General anaesthesia in eight patients with familial dysautonomia

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General anaesthesia in eight patients with familial dysautonoma.

FAMILIAL DYSAUTONOMIA, a rare syndrome typically involving Jewish children is probably transmitted as a simple autosomal recessive gene. 1-4 During the past 15 years, eight patients with familial dysautonomia underwent a total of 24 surgical procedures under general anaesthesia at The Hospital for Sick Children, Toronto. The purpose of this paper is to review our experience with these eight pat...

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Familial dysautonomia.

Familial dysautonomia is a rare syndrome of childhood affecting the nervous system. As the name suggests, dysfunction of the autonomic system is a prominent feature. It was first recognized as a separate entity by Riley, Day, Greeley and Langford (1949), the first large series being described by Riley (1952). So far, about 70 patients have been recorded. Though the fully developed syndrome is u...

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familial dysautonomia, report of 3 cases from iran and a discussion about their general and anaesthesia care

background: familial dysautonomia (fd) is a rare hereditary syndrome which is an autosomal recessive trait that typically affects jewish children. important signs and symptoms of the disorder include; diminished pain perception, absence of overflow tears, hypotonia, fainting cardiac arrhythmias and autonomic crisis. cases report : in this article we reported 3 cases of fd syndrome which had pre...

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Familial Dysautonomia (FD)

Familial dysautonomia (FD) is an autosomal recessive congenital neuropathy which results from poor development and progressive degeneration of the nervous system. The mutation responsible for FD was found at the 5�ss of intron 20 of the IKBKAP gene, encoding the I?B kinase complex-associated protein (IKAP...

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Familial Dysautonomia (FD)

Familial dysautonomia (FD) is an autosomal recessive congenital neuropathy which results from poor development and progressive degeneration of the nervous system. The mutation responsible for FD was found at the 5�ss of intron 20 of the IKBKAP gene, encoding the I?B kinase complex-associated protein (IKAP...

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ژورنال

عنوان ژورنال: Canadian Anaesthetists’ Society Journal

سال: 1971

ISSN: 0832-610X,1496-8975

DOI: 10.1007/bf03026019