In:
Pediatria. Journal named after G.N. Speransky, Pediatria, Ltd., Vol. 100, No. 4 ( 2021-08-06), p. 12-19
Abstract:
Atypical hemolytic uremic syndrome (aHUS) is an orphan disease caused by hyperactivation of the alternative complement pathway. Objective of the study: to assess the state of the «ADAMTS13 – von Willebrand factor (vWF) – platelets» system in children with aHUS. Materials and methods of research: [by the FRET method (fluorescence resonance energy transfer) for the FRETSVWF73 (Peptide Institude, Inc., Japan)] hydrolysis of the fluorescent substrate and ADAMTS13 antigen [by ELISA using TECHNOZYM® ADAMTS13 5450551 ELISA (Technoclone GmbH, Austria)] , vWF activity [for platelet agglutination (aggregation) in the presence of ristomycin (NPO Renam reagent kit for the ALAT-230LA-2 aggregometer, Russia)] and vWF antigen [by ELISA using the TECHNOZYM® vWF kit: Ag 5450201 ELISA (Technoclone GmbH , Austria)] . Results: there was a decrease in the activity and concentration of ADAMTS13 in 63% and 62% of patients, respectively. A decrease in vWF activity was noted in 44% of cases, an increase in its concentration – in 54% of children. Thrombocytopenia was diagnosed in 99% of children. Conclusion: the imbalance in the «ADAMTS13 – vWF – platelets» system supports the process of thrombus formation with the development of organ ischemia in aHUS under conditions of endothelial dysfunction. Reduced ADAMTS13 activity predicts the severity of the disease.
Type of Medium:
Online Resource
ISSN:
0031-403X
,
1990-2182
Uniform Title:
СИСТЕМА «АDAMTS13 – ФАКТОР ФОН ВИЛЛЕБРАНДА – ТРОМБОЦИТЫ» ПРИ АТИПИЧНОМ ГЕМОЛИТИКО- УРЕМИЧЕСКОМ СИНДРОМЕ У ДЕТЕЙ
DOI:
10.24110/0031-403X-2021-4
DOI:
10.24110/0031-403X-2021-100-4-12-19
Language:
Unknown
Publisher:
Pediatria, Ltd.
Publication Date:
2021
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