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ClinMed_I > Lecture 3c > Flashcards

Lecture 3c Flashcards

1
Q

Define hemophilia A.

A

Congenital deficiency of coagulation F8

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2
Q

Define hemophilia B.

A

Congenital deficiency of coagulation F9

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3
Q

What is the common symptom of hemophilia?

A

Recurrent hemarthoses(joint bleeding)
Easy bruising/bleeding

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4
Q

How does severe hemophilia present in infant males or early childhood?

A

Spontaneous bleeding into joints, soft tissue

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5
Q

Why is an associated negative development that could occur with hemophilia?

A

Develop inhibitors to the associated clotting factor
30% of pts in hemophilia A
<5% in hemophilia B

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6
Q

What is the normal range for F8?

A

50-150% (varies with age)

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7
Q

How do you treat hemophilia?

A

Plasma-derived or recombinant factor concentrates
Can be treated with prophylaxis
Severe hemophilia can come up to 3x a week

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8
Q

Can pts live a normal life with hemophilia?

A

Yes, if prophylactically treated/managed and educated

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9
Q

What is the MC cause of death in hemophilia pts?

A

Transfusion-obtained HIV/AIDs
Hepatitis/cirrhosis

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10
Q

What must hemophilic pts do to avoid complications?

A

Avoid contact sports
Monitor for S/S of bleeding
Home infusion technique
Managed by hematologist

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11
Q

What is the MC inherited bleeding disorder?

A

Von willebrand disease

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12
Q

How many types of von willebrand stages are there?

A

3
Type 1: quantitive(not enough)
Type 2: qualitative(dysfunctional)
Type 3: profound quantitive(near or total absence of VWF)

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13
Q

What is F11 deficiency also referred to as?

A

Hemophilia C

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14
Q

What does thrombocytopenia present as?

A

Mucous membrane related bleeding…
Epistaxis(nose bleed)
Gum bleeding
GI bleeds
Life-threatening cerebral hemorrhage

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15
Q

How can thrombocytopenia occur?

A

Increased destruction
Decreased production

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16
Q

What are examples that decrease production of platelets?

A

Congenital/Acquired bone marrow failure
Exposure to chemotherapy, irradiation
Marrow infiltration
Nutritional

17
Q

What are examples of destructive/consumption thrombocytopenia?

A
18
Q

How does splenomegaly/hypersplenism destroy platelets?

A

Aged platelets are normally destroyed by the spleen, but it is now unregulated

19
Q

What is the etiology of immune thrombocytopenia purpura?

A

Pts form auto-AB against Ag on the platelet surface (causing destruction)

20
Q

What lab do we order for suspicion of immune thrombocytopenia purpura?

A

CBC with peripheral blood smear

21
Q

Is there a cure for ITP?

A

No

22
Q

What are the treatments for ITP?

A

Corticosteroids(first line)
IVIG
Platelet transfusion(serious bleeds)
Splenectomy

23
Q

What drugs are the common cause of acquired/iatrogenic PLT defects?

A

Aspirin
Clopidogrel
NSAIDs

24
Q

What thrombotic microangiopathies (TMAs) are there?

A

Thrombotic thrombocytopenic purpura (TTP)
Hemolytic-uremic syndrome (HUS)

25
Q

How do you diagnose TTP?

A

CDC (should show elevated WBC)
Hg 8-9g/dL
PLT 20-50k
Peripheral smear (presence of schistocytes)
Coagulation studies, PT, aPTT are normal
D-dimer (normal-elevated)
Fibrinogen (high)
LDH (high)
Direct Coombs test negative
Hyperbilirubinemia

26
Q

What is the most common cause of acute renal failure in children?

A

Hemolytic-uremic syndrome(HUS)

27
Q

How does HUS present?

A

Prodromal gastroenteritis
Prodrome of fever, bloody diarrhea for 2-7 days before onset of renal failure
Irritability, lethargy
Seizures
Acute renal failure
Anuria
HTN
Edema/volume overload
Pallor

28
Q

What bacteria is the most common cause of HUS in NA and Western Europe?

A

E. coli serotype 0157:H7

29
Q

What disorders are associated with DIC?

A

Sepsis
Cancer, trauma, burns, or pregnancy-assocated morbidity
Aortic aneurysm or cavernous hemangiomas
Snake bites

ClinMed_I (15 decks)

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