Disorders Flashcards
1
Q
Bartter’s syndrome
A
AR: Na wasting
thick ascending loop of Henlee
defect in NaK2Cl transporter
Sx: hypokalemia, elevated renin and aldosterone, hypochloremic metabolic acidosis, increase PGE2, failure to thrive
some: hypomagnesemia, hypercalinuria
neonatal: polyhydramnios (fetal polyuria)
SALT CRAVING, DEAF
mutations: Na/K/2Cl, ROMK, CLCNKA, CLCKB, barttin
Tx: K and Mg if needed, COX inhibitors
2
Q
Gittleman’s syndrome
A
AR: Na wasting
DCT
mutation in Na/Cl cotransporter: presents later in life
Sx: hypokalemia, hypochloremic metabolic alkalosis, hypocalciuria
3
Q
glucosuria
A
cause: pregnancy, DM, familial renal glucosuria
Sx: thirst, nocturia due to osmotic diuresis
4
Q
familial renal glucosuria
A
mutation in SGLT1
5
Q
renal failure
A
loss of ability to balance salt and water
edema causes increase work load of heart leading to HF and pulmonary edema + acidemia and hyperkalemia
6
Q
diabetic nephropathy
A
MOST COMMON: ESRD
AA, native american, mexican
nephrotic: glomerular capillary wall deposition
pore size increased and charge difference reduced: proteinuria
chronic
Sx: HTN, increased then decreased GFR, microalbuminemia to macro
HE: increase measangial matrix, glomerular collapse, glomerulosclerosis; normal or increased glomeruli initially
risk: increases sig. when 1st relative of Type 1 DM has diabetic nephropathy
type 1: 10 years before develops
type 2: can’t tell how long
Tx: reduce BP and proteinuria; ACEI/ARB
3 types: glomerular, papillary, tubulointerstitial
7
Q
nephritic syndrome
A
endothelial cell
inflammation
UA: 1-2+ proteinuria, hematuria, RBC casts, dysmorphic RBC
spot urine protein creatinine ratio: 1
Sx: periorbital swelling, HTN, elevated BUN and Cr, oliguria
sub endothelial or mesangial: post-infectious glomerulonephritis, lupus nephritis, IgA nephropathy
BM: anti-GBM disease
necrotizing injury and inflammation: ANCA
8
Q
nephrotic syndrome
A
visceral epithelial cell (podocyte)
noninflammatory
EM: fusion of pedicels (effacement)
UA: 3+ to 4+, greater than 3.5 g/day, greater than 40 mg/hr/m2 in children, proteinuria, fatty cast
spot urine protein creatinine ratio: 10
Sx: pitting edema, hypercholesterolemia
podocyte injury: MCD, FSGS
sub epithelial complexes: membranous nephropathy
capillary wall: amyloidosis, light chain deposition disease, diabetic nephropathy
Tx: ACEI/ARB, tx hyperlipidemia, corticosteroids, Calcineurin inhibitors
9
Q
proteinuria
A
presents as foamy urine
due to barrier failure: large pore or loss of charge selectivity or abnormal circulating protein
10
Q
Diabetes Mellitus
A
impairment of insulin secretion and peripheral resistance to insulin
Sx: hyperglycemia (thirst, polyuria, weight loss, vision)
microvascular: CAD, cerebrovascular disease, PVD
Macro: nephropathy, neuropathy, retinopathy
increased thickness of BM and damaged capillaries: reduced Kf
macroalbuminuria: more likely to die than develop ESRD
renal complications: pyelonephritis, emphasematous pyelonephritis, type 4 RTA, neurogenic bladder
11
Q
lower urinary tract obstruction
A
stones, BPH
increases PBS and decreases GFR
12
Q
frequent emptying of bladder
A
decrease PBS and increases GFR
13
Q
low capillary flow
A
increases FF and therefore πG increases and GFR decreases
14
Q
Addison’s
A
absence of aldosterone
increased urinary excretion of NaCl
15
Q
Conn’s syndrome
A
aldosterone secreting tumor
increased Na reabsorption and K secretion: hypokalemia, hypernatremia, hypertension
16
Q
Liddle syndrome
A
AD pseudo hyperaldosteonism gain of function of beta or gamma ENac Sx: HTN, low renin and aldosterone, hypokalemia, metabolic alkalosis Tx: salt restriction
17
Q
metabolic alkalosis
A
increased pH, PCO2, HCO3
causes: loop/thiazide diuretics, vomiting, antacids, hyperaldosteronism
18
Q
non-anion gap metabolic acidosis
A
decrease pH, PCO2, HCO3 causes: HARDASS Hyperalimentation Addison Disease Renal tubular acidosis Diarrhea Acetazolamide Spironolactone Saline infusion mild to mod. renal insufficiency
19
Q
increased anion gap metabolic acidosis
A
decrease pH, PCO2, HCO3 causes: MUDPILES Methanol Uremia Diabetic ketoacidosis Propylene glycol Iron tablets or Isoniazid Lactic Acidosis (shock) Ethylene glycol Salicylates (Aspirin) renal failure: decreased NH4 excretion
20
Q
respiratory acidosis
A
increase pCO2, HCO3
decrease pH
causes: hypoventilation; hypercapnia: airway obstruction, acute lung disease, chronic lung disease, opioids, sedatives, weak respiratory muscles
Cl: changes inversely and equally to bicarb
21
Q
respiratory alkalosis
A
increase pH
decrease pCO2, HCO3
causes: hyperventilation: hysteria, hypoxemia, salicylates (early), tumor, pulmonary embolism
Cl: changes inversely and equally to bicarb
*preganancy: chronic
22
Q
type 2 renal tubular acidosis
A
proximal: loss of bicarbonate Faconi FEHCO3: greater than 10% urine pH: less than 5.5 Sx: hypokalemia
23
Q
type 1 renal tubular acidosis
A
distal: decreased acid secretion
Sx: hypokalemia, hypercalciuria, stones, failure to thrive
FEHCO3: less than 5%
urine pH: greater than 5.5
24
Q
type 4 renal tubular acidosis
A
aldosterone deficiency decreased acid excretion FEHCO3: less than 5-10% urine pH: greater or less than 5.5 Sx: HYPERkalemia
25
acute respiratory disorders
acidosis: increase pCO2 by 10 results in increase of bicarb by 1
alkalosis: decrease pCO2 by 10 results in decrease of bicarb by 2
* greater change in pH
26
chronic respiratory disorder
acidosis: increase pCO2 by 10 results in increase of bicarb by 4
alkalosis: decrease pCO2 by 10 results in decrease of bicarb by 5
27
Cl responsive metabolic alkalosis
urine Cl less than 20 mEq/l (usually less than 10 mEq/l)
28
Cl resistant metabolic alkalosis
urine Cl greater than 20 mEq/l (usually greater than 50 mEq/l)
29
hyperkalemia
Sx: weakness, ileum, EKG: peaked T wave; then wide QRS, short QT, long PR; absent P wave; V tach
causes:
1. increase intake
2. decrease renal excretion: renal failure, decreased tubular flow, distal tubular dysfunction, hypoaldosteronism
3. internal redistribution: insulin deficiency, B2 blocker, hypertonicity, acidosis, cell lysis
Tx:
1. stabilize heart: calcium gluconate
2. move K into cell: insulin, albuterol, bicarb
3. move K out of body: diuretics, kayexalate, dialysis
30
hypokalemia
Sx: weakness, rhabdomyolysis, ileus, HTN, nephrogenic diabetes insipidus, EKG: flat T wave; prominent U wave, depressed ST
chronic: asymptomatic
causes:
1. inadequate K intake
2. external loss: GI, cutaneous, renal
3. internal redistribution: insulin, catecholamine, alkalemia, cell proliferation
Tx: K, K sparing diuretics
31
hypokalemia normotensive disorders with metabolic alkalosis
```
loop and thiazide diuretics
vomiting
nasogastric suction
Bartter's syndrome
Gitelman's syndrome
```
32
hypokalemia normotensive disorders with metabolic acidosis
renal tubular acidosis (1 and 2)
| ureteral diversion
33
hypokalemia hypertensive disorders
hyper-reninemia: renal artery stenosis, renin secreting tumor
primary hyperaldosteronism: Conn's syndrome, adrenal hyperplasia or tumor, Cushing's syndrome (glucocorticoids can bind aldosterone receptor)
34
hyponatremia
Sx: lethargy, hyporefelxia, mental confusion
1. decrease ECFV: diarrhea, vomiting
2. NO CHANGE ECFV: HF, liver failure
renal Na loss: Una greater than 20 mEq/L
extrarenal Na loss: Una less than 20 mEq/L
Tx: isotonic saline infusion (slow)
35
nocturia
frequent urination during the night
| cause: disability to concentrate urine
36
central diabetes insipidus
```
Sx: dilute urine
cause: lack ADH
resting: high Posm, low Uosm
WD: increase Posm
ADH infusion: normal Posm, increase Uosm
```
37
nephrogenic diabetes insipidus
```
Sx: dilute urine
cause: CD do not respond to AVP
congenital: x-linked V2 or AQP2 mutation
acquired: defect in medullary interstitial tonicity or cAMP, AQP down regulation, pregnancy
resting: high Posm, low Uosm, high ADH
WD: increase Posm, increase ADH
ADH infusion: no change
```
38
polydipsia
```
obsessive water drinking
Sx: hyponatremia, coma, death
cause: loss of medullary hyperosmolarity
resting: low Posm, Uosm, ADH
WD: normal Posm, Uosm; increase ADH
ADH infusion: decrease Posm; increase Uosm
```
39
osmotic diuresis
cause: hyperosmotic plasma
resting: high Posm, Uosm
WD: increase Posm, Uosm, ADH
ADH infusion: Posm and Uosm remain high
40
Goodpasture's
nephritic with crescentic GN
anti-GBM: antibodies against NC1 domain of alpha3 (IV) chain
young adult, white, men (thicker BM)
IF: linear, IgG, C3
Sx: glomerulonephritis with hematuria and hemoptysis, azotemia, arthritis, anemia
Tx: plasmapheresis
41
effacement of pedicels
nephrotic syndrome
retraction of foot processes and loss of slit pore diaphragm so that long segments of capillary are invested by cytoplasm of a single podocyte
detachment from BM and BM degradation allow proteins to leak into urinary space
42
lupus nephritis
```
subendothelial: immune complex
HE: wire loops
rapidly progressive
IF: FULL HOUSE: IgG, IgM, IgA, C3, C4
Tx: glucocorticoids, cyclophosphamide, mycophenolate mofetil, hydroxychloroquine, aspirin
```
43
post-streptococcal glomerulonephritis
```
nephrotic
subendothelial to subepithelial:
anti-streptolysin O, anti-DNAse B
EM: subepithelial HUMPS
HE: endocapillary proliferation, neutrophils
IF: IgG, C3 (capillary and mesangium)
Sx: tea colored urine (RBCs), recent throat or skin infection (1-6 week), HTN, azotemia
LOW C3, normal C4: alternate pathway
```
44
metabolic (diabetic) glomerular injury
1. hyperglycemia causes non-enzymatic glycosylation of proteins resulting in thickened GBM and AGE (advanced glycation end-products)
2. AOPP (advanced oxidation products), renin-angiotensin, TGF-beta, AGE activate NADPH oxidases producing ROS leading to mesangial matrix production, podocyte injure and apoptosis and proteinuria
H&E: hyaline sclerosis; diffuse mesangial sclerosis
45
hemodynamic glomerular injury
supra-normal glomerular capillary pressures cause GBM thickening, mesangial cell hypertrophy and hyperplasia, and mesangial matrix production
causes hyaline sclerosis of afferent but not efferent arteriole: ischemic atrophy of glomerulus
46
arterionephrosclerosis
more common in A.A. due to apoL1
| HTN leads to globally sclerotic glomeruli
47
malignant HTN
```
black males ~40
HTN that causes end organ damage
greater than 200/120 mmHg
Sx: headache, vomiting, proteinuria, hematuria, scotoma (spots before eyes), renal failure
H&E: fibrinoid necrosis of arterioles leading to necrosis of glomeruli
onion skin
gross: flea-bitten kidney
P: can be fatal due to renal disease
MEDICAL EMERGENCY
```
48
diffuse glomerular disease
involving all or most of the glomeruli
49
focal glomerular disease
involving some glomeruli but not most
50
global glomerular disease
involving whole glomerulus
51
segmental glomerular disease
involving only part of glomerulus
52
proliferative glomerular disease
increased cell
| proliferating native cells but also infiltrating inflammatory cells
53
membranous glomerular disease
increase GBM without increased cells
54
membrano-proliferative glomerular disease
increased cells and GBM
| EM: TRAM TRACKS
55
membrano-proliferative glomerular disease
increased cells and GBM
56
pauci-immune glomerulonephritis
crescent: lymphocytes and monocytes in Bowman's space
antibodies are present but not visible on immunofluorescence or EM
ANCA
Sx: arthritis, arthralgia, myalgia, fatigue
57
microscopic polyangiitis
```
small vessel
nephritic: necrotizing injury of vascular and glomerular capillary wall
pauci-immune
P-ANCA
rapidly progressive
```
58
Churg-Strauss
```
small vessel
nephritic: necrotizing injury of vascular and glomerular capillary wall
pauci-immune
P-ANCA
rapidly progressive
```
59
Granulomatous with polyangiitis (Wegner's)
small vessel
nephritic: necrotizing injury of vascular and glomerular capillary wall
pauci-immune
C-ANCA
Sx: sinopulmonary renal syndrome, purpura
rapidly progressive
HE: normal, mesangial proliferative, segmental necrotizing, crescents (fibrin): tubulointerstitial GRANULOMAS
Tx: cyclophosphamide
60
C-ANCA
cytoplasmic
| Anti-proteinase3
61
P-ANCA
perinuclear
| Anti-MPO
62
IgA nephropathy
nephritic: mesangial
CURRENT infection
Sx: brown/red urine (macroscopic hematuria)
63
IgA nephropathy
| Berger's Disease
```
nephritic (variable): mesangial
CURRENT infection (1-2 days)
galactose deficient IgA1
HE: variable; mesangial expansion
IF: IgA
EM: mesangial deposits
Sx: brown/red urine (macroscopic hematuria)
```
64
membranous nephropathy
```
white male adults 4th-6th decade
nephrotic: subepithelial
Ab: PLA2, NEP
EM: SPIKE and DOME
immuno: IgG
HE: thick BM
2: Hep. B, syphilis, malaria, gold, penicillamine, captorpril, NSAID; lung, colon, skin cancer; SLE, sickle cell
risk for loss of renal function: male, greater than 10g/day proteinuria, HTN, azotemia, tubuointerstitial fibrosis, glomerulosclerosis
```
65
causes of iso-osmolar hyponatremia
pseudohyponatremia: severe hyperlipidemia and hyperproteinemia
post-transurethral prostatectomy, posthysteroscopic
66
causes of hypo-osmolar hypervolemic hyponatremia with decreased ECV
TBW and TBNa increase, TBW more than TBNa
| CHF, liver disease, nephrotic syndrome, renal disease
67
causes of hypo-osmolar hypervolemic hyponatremia with increased ECV
acute and chronic renal failure
68
causes of hypo-osmolar euvolemic hyponatremia
increase TBW; no change TBNa
causes: SIADH, drugs, glucocorticoid deficiency, hypothyroidism, primary polydipsia, lung disorders, CNS disorders and malignancy, drug induced water retention
69
causes of hypo-osmolar hypovolemic hyponatremia
decrease ECFand ECV
causes: diuretics, aldosterone deficit, diarrhea, vomiting, bleeding, excessive sweating with high water intake, pancreatitis, bowel obstruction, burns
severe hypokalemia
70
hypernatremia
Uosm less than 300 mOsm/kg: diabetes insipidus
Uosm greater than 500 mOsm/kg: extra-renal water loss, Na infusion, decrease osmotic diuresis
other: primary hyperaldosteronism, Cushing's, hypertonic hemodialysis, admin. of Na
71
tubular injury UA
microscopic hematuria, renal tubular epithelial cells, granular casts, specific gravity 1.010, Uosm 300
72
acute tubular necrosis (ATN)
non-inflammatory tubular injury
cause: ischemia or toxins (aminoglycosides (PT), contrast, amphotercin B)
proximal straight tubule and TALH
occlusion of tubular lumens with cells and casts
muddy brown granular casts
urine Na greater than 20
FeNa greater than 1
73
allergic interstitial nephritis (AIN)
inflammatory tubular injury
proliferation of interstitial fibroblast and matrix: TGF-B
UA: pyuria, eosinophiluria
cause: penicillin, cephalosporin, NSAIDS; infections, autoimmune, infection
Sx: fever, rash, joint pain, eosinophils, sterile pyuria
74
obstructive uropathy
tubular injury and possible RBC
75
mesangial pattern tubular disease
UA: hematuria, RBC casts without proteinuria
76
hereditary renal glucosuria
AR: PT
| mutation of SGLT2
77
Cystinuria
AR: PT: amino aciduria
mutation of brush border transporter responsible for reabsorption of cystine, ornithine, lysine, arginine
cystine stones
UA: cystine casts
78
X-linked hypophosphatemia
mutation of PHEX gene: increased FGF23
Sx: urinary Pi wasting, elevated serum alkaline phosphatase, normal Ca and calcitriol
presents: rickets in children and osteomalacia in adults
79
autosomal recessive hypophosphatmic Rickets
mutation that leads to increased FGF-23
| mutation in Na/Pi IIc transporter
80
oncogenic hypophosphatemic osteomalacia
aquired
| increased production of FGF-23 by tumors (fibromas, angiosarcomas, hemangiopericytomas)
81
Hartnup disease
defect in neutral amino acid transporter SLC6A19
| Sx: failure to thrive, photosensitivity, intermittent ataxia, nystagmus, tremor
82
vitamin D dependent rickets type 1
mutation of 1 alpha hydroxylase
| Sx: hypophosphatemia, rickets
83
Faconi syndrome
generalized PT dysfunction
Sx: aminoaciduria, glucosuria, hypophophatemia, hyperchloremic metabolic acidosis, hypokalemia, uricosuria
polyuria, polydipsia, volume depletion, arrhythmia, proteinuria, growth retardation, rickets, renal stones
inherited: cysinosis, lots of others
acquired: tenofovir, multiple myeloma, lots of of others
84
glucocorticoid-remediable hyperaldosteronism (GRA)
congenital
recombination of 11-B-hydroxysteroid dehydrogenase and aldosterone synthase: promoter that makes cortisol is stuck on gene that produces aldosterone
Sx: low renin HTN, hypokalemia
Tx: glucocorticoids
85
apparent mineralcorticoid excess (AME)
mutations in kidney isozyme of 11BHSD: increase cortisol in kidney that activates aldosterone receptor
Sx: low renin HTN, hypokalemia
Tx: glucocorticoids
86
PHA type 1
AD: mutation in mineralocorticoid receptor, childhood
AR: loss of function mutation in ENaC
Sx: hyperkalemia, hypernatremia, HYPOTENSION
Tx: Na, fluids, Kayexalate
mirror image of Liddle
87
PHA type II
Gordon syndrome
chloride shunt syndrome
mutation: WNK1, WNK4, ser/thr kinase that regulates Na/Cl cotransporter
Sx: hyperkalemia, hyperchloremic metabolic acidosis, HTN, low renin and aldosterone
mirror image of Gitelman
Tx: thiazides
88
renal tubular acidosis (RTA)
normal anion gap metabolic acidosis
| positive urine anion gap
90
Focal segmental glomerulosclerosis (FSGS)
```
A.A.
nephrotic: subepithelial immune complex
non-selective proteinuria
EM: podocyte effacement
H&E: focal, segmental
immuno: neg
suPAR, apoL1
Sx: may have HTN, half develop ESRD
2: HIV, parvo, heroin, lithium, pamidronate, partial nephrectomy
mutations: alpha-actinin-4, podocin, TRPC6, apoL1
```
90
minimal change disease (MCD)
```
children
nephrotic: podocyte injury
proteinuria: ALBUMIN
EM: foot process EFFACEMENT
H&E: normal
immuno: neg.
2: Hodkin's, NSAID, IFN-alpha
Tx: GLUCOCORTICOIDS
```
91
rapidly progressive glomerulonephritis
nephritic
renal failure over days/weeks
features of vasculitis
ex: ANCA vasculitis, lupus nephritis
92
chronic glomerulonephritis
HTN, renal insufficiency, proteinuria greater than 3 g/day
SHRUNKEN kidneys
ex: diabetic and HTN nephropathy
93
Alport syndrome (Hereditary nephritis)
GBM
X-linked COL4A5: male
Sx: hematuria, progressive proteinuria, ESRD
assoc.: hearing loss, lens abnormalities, platelet defects, esophageal leiomyomas
HE: focal, segmental or global glomerulosclerosis, interstitial fibrosis, foam cells
EM: BASKETWEAVE, focal effacement of podocytes
IF: neg.
94
hemolytic uremic syndrome (HUS)
hemolytic anemia, renal dysfunction , thrombocytopenia
ONION SKIN
HE: fibrin and platelets in lumen, fibrin in subintima and media of vessels
E. coli H7:O157 verotoxin
95
thrombocytpenia purpura (TTP)
FEVER, NEUROLOGIC, PURPURA
hemolytic anemia, renal dysfunction , thrombocytopenia,
ONION SKIN
HE: fibrin and platelets in lumen, fibrin in subintima and media of vessels
ADAMTS13 or autoimmune disorders
96
membranoproliferative glomeruluonephritis
asymptomatic, acute nephritic, nephrotic, crescentic rapidly progressive glomerulonephritis
subendothelial
EM: TRAM TRACKS
HE: hypercellular glomerulus, increased mesangium
immuno: C3, C4, IgG; monoclonal gammopathy (kappa or lambda)
Sx: HTN, decreased GFR
rheumatoid factor, ANA, LOW C3
immune complex mediated: classical pathway
complement mediated: alternative pathway
97
type 1 MPGN
most common
HE: mesangial hypercellularity, monocytes
sub endothelial and mesangial
IF: IgG
2: neoplasm, infection, collagen vascular disease
Hep. C; LOW C3 and C4
98
type 2 MPGN
```
dense deposit disease in capillary wall
deficiency of FACTOR H
C3c present
partial liphodystrophy: loss of fat upper body; macular deposits in eyes
LOW C3
```
99
type 3 MPGN
subedothelial and sub epithelial with GBM disruption and lamina dens layering
100
thin basement membrane
```
female
heterozygote COL4A5: defects in alpha-3 or 4 type IV
Sx: hematuria
HE: normal
IF: negative
EM: thin GBM
```
101
polyarteritis nodosa
medium vessel: infarcts
ANCA NEG.
assoc.: Hep B (and Hep C and Hairy cell leukemia)
Sx: arthralgia, fatigue weight loss, fever, abdominal pain, near, renal, HTN, skin lesions
HE: SEGMENTAL TRANSMURAL NECROTIZING vasculitis
102
What indicates ANCAs are pathogenic?
activate PMN
increase contact and adhesion with endothelial cells and vasculature structures
adhesion: B-2 integrin, Mac-1, Fc gamma
*endothelial cells are the primary target in small cell vasculitis
103
thrombotic microangiopathy (TMA)
normal PTT and PT (prolonged in DIC)
HUS, TTP
drugs, SLE, antiphospholipid, complement defects, HIV, cancer
104
class I lupus nephritis
minimal measngial
| RARE
105
class II lupus nephritis
mesangial proliferative
106
class III lupus nephritis
focal proliferative
107
class IV lupus nephritis
diffuse proliferative
| MOST COMMON
108
class V lupus nephritis
membranous
| nephrotic
109
class VI lupus nephritis
advanced sclerosing
110
scleroderma
females AA
fibrosis and vascular occlusion
mild renal involvement: dysfunction, proteinuria, HTN
renal crisis: accelerated arterial HTN and/or rapidly progressive oliguria renal failure
Risk factors for renal crisis: early diffuse systemic sclerosis, rapidly progressing, anti RNA polymerase Ab, steroids
HE: intimal and medial proliferation arcuate arteries, fibrinoid necrosis and thrombosis
111
acute kidney injury (AKI or ARF)
less than 1 month
Cr: greater than 0.5 mg/dl
usually asymptomatic
112
pre-renal ARF
```
decrease renal blood flow
BUN: Cr ratio: greater than 20
urine mOsm: greater than 500
urine Na: less than 25
FeNa less than 1
causes: volume depletion, CHF, shock, hepatorenal syndrome, renal artery stenosis and ACEI/ARB, NSAIDS
```
113
intrinsic renal ARF
glomerular: nephrotic and nephritic
tubular: ATN AIN
vasculitis
BUN: Cr ratio: less than 15
urine mOsm: less than 250
urine Na: greater than 20
muddy brown casts
114
post-renal ARF
obstruction of flow: voiding complaints
Dx: ultrasound
causes: prostate, cancer, neurogenic bladder,
115
hepatorenal syndrome
pre-renal AKI
cirrhosis: portal HTN leading to vasodilation and therefore decreased ECV and activation of renin
decrease BP with increased ECFV
UA and kidneys: normal; urine Na less than 10
Tx: liver transplant
116
chronic kidney disease
Sx: voiding complaints, HTN, edema, flank mass
SERIAL labs needed
SMALL kidneys, WAXY cast, PERIPHERAL NEUROPATHY
usually ASYMPTOMATIC, BONE change with hyperparathyroidism
high risk of CVD
Effects:
nervous system: asterixis, intellectual function, encephalopathy
hypoglycemia (decreased insulin degradation and decreased gluconeogenesis)
anemia
anorexia
drug metabolism effects
decreased Vit D: hypocalcemia, increase PTH
hyperphosphatemia
HYPERKALEMIA
metabolic ACIDOSIS NON ANION GAP
117
K over 7mmol/L
MEDICAL EMERGENCY
| Tx: IV calcium gluconate, IV insulin + glucose
118
autosomal dominant polycystic kidney disease
ADULT
slow growing cysts compress normal tissue and cause ischemic atrophy
HUGE kidneys
polycystin-1 or 2
ciliopathy, defective mechanosensigin of urine flow, dysregulation of cell adhesion
two hit genetics
119
autosomal recessive polycystic kidney disease
CHILDREN
cysts replace normal tissue in fetus
mutation: fibrocystin
pulmonary hypoplasia and death
120
nephronophthisis
medullary cystic disease
SMALL kidneys with numerous small cysts at corticomedullary junction and chronic tubulointerstitial nephritis and fibrosis
mutation: cilia components
most common genetic cause of ESRD in CHILDREN
121
kidney stones
```
males
dysuria, hematuria, restless, writhing in distress, N/V, flank pain
CT: sensitive
UA, ultrasound, xray
Tx: fluids
```
122
calcium stones
most: Ca oxalate; Ca Pi next
calcium oxalate crystals
cause: hypercalcemia, reduced citrate, excess Na
Tx: do NOT restrict dietary Ca, thiazide diuretics, Na restriction,, K citrate
123
Magnesium ammonium phosphate (Struvite) stone
or
triple phosphate
UTI with urease producing organism (Proteus, Klebsiella)
urine pH greater than 7 (ammonium produced by bacteria)
coffin lid crystal
Tx: infection
124
uric acid stones
```
cause: hyperuricemia
urine pH less than 5.5
diamond cyrstals
RADIOLUCENT
Tx: restrict protein, K citrate, allopurinol
```
125
cystine stones
decreased PT reabsorption
Tx: penicillamine, K citrate, alkalinize urine
hexagon crystals
CHILDREN
126
urinary tract obstruction
mechanical: intraluminal, intramural, extrinsic
functional: neurogenic
common locations: ureter crosses iliac vessels, enters bladder, ureterovesical junction
proximal to bladder: one kidney, normal Cr
distal: both kidneys, increased Cr
127
retroperitoneal fibrosis
can cause urinary tract infection
128
chronic bilateral partial urinary tract obstruction
polyuria, azotemia, nonunion gap metabolic acidosis, hyperkalemia, RTA
129
clear cell carcinoma
MOST COMMON
malignant
chromosome 3p: VHL
gross: solitary, yellow, cysts, necrosis, hemorrhage
HE: clear or eosinophilic cytoplasm, vascular
sporadic: translocation or deletion chromosome 3
hereditary: inactivated mutated VHL, hyper-methylation of VHL
risk: dialysis related renal cystic disease
grade indicates survival rate
130
oncocytoma
```
benign
renal epithelial neoplasm
large cells with lots of mitochondria
MAHOGANY BROWN with central STELLATE scar
may be multiple
```
131
renal carcinoma
male, smoker, older, HTN, obesity
tubule epithelial cells
encapsulated, necrosis, hemorrhage (rupture invaded vein, abnormal new vessels)
metastasis: perinephric fat, VENOUS, LUNG, BONE; next: lymph, liver, adrenal, brain
Sx: dull flank pain, hematuria, abdominal mass
132
papillary carcinoma
malignant
renal parenchymal tumor
bilateral, multifocal
sporadic: trisomy 7, 16, 17, loss Y
activated MET, translocation 1: PRCC oncogenes
hereditary: trisomy 7, activated MET
risk: dialysis related renal cystic disease
133
chromophobe carcinoma
large pale cells with prominent cell membranes
lower mortality
Hale's colloidal iron stain
134
renal pelvis carcinoma
urothelial: transitional cell carcinoma; can get squamous
CENTRAL location
smoker: get in bladder and can get higher in collecting system
135
Wilm's tumor (nephroblastoma)
KIDS: 2-5 yrs
embryonal kidney tumor
HE: mixture of cell types: blastemal, stromal, epithelial
ABDOMINAL MASS
136
WAGR
no iris, genital abnormalities, mental retardation
Wilm's tumor
deletion of WT1 gene
137
Denys-Drash
gonadal dysgenesis
Wilm's tumor
inactivating mutation WT1 gene
138
Beckwith-Wiedemann
Wilm's tumor
one sided organomegaly
genetic imprinting doesn't silence maternal allele that controls IGF2
139
light chain disease
nephrotic: glomerular capillary wall deposition
MONOCLONAL
precipitation of Ig chains without elongation
MULTIPLE MYELOMA
Sx: proteinuria, renal failure, tubuolinterstitial syndrome, cardiomegaly, hepatomegaly, sicca syndrome, peripheral neuropathy, GI, pulmonary nodules, arthopathy
140
sickle cell nephropathy
val for glutamate: 6th aa beta global chain
polymerize with low O2 and sickle
early: glomerular hypertrophy, HEMOSIDERIN, focal hemorrhage or necrosis
late: interstitial inflammation, edema, fibrosis, tubular atrophy papillary infarcts
end: FSGS
vaso-occlusive
Sx: pain crises, hemolysis, reticulocytosis, hyperbilirubinemia, elevated lactate dehydrogenase, low haptoglobin
SICKLE cell, Howell- Jolly
141
amyloidosis
deposition of proteinaceous material in vessels then interstitium
BETA-PLEATED SHEETS
nonproliferative, noninflammatory glomeruopathy
vascular; tubular atrophy and interstitial fibrosis
APPLE GREEN BIREFRINGENCE
CONGO RED
142
HIV associated nephropathy
```
progressive azotemia, proteinuria
COLLAPSING FSGS with microcystitic tubular dilation, interstitial inflammation, fibrosis
Nef and Vpr viral genes
ApoL1
Tx: HAART, ACEI/ARB
most still develop ESRD
```
143
cryoglobulinemia
HEP. C
immune complexes: RF, IgG, HCV RNA, complement of endothelial surfaces
precipitate at 4 degrees
MEMBRANOPROLIFERATIVE
Sx: palpable purpura, arthralgia, MPGN, lymphoma, peripheral neuropathy
MELTZER's TRIAD
lymphocyte infection leads to IgM secreting clones
144
AL amyloid
```
primary
older
MOST COMMON
MONOCLONAL LIGHT chains
urine: BENCE-JONES proteins
assoc. MULTIPLE MYELOMA; PLASMA CELL DYSCRASIS
Sx: weak, weight loss, NEPHROTIC, peripheral neuropathy, large kidneys and insufficiency, GI, heart
Tx: melphalan, dexamethosone
```
145
AA amyloid
```
reactive systemic
secondary to CHRONIC INFLAMMATION: RA, IBS, ankylosing spondylitis
IL-1 and IL-6
Sx: kidney and GI
Tx: eprodisate
```
146
Abeta2m amyloid
long term hemodialysis
| Sx: synovium, joints and tendon sheaths
147
ATTR amyloid
senile or familial (AA)
transthyretin
HEART and pulmonary blood vessels
Sx: heart failure or arrhythmia
148
Abeta amyloid
Alzheimer's
149
stage 1 diabetic nephropathy
hyperfiltration, increase GFR
| increase kidney size
150
stage 2 diabetic nephropathy
glomeruli show damage and microalbuminuria
151
stage 3 diabetic nephropathy
albumin excretion rate exceeds 200 mg/min
increase serum BUN and Cr
increase BP in some
152
stage 4 diabetic nephropathy
```
GFR decreases less than 75 ml/min
proteinuria
high BP
further increase BUN and Cr
17 years to develop
```
153
stage 5 diabetic nephropathy
kidney failure or ESRD
GFR less than 10 ml/min
23 years to develop
154
diffuse glomerular diabetic nephropathy
early, less sever
MOST COMMON
identical to HTN and aging glomerulopathy
capillary BM thickening and increased mesangial matrix: begins in stalk of glomerular tuft
155
nodular glomerular diabetic nephropathy
after 10 years DM
superimposed on glomerulopathy
KIMMELSTIEL WILSON nodules and HYALINE sclerosis of efferent and afferent arterioles
156
papillary diabetic nephropathy
pyelonephritis
| papillary necrosis
157
tubulo-interstitial diabetic nephropathy
tubular basement membrane thickening
| interstitial fibrosis
158
pyelonephritis
infection of kidney: infected tubules
| UA: pyuria and bacteruria
159
acute urethral syndrome
does NOT have significant bacteriuria
cause: chlamydia, bacterial lower UTI
Sx: dysuria
160
UTI
females (opp. in infants)
intercourse, SPERMICIDE + diaphragm, OBSTRUCTION, trauma, VESICULO-URTERAL REFLUX, intrumentation
pregnancy: progression more likely, higher bacteriuria; need to check for pyelonephritis
ASCENDING infection, hematogenous much less likely
pathogenesis: adhesion (type 1 and P-fimbriae), colonization, invasion, phase variation
complication: sepsis, abscess, chronic renal insufficiency, struvite renal calculi, recurrent infections
161
What causes increase in UTI with age?
bacteriuria
male: BPH
female: decrease estrogen and vaginal acidity
162
lower UTI
freq. of urination, dysuria, turbid urine, suprapubic discomfort, hematuria, asymptomatic cystitis
Tx: female 1-3 days, males 1 week
163
upper UTI
fever, chills, CVAT, asymptomatic pyelonephritis
| Tx: 1-6 weeks
164
asymptomatic bacteriuria
DO NOT TREAT in elderly or others
