Matches in SemOpenAlex for { <https://semopenalex.org/work/W2461529307> ?p ?o ?g. }
Showing items 1 to 81 of
81
with 100 items per page.
- W2461529307 abstract "Maple syrup urine disease (MSUD) is a rare autosomal recessive inherited metabolic disorder. The main toxic amino acid leucine and its metabolites build up in the blood, precipitated by fasting, surgery and sepsis. This can result in confusion, seizure activity and potential cerebral oedema. We hope to raise awareness and understanding of the condition and its management for surgical teams who may encounter patients with MSUD. Medical therapy aims to rapidly reduce toxic metabolites by restricting dietary intake of plasma-branched amino acids and supplementing with amino acids mixtures free of leucine, isoleucoine and valine. Strict dietary management, restricting catabolism and promoting anabolism, and monitoring nutritional status are fundamental to long term growth, development and health maintenance. When considering surgery in a patient with MSUD, either electively or as an emergency, close monitoring is required. Advice from a metabolic expert is preferable. Fasting should be minimized where possible, the operation should be performed promptly and effectively and any sepsis should be treated. Daily monitoring of branched-chain amino acid levels and monitoring or nutritional and fluid intake are essential. Emergency feeding regimens, either orally, via nasogastric or total parenteral nutrition is required. Dialysis can filter toxic metabolites from the blood. When patients with maple surgery urine disease require surgery a multi-disciplinary team approach is required. Under the advice of a metabolic team, the surgical team needs to work closely with the anaesthetic and dietetic teams. With correct monitoring and nutritional support patients can make a full recovery following emergency or elective surgery." @default.
- W2461529307 created "2016-07-22" @default.
- W2461529307 creator A5007418015 @default.
- W2461529307 creator A5044757199 @default.
- W2461529307 date "2014-08-04" @default.
- W2461529307 modified "2023-09-27" @default.
- W2461529307 title "Peri-Operative Management Of Maple Syrup Urine Disease: The Surgical Perspective" @default.
- W2461529307 cites W1921797022 @default.
- W2461529307 cites W1953493409 @default.
- W2461529307 cites W1986095977 @default.
- W2461529307 cites W2038783671 @default.
- W2461529307 cites W2055649458 @default.
- W2461529307 cites W2074551630 @default.
- W2461529307 cites W2137680310 @default.
- W2461529307 cites W2158245071 @default.
- W2461529307 cites W2166078809 @default.
- W2461529307 cites W3216228406 @default.
- W2461529307 hasPublicationYear "2014" @default.
- W2461529307 type Work @default.
- W2461529307 sameAs 2461529307 @default.
- W2461529307 citedByCount "0" @default.
- W2461529307 crossrefType "journal-article" @default.
- W2461529307 hasAuthorship W2461529307A5007418015 @default.
- W2461529307 hasAuthorship W2461529307A5044757199 @default.
- W2461529307 hasConcept C126322002 @default.
- W2461529307 hasConcept C141071460 @default.
- W2461529307 hasConcept C177713679 @default.
- W2461529307 hasConcept C185592680 @default.
- W2461529307 hasConcept C2776580952 @default.
- W2461529307 hasConcept C2777573094 @default.
- W2461529307 hasConcept C2778384902 @default.
- W2461529307 hasConcept C2779214519 @default.
- W2461529307 hasConcept C2780026642 @default.
- W2461529307 hasConcept C31174226 @default.
- W2461529307 hasConcept C515207424 @default.
- W2461529307 hasConcept C55493867 @default.
- W2461529307 hasConcept C71924100 @default.
- W2461529307 hasConcept C75141282 @default.
- W2461529307 hasConceptScore W2461529307C126322002 @default.
- W2461529307 hasConceptScore W2461529307C141071460 @default.
- W2461529307 hasConceptScore W2461529307C177713679 @default.
- W2461529307 hasConceptScore W2461529307C185592680 @default.
- W2461529307 hasConceptScore W2461529307C2776580952 @default.
- W2461529307 hasConceptScore W2461529307C2777573094 @default.
- W2461529307 hasConceptScore W2461529307C2778384902 @default.
- W2461529307 hasConceptScore W2461529307C2779214519 @default.
- W2461529307 hasConceptScore W2461529307C2780026642 @default.
- W2461529307 hasConceptScore W2461529307C31174226 @default.
- W2461529307 hasConceptScore W2461529307C515207424 @default.
- W2461529307 hasConceptScore W2461529307C55493867 @default.
- W2461529307 hasConceptScore W2461529307C71924100 @default.
- W2461529307 hasConceptScore W2461529307C75141282 @default.
- W2461529307 hasIssue "1" @default.
- W2461529307 hasLocation W24615293071 @default.
- W2461529307 hasOpenAccess W2461529307 @default.
- W2461529307 hasPrimaryLocation W24615293071 @default.
- W2461529307 hasRelatedWork W168195304 @default.
- W2461529307 hasRelatedWork W179677471 @default.
- W2461529307 hasRelatedWork W1921691366 @default.
- W2461529307 hasRelatedWork W1963927792 @default.
- W2461529307 hasRelatedWork W2025398217 @default.
- W2461529307 hasRelatedWork W2067458921 @default.
- W2461529307 hasRelatedWork W2098790252 @default.
- W2461529307 hasRelatedWork W2101229643 @default.
- W2461529307 hasRelatedWork W2281688390 @default.
- W2461529307 hasRelatedWork W2408990894 @default.
- W2461529307 hasRelatedWork W2410030919 @default.
- W2461529307 hasRelatedWork W2411550701 @default.
- W2461529307 hasRelatedWork W2411903247 @default.
- W2461529307 hasRelatedWork W2416088883 @default.
- W2461529307 hasRelatedWork W2418351398 @default.
- W2461529307 hasRelatedWork W2472285059 @default.
- W2461529307 hasRelatedWork W3029295690 @default.
- W2461529307 hasRelatedWork W3032491949 @default.
- W2461529307 hasRelatedWork W3151965938 @default.
- W2461529307 hasRelatedWork W71885790 @default.
- W2461529307 hasVolume "32" @default.
- W2461529307 isParatext "false" @default.
- W2461529307 isRetracted "false" @default.
- W2461529307 magId "2461529307" @default.
- W2461529307 workType "article" @default.