(F) L4: Renal Function (Part 3: Diseases)

Question 1

These may be secondary to infection, autoimmune diseases, or genetic diseases leading to the dysfunction of filtration causing the presence of cells and proteins in urine

Answer 1

Glomerular Disorders

Question 2

Give the 13 Glomerular Disorders

Answer 2

1. Acute Glomerulonephritis (AGN)
2. Rapidly Progressive/Crescentic Glomerulonephritis (RPGN)
3. Goodpasture Syndrome
4. Granulomatosis with Polyangiitis or Wegener’s Granulomatosis (GPA)
5. Henoch-Schonlein Purpura
6. Membranous Glomerulonephritis (MGN)
7. Chronic Glomerulonephritis
8. Berger’s Disease
9. Nephrotic Syndrome
10. Minimal Change Disease
11. Focal Segmental Glomerulosclerosis
12. Alport Syndrome
13. Diabetic Nephropathy

Question 3

Glomerular Disorders

• aka Post Streptococcal Nephritis (secondary to S. pyogenes)
• Deposition of immune complexes for the Group A (pyogenes) Strep infection on the glomerular membrane
• Presents with macroscopic hematuria, proteinuria, RBC and granular casts, and oliguria

Answer 3

Acute Glomerulonephritis (AGN)

Question 4

Glomerular Disorders

This test involves the complement with the activation of the membrane attack complex (the lysis antigen)

Answer 4

Antistreptolysin O (ASO) for Acute Glomerulonephritis (AGN)

Question 5

Glomerular Disorders

• Hematuria and proteinuria (albumin < 3g/day causes edema)
• Decreased GFR with sodium retention (hypertension)
• Presence of RBC casts (pathognomonic to this disease)
• Associated with a recent infection yet idiopathic

Answer 5

Acute Glomerulonephritis (AGN)

Question 6

Glomerular Disorders

• The deposition of immune complexes from systemic immune disorders on the glomerular membranes caused by systemic autoimmune diseases such as SLE
• Presents with macroscopic hematuria, proteinuria, and RBC casts

Answer 6

Rapidly Progressive/Crescentic Glomerulonephritis (RPGN)

Question 7

Glomerular Disorders

• Has an anti-glomerular basement membrane antibody
• A cytotoxic antibody attaches to the glomerular and alveolar basement membrane (lungs and kidneys are affected)
• Presents with macroscopic hematuria, proteinuria, and RBC casts

Answer 7

Goodpasture Syndrome

Question 8

Glomerular Disorders

What 3 diseases are characterized by the presence of antibodies against the glomerular basement membrane?

Answer 8

1. Acute Glomerulonephritis (AGN)
2. Rapidly Progressive/Crescentic Glomerulonephritis (RPGN)
3. Goodpasture Syndrome

Question 9

Glomerular Disorders

• Has an antineutrophilic cytoplasmic autoantibody
• Is also associated with primary sclerosing cholangitis
• Binds to neutrophils in vascular walls producing damage to small vessels in the lungs and glomerulus
• Presents with macroscopic hematuria, proteinuria, and RBC casts

Answer 9

Granulomatosis with Polyangiitis or Wegener’s Granulomatosis (GPA)

Question 10

Glomerular Disorders

Goodpasture Syndrome and Granulomatosis with Polyangiitis or Wegener’s Granulomatosis (GPA) are almost identical except for their antibodies, which are those?

Answer 10

Goodpasture: Anti-Glomerular Basement Membrane Ab
GPA: Anti-Neutrophilic Cytoplasmic AutoAb

Question 11

Glomerular Disorders

• Common in children following a viral respiratory infection
• Presents with decreased platelets and vasculitis (IgA vasculitis; inflammation of BVs due to IgA deposition)
• Every BV is affected because of the deposition
• Presents with macroscopic hematuria, proteinuria, and RBC casts

Answer 11

Henoch-Schonlein Purpura

Question 12

Glomerular Disorders

What 5 conditions present with macroscopic hematuria, proteinuria, and RBC casts?

Answer 12

1. Acute Glomerulonephritis (AGN)
2. Rapidly Progressive/Crescentic Glomerulonephritis (RPGN)
3. Goodpasture Syndrome
4. Granulomatosis with Polyangiitis or Wegener’s Granulomatosis (GPA)
5. Henoch-Schonlein Purpura

First 5 on the list basically

Question 13

Glomerular Disorders

• There is thickening of the Glomerular Basement Membrane (GBM) due to IgG deposition
• Since IgG is triggered, the complement is also triggered which causes cell lysis
• Presents with macroscopic hematuria and elevated proteins

Answer 13

Membranous Glomerulonephritis (MGN)

Question 14

Glomerular Disorders

• There is a marked decrease in renal function resulting from glomerular damage precipitated by other renal disorders
• Is secondary to chronic diseases like hypertension and uncontrolled diabetes (hyperglycemia)
• Presents with hematuria, proteinuria, glucosuria, and casts (cellular, granular, waxy, and broad)

Answer 14

Chronic Glomerulonephritis

Question 15

Glomerular Disorders

• Aka IgA nephropathy
• The most common cause of glomerulonephritis
• Increased serum IgA levels cause a depositioning of IgA on the GBM
• Predominantly affects the kidneys of adults and young adults

Answer 15

Berger’s Disease

Question 16

Glomerular Disorders

Deposition of IgA
A. Henoch-Schonlein Purpura
B. Berger’s Disease
C. Both
D. Neither

Answer 16

C. Both

Question 17

Glomerular Disorders

Early or Late Stage Berger’s Disease?
1. Macroscopic and microscopic hematuria
2. Hematuria, proteinuria, glucosuria, and casts (cellular, granular, waxy, and broad)

Answer 17

1. Early stage
2. Late stage

Question 18

Glomerular Disorders

• There is a disruption of the shield of negativity and damage to the tightly fitting podocytes resulting in massive loss of proteins and lipids
• There is excessive proteinuria (3.5 to 20 g/day) and hypoalbuminemia (which causes edema)
• Also manifests as hyperlipidemia and lipiduria
• Presents with heavy proteinuria, microscopic hematuria, RTE, oval fat bodies, and fatty and waxy casts

Answer 18

Nephrotic Syndrome

Question 19

Glomerular Disorders

In nephrotic syndrome, in a patient’s SERUM, what are the levels of albumin and A2-macroglobulin?

Answer 19

• Albumin - decreased (excreted out due to impaired shield of negativity)
• A2-Macroglobulin - increased (unable to pass due to a MW of 700,000 Da)

Question 20

Glomerular Disorders

In nephrotic syndrome, in a patient’s URINE, what are the levels of albumin and A2-macroglobulin?

Answer 20

• Albumin - increased (excreted out due to impaired shield of negativity)
• A2-Macroglobulin - decreased (unable to pass due to a MW of 700,000 Da)

Question 21

Glomerular Disorders

• Aka Lipoid nephrosis
• Mostly idiopathic however it may be secondary to allergic reactions and immunization
• T-cells release cytokines that can damage the podocytes
• There is little cellular change in the glomerulus but less tightly fitting podocytes
• Presents with heavy proteinuria, edema, transient hematuria, fat droplets, and normal NPNs

Answer 21

Minimal Change Disease

Question 22

Glomerular Disorders

There is a disruption of podocytes in certain areas of the glomeruli associated with heroin and analgesic abuse & AIDS

Answer 22

Focal Segmental Glomerulosclerosis

Question 23

Glomerular Disorders

• An inherited disorder showing a lamellated & thinning GBM caused by a defect in the gene that codes for type 4 collagen
• Primary urinalysis results are that of nephrotic syndrome: heavy proteinuria, microscopic hematuria, RTE, oval fat bodies, and fatty and waxy casts

Answer 23

Alport Syndrome

Question 24

Glomerular Disorders

Patients with Alport Syndrome have a genetic defect on the gene that codes for what?

Answer 24

Type 4 Collagen

Question 25

# Glomerular Disorders - Aka Kimmelstiel-Wilson Disease - The most common cause of end stage renal disease - There is GBM thickening and an accumulation of solid substances around the capillary tuft due to a deposition of glycosylated proteins - Primary urinalysis results are that of chronic glomerunonephritis: hematuria, proteinuria, glucosuria, and casts (cellular, granular, waxy, and broad)

Answer 25

Diabetic Nephropathy

Question 26

# Glomerular Disorders Thickening of the GBM A. Alport Syndrome B. Diabetic Nephropathy C. Both D. Neither

Answer 26

B. Diabetic Nepropathy (Alport is thinning)

Question 27

# Glomerular Disorders Microscopic hematuria A. Nephrotic Syndrome B. Alport Syndrome C. Chronic Glomerulonephritis D. A and B E. B and C F. A and C

Answer 27

D. A and B

Question 28

Disorders that decrease the efficacy of reabsorption and secretion

Answer 28

Tubular Disorders

Question 29

Give the 7 tubular disorders

Answer 29

1. Acute Tubular Necrosis (ATN) 2. Fanconi Syndrome 3. Uromodulin-associated Kidney Disease 4. Nephrogenic DI 5. Renal Tubular Acidosis (RTA) 6. Renal Tubular Infection (E. coli) 7. Renal Tubular Obstruction

Question 30

# Tubular Disorders - There is damage to the renal tubular cells caused by ischemia (lack of oxygenation) or toxic agents - Presents with microscopic hematuria, proteinuria, RTE, and casts (RTE, hyaline, granular, waxy, and broad)

Answer 30

Acute Tubular Necrosis (ATN)

Question 31

# Tubular Disorders - A generalized defect in renal tubular reabsorption - Is inherited in association with cystine disorders and Hartnup disease or acquired through exposure to toxic agents (heavy metals) - Is secondary to multiple myeloma or renal transplantation

Answer 31

Fanconi Syndrome

Question 32

# Tubular Disorders - An inherited defect in the production of normal uromodulin (aka Tamm-Horsfall protein) by RTEs - Increased uric acid causes gout

Answer 32

Uromodulin-Associated Kidney Disease

Question 33

# Tubular Disorders An inherited defect of tubular response to ADH or acquired from medications

Answer 33

Nephrogenic DI

Question 34

# Tubular Disorders Tubules are unable to secrete acids leading to acidic blood pH

Answer 34

Renal Tubular Acidosis (RTA)

Question 35

# Tubular Disorders Infection of the upper urinary tract (pyelonephritis)

Answer 35

Renal Tubular Infection (E. coli)

Question 36

# Tubular Disorders Tumors, renal calculi, and stones | Note: Calcium oxalate is the most common

Answer 36

Renal Tubular Obstruction

Question 37

Diseases that result in tissue inflammation that may affect the kidneys

Answer 37

Interstitial Diseases

Question 38

Give the 3 interstitial diseases

Answer 38

1. Cystitis 2. Pyelonephritis 3. Acute Interstitial Nephritis (AIN)

Question 39

# Interstitial Diseases Infection of the bladder

Answer 39

Cystitis

Question 40

# Interstitial Diseases - Infection of the renal tubules, interstitium, and renal pelvis - May be acute or chronic

Answer 40

Pyelonephritis

Question 41

# Interstitial Diseases How do you differentiate Cystitis and Pyelonephritis?

Answer 41

Pyelonephritis has WBC casts

Question 42

# Interstitial Diseases - Allergic inflammation of the interstitium secondary to medications - We expect elevated eosinophils

Answer 42

Acute Interstitial Nephritis (AIN)

Question 43

The most severe disease that is aka end stage renal disease

Answer 43

Renal Failure

Question 44

Give the 2 types of renal failure

Answer 44

1. Acute 2. Chronic

Question 45

# Renal Failure - Sudden loss of renal function - May be a toxic or hypoxic insult to the kidney - Signs include: oliguria, azotemia, onset of uremic syndrome or ESRD, peripheral edema, hypertension, metabolic acidosis, and CHF - There is reduced GFR (< 10mL/min) which is caused by decreased tubular secretion

Answer 45

Acute Renal Failure

Question 46

# Renal Failure - Secondary to chronic renal disease like chronic glomerulonephritis and diabetic nephropathy - A gradual decline in renal operation - There is scarring of the collecting tubules - Prevents salt and water reabsorption (polyuria)

Answer 46

Chronic Renal Failure

Question 47

# Give the stage of chronic kidney disease Description: At increased risk GFR (mL/min per 1.73m2): ≥ 90 (with risk factors)

Answer 47

None

Question 48

# Give the stage of chronic kidney disease Description: Kidney damage with normal or unequal GFR GFR (mL/min per 1.73m2): > 90 Manual: silent deterioration of renal status

Answer 48

Stage 1

Question 49

# Give the stage of chronic kidney disease Description: Kidney damage with normal or decreased GFR GFR (mL/min per 1.73m2): 60-89 Manual: 50% reduction in normal functioning

Answer 49

Stage 2

Question 50

# Give the stage of chronic kidney disease Description: Moderately decreased GFR GFR (mL/min per 1.73m2): 30-59 Manual: impending renal failure (anemia systemic acidosis)

Answer 50

Stage 3

Question 51

# Give the stage of chronic kidney disease Description: Severely decreased GFR GFR (mL/min per 1.73m2): 15-29 Manual: uremic syndrome

Answer 51

Stage 4

Question 52

# Give the stage of chronic kidney disease Description: Kidney failure GFR (mL/min per 1.73m2): < 15

Answer 52

Stage 5

Question 53

# Additional Diseases - Aka Diabetic nephropathy - Manifests 15-20 years after diagnosis - There are heavy demands made by the kidney to diurese - Microalbuminuria (10-15 years) with increased pores

Answer 53

Diabetes Mellitus

Question 54

# Additional Diseases - Decreased perfusion - Increased hypernatremia - Stimulation of the RAAS

Answer 54

Renal Hypertension

Question 55

# Tubular Disorders Inherited conditions A. Acute Tubular Necrosis B. Uromodulin-associated Kidney Disease C. Both D. Neither

Answer 55

B. Uromodulin-associated Kidney Disease The inherited conditions are: 1. Fanconi 2. Uromodulin-associated 3. Nephrogenic DI

Question 56

# Glomerular Disorders Autoimmune disorders A. Acute Glomerulonephritis B. Rapidly Progressive (Crescentic) Glomerulonephritis C. Both D. Neither

Answer 56

B. Rapidly Progressive (Crescentic) Glomerulonephritis Other autoimmune disorders: - Goodpasture Syndrome - Granulomatosis with Polyangiitis/Wegener's Granulomatosis (GPA)