Kidney Path Flashcards

(84 cards)

1
Q

nephrotic syndrome

A
severe proteinuria
hypoalbuminemia
generalized edema
hyperlipidemia
lipiduria
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

nephritic syndrome

A
hematuria
azotemia
oliguria
hypertension
proteinuria
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

causes of nephrotic syndrome

A
Minimal Change Disease (MCD)
Membranous Glomerulonephritis
(MGN)
Focal Segmental
Glomerulosclerosis (FSGS)
Membranoproliferative
Glomerulonephritis (MPGN)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Minimal Change Disease (MCD)

A

n most frequent cause of the nephrotic
syndrome in children
n glomeruli have normal appearance under
the light microscope, but electron
microscopy reveals diffuse loss of visceral
epithelial foot processes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what is universal to nephrotic syndrome

A

fusion of podocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Minimal Change Disease (MCD) path

A

current evidence points to a disorder of T cells
-elaborate factors (IL-8, TNF, etc.?) that affect
nephrin synthesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Minimal Change Disease (MCD) gold standard for diagnosis

A

EM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Minimal Change Disease (MCD) immunofluorescence?

A

no

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Minimal Change Disease (MCD) clinical course

A

Insidious development of nephrotic syndrome in
otherwise healthy child (peak age, 2-6)
-Usually follows respiratory infection or prophylactic
immunization; assoc. w/atopic disorders
Selective proteinuria (mainly albumin)
Good prognosis
>90% respond to short course of corticosteroids
<5% develop chronic renal failure after 25 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Membranous Glomerulonephritis

MGN

A
most common cause of
nephrotic syndrome in
adults (peak age, 30-50)
n glomeruli appear normal
early in the disease, but
develop diffuse thickening
of the capillary walls
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Membranous Glomerulonephritis

(MGN) path

A

form of chronic immune complex nephritis
-idiopathic forms (85% of cases) are mainly autoimmune
*antibodies react to antigens in the glomerulus components, or to
antigens that have become trapped there
-may also be due to circulating complexes of known exogenous
or endogenous antigen
the membrane attack complex of complement (C5b-C9)
activates mesangial and epithelial cells, causing them to
liberate proteases and oxidants that damage glomerular
capillaries

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Membranous Glomerulonephritis

(MGN) key to dianosis

A

silver stain

detects collagen in BM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Membranous Glomerulonephritis

(MGN) clinical course

A

Insidious development of nephrotic syndrome
Proteinuria is nonselective and does not respond
to corticosteroid therapy
Variable course
40% suffer progressive disease ending in renal failure
after 2-10 years
10-30% follow benign course with partial or complete
remission

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Focal Segmental

Glomerulosclerosis (FSGS)

A

characterized by sclerosis affecting some
but not all glomeruli and involving only
segments of each glomerulus
most well-known association is with HIV
patients and IV drug users, but occurs in
all ages (more common in African-
American populations)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Focal Segmental

Glomerulosclerosis (FSGS) path

A

unknown (some have suggested it is an
aggressive variant of minimal change
disease)
circulating mediator is likely responsible
-proteinuria recurs soon after renal transplant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Focal Segmental

Glomerulosclerosis (FSGS) diagnosis

A

IF usually isn’t done, but detects IgM and

C3 in sclerotic segments

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Focal Segmental

Glomerulosclerosis (FSGS) clinical course

A

May be a primary disease or result from other
forms of GN (esp. IgA nephropathy)
Proteinuria is nonselective and has poor
response to corticosteroid therapy
Higher incidence of hematuria and hypertension
(more likely to evolve from nephritic syndromes)
Prognosis is poor, with children faring slightly
better than adults
-50% suffer renal failure after 10 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Membranoproliferative

Glomerulonephritis (MPGN)

A

may be nephritic, nephrotic, or mixed
characterized by alterations in the GBM
and mesangium plus proliferation of
glomerular cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Membranoproliferative

Glomerulonephritis (MPGN) Type I

A

(2/3rds of cases)
Circulating immune complexes of unknown antigen
-Associated with hepatitis B and C, SLE, infected
atrioventricular shunts, chronic lymphocytic leukemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Membranoproliferative

Glomerulonephritis (MPGN) Type II

A

Less clear, but serum of patients has a factor called C3
nephritic factor (C3NeF)
Activates the alternative complement pathway by stabilizing
C3 convertase
hypocomplementemia, but normal C1, C2, C4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Membranoproliferative

Glomerulonephritis (MPGN) key to diagnosis

A

“doubled” GBM on silver stain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q
Membranoproliferative
Glomerulonephritis (MPGN) EM varies by type
A
Type I: subendothelial
deposits
Type II: dense
deposits in center of
GBM and under
endothelium
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Causes of Nephritic Syndrome

A
Acute Proliferative (Poststreptococcal)
Glomerulonephritis (PGN)
Rapidly Progressive (Crescentic)
Glomerulonephritis (RPGN or CrGN)
IgA Nephropathy (Berger Disease)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q
Acute Proliferative (Poststreptococcal)
Glomerulonephritis (PGN)
A

Typically caused by immune complexes of
exogenous or endogenous antigens
-Prototype exogenous pattern = 1-4 weeks after a
streptococcal infection
*Only “nephritogenic” strains of group A β-hemolytic
streptococci associated
*Most cases follow skin or pharynx infection
Children affected more frequently than adults

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
``` Acute Proliferative (Poststreptococcal) Glomerulonephritis (PGN) path ```
``` Immune complex formation, but unclear if complexes are simply trapped as they pass through the glomerulus, or if C3 is deposited on GBM before IgG subepithelial humps ```
26
``` Acute Proliferative (Poststreptococcal) Glomerulonephritis (PGN) Clinical Course ```
Abrupt onset, with malaise, slight fever, nausea, and nephritic syndrome Hematuria: urine appears smoky brown Hypocomplementemia; elevated serum antistreptolysin O titers in poststreptococcal cases Children: complete recovery in most (95%) Adults: complete recovery (60%); many develop RPGN or chronic GN
27
``` Rapidly Progressive (Crescentic) Glomerulonephritis (RPGN or CrGN) ```
Clinical syndrome (not a specific etiologic form of GN) Characterized by rapid and progressive loss of renal function associated with severe oliguria and (if untreated) death from renal failure in weeks to months All types demonstrate crescents (proliferation of parietal cells/migration of monocytes into Bowman's space)
28
``` Rapidly Progressive (Crescentic) Glomerulonephritis (RPGN or CrGN) type I ```
anti-GBM disease Type IV collagen is target Linear deposits of IgG (sometimes C3 also) on GBM In some patients, anti-GBM antibodies also bind to pulmonary alveolar capillary basement membranes = GOODPASTURE SYNDROME -Goodpasture antigen is noncollagenous component of Type IV collagen -Males > females; peak age is 20-40 y.o. -Pulmonary involvement precedes renal disease *Pulmonary hemorrhage and recurrent hemoptysis -Plasmapheresis is beneficial (removes pathogenic antibodies) Least common of all CrGN types
29
``` Rapidly Progressive (Crescentic) Glomerulonephritis (RPGN or CrGN) Type II CrGN ```
Complication of any immune complex nephritides (PGN, SLE, IgA nephropathy) Granular IF pattern
30
``` Rapidly Progressive (Crescentic) Glomerulonephritis (RPGN or CrGN) Type III GrGN (pauci-immune type CrGN) ```
Component of a systemic vasculitis, like microscopic polyangiitis or Wegener granulomatosis, or idiopathic Most have serum ANCAs No anti-GBM antibodies or immune complexes
31
``` Rapidly Progressive (Crescentic) Glomerulonephritis (RPGN or CrGN) ```
Crescents obliterate the Bowman's space | and compress the glomeruli
32
``` Rapidly Progressive (Crescentic) Glomerulonephritis (RPGN or CrGN) clinical course ```
Typical nephritic syndrome, but oliguria and azotemia more pronounced Rapid progression to severe renal failure; longterm dialysis or transplantation required Prognosis roughly related to crescent number (<80% crescents means better prognosis) and type
33
IgA Nephropathy (Berger Disease)
Most common glomerular disease, and one of most common causes of recurrent hematuria Usually affects children and young adults (mostly males) Typical presentation = gross hematuria that occurs 1-2 days after a nonspecific respiratory tract infection; disappears, but recurs every few months
34
IgA Nephropathy (Berger Disease) path
Genetic or acquired abnormality of IgA production and clearance -IgA synthesis is increased in response to respiratory or GI exposure to antigen *IgA nephropathy occurs with increased frequency in patients with celiac sprue and liver disease -IgA and IgA complexes are entrapped in the mesangium, where they activate the alternative complement pathway and cause glomerular injury Proposed to be a variant of Henoch-Schönlein purpura
35
IgA Nephropathy (Berger Disease) clinical course
``` Typically presents with loin pain, gross hematuria; usually after respiratory, gastrointestinal, or urinary infection Some develop slow progression to chronic renal failure in 20 years ```
36
Hereditary Nephritis
Group of hereditary familial renal diseases Best studied is Alport syndrome -GBM defect due to mutation in type IV collagen components -Nephritis accompanied by nerve deafness and eye disorders, including lens dislocation, posterior cataracts, and corneal dystrophy -Males affected more frequently, more severely Patients present at age 5-20 y.o., and develop renal failure by 20-50 yoa Heterogenous inheritance (X-linked, autosomal recessive, or autosomal dominant)
37
Chronic Glomerulonephritis
``` The final stage of many forms of glomerular disease; characterized by progressive renal failure, uremia, and untimely death Usually from rapidly progressive GN, focal glomeulosclerosis, membranoproliferative GN ```
38
signs & symptoms of Chronic Glomerulonephritis
``` decreased renal acuity cramps restless leg sleep disturbances bone disease itching anemia ```
39
Chronic Glomerulonephritis morphology
Kidneys small with | granular surface
40
Chronic Glomerulonephritis clinical course
``` Usually undiscovered until late in its course, when symptoms of renal insufficiency develop (proteinuria, hypertension, azotemia, anasarca, anemia, anorexia) Urinalysis: broad waxy casts Dialysis and renal transplantation required ```
41
Chronic Diabetic Glomerulopathy risk factors
Poor glycemic control hypertension diabetic retinopathy (high correlation)
42
Diabetic Glomerulopathy path
``` Nonenzymatic glycosylation (NEG) -glomerular and tubular BMs -arterioles (efferent 1st) osmotic damage to glomerular capillary endothelial cells - glucose → osmotically active sorbitol hyperfiltration damage to mesangium diabetic microangiopathy ```
43
Diabetic Glomerulopathy clinical features
Microalbuminuria -first lab manifestation (usually after ~10 y of poor glycemic control) -microalbuminuria dipsticks detect albumin levels 1.5-8 mg/dL increased susceptibility to acute and chronic pyelonephritis infarction of renal papillae = papillary necrosis most common cause of chronic renal failure in U.S.
44
Renal amyloidosis
B-2 microglobulin
45
Acute Pyelonephritis
``` if obstruction prevents draining, the renal pelvis, calyces, and ureter may fill with exudate = pyonephrosis -pus under pressure may develop ischemic and suppurative necrosis of the renal pyramid tips = papillary necrosis -more common in diabetics papillary necrosis ```
46
Acute Pyelonephritis clinical course
Fever, chills, malaise Dysuria, frequency, and urgency Costovertebral angle (CVA) tenderness Urinalysis shows WBC casts and pyuria
47
Chronic Pyelonephritis
``` Due to recurrent infections promoted by chronic obstruction or reflux -Chronic Obstructive Pyelonephritis -Chronic Reflux-Associated Pyelonephritis Important cause of chronic renal failure ```
48
Chronic Pyelonephritis morphology
``` Uneven scarring of the pelvis and/or calyces -Leads to blunted calyces ```
49
Chronic Pyelonephritis clinical course
Unnoticed until renal insufficiency begins; may cause hypertension n Some develop FSGS
50
Acute Drug-Induced Interstitial | Nephritis (aka, Acute TIN)
Adverse reaction to drugs that begins ~15 days (range, 2-40 days) after exposure -Synthetic antibiotics, especially penicillins -Diuretics (thiazides) -Nonsteroidal anti-inflammatory agents (phenylbutazone)
51
Acute Drug-Induced Interstitial | Nephritis (aka, Acute TIN) path
Drugs acts as haptens that covalently bind to cytoplasmic or extracellular component of tubular cells and become immunogenic eosinophils in the urine help support the diagnosis
52
Acute Drug-Induced Interstitial | Nephritis (aka, Acute TIN) Clinical Course
Fever Eosinophilia (may be transient) Rash (only ~25% of patients) Renal abnormalities: hematuria, minimal or no proteinuria, leukocytouria A rising serum creatinine level or acute renal failure with oliguria develops in about 50% of cases (esp. elderly) Withdrawal of offending drug results in recovery (may take months)
53
Analgesic Nephropathy
``` Seen in patients consuming large quantities of analgesics -Most consume mixtures containing some combination of phenacetin, aspirin, acetaminophen, caffeine, and codeine for long periods papillary necrosis ```
54
Analgesic Nephropathy clinical course
``` Chronic renal failure (more common in those with preexisting renal disease), hypertension, anemia Increased incidence of transitional cell carcinoma ```
55
Acute Tubular Necrosis
The most common cause of acute renal failure Characterized by destruction of tubular epithelial cells Generally a reversible lesion that arises in a variety of clinical settings: Ischemic Type = due to decreased blood flow (e.g., severe hemorrhage, shock, dehydration) Nephrotoxic Type = death of tubular cells due to poisons, drugs
56
Acute Tubular Necrosis: Nephrotoxic Type
Aminoglycosides (#1 cause) and other drugs IV radiographic contrast agents (#2 cause) Heavy metals (e.g., mercury, gold, lead) Organic solvents (e.g., carbon tetrachloride) Ethylene glycol Mushroom poisoning Pesticides Myoglobin (from crushing injuries)
57
Acute Tubular Necrosis Clinical Course
Oliguria and elevation of blood urea nitrogen and creatinine Metabolic acidosis and hyperkalemia Urinalysis: dirty brown granular casts and epithelial casts Prognosis is excellent in nephrotoxic ATN if patient survives the disease responsible
58
normal BUN & creatinine
BUN: 7-18 mg/dL creatinine = 0.6-1.2 mg/dL Ratio should be ~15:1
59
Pre-renal azotemia
caused by a decrease in cardiac output to kidneys (e.g., blood loss, CHF) that ↓GFR BUN:Cr > 15
60
Renal (intrinsic) azotemia
caused by damage to the kidneys (e.g., ATN, chronic renal failure) BUN:Cr ≤ 15
61
Post-renal azotemia
↓GFR due to obstruction and increased tubular pressure causes backdiffusion of urea into blood BUN:Cr > 15 **persistent obstruction will lead to renal azotemia
62
Benign Nephrosclerosis
``` term for the renal changes observed in benign hypertension -always associated with hyaline arteriolosclerosis * homogeneous, pink hyaline thickening of the vessel lumen ```
63
Benign Nephrosclerosis morphology
``` kidneys atrophy --> grain leather appearance does not cause severe damage to kidneys -↓ GFR -loss of concentrating ability ```
64
Malignant nephrosclerosis
associated with malignant hypertension -always associated with hyperplastic arteriolosclerosis
65
Malignant nephrosclerosis morphology
small, pinpoint petechial hemorrhages cover the kidney surface -“flea-bitten kidney"
66
Malignant nephrosclerosis clinical course
Diastolic pressure >120 mm Hg, papilledema, encephalopathy, cardiovascular abnormalities, renal failure At onset, there is marked proteinuria and hematuria, but no significant alteration in renal function -quickly progresses to renal failure, so treat as medical emergency ~50% survive 5 years; 90% of deaths caused by uremia
67
Simple Cysts
``` occur as multiple or single cystic spaces in renal cortex -~1-5 cm in diameter; filled with clear fluid no clinical significance; common postmortem finding main importance is differentiation from tumors (usually simply via ultrasound) ```
68
Dialysis-associated cystic change
occur in patients with end-stage renal disease that have undergone prolonged dialysis present in both cortex and medulla, and may bleed, causing hematuria increase risk for adenomas and adenocarcinomas
69
``` Autosomal Dominant (Adult) Polycystic Kidney Disease ```
``` multiple expanding cysts of both kidneys that destroy underlying parenchyma important cause of chronic renal failure (~10% of cases) ```
70
``` Autosomal Dominant (Adult) Polycystic Kidney Disease path ```
caused by inheritance of an autosomal dominant polycystin (PKD) gene, either PKD1 (90% of families) or PKD2 -both genes encode proteins of unknown function, but have homology to those involved in cell-cell or cellmatrix adhesion pressure of expanding cysts leads to ischemic atrophy of renal parenchyma
71
``` Autosomal Dominant (Adult) Polycystic Kidney Disease clinical course ```
``` usually does not produce symptoms until the 4th decade -flank pain; "heavy, dragging sensation" -intermittent gross hematuria -superimposed hypertension and urinary infection are most important complications Berry aneurysms of the circle of Willis present in 10-30% of patients - high incidence of subarachnoid hemorrhage Asymptomatic liver cysts in 1/3rd of patients ```
72
``` Autosomal Dominant (Adult) Polycystic Kidney Disease prognosis ```
``` better than other most chronic renal diseases -slow progression; renal failure occurs, on average, at 50 yoa, but variable - treat with renal transplantation ```
73
``` Autosomal Recessive (Childhood) Polycystic Kidney Disease path ```
rare polycystic kidney disease with perinatal, neonatal, infantile, and juvenile categories caused by inheritance of two mutated copies of the fibrocystin gene (PKHD1) - unknown function, but may be a receptor with role in collecting-duct and biliary differentiation
74
``` Autosomal Recessive (Childhood) Polycystic Kidney Disease presentation ```
numerous small cysts in the cortex and medulla; give kidney sponge-like appearance - all cysts derive from collecting tubules
75
``` Autosomal Recessive (Childhood) Polycystic Kidney Disease clinical course ```
``` perinatal and neonatal forms are most common - cysts present at birth - may die quickly from pulmonary or renal failure - patients surviving infancy develop liver cirrhosis ```
76
Urolithiasis | Renal Calculi, Stones
stones may arise at any level in the urinary tract, but the kidney is the most common site affect 5-10% of Americans in their lifetime - men > women; peak age: 20-30s -most commonly contain calcium oxalate (70%), followed by magnesium ammonium phosphate (struvite), uric acid, and cystine
77
Urolithiasis | (Renal Calculi, Stones) path
increased urine concentration of the stone's constituents so that it exceeds their solubility in urine (supersaturation) e.g., hypercalciuria associated with calcium oxalate stones
78
Magnesium ammonium phosphate (struvite) stones
``` associated with UTIs caused by urea-splitting bacteria (e.g., Proteus sp.) bacteria also serve as nidus for stone formation ```
79
Uric acid stones
predisposition with gout, diseases involving rapid cell turnover (leukemias), and low urine pH (<5.5)
80
Cystine stones
genetic defect in renal transport of amino acids
81
Urolithiasis | (Renal Calculi, Stones) clinical course
``` renal or ureteral colic - flank pain radiating toward the groin gross hematuria predispose to bacterial infection; large stone may cause hydronephrosis ```
82
Hydronephrosis
dilation of the renal pelvis and calyces, with atrophy of the parenchyma, caused by obstruction to urine outflow
83
Hydronephrosis morphology
kidney may be massively enlarged renal parenchyma is compressed and atrophied; papillae are obliterated and pyramids are flattened
84
Hydronephrosis clinical course
``` complete bilateral obstruction (below ureters) causes anuridia, but partial bilateral obstruction causes polyuria (due to defects in tubular concentrating mechanism) unilateral obstruction often remains silent for long period reversible if obstruction is removed within a few weeks ```