Myocardial infarction is a complication of factor H-associated atypical HUS

被引:42
作者
Sallee, Marion [1 ]
Daniel, Laurent [2 ]
Piercecchi, Marie-Dominique [3 ]
Jaubert, Dominique [1 ]
Fremeaux-Bacchi, Veronique [4 ]
Berland, Yvon [1 ]
Burtey, Stephane [1 ]
机构
[1] Univ Med, Ctr Nephrol & Transplantat Renale, AP HM, Hop Concept, Marseille, France
[2] Hop Enfants La Timone, AP HM, Serv Anat Pathol, Marseille, France
[3] Hop Enfants La Timone, AP HM, Serv Med Legale & Droit Med, Marseille, France
[4] Hop Georges Pompidou, AP HP, Serv Immunol, Paris, France
关键词
atypical haemolytic-uraemic syndrome; coronary thrombotic microangiopathy; factor H mutation; sudden death; HEMOLYTIC-UREMIC SYNDROME; THROMBOTIC THROMBOCYTOPENIC PURPURA; CARDIAC-TAMPONADE; MUTATIONS; CARDIOMYOPATHY; FAILURE;
D O I
10.1093/ndt/gfq160
中图分类号
R3 [基础医学]; R4 [临床医学];
学科分类号
1001 ; 1002 ; 100602 ;
摘要
Cardiac complications are frequently seen in thrombotic thrombocytopaenic purpura related to ADAMTS13 deficiency. We describe the case of a 43-year-old woman who was diagnosed with an atypical haemolytic uraemic syndrome (aHUS) associated with a pathogenic mutation in the factor H gene (C623S). After 15 days of treatment, she suffered a sudden cardiac arrest and died despite intensive resuscitation attempts. She showed only one cardiovascular risk factor, hypercholesterolaemia. Her sudden death was secondary to cardiac infarction related to a coronary thrombotic microangiopathy. This is the first case of aHUS related to a mutation in the factor H gene associated with cardiac microangiopathy. This case emphasizes the need to screen for cardiac complication during the treatment of aHUS.
引用
收藏
页码:2028 / 2032
页数:5
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