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Arch Pediatr Adolesc Med. 2006;160:706.

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Denouement and Discussion: Ehlers-Danlos Syndrome, Classical Type

Ehlers-Danlos syndrome is a heterogenous group of inherited connective tissue disorders characterized by the variable expression of hyperextensible skin, joint hypermobility, and tissue fragility.¹ Ehlers-Danlos syndrome is thought to occur as frequently as 1 in 5000 and can affect any sex or race.² In 1997, a revised classification of Ehlers-Danlos syndrome resulted in a simplified classification of 6 types.³ The new classification system proposed specific major and minor diagnostic criteria for each type and also included newly available molecular and biochemical data.³ The 6 types include classical, hypermobility, vascular, kyphoscoliosis, arthrochalasia, dermatosparaxis, and other.³ The inheritance pattern is either autosomal dominant or autosomal recessive depending on the specific subtype. Severity of symptoms can range from extremely mild to disabling. Because of the varied clinical symptoms, diagnosis can be difficult to make.

CLINICAL FEATURES
The classical type of Ehlers-Danlos syndrome is the most common.¹ It is usually transmitted in an autosomal dominant pattern. . . . [Full Text of this Article]

ASSOCIATED SYSTEMIC FEATURES

PATHOGENESIS

MANAGEMENT

AUTHOR INFORMATION

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Arch Pediatr Adolesc Med. 2006;160(7):705.
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