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Old 11-01-2011
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Question Died by aortic rupture!

An unconscious 25-year-old man is brought to the emergency department (ED) by a police officer. He reports having last seen the man working in his yard about 4 hours earlier. Initial physical examination showed blood pressure of 60/40 mm Hg, pulse of 150/min, and respiratory rate of 10/min. He is of normal height. The man died after 1 one hour. Autopsy reveals extremely flexible joints and skin, although fingers are of normal length and there are no appreciable spinal abnormalities. A number of bruises, not present on admission, are noted on his thorax. The cause of death is determined to be rupture of the abdominal aorta.

A) Elastin
B) Fibrillin
C) Type I collagen
D) Type III collagen
E) Type IV collagen
F) Dynein
G) Spectrin
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Old 11-01-2011
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D -Ehler danols??
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Old 11-02-2011
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E. Type IV collagen
I'm thinking about the Type IV collagen formation disorder.
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Old 11-02-2011
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i think its E, type IV collagenn
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Old 11-02-2011
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Hi Evergreen, could you give out the right answer with explanation please?
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Old 11-02-2011
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Correct Answer Answer

Quote:
Originally Posted by belindalimm View Post
Hi Evergreen, could you give out the right answer with explanation please?
The correct answer is D. Aortic aneurysm can be sequelae of Ehlers-Danlos syndrome which is caused by an autosomal dominant genetic defect in collagen, which results in join laxity and poor tissue integrity. Synthesis of type III collagen is most frequently defective in this syndrome.

A is not correct. A defect in elastin synthesis would result in an increase in tissue rigidity as opposed to an in increase in elasticity, as is observed in this patient.

Mutation in Fibrillin is observed in Marfan Syndrome. The skin finding in this patient is not consistent with this syndrome. In Marfan patients are tall with long extremities and have scoliosis. Aortic dissection is possible complication in these patients.

Defect in Type I collagen --> Osteogenesis imperfecta
Defect in Type IV collagen --> Alport's syndrome (Mostly X-linked recessive): Nephritis, deafness and ocular disturbances
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ooops!! u kno wat i was thinkn..ehler danlos type IV!! haha..thats y i went with option E.
dumb dumb dumb
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Old 11-02-2011
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This is my way to remember these a long time ago:
Osteogenesus 1mperfecta
3hl3r-Danlos
Al4t
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