| Fucosidosis and anesthesia. | |
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MedLine Citation:
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PMID: 17768480 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Fucosidosis is a rare, autosomal recessive lysosomal storage disorder caused by a severe deficiency of a-L-fucosidase. Patients usually have some problems with glycoprotein storage in the brain and other organs, and some structural abnormalities that need special consideration in anesthesia. It has 2 types, the early onset or infantile, and the juvenile. Here we present an 8-year old girl with deformities in the maxillofacial region, with big tongue, small and retracted chin, saddle nose, and short neck that could not be extended, causing difficult intubation, and congenital cardiac problems requiring a special anesthetic strategy. |
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Authors:
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Alireza E Soltani; Reza S Moharari; Reza Ghaffari; Hamid Zahedi; Mojgan Hajmahmoodi |
Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Saudi medical journal Volume: 28 ISSN: 0379-5284 ISO Abbreviation: Saudi Med J Publication Date: 2007 Sep |
Date Detail:
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Created Date: 2007-09-04 Completed Date: 2008-02-05 Revised Date: 2008-06-23 |
Medline Journal Info:
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Nlm Unique ID: 7909441 Medline TA: Saudi Med J Country: Saudi Arabia |
Other Details:
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Languages: eng Pagination: 1446-8 Citation Subset: IM |
Affiliation:
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Department of Anesthesia, Children Medical Center, University of Tehran, Tehran, Iran. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Anesthesia
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methods* Child Female Fucosidosis / pathology*, surgery Glaucoma / etiology, surgery Humans |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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