Matches in SemOpenAlex for { <https://semopenalex.org/work/W2086723976> ?p ?o ?g. }
Showing items 1 to 70 of
70
with 100 items per page.
- W2086723976 abstract "Microangiopathic haemolytic anaemia (MAHA), as part of thrombotic microangiopathy (TMA) post bone marrow transplant, is well documented yet its management continues to challenge clinicians. Outside the transplant setting, classical thrombotic thrombocytopenia is often successfully treated with plasma exchange therapy, but there is evidence to suggest that is not beneficial for post–transplant patients particularly when acute graft-versus-host disease (GVHD) is present. Post–transplant TMA is characterised by MAHA, thrombocytopenia, elevated LDH and abnormal renal function. Fever and neurological impairment may also feature. In children, management has mainly been supportive, with a focus on withdrawing risk factors. The aetiology is unconfirmed and is likely to be multifactorial. Infection, GVHD, irradiation and immunosuppressive drugs have been implicated as has the use of ATG in conditioning and CMV seronegativity. Outside of the transplant setting it is thought that inappropriate complement pathway is central to the development and treatment of TMAs and it has been hypothesised that complement dysregulation caused by ciclosporin may be responsible for some cases of post-transplant TMA. Eculizumab is a monoclonal antibody that acts on the complement pathway by preventing the cleavage of C5 into C5a and C5b, which forms part of a cytotoxic membrane attack complex. Eculizumab is licensed for use in adults and adolescents to treat atypical haemolytic uraemic syndrome and is the standard treatment for paroxysmal nocturnal haemoglobinuria in adults with emerging reports of its use to treat children with the same condition. There is also increasing evidence to support its use in children with atypical haemolytic uraemic syndromeand adult patients with TMA following renal transplant. We present the case of an 18-month-old boy with Mucopolysaccharidosis Type 1 who following a bone marrow transplant, developed anaemia, thrombocytopenia, renal impairment with difficult to manage hypertension and raised LDH. A diagnosis of MAHA was reached and eculizumab was given after minimal improvement was seen after discontinuing ciclosporin. This resulted in an improvement in blood pressure and renal function, reduction in LDH and urine protein–creatinine ratio and reduced need for transfusion." @default.
- W2086723976 created "2016-06-24" @default.
- W2086723976 creator A5030719286 @default.
- W2086723976 creator A5045080781 @default.
- W2086723976 creator A5074403474 @default.
- W2086723976 date "2015-04-01" @default.
- W2086723976 modified "2023-09-26" @default.
- W2086723976 title "G488(P) A novel use for eculuzimab: successful treatment of a child with transplant associated microangiopathic haemolytic anaemia" @default.
- W2086723976 doi "https://doi.org/10.1136/archdischild-2015-308599.441" @default.
- W2086723976 hasPublicationYear "2015" @default.
- W2086723976 type Work @default.
- W2086723976 sameAs 2086723976 @default.
- W2086723976 citedByCount "1" @default.
- W2086723976 countsByYear W20867239762015 @default.
- W2086723976 crossrefType "journal-article" @default.
- W2086723976 hasAuthorship W2086723976A5030719286 @default.
- W2086723976 hasAuthorship W2086723976A5045080781 @default.
- W2086723976 hasAuthorship W2086723976A5074403474 @default.
- W2086723976 hasBestOaLocation W20867239761 @default.
- W2086723976 hasConcept C111684460 @default.
- W2086723976 hasConcept C126322002 @default.
- W2086723976 hasConcept C137627325 @default.
- W2086723976 hasConcept C203014093 @default.
- W2086723976 hasConcept C2777878052 @default.
- W2086723976 hasConcept C2777991916 @default.
- W2086723976 hasConcept C2778585876 @default.
- W2086723976 hasConcept C2779134260 @default.
- W2086723976 hasConcept C2781127562 @default.
- W2086723976 hasConcept C71924100 @default.
- W2086723976 hasConcept C8891405 @default.
- W2086723976 hasConcept C89560881 @default.
- W2086723976 hasConceptScore W2086723976C111684460 @default.
- W2086723976 hasConceptScore W2086723976C126322002 @default.
- W2086723976 hasConceptScore W2086723976C137627325 @default.
- W2086723976 hasConceptScore W2086723976C203014093 @default.
- W2086723976 hasConceptScore W2086723976C2777878052 @default.
- W2086723976 hasConceptScore W2086723976C2777991916 @default.
- W2086723976 hasConceptScore W2086723976C2778585876 @default.
- W2086723976 hasConceptScore W2086723976C2779134260 @default.
- W2086723976 hasConceptScore W2086723976C2781127562 @default.
- W2086723976 hasConceptScore W2086723976C71924100 @default.
- W2086723976 hasConceptScore W2086723976C8891405 @default.
- W2086723976 hasConceptScore W2086723976C89560881 @default.
- W2086723976 hasLocation W20867239761 @default.
- W2086723976 hasOpenAccess W2086723976 @default.
- W2086723976 hasPrimaryLocation W20867239761 @default.
- W2086723976 hasRelatedWork W10393505 @default.
- W2086723976 hasRelatedWork W11937183 @default.
- W2086723976 hasRelatedWork W2072826018 @default.
- W2086723976 hasRelatedWork W2073597868 @default.
- W2086723976 hasRelatedWork W2089750828 @default.
- W2086723976 hasRelatedWork W2100348771 @default.
- W2086723976 hasRelatedWork W2296548580 @default.
- W2086723976 hasRelatedWork W2511556555 @default.
- W2086723976 hasRelatedWork W2598352713 @default.
- W2086723976 hasRelatedWork W2604040070 @default.
- W2086723976 hasRelatedWork W2615570676 @default.
- W2086723976 hasRelatedWork W2916800519 @default.
- W2086723976 hasRelatedWork W2965651562 @default.
- W2086723976 hasRelatedWork W2971375901 @default.
- W2086723976 hasRelatedWork W2973470021 @default.
- W2086723976 hasRelatedWork W3025198264 @default.
- W2086723976 hasRelatedWork W3102726713 @default.
- W2086723976 hasRelatedWork W3122877358 @default.
- W2086723976 hasRelatedWork W3129593041 @default.
- W2086723976 hasRelatedWork W2184531469 @default.
- W2086723976 isParatext "false" @default.
- W2086723976 isRetracted "false" @default.
- W2086723976 magId "2086723976" @default.
- W2086723976 workType "article" @default.