dc.contributor.author | Cayci, F. Semsa | |
dc.contributor.author | Cakar, Nilgun | |
dc.contributor.author | Hancer, Veysel Sabri | |
dc.contributor.author | Uncu, Nermin | |
dc.contributor.author | Acar, Banu | |
dc.contributor.author | Gur, Gokce | |
dc.date.accessioned | 2019-08-13T12:10:23Z | |
dc.date.accessioned | 2019-08-13T16:03:23Z | |
dc.date.available | 2019-08-13T12:10:23Z | |
dc.date.available | 2019-08-13T16:03:23Z | |
dc.date.issued | 2012 | |
dc.identifier.issn | 0931-041X | |
dc.identifier.uri | https://dx.doi.org/10.1007/s00467-012-2283-9 | |
dc.identifier.uri | http://hdl.handle.net/11446/3074 | |
dc.description | WOS: 000310829700022 | en_US |
dc.description | PubMed ID: 22903728 | 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 | en_US |
dc.identifier.doi | 10.1007/s00467-012-2283-9 | en_US |
dc.rights | info:eu-repo/semantics/closedAccess | en_US |
dc.title | Eculizumab therapy in a child with hemolytic uremic syndrome and CFI mutation | en_US |
dc.type | article | en_US |
dc.relation.journal | PEDIATRIC NEPHROLOGY | en_US |
dc.department | DBÜ | en_US |
dc.identifier.issue | 12 | en_US |
dc.identifier.volume | 27 | en_US |
dc.identifier.startpage | 2327 | en_US |
dc.identifier.endpage | 2331 | en_US |
dc.contributor.authorID | 0000-0003-2994-1077 | en_US |
dc.relation.publicationcategory | Makale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı | en_US |
dc.department-temp | [Cayci, F. Semsa -- Cakar, Nilgun -- Uncu, Nermin -- Acar, Banu -- Gur, Gokce] Ankara Child Hlth Hematol Oncol Educ & Res Hosp, Dept Pediat Nephrol, Ankara, Turkey -- [Hancer, Veysel Sabri] Istanbul Bilim Univ, Fac Med, Dept Med Biol & Genet, Istanbul, Turkey | en_US |