Galic Slobodan, Csuka Dorottya, Prohászka Zoltán, Turudic Daniel, Dzepina Petra, Milosevic Danko
University Hospital Centre Zagreb, Kispaticeva, Zagreb.
Research Laboratory, 3rd Department of Internal Medicine and MTA-SE Research Group of Immunology and Hematology, Hungarian Academy of Sciences and Semmelweis University, Budapest, Hungary.
Medicine (Baltimore). 2019 Jan;98(4):e14105. doi: 10.1097/MD.0000000000014105.
This article describes a child with a life-threatening multiorgan failure with disseminated intravascular coagulation (DIC) and massive complement consumption. To our knowledge this therapeutic approach was for the first time effectively applied in a pediatric patient.
A 14-month-old boy was presented with a severe, rapidly progressing, life-threatening disease because of sudden onset of fever, hemathemesis, hematuria, and bloody diarrhoea alongside fast spreading hematomas and general corporeal edema.
The most plausible diagnosis in our patient is Clostridium difficile sepsis-induced thrombotic microangiopathy alongside with DIC and consumption coagulopathy. The diagnosis was confirmed by positive C difficile bacteria strain in coproculture, clinical, and laboratory tests affirming DIC and global complement activation and consumption.
The patient was treated with antibiotics (Metronidazole, Vancomycin), plasmapheresis, dialysis, methylprednisolone, mycophenolate mofetil, and Eculizumab.
The child is in fair overall condition in a 2 year follow-up with no complications save chronic renal failure.
In rare cases of sepsis with massive complement consumption, a case-sensitive Eculizumab therapy may be at least considered after the resolution of life-threatening multiorgan failure. The application of this drug can be performed only after sepsis induced disease is put under control. A fast withdrawal of Eculizumab after control of massive complement consumption is recommended to prevent triggering of second sepsis reactivation.
本文描述了一名患有危及生命的多器官功能衰竭、弥散性血管内凝血(DIC)和大量补体消耗的儿童。据我们所知,这种治疗方法首次在儿科患者中得到有效应用。
一名14个月大的男孩因突然发热、咯血、血尿、血性腹泻,同时伴有迅速扩散的血肿和全身水肿,出现了一种严重的、进展迅速的、危及生命的疾病。
我们患者最合理的诊断是艰难梭菌败血症诱发的血栓性微血管病,同时伴有DIC和消耗性凝血病。粪便培养中艰难梭菌菌株呈阳性,临床和实验室检查证实了DIC以及全身补体激活和消耗,从而确诊。
该患者接受了抗生素(甲硝唑、万古霉素)、血浆置换、透析、甲泼尼龙、霉酚酸酯和依库珠单抗治疗