Glycosylated Ferritin in Differential Diagnosis of Still's Disease, Sepsis and Other Macrophagic Activation Syndromes.
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 入组人数
- 60
- 试验地点
- 1
- 主要终点
- Fraction of glycosylated ferritin in serum
研究概览
简要总结
In healthy subjects, from 50 to 80 % of the serum ferritin is glycosylated [1, 2] . A decrease in the percentage of ferritin glycosylation can be observed in inflammatory diseases, malignancies, infections, or liver disease but is rarely less than 20% [3 , 4] . Percentage of glycosylated ferritin below 20% have been described in patients with adult Still's disease and haemophagocytosis lymphohistiocytic syndromes (HLH).
The glycosylated ferritin has been included in the diagnostic criteria for Still's disease in adults. A cut-off of less than 20 % has a sensitivity and specificity of 72 and 69 % respectively , and 35 and 94 % when combined with a total ferritin level greater than 5 times normal value. This parameter was also suggested to be a more specific marker to confirm a diagnosis of HLH than a high ferritin level ( > 500μg / L). However, several limitations of this parameter were highlighted, some conditions making its interpretation difficult : particularly in cases of major hepatic cytolysis and severe sepsis (miliary tuberculosis, lymphoma and disease Adult Still).
It is not always possible to distinguish severe sepsis, HLH syndrome and Still's disease.
A fine analysis of various glycoforms components of ferritin could be used to distinguish different subgroups of patients. Few data are available on the mechanism of secretion and glycosylation of ferritin, but the investigators assume that the glycosylation patterns of ferritin may vary between different disease states and reflect distinct underlying pathophysiological mechanisms.
详细描述
The objectives of this study are:
- . To evaluate the diagnostic performance of the assay of the glycosylated ferritin under HLH syndrome and Still's disease in adults.
- . To study the different glycoforms of ferritin from serum of patient with hyperferritinemia.
Materials and methods
- Patient groups studied and control group
- Any suspicion of HLH or Still's disease syndrome in adults
- Control groups: sepsis, severe sepsis, septic shock, inflammation ( lupus, rheumatoid arthritis), severe hepatic necrosis, hematologic malignancy at diagnosis or relapse
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- — 至 85 Years(Child, Adult, Older Adult)
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Suspicion of sepsis, macrophagic activation syndrome, Still's disease or hyperferritinemia (malignant disease, hepatic cytolysis)
排除标准
- •Normal ferritin level
结局指标
主要结局
Fraction of glycosylated ferritin in serum
时间窗: One year
次要结局
未报告次要终点
研究者
Francis Corazza
Head of laboratory of immunology
Brugmann University Hospital
