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*MHD Exam 5 > Systemic Diseases w/ Glomerular Involvement > Flashcards

Systemic Diseases w/ Glomerular Involvement Flashcards

1
Q

Do children typically get primary glomerular diseases or secondary to systemic diseases?

A

primary

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2
Q

There is kidney involvement in many systemic diseases, including:

A
  • Diabetic nephropathy
  • Amyloidosis
  • SLE
  • Thrombotic microangiopathies
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3
Q

the most common cause of ESRD in the United States

A

diabetic nephropathy

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4
Q

Diabetic nephropathy is a clinical syndrome characterized by:

A
  • Proteinuria
  • Progressive decline in GFR
  • HTN
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5
Q

In diabetic nephropathy, thickening of the vascular BM in which arteriole is greater?

A

efferent arteriole

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6
Q

What is the primary pathogenic feature associated with diabetic nephropathy?

A

nonenzymatic glycosylation of the vascular basement membrane resulting in hyaline arteriosclerosis (thickening of the lamina densa impairs diffusion of oxygen and nutrients)

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7
Q

characteristic nodule associated with diabetic nephropathy

A

Kimmelstiel-Wilson (KW) nodules

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8
Q

What is diabetic glomerulosclerosis?

A

progressive thickening of the glomerular basement membrane and increase in mesangial matrix eventually forming KW nodules

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9
Q

In diabetic nephropathy, overt _______ correlates with ________.

A

nephropathy; retinopathy

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10
Q

What are the protein quality control systems in place to remove abnormal proteins in the body?

A
  • proteasomes (intracellular)

- macrophages (extracellular)

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11
Q

Explain what fibrillogenesis is and which renal disorders it is relevant to.

A
  • Fibrillogenesis is a conformational shift in protein structure from an alpha helix to a beta-pleated sheet (which is hydrophobic, insoluble, non-functional, resistant to degradation, and “fibrillar”)
  • Fibrillogenesis is the basis behind the amyloidoses
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12
Q

What are the most common amyloidoses?

A
  • Aβ (Amyloid β protein)
  • AL (Amyloid Light chain)
  • AA (Amyloid A protein)
  • ATTR (Amyloid Transthyretin)
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13
Q

Alzheimer’s disease is which type of amyloidosis?

A

Aβ

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14
Q

What is the typical clinical presentation of systemic amyloidosis?

A
  • multisystem
  • nephrotic syndrome, cardiac failure, arrhythmia, peripheral neuropathy
  • external signs=rare (macroglossia, periorbital purpura, submandibular swelling, shoulder pad, nail lesions/dystrophy``)
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15
Q

represents 85% of systemic amyloidoses in the developed world

A

AL

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16
Q

With AL, systemic deposits affect which two major systems?

A
  • kidneys (70% with nephrotic syndrome)

- heart (60% with heart failure and arrhythmias)

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17
Q

AL involves clonal plasma cell proliferation, causing which 2 major conditions?

A
  • Plasma cell dyscrasia (85% of AL)

- Multiple myeloma (15% of AL)

18
Q

also known as “reactive” amyloidosis

A

AA

19
Q

AA amyloidosis is derived from which protein?

A

SAA (Serum Amyloid A)

20
Q

What leads to a sustained elevation of SAA?

A

Long-standing inflammation, as in RA, IBD, Crohn’s, heroin addiction, chronic infection

21
Q

Familial AA is also known as:

A

Familial Mediterranean Fever (FMF)

22
Q

Familial AA is characterized by mutations in which gene?

A

MEFV gene (regulates innate immunity)

23
Q

Hereditary amyloidosis is derived from:

A

transthyretin (TTR)

24
Q

What happens in hereditary amyloidosis?

A

A mutation in transthyretin (TTR) destabilizes the tetramer, causing TTR to aggregate and deposit in the liver (>95%), choroid plexus, and eye

25
Q

the most common hereditary amyloidosis in the US

A

ATTR

26
Q

What does ATTR mimic?

A

AL (danger of misdiagnosis!)

27
Q

What are some of the systemic effects of ATTR?

A
  • polyneuropathies (sensory, autonomic)
  • GI
  • Carpal tunnel syndrome
  • Enlarged heart
28
Q

How are amyloidoses diagnosed?

A
  • Detection w/ (+) Congo red

- Typing of amyloid protein

29
Q

Hemodialysis-associated amyloidosis is derived from:

A

β2 microglobulin (not effectively removed by dialysis); can cause peri-arthritis, carpal tunnel syndrome, and tenosynovitis

30
Q

Amyloid is fibrillary ONLY by:

A

electron microscopy

31
Q

What is a common “surrogate” site biopsy for amyloidosis?

A

subcutaneous fat (typically from periumbilical abdomen)

32
Q

What is the point of ordering a kidney biopsy for SLE patients?

A
  • to determine severity of kidney involvement and potential for reversibility of lesions
  • to determine treatment options
  • to determine prognosis
33
Q

What is the glomerular involvement of lupus nephritis?

A

ranges from mild (class I or II) to proliferative glomerulonephritis (class III, IV); only ~10% of patients develop lupus membranous nephropathy with nephrotic syndrome (class V)

34
Q

What are the vascular diseases that often have renal involvement?

A
  • benign nephrosclerosis
  • HTN
  • atherosclerosis and atheroemboli
  • vasculitis
  • Thrombotic Microangiopathies (TMA)
35
Q

What are the 2 mechanisms of Drug-Induced Thrombotic Microangiopathy?

A

1) immune-mediated reactions

2) dose- or duration-related toxic reactions

36
Q

What are the hallmarks of DITMA?

A

-acute deterioration
-development of acute kidney injury
-acute thrombocytopenia
-hemolytic anemia
-no diarrhea
(many patients w/ DITMA are found to have an underlying genetic complement defect)

37
Q

How can malignant HTN lead to TMA?

A

Malignant HTN causes fibrinoid necrosis and narrowing of the lumen, which increases mechanical injury (shearing). This is what leads to TMA.

38
Q

one of the main causes of acute kidney injury in children

A

typical HUS

39
Q

What is the typical clinical presentation of TMAs?

A
  • microangiopathic hemolytic anemia (MAHA)
  • thrombocytopenia
  • renal failure (some)
40
Q

What is the difference between typical and atypical HUS?

A
  • typical: caused by Shiga toxin E. coli; associated with diarrhea
  • atypical: complement-mediated (unregulated/excessive activation of alternative complement pathway leading to complement-mediated injury); no diarrhea

*MHD Exam 5 (14 decks)

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