dc.contributor.author | Çaycı, F. Şemsa | |
dc.contributor.author | Çakar, Nilgün | |
dc.contributor.author | Hançer, Veysel Sabri | |
dc.contributor.author | Uncu, Nermin | |
dc.contributor.author | Acar, Banu | |
dc.contributor.author | Gür, Gökçe | |
dc.date.accessioned | 2014-11-27T14:01:29Z | |
dc.date.available | 2014-11-27T14:01:29Z | |
dc.date.issued | 2012 | |
dc.identifier.citation | Cayci FS, Cakar N, Hancer VS, Uncu N, Acar B, Gur G. Eculizumab therapy in a child with hemolytic uremic syndrome and CFI mutation. Pediatric Nephrology. 2012; 27(12):2327-2331. doi: 10.1007/s00467-012-2283-9. | en_US |
dc.identifier.issn | 0931-041X | |
dc.identifier.uri | http://link.springer.com/article/10.1007%2Fs00467-012-2283-9 | en_US |
dc.identifier.uri | https://hdl.handle.net/11446/571 | en_US |
dc.description | İstanbul Bilim Üniversitesi, Tıp Fakültesi. | en_US |
dc.description.abstract | Background
Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in childhood. It usually occurs after a prodromal episode of diarrhea and it leads to significant morbidity and mortality during the acute phase. However, cases that start as diarrhea-positive HUS whose renal function fail to recover should be screened for genetic disorders of the complement system, which is called atypical HUS (aHUS).
Case-Diagnosis/Treatment
We herein report a 10-year-old girl, who initially came with bloody diarrhea and had features of HUS with delayed renal and hematological recovery despite plasma therapy. Eculizumab (600 mg/week) was initiated on day 15 for atypical presentation and later a complement factor I (CFI) mutation was detected. The girl recovered diuresis within 24 h and after the third eculizumab infusion, hemoglobin, platelet, and C3 levels normalized; renal function improved; and proteinuria completely disappeared in 2 weeks.
Conclusion
It is our belief that eculizumab can be the treatment of choice in children who have plasma exchange-refractory HUS with defective regulation of the alternative complement pathway. | en_US |
dc.language.iso | eng | en_US |
dc.publisher | Springer Verlag | en_US |
dc.rights | info:eu-repo/semantics/embargoedAccess | en_US |
dc.title | Eculizumab therapy in a child with hemolytic uremic syndrome and CFI mutation | en_US |
dc.type | report | en_US |
dc.relation.journal | Pediatric Nephrology | en_US |
dc.department | DBÜ, Tıp Fakültesi | en_US |
dc.identifier.issue | 12 | |
dc.identifier.volume | 27 | |
dc.identifier.startpage | 2327 | |
dc.identifier.endpage | 2331 | |
dc.contributor.authorID | TR132685 | en_US |
dc.contributor.authorID | TR43513 | en_US |
dc.relation.publicationcategory | Diğer | en_US |