Document Detail

The effect of rocuronium and sugammadex on neuromuscular blockade in a child with congenital myotonic dystrophy type 1.
MedLine Citation:
PMID:  23763618     Owner:  NLM     Status:  Publisher    
Myotonic dystrophy type 1 (MD1) is the commonest muscular dystrophy found in adults; however, it may present in the neonatal period with hypotonia, talipes, poor feeding, and respiratory failure. Inheritance is autosomal dominant with a defect in the DMPK gene found on the long arm of chromosome 19 with variable expansion of the cytosine-thymine-guanine (CTG) triplet repeat. A 14-month-old boy with congenital MD type 1 was scheduled for percutaneous endoscopic gastrostomy (PEG) insertion, orchidopexy, and division of tongue-tie. Following induction of anesthesia, acceleromyography was used to monitor neuromuscular function. This revealed a very rapid onset of profound neuromuscular block which lasted significantly longer than would be expected in a child without MD1. Sugammadex reversed the block rapidly. The anesthetic management of children with MD1 has been well described but not the acceleromyographic monitored use of rocuronium and its subsequent reversal with the new cyclodextrin sugammadex.
Amelia Pickard; Clinton Lobo; Peter A Stoddart
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-6-14
Journal Detail:
Title:  Paediatric anaesthesia     Volume:  -     ISSN:  1460-9592     ISO Abbreviation:  Paediatr Anaesth     Publication Date:  2013 Jun 
Date Detail:
Created Date:  2013-6-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9206575     Medline TA:  Paediatr Anaesth     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2013 John Wiley & Sons Ltd.
Department of Anaesthesia, Bristol Royal Hospital for Children, Bristol, UK.
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