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KMID : 0356919930260051055
Korean Journal of Anesthesiology
1993 Volume.26 No. 5 p.1055 ~ p.1058
A Case Report of Marfan Syndrome under General Anesthesia


Abstract
We experienced a case of Cesarean section of Marfan syndrome under the general anesthesia. Marfan syndrome is an autosomal dominant trait characterized by generalized defect of connective tissue, which is mediated by a structural deficiency in
Type
1
collagen. Its prevalence has been estimated at 1 to 5 in 100,000. Expression of the trait is variable but the most common diagnostic signs are disproportionate length of long bones, hypermobility of joints, prolapsed lens, and cardiac murmurs,
most
often the systolic click-murmur of mitral valve prolapse.
The importance in anesthetic management of these patents should be chosen to prevent any hypertension in response to intubation and surgery, and handled very gently, with minimal stress placed on the connective tisues.
KEYWORD
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