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MT2 > HEMA 2 - CONNECTIVE TISSUE DISORDERS > Flashcards

HEMA 2 - CONNECTIVE TISSUE DISORDERS Flashcards

1
Q

Hereditary connective tissue disorder (autosomal dominant or recessive traits)

A

EHLERS-DANLOS SYNDROME (EDS)

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2
Q

prevelance of EHLERS-DANLOS SYNDROME (EDS)

A

1/10,000 to 1/25,000

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3
Q

_______ to _______ EDS patients in the US

A

20,000 – 50,000

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4
Q

Signs and Symptoms of EDS

A

o Joint Hypermobility
o Skin Hyperextensibility
o Tissue Fragility
o Pain

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5
Q

a disease caused by a deficiency of Vitamin C or Ascorbic Acid.

A

Scurvy

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6
Q

Signs of Scurvy

A
  • Muscle Weakness
  • Joint and Muscle Aches
  • A rash on the legs
  • Bleeding Gums
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7
Q

scurvy is prevented with 10 mg of ______ per day

A

Vitamin C

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8
Q

scurvy first present with

A

o Fatigue
o Formation of spots on the skin
o Spongy gums
o Bleeding from mucous membranes

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9
Q

scurvy patient feels

A

o depressed
o pale
o partially immobilized

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10
Q

Advanced scurvy shows

A

o Open suppurating (pus filled) wounds
o Loss of teeth
o Yellow skin
o Fever
o Neuropathy
o Death (caused by bleeding)

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11
Q
  • Easy bruising
  • Extravasated erythrocytes and increased perivascular inflammation.
A

ACTENIC (SENILE) PURPURA

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12
Q

increased vascular fragility.

A

VESSEL ABNORMALITIES

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13
Q

manifested by petechial hemorrhages of skin/mucous membranes.

A

VESSEL ABNORMALITIES

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14
Q

in vessel abnormalities, bleeding time, plt count, PT, aPTT is _____

A

normal

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15
Q

vessel abnormalities is not life threatening ______

A

bleeding

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16
Q

congenital vessel abnormalities

A

Ehlers-Danlos syndrome (AD)
Hereditary hemorrhagic telangiectasia (AD)

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17
Q

drug reaction in hypersensitivity vasculitis

A

immune complex deposit in vessel walls

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18
Q

hypersensitivity vasculitis includes

A

Henoch-Schonlein purpura

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19
Q

acquired vessel abnormalities

A

Hypersensitivity Vasculitis
Scurvy (vit C deficiency)

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20
Q

Acquired disorders caused by an underlying disease of condition.

A

ACQUIRED DISORDERS OF THE VASCULAR SYSTEM

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21
Q

ACQUIRED DISORDERS OF THE VASCULAR SYSTEM includes

A
  • Senile Purpura
  • Cushing syndrome and Corticosteroid therapy
  • Scurvy
22
Q

Ecchymoses that appear with unrecognized or minor trauma in elderly individuals.

A

Senile Purpura

23
Q

Extracellular matrix components of the skin degenerate → loss of supportive collagen fibrils → capillaries burst with minor pressure.

A

Senile Purpura

24
Q

Excess endogenous glucocorticosteroids (Cushing Syndrome) → break down in collagen

A

Cushing syndrome and Corticosteroid therapy

25
Q

Exogenous (therapeutic) glucocorticosteroids → breakdown in collagen.

A

Cushing syndrome and Corticosteroid therapy

26
Q

defiency of vitamin C for collagen synthesis → abnormal collagen production → vascular fragility and bleeding.

A

Scurvy

27
Q

Hereditary Hemorrhagic Telangiectasia is also known as

A

OSLER-WEBER-RENDU DISEASE

28
Q

Osler-Weber-Rendu Disease is autosomal _____

A

dominant

29
Q

OSLER-WEBER-RENDU DISEASE is a Telangiectasia of _________, __________, ___________, _____, ______ and __________

A

dorsum of tongue, oral cavity, buccal mucosa, lips, plate and nasal mucosa.

30
Q

osler-weber-rendu disease is apparent at ______

A

puberty

31
Q

Lung, Liver and GI tract arterio-venous malformations.

A

OSLER-WEBER-RENDU DISEASE

32
Q

OSLER-WEBER-RENDU DISEASE treatment

A

regular iron therapy, laser therapy.

33
Q

Rare complication of hepatic hemangioma in adults

A

Kasabach-Merritt Syndrome

34
Q

Kasabach-Merritt Syndrome consists of

A

o Intravascular Coagulation
o Clotting
o Fibrinolysis (sequestration of platelets) within the hemangioma.

35
Q

Kasabach-Merritt Syndrome progresses to secondary increased _______ and ________.

A

systemic fibrinolysis ; thrombocytopenia

36
Q

Kasabach-Merritt Syndrome increases risk of bleeding complication including _________.

A

intracranial hemorrhage

37
Q

Kasabach-Merritt Syndrome may lead to _____

A

DIC or Disseminated intravascular coagulation

38
Q

Kasabach-Merritt Syndrome management - definitive: the syndrome is reversible after removal of the _________.

A

hemangioma

39
Q

Kasabach-Merritt Syndrome management - supportive: __________ and ______

A

platelet transfusions and FFPs

40
Q

Kasabach-Merritt Syndrome management - in non-resectable cases: ______, _________, __________, ________

A

arterial embolization, corticosteroids, alpha-interferon, chemotherapy.

41
Q

This is a form of vasculitis that is a grouping of conditions that develops inflammation in the blood vessels.

A

HENOCH SCHONLEIN PURPURA

42
Q

HENOCH SCHONLEIN PURPURA is a type of inflammation can cause bleeding in the blood vessels that are small and referred to as _______.

A

capillaries

43
Q

In Henoch Schonlein Purpura, the capillaries that are affected are in ____, _______, _____ and _____

A

joints, skin, kidneys and intestines.

44
Q

In Henoch Schonlein Purpura, the major indication is a rash that is ______ in color, normally on the lower area of the _____ as well as ______.

A

purplish ; legs ; buttocks

45
Q

The Henoch Schonlein Purpura condition can cause pain in the ______ as well as _____ that ache and with some, __________.

A

abdomen ; joints ; kidney complications

46
Q

A level which is elevated of a distinctive protein called ___ can advise Henoch-schonlein purpura, but it is not conclusive.

A

IgA

47
Q

Other indications of Hennoch-Schonlein Purpura can be a high _________ – denoted as sed rate.

A

erythrocyte sedimentation rate

48
Q

This measures how fast red blood cells drop to the end in a tube of blood in an hour, and indicates the ________ level in the body.

A

erythrocyte sedimentation rate ; inflammation

49
Q
  • generalized hypersensitivity vasculitis.
  • uncertain cause.
A

HENOCH-SCHONLEIN PURPURA

50
Q

CLINICAL Sx of Henoch-Schonlein:

A
  • purpura
  • colicky abdominal pain
  • polyarthralgia
  • acute glomerulonephritis

MT2 (45 decks)

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