
- causes
- mineralocorticoid excess
- ACTIVE diuretic use
- Bartter syndrome
- Gitelman syndrome
- Cushing syndrome
- hypokalemia
253
treatment of chloride RESISTANT metabolic alkalosis
- treat cause
| - NaCl INeffective
254
medications that cause CaPO4 stones
- topiramate
- zonisamide
- acetazolamide
255
cause of indinavir stones
indinavir
256
cause of atazanavir stones
atazanavir
257
medications that cause CaOxalate stones
- Orlistat
| - vitamin C
258
laxative abuse causes what kind of stones
ammonium acid urate stones
259
cause of sulfa stones
sulfa medications
260
Gitelman syndrome is like taking which diuretic
thiazide diuretics
261
Bartter syndrome is like taking which diuretic
loop diuretics
262
Gitelman syndrome abnormal transporter
NaCl cotransporter
263
Bartter syndrome abnormal transporter
NaK2Cl cotransporter
264
Bartter age group
childhood
265
Gitelman age group
adulthood
266
Bartter genetics
AR
267
Gitelman genetics
AR
268
Bartter and Gitelman both require what to be ruled out first?
diuretic abuse
269
Gitelman is a/w
- hypomagnesemia (renal Mg2+ wasting)
| - hypocalciuria
270
Bartter is a/w
hypERcalciuria
271
Bartter and Gitelman treatment
amiloride or spironolactone
272
other treatment for Bartter and why
- NSAIDs
| - PGs elevated in Bartter
273
AA amyloidosis histopathology
- Congo red stain positive
- no light chain restriction
- amyloid A protein positive
274
AA amyloidosis seen in
- chronic skin infection; IVDA skin poppers
- chonic OM
- TB
275
AA amyloidosis seen in which conditions
- chronic inflammatory diseases
- Crohn's disease
- RA
- familial Mediterranean fever (FMF); renal amyloidosis
276
AA amyloidosis can be seen in which hereditary condition?
hereditary fibrinogen A
277
what conditions and medications should you think of in proximal RTA?
- MM
- amyloidosis
- Fanconi syndrome
- tenofovir
- ifosfamide
- cisplatin
278
K+ level in proximal RTA
low
279
what treatment should initially be avoided in proximal RTA as to not worsen hypokalemia?
bicarbonate
280
proximal RTA is benign, except in
children - stunts growth
281
urine pH in proximal RTA once steady state is reached
< 5.5 (appropriately low to maintain homeostasis)
282
treatment for proximal RTA
10-15 meq/kg/day bicarb and K+ citrate
283
fibromuscular dysplasia epidemiology
- usually young women
| - rare in blacks
284
what is icodextrin, and what is it metabolized into?
- glucose polymer
| - metabolized to maltose
285
how does UF occur w/ icodextrin?
from colloid oncotic osmotic pressure
286
can occur in up to 10% of patients on icodextrin
rash
287
what is a rare complication of icodextrin?
sterile peritonitis
288
icodextrin can be useful in what situation?
type 1 UF failure for high solute transporters
289
through what pores does UF occur w/ icodextrin use?
medium sized, 4-6 nm, interendothelial cell pores
290
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
291
what are the 2 systemic causes that can lead to a decreased Kt/V?
- access recirculation
| - cardiopulmonary recirculation, especially at lower CO
292
cardiopulmonary recirculation in HD can occur w/?
CVC
293
MCC of recirculation in AVF or AVG
outflow stenosis
294
Kt/V not affected by
- pre-dialysis BUN
- diet compliance
- frequency of HD
295
how is Kt/V achieved?
diffusive clearance AND convective clearance from UF removal
296
Kt/V increased by what factors?
- increase in BFR
- increase in DFR
- time on HD
- mass transfer of dialyzer (size)
297
Kt/V treatment goal which ONLY applies to 3x/week HD
1.2
298
Kt/V decreased by what factors?
- access recirculation
| - dialyzer fiber clotting
299
often preceded by a period of type 1 (high effective peritoneal surface area) UF failure
encapsulating peritoneal sclerosis
300
can present early w/ bloody PD fluid
encapsulating peritoneal sclerosis
301
can present later w/ peritoneal calcification or intestinal obstruction w/ complete cocooning of peritoneal membrane
encapsulating peritoneal sclerosis
302
treatment for encapsulating peritoneal sclerosis
- switch to HD and start steroids
- tamoxifen if diagnosed early
- surgery for bowel obstruction
303
can occur in PD patients even after transplant
encapsulating peritoneal sclerosis
304
complication of PD a/w high mortality rate
encapsulating peritoneal sclerosis
305
calcific uremic arteriolopathy (calciphylaxis) usually occurs in what tissue type?
fatty tissues
306
calcific uremic arteriolopathy (calciphylaxis) sex distribution
women > men
307
calcific uremic arteriolopathy (calciphylaxis) signs/symptoms
- painful
- very tender
- indurated nodules
- subcutaneous plaques that eventually become necrotic
308
calcific uremic arteriolopathy (calciphylaxis) diagnosis
- bone scan which shows soft tissue calcium deposition
| - biopsy should be avoided d/t poor healing
309
risk factors for calcific uremic arteriolopathy (calciphylaxis)
- warfarin
- corticosteroids
- vitamin D analogs
- possibly severe hyperparathyroidism
310
calcific uremic arteriolopathy (calciphylaxis) treatment
- remove risk factors
- sodium thiosulfate (12.5-25 g IV) after HD
- hyperbaric O2 may help wound healing
311
least common cause of all infectious peritonitis
fungal peritonitis (3-6%)
312
major risk factor for fungal peritonitis
antibiotic use to treat bacterial peritonitis
313
fungal peritonitis can be avoided during treatment of bacterial peritonitis by doing what?
antifungal ppx
314
fungal peritonitis treatment
- prolonged antifungal tx
| - prompt PD catheter removal
315
fungal peritonitis is more common after what?
gram negative peritonitis
316
fungal peritonitis commonly results in
PD dropout and switch to HD
317
decrease in Kt/V in a new start HD patient is usually d/t what?
loss of residual renal function (RRF) over time
318
what can slow down loss of residual renal function (RRF) in PD patients?
ACEI
319
should be used for V in PD patients
ideal body weight
320
not affected by PET transport group types
Kt/V
321
survival in PD patients is related to what?
renal-Kt/V >> PD Kt/V (RRF enhances survival)
322
polyuria definition
UOP > 3 L/24 hours
323
polyuria
- > 1000 mOsm/day
- osmotic diuresis
- causes
- glucose
- mannitol
- urea
- resolving ATN
- diuretics
- propylene glycol
324
polyuria
- < 800 mOsm/day
- water diuresis
- next step
water deprivation test
325
polyuria
- < 800 mOsm/day
- water diuresis
- Na+ > 140 meq/l
- water deprivation test shows NO change in UOsm
give DDAVP
326
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
327
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
328
polyuria
- < 800 mOsm/day
- water diuresis
- Na+ LESS THAN 140 meq/l
- water deprivation test shows INCREASE in UOsm
primary polydipsia
329
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
330
transplant medication adverse effects;
| - cyclosporine
- HTN
- hyperkalemia
- HLD
- renal dysfunction
- gingival hyperplasia
- hirsutism
331
transplant medication drug-drug interactions;
| - cyclosporine
- diltiazem
- verapamil
- increases CSA level
332
transplant medication adverse effects;
| - tacrolimus
- HTN (Na+ retention)
- hyperkalemia
- tremor
- renal dysfunction
- hyperglycemia
- alopecia
333
transplant medication drug-drug interactions;
| - tacrolimus
- diltiazem
- verapamil
- increases FK level
334
transplant medication adverse effects;
| - sirolimus
- proteinuria
- HLD
- cytopenia
- slow wound healing
- pulmonary infiltrates
335
transplant medication adverse effects;
| - mycophenolate
- GI upset
- myelosuppression
- hepatotoxic
- teratogenic
336
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
337
AMR often occurs when?
after decreasing IS therapy
338
AMR diagnosis
- peritubular capillaritis
- +C4d staining
- +DSA
339
AMR treatment
- steroids
- plasmapheresis
AND
- IVIG
340
acute cellular (T-cell) mediated rejection pathogenesis
T-cell reaction to donor histocompatibility Ags
341
acute cellular (T-cell) mediated rejection grading criteria
Banff criteria
342
Banff criteria
grades
- interstitium
- tubules
- arteries
343
acute cellular (T-cell) mediated rejection treatment
- steroids
- increase IS
and/or
- anti-thymocyte globulin OR alemtuzumab
344
immunosuppression medications mechanism of action
| - belatacept
binds CD80 and CD86 which blocks co-stimulation of T-cells
345
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
346
immunosuppression medications mechanism of action
| - basiliximab
IL-2 receptor blocker
347
immunosuppression medications mechanism of action
| - azathioprine
disrupts purine synthesis
348
immunosuppression medications mechanism of action
| - mycophenolate mofetil (MMF)
disrupts purine synthesis
349
causes of SIADH
- malignancy
- pulmonary issues
- CNS issues
- nausea
- pain
- opiates
- ecstasy
- SSRIs
350
should be ruled out in evaluation of SIADH
- adrenal insufficiency
| - hypothyroidism
351
volume state in SIADH
euvolemic
352
serum Osm in SIADH
hypoosmolar, < 275 mOsm/kg
353
urine Osm and UNa+ in SIADH
> 100 mOsm/kg and > 40 mmol/l
354
SIADH treatment
- fluid restriction
- oral NaCl or urea
- 3% saline
- V2 antagonists (not acutely)
- demeclocycline
355
distal, type 1, RTA
| - K+ level
LOW
356
distal, type 1, RTA
| - bicarb level
NAGMA, usually < 10
357
distal, type 1, RTA
| - urine pH
> 5.5 (inappropriately HIGH)
358
distal, type 1, RTA
| - urine AG (INDIRECT way to measure urine NH4+)
POSITIVE; corresponds to LACK of urine NH4+
359
distal, type 1, RTA
| - causes
- Sjogren
- SLE
- amphotericin
- topiramate
360
abused substance that mimics distal, type 1, RTA
toluene (found in paints, paint thinners, fingernail polish, lacquers, adhesives, and rubber)
361
distal, type 1, RTA
| - a/w
- nephrolithiasis
- nephrocalcinosis
- CKD
362
distal, type 1, RTA
| - treatment
1-2 meq/kg/d of base, usually K+ citrate
363
proximal, type 2, RTA
| - K+ level
LOW
364
proximal, type 2, RTA
| - bicarb level
NAGMA, usually 12-20
365
distal, type 1, RTA
| - pathophysiology
unable to secrete NH4+
366
proximal, type 2, RTA
| - pathophysiology
decreased proximal HCO3 reabsorption (lower threshold for reabsorption)
367
proximal, type 2, RTA
| - urine pH once steady state is reached
5 (appropriately LOW)
368
proximal, type 2, RTA
| - urine AG (INDIRECT way to measure urine NH4+)
NEGATIVE; corresponds to PRESENCE of urine NH4+
369
proximal, type 2, RTA
| - a/w
Fanconi syndrome
370
proximal, type 2, RTA
| - effects in adults
benign
371
proximal, type 2, RTA
| - effects in children
stunts growth
372
proximal, type 2, RTA
| - treatment
- 5-10 meq/kg/day of base
| - potassium-sparing diuretic (amiloride, or spironolactone)
373
RTA type 4 (hypoaldosteronism)
| - K+ level
HIGH
374
RTA type 4 (hypoaldosteronism)
| - bicarb level
usually > 17
375
RTA type 4 (hypoaldosteronism)
| - pathophysiology
low aldosterone release and/or response
376
RTA type 4 (hypoaldosteronism)
| - urine AG
variable
377
RTA type 4 (hypoaldosteronism)
| - causes
- DM
- NSAIDs
- cyclosporine
- old age
378
RTA type 4 (hypoaldosteronism)
| - treatment
- loop diuretic
- bicarb
- mineralocorticoid
AND
- K+ binding resin
379
causes of osmotic nephropathy
- sucrose containing IVIG
- hydroxyethyl starch (HES)
- dextran
380
osmotic nephropathy LM on kidney biopsy
swollen tubular cells packed w/ numerous cytoplasmic vacuoles
381
osmotic nephropathy EM on kidney biopsy
vacuoles with phagolysosomes containing the offending agent (sucrose, HES, etc)
382
medications that decrease calcineurin inhibitor level
- rifampin
- barbiturates
- phenytoin
- carbamazepine
- nafcillin
- trimethoprim
- imipenem
- cephalosporins
- St. John wort
- INH
383
medications that increase calcineurin inhibitor level
- verapamil
- diltiazem
- ketoconazole
- fluconazole
- erythromycin
- grapefruit
- amiodarone
- carvedilol
- dapsone
384
medications and condition that increase calcineurin inhibitor toxicity
- amphotericin
- aminoglycosides
- NSAIDs
- lovastatin
- rhabdomyolysis**
385
Liddle syndrome pathophysiology
- GOF mutation
| - ENaC overactivity
386
apparent mineralocorticoid excess pathophysiology
11β-HSD2 deficiency
387
glucocorticoid remediable aldosteronism pathophysiology
- chimeric gene fusion (CYP11B1 and CYP11B2)
- zona fasciculata defect
- ACTH receptor overstimulated by cortisol
388
renal artery stenosis renin/aldosterone pathophysiology
renal parenchymal ischemia
389
- 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
390
pregnancy-associated TMA
- > 24 weeks
- HTN, edema
- effect of delivering fetus
- preeclampsia
| - improved
391
pregnancy-associated TMA
- > 27 weeks
- liver dysfunction and coagulopathy
- effect of delivering fetus
- acute fatty liver (AFL)
| - improved
392
pregnancy-associated TMA
- > 34 weeks
- elevated LFTs (not bili), and severe MAHA
- effect of delivering fetus
- HELLP
| - improved
393
pregnancy-associated TMA
- postpartum
- renal failure
- effect of delivering fetus
- HUS
| - none
394
pregnancy-associated TMA
- < 24 weeks
- neurological symptoms
- effect of delivering fetus
- TTP
| - none
395
calculator for estimating likelihood of severe ADAMTS13 deficiency in adults w/ suspected TTP
PLASMIC score
396
treatment for suspected TTP
plasmapheresis
397
treatment for drug-induced TMA
- quinine-containing beverage
- IVDU
- chemotherapy
- CNI
- d/c medication
| - may be able to avoid plasmapheresis
398
treatment for postpartum complement-mediated TMA
prompt anti-complement treatment (eculizumab)
399
alternative treatment for TMA if plasmapheresis not available
plasma infusion until transfer to another hospital
400
alternative treatment for TMA if anaphylactic reaction to plasma
factor VIII concentrate (contains ADAMTS13) and apheresis (plasma removal)
401
alternative treatment for TMA if can't give any blood products for religious reasons
intensive IS and apheresis
402
how is adequate response to plasmapheresis assessed?
normalization of platelet count
403
eculizumab is removed by
plasmapheresis
404
what ppx must be given prior to eculizumab administration?
- abx x 2 weeks before vaccine is effective
| - meningococcal vaccine
405
cause for primary FSGS
- specific cause unknown
| - ? circulating permeability factors
406
cause for familial/genetic FSGS
mutation in podocyte genes (eg podocin, nephrin)
407
causes for virus-associated FSGS
- HIV-1
- parvovirus B19
- SM 40
- CMV
- EBV
408
causes for drug-induced FSGS
- heroin
- interferon alfa, beta, and gamma
- lithium
- pamidronate
- sirolimus
- CNIs
- anabolic steroids
409
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
410
causes for adaptive FSGS
| - conditions w/ initially normal renal mass
- HTN
- AED
- TMA
- RAS
- cyanotic congenital heart disease
- sickle cell anemia
411
the adaptive form of FSGS is mediated by adaptive structural-functional responses to glomerular hypertension caused by
elevated glomerular capillary pressures and flows
412
initial urine pH in proximal, type 2, RTA
alkaline, 8
413
urine NH4+ level in proximal, type 2, RTA once steady state is reached
HIGH
414
some studies have indicated that magnesium wasting in Gitelman syndrome may be d/t down-regulation of what?
TRPM6 in DCT
415
expected acid-base disturbance in aspirin OD
respiratory alkalosis w/ compensatory metabolic acidosis
416
best initial test for a patient suspected of having pheochromocytoma
24-hour URINARY fractionated metanephrines
- 98% sensitivity
- 98% specificity
417
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
418
what treatment should a patient with pheochromocytoma receive prior to surgery?
- alpha and beta blockers
| - HIGH SALT diet (to mitigate volume contraction and orthostasis)
419
generally, if problem on PD is inadequate solute clearance, what is the next best step?
increase volume in each exchange
420
ACCOMPLISH trial showed combination of which antihypertensives reduced cardiovascular morbidity and mortality, and slowed CKD progression?
ACEI and CCB
421
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
422
renal and GU abnormalities and associated cause
| - Turner's syndrome
horseshoe kidney
423
renal and GU abnormalities and associated cause
| - epispadias
bladder extrophy
424
renal and GU abnormalities and associated cause
- undescended testes
- inguinal hernias
- CHARGE syndrome
hypospadias
425
renal and GU abnormalities and associated cause
- WAGR
- aniridia
- GU malformation
- intellectual disability
Wilm's tumor
426
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)
427
inulin clearance (GFR) formula
- inulin is neither secreted NOR reabsorbed
U(inulin)/P(inulin) x V (urine flow rate)
428
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)
429
respiratory alkalosis
| - for every decrease in PCO2 below 40, bicarb decreases by
2-3
430
respiratory acidosis
| - for every increase in PCO2 above 40, bicarb increases by
2-3
