HEMA 2 - CONNECTIVE TISSUE DISORDERS Flashcards
Hereditary connective tissue disorder (autosomal dominant or recessive traits)
EHLERS-DANLOS SYNDROME (EDS)
prevelance of EHLERS-DANLOS SYNDROME (EDS)
1/10,000 to 1/25,000
_______ to _______ EDS patients in the US
20,000 – 50,000
Signs and Symptoms of EDS
o Joint Hypermobility
o Skin Hyperextensibility
o Tissue Fragility
o Pain
a disease caused by a deficiency of Vitamin C or Ascorbic Acid.
Scurvy
Signs of Scurvy
- Muscle Weakness
- Joint and Muscle Aches
- A rash on the legs
- Bleeding Gums
scurvy is prevented with 10 mg of ______ per day
Vitamin C
scurvy first present with
o Fatigue
o Formation of spots on the skin
o Spongy gums
o Bleeding from mucous membranes
scurvy patient feels
o depressed
o pale
o partially immobilized
Advanced scurvy shows
o Open suppurating (pus filled) wounds
o Loss of teeth
o Yellow skin
o Fever
o Neuropathy
o Death (caused by bleeding)
- Easy bruising
- Extravasated erythrocytes and increased perivascular inflammation.
ACTENIC (SENILE) PURPURA
increased vascular fragility.
VESSEL ABNORMALITIES
manifested by petechial hemorrhages of skin/mucous membranes.
VESSEL ABNORMALITIES
in vessel abnormalities, bleeding time, plt count, PT, aPTT is _____
normal
vessel abnormalities is not life threatening ______
bleeding
congenital vessel abnormalities
Ehlers-Danlos syndrome (AD)
Hereditary hemorrhagic telangiectasia (AD)
drug reaction in hypersensitivity vasculitis
immune complex deposit in vessel walls
hypersensitivity vasculitis includes
Henoch-Schonlein purpura
acquired vessel abnormalities
Hypersensitivity Vasculitis
Scurvy (vit C deficiency)
Acquired disorders caused by an underlying disease of condition.
ACQUIRED DISORDERS OF THE VASCULAR SYSTEM
ACQUIRED DISORDERS OF THE VASCULAR SYSTEM includes
- Senile Purpura
- Cushing syndrome and Corticosteroid therapy
- Scurvy
Ecchymoses that appear with unrecognized or minor trauma in elderly individuals.
Senile Purpura
Extracellular matrix components of the skin degenerate → loss of supportive collagen fibrils → capillaries burst with minor pressure.
Senile Purpura
Excess endogenous glucocorticosteroids (Cushing Syndrome) → break down in collagen
Cushing syndrome and Corticosteroid therapy
Exogenous (therapeutic) glucocorticosteroids → breakdown in collagen.
Cushing syndrome and Corticosteroid therapy
defiency of vitamin C for collagen synthesis → abnormal collagen production → vascular fragility and bleeding.
Scurvy
Hereditary Hemorrhagic Telangiectasia is also known as
OSLER-WEBER-RENDU DISEASE
Osler-Weber-Rendu Disease is autosomal _____
dominant
OSLER-WEBER-RENDU DISEASE is a Telangiectasia of _________, __________, ___________, _____, ______ and __________
dorsum of tongue, oral cavity, buccal mucosa, lips, plate and nasal mucosa.
osler-weber-rendu disease is apparent at ______
puberty
Lung, Liver and GI tract arterio-venous malformations.
OSLER-WEBER-RENDU DISEASE
OSLER-WEBER-RENDU DISEASE treatment
regular iron therapy, laser therapy.
Rare complication of hepatic hemangioma in adults
Kasabach-Merritt Syndrome
Kasabach-Merritt Syndrome consists of
o Intravascular Coagulation
o Clotting
o Fibrinolysis (sequestration of platelets) within the hemangioma.
Kasabach-Merritt Syndrome progresses to secondary increased _______ and ________.
systemic fibrinolysis ; thrombocytopenia
Kasabach-Merritt Syndrome increases risk of bleeding complication including _________.
intracranial hemorrhage
Kasabach-Merritt Syndrome may lead to _____
DIC or Disseminated intravascular coagulation
Kasabach-Merritt Syndrome management - definitive: the syndrome is reversible after removal of the _________.
hemangioma
Kasabach-Merritt Syndrome management - supportive: __________ and ______
platelet transfusions and FFPs
Kasabach-Merritt Syndrome management - in non-resectable cases: ______, _________, __________, ________
arterial embolization, corticosteroids, alpha-interferon, chemotherapy.
This is a form of vasculitis that is a grouping of conditions that develops inflammation in the blood vessels.
HENOCH SCHONLEIN PURPURA
HENOCH SCHONLEIN PURPURA is a type of inflammation can cause bleeding in the blood vessels that are small and referred to as _______.
capillaries
In Henoch Schonlein Purpura, the capillaries that are affected are in ____, _______, _____ and _____
joints, skin, kidneys and intestines.
In Henoch Schonlein Purpura, the major indication is a rash that is ______ in color, normally on the lower area of the _____ as well as ______.
purplish ; legs ; buttocks
The Henoch Schonlein Purpura condition can cause pain in the ______ as well as _____ that ache and with some, __________.
abdomen ; joints ; kidney complications
A level which is elevated of a distinctive protein called ___ can advise Henoch-schonlein purpura, but it is not conclusive.
IgA
Other indications of Hennoch-Schonlein Purpura can be a high _________ – denoted as sed rate.
erythrocyte sedimentation rate
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.
erythrocyte sedimentation rate ; inflammation
- generalized hypersensitivity vasculitis.
- uncertain cause.
HENOCH-SCHONLEIN PURPURA
CLINICAL Sx of Henoch-Schonlein:
- purpura
- colicky abdominal pain
- polyarthralgia
- acute glomerulonephritis
