10.24 Flashcards

1
Q

What type of collagen forms the backbone of the GBM?

A

Type IV collagen (COL4A1-COL4A6)

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2
Q

What is azotemia?

A

High in nitrogen byproducts–BUN and creatinine

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3
Q

What is uremia?

A

Azotemia + clinical symptoms (gastroenteritis, anemia, periph. neuropathy, pruritis, pericarditis, etc)

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4
Q

What is the presentation of nephritic syndromes?

A

Hematuria
Mild/moderate proteinuria
HTN

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5
Q

What is the presentation of nephrotic syndromes?

A
>3.5g/day proteinuria
Hypoalbuminemia
Edema
Hyperlipidemia
Lipiduria
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6
Q

What are signs of Acute Renal Failure (ARF)?

A

Recent onset azotemia (increases in BUN/Cr)

Oliguria or anuria

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7
Q

What causes ARF?

A

Glomerular, tubulointerstitial, or vascular disease

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8
Q

What are signs of Chronic Renal Failure (CRF)?

A

Prolonged symptoms of uremia

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9
Q

What are the symptoms of renal tubular defects?

A

Polyuria
Nocturia
Electrolyte imbalances (metabolic acidosis)

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10
Q

What causes renal tubular defects?

A

Inherited (RTA, cystinuria)

Acquired (lead)

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11
Q

When do renal tumors typically present?

A

Late…not good

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12
Q

What are the 4 stages of Chronic Renal Failure?

A

Diminished renal reserve
Renal Insufficiency (CRI)
Renal Failure (CRF)
End-Stage Renal Disease (ESRD)

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13
Q

What are the symptoms of Diminished renal reserve?

A

GFR of 50% normal
Normal range BUN/Cr
Asymptomatic

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14
Q

What are the symptoms of CRI?

A

GFR is 20-50% normal
Azotemia
Anemia
HTN

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15
Q

What are the symptoms of CRF?

A

GFR <20-25% normal
Edema
Metabolic acidosis
Uremia

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16
Q

What are the symptoms of ESRD?

A

GFR <5% normal

Terminal stage of uremia

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17
Q

What causes pre-renal increases in BUN?

A
Increased synthesis of urea: 
Catabolism--burns, fever, stress
High protein diet
GI bleed
Hemolyis
Malignancy
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18
Q

What causes renal increases in BUN?

A

Glomerular disease
ATN (Acute Tubular Necrosis)
Interstitial disease

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19
Q

What causes post-renal increases in BUN?

A

Urinary tract obstruction

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20
Q

What causes pre-renal increases in serum creatinine?

A
Increased synthesis (muscle hypertrophy/necrosis [and similar], high meat diet)
Decreased renal perfusion (CHF, hypotension/shock, etc)
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21
Q

What causes post-renal increases in serum creatinine?

A

Urinary tract obstruction

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22
Q

Is creatinine a good indicator of GFR?

A

Yes…but exceeds actual by 10-40%–creatinine is secreted in the tubules

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23
Q

What causes a high BUN:Cr ratio?

A

Pre-renal conditions–increased proximal urea reabsorption (accompanies the reabsorption of water)

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24
Q

What would a FeNa of <1.0% indicate?

A

Pre-renal condition

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25
Q

What would a FeNa of >2.0% indicate?

A

ATN

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26
Q

What protein does a urine dipstick measure?

A

Albumin

27
Q

What causes false positives for protein in a urine dipstick test?

A

Alkaline urine
Gross hematuria
Dilute urine

28
Q

What does an acid precipitation detect?

A

Albumin

Globulins–light chains

29
Q

What causes false positives in an acid precipitation test?

A

Hematuria

Some meds

30
Q

What conditions can cause proteinuria other than renal disease?

A

CHF
Massive obesity
Constrictive pericarditis
Renal vein thrombosis

31
Q

What would the proteinuria pattern of a glomerular condition look like?

A

Albumin
Small globulins–usually reabsorbed with normal tubular function

(protein level may be in nephrotic range)

32
Q

What would the proteinuria pattern of a tubular condition look like?

A

Beta-2-microglobulin (1-2gms/24hrs)

33
Q

What percentage of people are born with urinary tract malformations?

A

10%

34
Q

What percentage of CRF in kids is caused by dysplasia and hypoplasia?

A

20%

35
Q

Are horseshoe kidneys usually fused at the upper or lower poles?

A

Lower poles (9:1)

36
Q

How common are horseshoe kidneys?

A

0.2-0.1%

37
Q

What are the gross characteristics of cystic renal dysplasia?

A

Enlarged
Multi-cystic
Irregular

38
Q

What are the micro characteristics of cystic renal dysplasia

A

Undifferentiated mesenchyme
Cartilage
Immature collecting ductules
Variably sized cysts lined by flattened epithelium

39
Q

What genes are possibly affected in Autosomal Dominant Polycystic Kidney Disease (PCKD)?

A

PKD1 (on 16p13.3)…80-85% of cases

PKD2 (on 4q21)…10-15% of cases

40
Q

What does PKD1 encode for?

A

Polycystin 1…cell/cell or cell/matrix interactions

On cilia of tubular cells

41
Q

What does PKD2 encode for?

A

Polycystin 2…acts as a nonspecific calcium-permeable channel

On cilia of tubular cells

42
Q

When is the onset of Autosomal Dominant PCKD?

A

Usually between 40 and 75

Renal failure in <75% by age 75

43
Q

What is destroyed due to multiple expanding cysts?

A

Renal parenchyma

44
Q

What are the gross characteristics of AD PCKD?

A

Bilaterally enlarged kidneys

Look like bags of cysts

45
Q

What are the micro characteristics of AD PCKD?

A

Cysts with variable lining

Arise from tubules throughout the nephron
Normal parenchyma presents between cysts

46
Q

What is the clinical presentation of AD PCKD?

A

Asymptomatic/pain/hematuria

47
Q

What are some extra-renal anomalies associated with AD PCKD?

A

Liver cysts (40%)
Intracranial berry aneurysms–causes deaths in 4-10% of PCKD patients
Mitral valve prolapse in 20-25%

48
Q

What are the categories of AR PCKD?

A

Perinatal
Neonatal
Infantile
Juvenile

49
Q

Patients in which categories survive infancy?

A

Infantile (die before adolescents)

Juvenile (die shortly after adolescents)

50
Q

What are the gross features of AR PCKD?

A

Enlarged
Smooth externally
Sections show many small cysts in cortex and medulla

51
Q

What are the micr features of AR PCKD?

A

Dilation of all collecting tubules…uniform cuboidal lining of cysts

52
Q

What other anomalies are associated with AR PCKD?

A

Liver cysts
Bile duct proliferations
Congenital hepatic fibrosis–in infantile and juvenile

53
Q

Who gets Medullary Sponge Kidney?

A

Adults

54
Q

What happens with Medullary Sponge Kidney?

A

Multiple cystic dilatations of collecting ducts in the medulla form

55
Q

What causes Medullary Sponge Kidney?

A

Can be incidental

Or associated with hematuria, infection, or stones

56
Q

How does the kidney function with Medullar Sponge Kidney?

A

Normally

57
Q

What are the gross features of Medullary Sponge Kidney?

A

Dilated papillary ducts in the medulla

58
Q

What are the micro features of Medullary Sponge Kidney?

A

Cysts lined by cuboidal or transitional epithelium

59
Q

What is Nephronophthisis-Uremic Medullary Cystic Disease Complex?

A

A progressive disorder with onset in childhood

60
Q

What are the features of Nephronophthisis-Uremic Medullary Cystic Disease Complex?

A

Cysts in the medulla associated with cortical tubular atrophy and interstitial fibrosis

61
Q

How do children present with Nephronophthisis-Uremic Medullary Cystic Disease Complex?

A

Polyuria and polydipsia–because of tubular defect

62
Q

What is the concern with Acquired (dialysis-associated) Cystic Disease?

A

Development of renal cell carcinoma within the cysts (7% of dialysis patients w/in 10 years)

63
Q

What is a simple cyst?

A

1-5cm
Filled with clear fluid
Single layer of cuboidal or flattened epithelium line the cysts

64
Q

What is the important thing about simple cysts?

A

Making sure they aren’t tumors