Nephrotic & Nephritic Syndromes Flashcards Preview

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Flashcards in Nephrotic & Nephritic Syndromes Deck (43)
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1
Q

What are the clinical features of nephrotic syndrome?

A
  1. Hypoalbuminemia –> pitting edema
  2. Hypogammaglobulinemia –> increased risk of infection
  3. Loss of antithrombin III –> hypercoagulable state
  4. Hyperlipidemia & hypercholesterolemia –> fatty casts in urine
2
Q

What is the most common cause of nephrotic syndrome in children?

A

Minimal change disease

3
Q

What causes minimal change disease? What is it associated with?

A

Idiopathic; Hodgkin lymphoma

4
Q

Minimal change disease results in which findings on

  1. H&E stain
  2. EM
  3. IF
A
  1. Normal glomeruli on H&E
  2. Effacement of foot processes on EM
  3. Negative IF
5
Q

Why do patients with minimal change disease respond well to steroids?

A

Damage to foot processes mediated by cytokines from T cells

6
Q

What demographic population is focal segmental glomerulosclerosis seen in?

A

Hispanics & African Americans; adults overall

7
Q

What is focal segmental glomerulosclerosis usually associated with?

A
  1. HIV
  2. Heroin use
  3. Sickle cell disease
8
Q

What is the most common cause of nephrotic syndrome in Caucasian adults?

A

Membranous nephropathy or membranous glomerulonephritis

9
Q

What is membranous nephropathy associated with?

A
  1. Hep B or C
  2. Solid tumors
  3. SLE
  4. Drugs: NSAIDs and penicillamine
10
Q

Where are immune complex deposits located in membranous nephropathy?

A

Subepithelial (beneath podocytes)

11
Q

How does membranous nephropathy appear on EM?

A

Spike and dome appearance

Spikes = immune complexes; dome = additional BM laid down by podocytes

12
Q

What is the pathogenesis of membrane thickening in membranous nephropathy?

A

Subepithelial immune deposits –> activate complement –> membrane thickening

13
Q

Focal segmental glomerulosclerosis results in which findings on

  1. H&E stain
  2. EM
  3. IF
A
  1. Sclerosis focally and segmentally
  2. Effacement of foot processes
  3. Negative IF
14
Q

Membranous nephropathy results in which findings on

  1. H&E stain
  2. EM
  3. IF
A
  1. Thickened glomerular BM
  2. Spike and dome appearance
  3. Granular IF
15
Q

Membranoproliferative glomerulonephritis results in which findings on

  1. H&E stain
  2. EM
  3. IF
A
  1. Thick glomerular BM; increased number of cells/nuclei
  2. Immune complex deposits (subendothelial or intramembranous)
  3. Granular IF
16
Q

What are some differences between Type I & II membranoproliferative glomerulonephritis?

A

Type I: subendothelial deposits; associated with HBV & HCV & more often tram track appearance
Type II = Dense Deposit Disease: intramembranous deposits; associated with C3 nephritic factor

17
Q

How does hyaline arteriosclerosis occur in diabetes mellitus? Where in the glomerulus is it more likely to occur?

A

High serum glucose –> nonenzymatic glycosylation of vascular BM
*Efferent arteriole affected more –> higher glomerular filtration pressure –> microalbuminuria ———> nephrotic syndrome

18
Q

Renal disease due to diabetes mellitus is characterized by what on H&E?

A

Sclerosis of mesangium + formation of Kimmelstiel-Wilson nodules

19
Q

What medication can be administered to reduce the progression of hyperfiltration-induced damage in DM associated renal disease?

A

ACE inhibitors

20
Q

Where in the kidney do amyloid deposits associated with systemic amyloidosis deposit?

A

Mesangium; also see nodules similar to DM

21
Q

What is the main pathology in nephritic syndrome? What clinical symptoms does it lead to?

A

Inflammation and bleeding of glomerulus

  1. Limited proteinuria (< 3.5 g/day)
  2. Oliguria & azotemia
  3. Salt retention –> periorbital edema & HTN
  4. RBC casts & dysmorphic RBCs in urine
22
Q

What does a kidney biopsy depict in a patient with nephritic syndrome?

A

Hypercellular, inflamed glomeruli

23
Q

What are the main molecules mediating damage in nephritic syndrome?

A

Immune complex deposition –> complement activation –> C5a attracts neutrophils –> hypercellular, inflamed glomeruli

24
Q

Where does immune complex deposition occur in post-streptococcal glomerulonephritis?

A

Subepithelial humps on EM

25
Q

What is the clinical presentation of someone with poststreptococcal glomerulonephritis?

A

2-3 weeks after impetigo or pharyngitis –> hematuria, oliguria, periorbital edema, HTN

26
Q

What comprises crescents seen in Rapidly Progressive Glomerulonephritis?

A

Fibrin and macrophages within Bowman’s capsule (outside glomerulus)

27
Q

A linear IF pattern is indicative of what? What is the linear pattern due to?

A

Goodpasture syndrome; IgG antibodies against BM create a sharp line giving a linear pattern

28
Q

A granular IF pattern is indicative of what? What is the granular pattern due to?

A

Immune complex deposition: PSGN, diffuse proliferative glomerulonephritis, membranous nephropathy, membranoproliferative glomerulonephritis; immune complexes are larger, have different solubilities and do not fit in glomerulus giving this granular pattern

29
Q

What are the clinical signs of Goodpasture syndrome? Which population of individuals is it classically seen in?

A

Hemoptysis and hematuria; antibodies against glomerular & alveolar basement membrane commonly in young adult males

30
Q

What is the most common type of renal disease in SLE?

A

Diffuse proliferative glomerulonephritis

31
Q

Where does immune complex deposition occur in diffuse proliferative glomerulonephritis?

A

Usually subendothelial

32
Q

Which rapidly progressive glomerulonephritis diseases does a negative IF suggest?

A

Wegener’s granulomatosis = granulomatosis with polyangitis, microscopic polyangitic, Churg-Strauss syndrome

33
Q

What are clinical features of Wegener’s granulomatosis or granulomatosis with polyangitis?

A
  1. Glomerular nephritis
  2. Hemoptysis
  3. Nasal pharyngeal symptoms: sinusitis, rhinorrhea
  4. Myalgias
34
Q

Positive c-ANCA is associated with which disease?

A

Wegener granulomatosis

35
Q

Positive p-ANCA is associated with which disease?

A

Microscopic polyangiitis & Churg-Strauss syndrome

36
Q

What are clinical features of Churg-Strauss Syndrome?

A
  1. Granulomatous inflammation
  2. Eosinophilia
  3. Asthma
    * Distinguish Churg-Strauss from microscopic polyangiitis
37
Q

Where in the kidney do IgA immune complexes associated with IgA nephropathy deposit?

A

Mesangium of glomeruli

38
Q

What is the most common nephropathy worldwide?

A

IgA nephropathy = Berger disease

39
Q

What is the clinical presentation for IgA nephropathy? When does it usually present?

A

Presents in childhood –> episodic gross or microscopic hematuria with RBC casts
*Episodic bc occur after mucosal infections (IgA elevated then)

40
Q

What are the consequences of IgA nephropathy?

A

May slowly progress to chronic renal failure

41
Q

What is the inheritance pattern for Alport Syndrome?

A

X-linked recessive

42
Q

What is the defect in Alport syndrome? What effect does this have on the glomerulus?

A

Defect in type IV collagen; results in thinning & splitting of glomerular BM

43
Q

What do people with Alport syndrome present with?

A
  1. Isolated hematuria
  2. Sensorineural hearing loss
  3. Ocular disturbances
  4. Family history of hematuria, dialysis/transplant
  5. Anterior lenticonus