Review Note

Last Update: 10/29/2024 03:23 AM

Current Deck: PGY 2 Path Review::Exam 2: Dec 6::Quizlet::path exam 2::BRS Pathology - Ch. 1-4

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Hurler syndrome
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Deficiency of a-L-iduronidase

Accumulation of mucopolysaccharides heparin sulfate, dermatan sulfate

- Progressive deterioration
- Hepatosplenomegaly
- Dwarfism
- Gargoyle-like facies
- Stubby fingers
- Corneal clouding
- Progressive mental retardation
- Death by 10 years
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