Mucopolysaccharidosis II : Colligative - Practical Wisdom in Anesthesia, Surgery, and Medicine.

Aliases:

Gargoylism, Hunter Syndrome

Hunter Syndrome Gargoylism

Hunter's Syndrome

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Overview

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Definition, Pathophysiology

Systemic lysosomal storage disease marked by progressive physical deterioration and caused by a deficiency of L-sulfoiduronate sulfatase. This disease differs from MUCOPOLYSACCHARIDOSIS I by slower progression, lack of corneal clouding, and X-linked rather than autosomal recessive inheritance. The mild form produces near-normal intelligence and life span. The severe form usually causes death by age 15.
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