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Renal Week 3 > The Kidney in Systemic Disease II > Flashcards

The Kidney in Systemic Disease II Flashcards

1
Q

What causes sickle cell?

A

Substitution of a valine for glutamic acid as the sixth amino acid of the beta globin chain, which produces a hemoglobin tetramer (alpha2/beta S2) that is poorly soluble when deoxygenated

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2
Q

How does sickle cell present?

A
  • painful ‘crises’
  • symptomatic anemia
  • renal involvement
  • leg ulcers
  • recurrent priapism
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3
Q

What labs would you see with sickle cell?

A

mild to moderate anemia,
reticulocytosis of 3 to 15 percent, unconjugated hyperbilirubinemia,

elevated serum lactate dehydrogenase, and

low serum haptoglobin.

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4
Q

What do Howell-Jolly body presence in SCD suggest?

A

reflecting hyposplenia secondary to repeated splenic infarctions

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5
Q

What are the renal manifestations of SCD?

A

Microalbuminuria reaches a prevalence of approximately 60% in patients with hgb SS disease those over 45 years

increased GFR into young adulthood, followed by decreasing GFR from the 40s on

Impaired distal H+ ion and K+ secretion

-Supranormal PT function leading to increased creatinine secretion and hyperphosphatemia

Hyposthenuria

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6
Q

Sickling crises are commonly initiated in the renal medulla. Why?

A

The relative hypoxia (partial pressure of oxygen 10–35 mmHg), acidosis, and hyperosmolarity of the inner medulla may result in polymerization of deoxygenated hemoglobin S and subsequent sickling of erythrocytes

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7
Q

Progression of renal involvement of SCD?

A

4 to 12% of patients with hgb SS disease will develop end-stage renal disease (ESRD)

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8
Q

What is early stage sickle cell nephropathy characterized by?

A

Glomerular hypertrophy,

hemosiderin deposits,

focal areas of hemorrhage or necrosis

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9
Q

What is late stage sickle cell nephropathy characterized by?

A

Interstitial inflammation,

edema, fibrosis, tubular atrophy, and

papillary infarcts

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10
Q

What is end stage sickle cell nephropathy characterized by?

A

glomerular enlargement and

focal segmental glomerulosclerosis

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11
Q

What causes amyloidosis?

A

Misfolded proteins (chemically diverse, but physically similar), all folded into beta-pleated sheets,

Usually first deposited in blood vessel walls, then interstitium, where it squeezes the cells to death, eventually leading to organ failure

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12
Q

What is a rare cancer that only presents in sickle cell patients?

A

medullary carcinoma of the kidney

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13
Q

What do medullary carcinomas of the kidney arise from?

A

collecting duct system

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14
Q

What patient population gets medullary carincomas of the kidney?

A

avg age 21-24; 75% male

75-89% in the right kidney

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15
Q

Prognosis of medullary carincomas of the kidney?

A

very aggressive (death within 4-6 months)- resistant to radiotherapy and chemo

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16
Q

Describe the histology of renal amyloidosis.

A
  • nonproliferative

- noninflammatory glomerulopathy

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17
Q

So what does renal amyloidosis show on urinalysis?

A

bland urinalysis (no pyuria) with proteinuria

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18
Q

T or F. Amyloidosis is associated with kidney shrinkage

A

F. It is associated with enlargement, as is diabetic nephropathy and HIV nephropathy

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19
Q

What causes AL amyloidosis?

A

primary disorders of immunoglobulin production (MM, Waldenstrom’s amcroglobulinemia)

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20
Q

What causes AA amyloidosis?

A

more secondary

  • persistent acute inflammation
  • Cancers
  • Familial Mediterranean Fever
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21
Q

Where does amyloidosis affect first in the kidneys?

A

the vessels first, and then into the interstitium

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22
Q

After confirmation of the presence of amyloid in tissue the next step must be what?

A

Assessment of the type of amyloid involved.

23
Q

Amyloid detected in a patient with longstanding rheumatoid arthritis and a nephrotic syndrome is almost undoubtedly __ type.

A

AA

24
Q

A patient with amyloid and clinical signs such as shoulder pads and glossomegaly most likely will have __ type.

A

AL

25
Q

What is ATTR amyloidosis caused by?

A

mutation in the transthyretin gene (TTR)

26
Q

A patient with chronic renal failure and often occurs in patients who are on dialysis for many years, tends to deposit in synovium and joints most likely has __ type.

A

Beta-2 microglobulin

27
Q

IHC is a method for characterizing amyloidosis and is most useful for ___ type

A

AA, in cases of AL and ATTR (need DNA analysis) it is less specific

28
Q

When does ALA usually present? How?

A

avg age is 64 yo. Presents with weakness, weight loss, nehrotic syndrome, and peripheral and autonomic neuropathy

not hypertensive

29
Q

Are the kidneys small or big in ALA?

A

enlarged

30
Q

How is ALA treated?

A

low dose PO melphalan and dexamethasome

or high dose melphalan with autologous set cell transplant in patients less than 50 yo

31
Q

Medium survival of ALA?

A

10 months

32
Q

How is AAAmy diagnosed?

A

renal biopsy (can follow serum amyloid A levels)

33
Q

How is AAAmy treated?

A

Eprodisate

34
Q

What does Eprodisate do?

A

limits deposition of amyloid A fibrils by interfering with their interaction with tissue glycosaminoglycans and slows renal decline

35
Q

What do AAAmy patients die from?

A

infection or dialysis infection

36
Q

What type of amyloidosis is most related to ESRD?

A

AB2M amyloidosis

37
Q

What is light chain deposition disease?

A

A NON-amyloid monoclonal immunoglobulin deposition disease (MIDD)

38
Q

T or F. Light chain deposition disease is congo red negative

A

T.

39
Q

Light chain deposition disease is most associated with what?

A

Multiple myeloma

40
Q

How does light chain deposition disease present?

A
  • proteinuria and renal failure
  • may have hepatomegaly or cardiomegaly or CHF
  • many more
41
Q

What are the viral-associated kidney diseases?

A
  • HIVAN

- Cryoglobulinemia

42
Q

HIVAN predisposes to what race?

A

AA (due to APOL1 mutation that persists because of protection against Trypanosoma induced african sleeping sickness)

43
Q

How does HIVAN present?

A
  • progressive azotemia
  • significant proteinemia
  • minimal peripheral edema
44
Q

HIVAN is classically a _________

A

collapsing form or focal segmental glomerulosclerosis (not crescent forming) with prominent microcytic tubular dilation and interstitial inflammation and fibrosis

45
Q

What causes HIVAN?

A

expression of viral genes, especially Nef and Vpr, in renal epithelial cells in HIV-1

46
Q

How is HIVAN treated?

A
  • start HAART
  • give ACEI or ARB

most will develop ESRD despite HAART treatment

47
Q

Mixed cryoglobulinemia type II isa associated with what?

A

Hep C

48
Q

What is Mixed cryoglobulinemia type II?

A

B-cell lymphoproliferative disorder characterized by the deposition of immune complexes containing rheumatoid factor (RF), IgG, HCV RNA, and complement on endothelial surfaces, eliciting vascular inflammation

49
Q

What is Meltzer’s triad for Mixed cryoglobulinemia type II?

A
  • purpura (most common)
  • arthralgia
  • weakness

Only seen in 25-30% of patients

50
Q

Is Mixed cryoglobulinemia type II more common in men or women?

A

slightly more in women (1.5:1)

51
Q

When does Mixed cryoglobulinemia type II typically present?

A

60s

52
Q

How do the symptoms progressive in Mixed cryoglobulinemia type II?

A

tend to wax and wane, often lasting 1-2 weeks and then recurring 1-2x per month

53
Q

Cryoglobulinemia causes a ____ pattern of injury

A

membranoproliferative

Renal Week 3 (16 decks)

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