Matches in SemOpenAlex for { <https://semopenalex.org/work/W152059350> ?p ?o ?g. }
Showing items 1 to 68 of
68
with 100 items per page.
- W152059350 endingPage "9" @default.
- W152059350 startingPage "364" @default.
- W152059350 abstract "The macrothrombocytopenias make up a heterogeneous group of disease involving thrombocytopenia and giant platelets; other clinical or laboratory findings, such as hereditary nephritis, sensorineural hearing loss, leukocyte inclusions, and cataracts, may also be present. The tendency to bleeding is highly variable and is due to decreased expression of the GP1b-V-IX complex on the surface of platelets, leading to altered platelet-vessel wall and platelet-platelet interactions. The 5 autosomal dominant giant-platelet disorders that are associated with macrothrombocytopenia are May-Hegglin anormaly, Epstein, Fechtner, and Sebastian syndromes, and Alport-like syndrome with macrothrombocytopenia. The mutation responsible is in gene 9 (MYH9) coding for the nonmuscle myosin heavy chain IIA that has been identified in the long arm of chromosome 22 (22q12.3-q13.2). The most recently described macrothrombocytopenia is Sebastian syndrome, consisting of thrombocytopenia with giant platelets and leukocyte inclusions. We report the case of a woman with Sebastian syndrome scheduled for abdominoperineal resection for rectal carcinoma. Preoperative studies revealed isolated thrombocytopenia (35,000 platelets/microL) and a mean platelet volumen of 13 fL. Preoperative prophylactic platelet transfusion was carried out with no adverse events. After a postoperative transfusion of packed red cells, needed because of abundant bleeding, clinical course continued to be satisfactory. The anesthetic implications of this syndrome are not well known because few cases have been reported in the literature, and none was found that describes anesthetic management. Nevertheless, thrombocytopenia and the tendency to bleeding present challenges to the anesthesiologist." @default.
- W152059350 created "2016-06-24" @default.
- W152059350 creator A5001405794 @default.
- W152059350 creator A5008993619 @default.
- W152059350 creator A5026200558 @default.
- W152059350 creator A5057920246 @default.
- W152059350 creator A5081311522 @default.
- W152059350 creator A5087630579 @default.
- W152059350 date "2003-10-14" @default.
- W152059350 modified "2023-09-27" @default.
- W152059350 title "[Anesthesia in Sebastian syndrome: a new hereditary macrothrombocytopenia]." @default.
- W152059350 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/14552110" @default.
- W152059350 hasPublicationYear "2003" @default.
- W152059350 type Work @default.
- W152059350 sameAs 152059350 @default.
- W152059350 citedByCount "0" @default.
- W152059350 crossrefType "journal-article" @default.
- W152059350 hasAuthorship W152059350A5001405794 @default.
- W152059350 hasAuthorship W152059350A5008993619 @default.
- W152059350 hasAuthorship W152059350A5026200558 @default.
- W152059350 hasAuthorship W152059350A5057920246 @default.
- W152059350 hasAuthorship W152059350A5081311522 @default.
- W152059350 hasAuthorship W152059350A5087630579 @default.
- W152059350 hasConcept C126322002 @default.
- W152059350 hasConcept C141071460 @default.
- W152059350 hasConcept C142724271 @default.
- W152059350 hasConcept C2776689292 @default.
- W152059350 hasConcept C2778212984 @default.
- W152059350 hasConcept C2779216453 @default.
- W152059350 hasConcept C2910090564 @default.
- W152059350 hasConcept C2993802102 @default.
- W152059350 hasConcept C42219234 @default.
- W152059350 hasConcept C71924100 @default.
- W152059350 hasConcept C89560881 @default.
- W152059350 hasConcept C90924648 @default.
- W152059350 hasConceptScore W152059350C126322002 @default.
- W152059350 hasConceptScore W152059350C141071460 @default.
- W152059350 hasConceptScore W152059350C142724271 @default.
- W152059350 hasConceptScore W152059350C2776689292 @default.
- W152059350 hasConceptScore W152059350C2778212984 @default.
- W152059350 hasConceptScore W152059350C2779216453 @default.
- W152059350 hasConceptScore W152059350C2910090564 @default.
- W152059350 hasConceptScore W152059350C2993802102 @default.
- W152059350 hasConceptScore W152059350C42219234 @default.
- W152059350 hasConceptScore W152059350C71924100 @default.
- W152059350 hasConceptScore W152059350C89560881 @default.
- W152059350 hasConceptScore W152059350C90924648 @default.
- W152059350 hasIssue "7" @default.
- W152059350 hasLocation W1520593501 @default.
- W152059350 hasOpenAccess W152059350 @default.
- W152059350 hasPrimaryLocation W1520593501 @default.
- W152059350 hasRelatedWork W152059350 @default.
- W152059350 hasRelatedWork W1968481366 @default.
- W152059350 hasRelatedWork W2029147896 @default.
- W152059350 hasRelatedWork W2168817958 @default.
- W152059350 hasRelatedWork W2330246311 @default.
- W152059350 hasRelatedWork W2341998416 @default.
- W152059350 hasRelatedWork W2416584307 @default.
- W152059350 hasRelatedWork W2952789016 @default.
- W152059350 hasRelatedWork W4236136620 @default.
- W152059350 hasRelatedWork W54221465 @default.
- W152059350 hasVolume "50" @default.
- W152059350 isParatext "false" @default.
- W152059350 isRetracted "false" @default.
- W152059350 magId "152059350" @default.
- W152059350 workType "article" @default.