PBL Objectives 2 Flashcards

1
Q

What is a good Ddx for HSP?

A
  • Idiopathic thrombocytopenic purport (ITP)
  • Hypersensitivity vasculitis
  • Wegener granulomatosis
  • Systemic lupus erythematosus (SLE)
  • Rheumatoid arthritis
  • Rheumatic fever
  • Hemolytic uremic syndrome (HUS)
  • Polyarteritis nodosa
  • IgA Nephropathy (Berger’s)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Why wasn’t this case Polyarteritis nodosa?

A

May have similar clinical features to HSP, but uncommon in children

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Why wasn’t this case Hemolytic uremic syndrome (HUS)?

A
  • May have similar clinical features
  • Rash may also have petechiae
  • Diarrhea is common in HUS
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What lab findings are found in HUS?

A
  • Hemolytic anemia w/ elevated reticulocyte counts, low haptoglobin levels, low platelets
  • Stool studies may be helpful in HUS (these studies are normal in HSP and abnormal in HUS)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Why wasn’t this case Rheumatic fever?

A
  • May have similar clinical features to HSP

- Rash in rheumatic fever is erthrma marginatum and not palpable purpura

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What lab values are found in HSP and Rheumatic fever?

A

Increased antistreptolysin-O antibody titers have been reported in both diseases –> making them difficult to distinguish

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Why wasn’t this case Rheumatoid arthritis?

A
  • Similar clinical features to HSP
  • However, if rash is present in rheumatoid arthritis it is usually not palpable purpura
  • RA is seen more often in adults
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What lab values are seen in RA and HSP?

A

Rheumatoid factor has been reported in both –> difficult to distinguish

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Why wasn’t this case SLE?

A

May have similar clinical features to HSP but uncommon in children

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What lab findings can help distinguish SLE from HSP?

A
  • Antinuclear antibodies
  • Antineutrophil cytoplasmic antibodies
  • Complement levels
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Why wasn’t this case Wegener granulomatosis?

A

Similar clinical features to HSP, but uncommon in children

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What lab findings can help distinguish Wegener’s from HSP?

A
  • Antinuclear antibodies
  • Antineutrophil cytoplasmic antibodies (c-ANCA)
  • Complement levels
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Why wasn’t this case Hypersensitivity vasculitis?

A

Hypersensitivity vasculitis shows NO renal involvement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What lab findings help distinguish HSP from hypersensitivity vasculitis?

A

-Skin biopsy may show Leukocytoclastic vasculitis, but NO IgA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Why wasn’t this case Idiopathic thrombocytopenic Purport (ITP)?

A

Rash of ITP may also have petechiae

-Arthralgias and abdominal pain are uncommon

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What lab findings help distinguish ITP from HSP?

A

-Platelet levels are low in ITP, but normal in HSP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is the major difference between genetic association studies and GWAS studies?

A
  • Genetic association implies that one gene or a few is being looked at
  • GWAS is massive amounts of data
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What are genetic association studies and GWAS both limited by?

A

The influence of rare associations that might impact the phenotype

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What does linkage analysis require?

A

Family members!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Why isn’t linkage analysis used for HSP studies?

A

HSP is so loosely linked to family members so it would be hard to get enough to study that way

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is the mechanism behind proteinuria in HSP?

A
  1. Injury to glomerular epithelial cells (thru IC deposition and inflammation)
  2. Effacement of podocytes
  3. Breakdown in the glomerular filtration barrier
  4. Increased permeability of the glomerulus to large molecules such as albumin
22
Q

What is the mechanism behind hematuria in HSP?

A
  1. Immune complexes deposit in small vessels within the kidneys (afferent or efferent arterioles)
  2. Inflammation
  3. Necrosis of vessels
  4. Leakage of blood into urine
23
Q

What areas of the nephron are affected by HSP?

A

-Immune complexes are deposited in the glomerulus –> mesangium, subendotehlium and subepithelium

24
Q

What do immunofluorescence studies reveal in disease with immune complex deposition?

A

Granular pattern of staining characteristic of immune complex deposition

  • Frequently it demonstrates cellular proliferation and influx of leukocytes within the glomerular tuft, in addition to crescent formation
  • These patients usually cannot be helped with plasmapheresis and require treatment for the underlying disease
25
Q

What must interactions with children be?

A

Developmentally appropriate

26
Q

“The goal of care is to assist children to. . .”

A

”. . .manage changes in their health states in a manner which enhances growth.”

27
Q

What stage of cognitive development is a 9 year old probably in?

A

Piaget’s concrete operations stage of cognitive development (where cause and effect are highlighted and thinking into the future is not a strength)

28
Q

What does Erik Erikson think about a 9 year olds development?

A

Emotional tasks of children at this age center around industry (and competence) versus inferiority

29
Q

What do school aged children like to demonstrate?

A

Competence in day to day life

  • -> why large motor activities are important
  • School = job
  • Learning new things is task-like
  • Like to order, categorize
  • Like to collect things
  • Categorizing is a precursor to formal operations –> but that will take time to develop
30
Q

What can you not ask children?

A

If they want to be treated. They have no choice –> this is the responsibility of the parent.

31
Q

How can we give children a sense of industry and competence?

A

By offering them the opportunity to control as much as is reasonable.

32
Q

What is an example of give a child industry and competence (control)?

A

Do you want to sit up or lie down when I do X?
Would you like to hold your mom’s hand while we do X?
How about you hold this band-aid until I need it?
-Rely on their task orientation and be SPECIFIC about what you need them to do

33
Q

What should you NEVER ask a parent to do?

A

Restrain their child

  • This is not their role, no matter how convenient
  • Staff people are around to do that
  • Role of parent is to comfort the child
34
Q

What should you always do when the procedure is done?

A

Always comment that the child did well, even if there was a lot of screaming and crying —> tell them it helped you do the procedure

35
Q

What should you remember with kids and procedures?

A
  • BE SPECIFIC

- THEY ARE CAUSE AND EFFECT ORIENTED

36
Q

What causes abdominal pain in HSP?

A

Type III hypersensitivity –> deposition into small vessels of GI tract –> destruction of vessels –> ischemia –> pain

37
Q

What causes a rash on the legs?

A

Progressing from red –> purple –> brown

38
Q

What causes tiredness in HSP?

A

IL-6 (predominantly)

39
Q

What causes dark stools/fecal occult blood in HSP?

A

Type III Hypersensitivity –> deposition into small vessels of the GI tract –> destruction of vessels –> leakage of blood into still

40
Q

What causes hypotension in HSP?

A

GI distubances –> N/V –> low blood volume –> low bp

41
Q

What causes elevated WBCs in HSP?

A

Elevated cytokines IL-1, 2, 17, 22, TNF(alpha) –> upregulation and chemotaxis of WBCs

42
Q

What causes elevated eosinophils?

A

Th2 secretes IL4, 5 –> Eosinophilia

43
Q

What causes elevated ESR?

A

Increase in IgA and Complement –> Increased repulsion of RBCs –> longer duration for sedimentation –> increased ESR

44
Q

What causes Hematuria in HSP?

A

Glomerulus injury from C3 deposit (Alt/Lectin pathway) + Controversy about involvement of the rest: IL-1,6, cytokines, chemokines, adhesion factors, PDGF, TNF, Leukotrienes, Membrane Attack Complexes (MAC 5b-9). These have been implicated to cause injury to the glomerulus –> leakage of blood into urine –> Hematuria

45
Q

What causes elevated IgA in HSP?

A

APC presentation –> CD4+ activation –> CD4+ T cell interaction with B cell (IL-2, 4, 10, CD40/CD40L, CD4+/MHC II) –> Formation of IgM against antigen –> class switch to IgA

46
Q

What causes Leukocytoclastic vasculitis in HSP?

A

White cells are being recruited to the immune complexes in the vasculature. There’s damage (Leukocytoclastic = damage to WBC)

47
Q

What causes Mural Fibrin Deposition?

A

Destruction of small vessels –> Coagulation cascade –> Mural fibrin on vessels

48
Q

What caused increased mesangial matrix in HSP?

A

Hyperglycemia (glucose stimulates collagen formation), alterations in Angiotensin II, and immune complex depositions into the mesangial matrix can all cause the matrix to expand
-In our case, the immune complex deposition was likely a key factor

49
Q

What causes protein in the urine in HSP?

A

Glomerulus injury form C3 deposit (Alt/Lectin pathway) + controversy abou involvement of rest IL1, 6, cytokines, chemokines, adhesion factors, PDGF, TNF, Leukotrienes, Membrane Attack Complex (MAC 5b-9). These have been implicated to cause the injury to the glomerulus –> increased filtration of protein –> protein in urine

50
Q

What is the mechanism behind the rash in HSP?

A
  • Extravasation of RBC caused by WBC damage to vessel wall

- Color change is hemosiderin from RBCs taken up by macrophages