منابع مشابه
Atypical HUS and complement dysregulation.
I n 1998, a linkage analysis in three families with atypical hemolytic uremic syndrome (aHUS) demonstrated segregation to the RCA (regulators of complement activation) cluster of genes on 1q32 (1). In one of these families, a missense mutation was found in the gene (CFH) encoding the soluble complement regulator factor H. Subsequently, more than 50 different CFH mutations have been described bo...
متن کاملHUS and atypical HUS.
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by intravascular hemolysis, thrombocytopenia, and acute kidney failure. HUS is usually categorized as typical, caused by Shiga toxin-producing Escherichia coli (STEC) infection, as atypical HUS (aHUS), usually caused by uncontrolled complement activation, or as secondary HUS with a coexisting disease. In recent years,...
متن کاملAtypical HUS caused by anti-complement factor H antibody: a hematologist's perspective
Hemoglobin 4.5 g/dL 11.5–15.5 g/dL MCV 98 fL 77–94 fL RBC count 3.2×10/L 4.0–5.2×1012/L Total leukocyte count 10.3×10/L 5.0–13.0×109/L Platelet count 60×10/L 170–450×109/L Reticulocyte production index 7.2 0.5–2.5 Urea 284 mg/dL 20–40 mg/dL Creatinine 6.1 mg/dL 1–2 mg/dL Uric acid 24.1 mg/dL 2–4 mg/dL Total bilirubin 3.3 mg/dL Up to 1.0 mg/dL AST 127 U/L 0–35 U/L ALT 30 U/L 0–35 U/L LDH 3,876 U...
متن کاملA new therapeutic strategy for atypical HUS.
In addition to the observations ofMerrill et al, several other articles published during 2014 to 2017 have reported that discontinuing eculizumab in patients during remission resulted in relapse in only ;one-third of patients. Eculizumab is a monoclonal antibody directed against complement component C5 (see figure) that is effective in inducing remission in pediatric and adult patients with acu...
متن کاملSuper factor B-gets atypical HUS.
a common ligand with GPVI and requires previous activating signals to adopt a high-affinity conformation needed for colla-gen adhesion. Mazzucato et al make an important contribution by showing that, as platelets interact with collagen under flow, ␣21 receptors generate calcium signals that are distinct from those generated by GPVI. Surprisingly, ␣21-dependent calcium signals are even generat...
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ژورنال
عنوان ژورنال: Japanese Journal of Thrombosis and Hemostasis
سال: 2016
ISSN: 0915-7441,1880-8808
DOI: 10.2491/jjsth.27.553