Clinical observation of a patient with thrombotic thrombocytopenic purpura with renal and intestinal lesions

The article presents a brief description of a rare disease - thrombotic thrombocytopenic purpura (Moshkovits - disease), which is based on the deficiency of ADAMTS-13 metalloproteinase, leading to the development of thrombotic microangiopathy with the defeat of vital organs. The article also describ...

Ausführliche Beschreibung

Gespeichert in:
Bibliographische Detailangaben
Veröffentlicht in:Terapevtic̆eskii arhiv 2019-07, Vol.91 (7), p.106-110
Hauptverfasser: Stolyar, A G, Avdonin, P V, Valamina, I E, Stolyarevich, E S
Format: Artikel
Sprache:eng ; rus
Schlagworte:
Online-Zugang:Volltext
Tags: Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
Beschreibung
Zusammenfassung:The article presents a brief description of a rare disease - thrombotic thrombocytopenic purpura (Moshkovits - disease), which is based on the deficiency of ADAMTS-13 metalloproteinase, leading to the development of thrombotic microangiopathy with the defeat of vital organs. The article also describes the clinical observation of a patient with the Moshkovits - disease. The features of the above observation are involvement in the pathological process of the kidneys and intestines, while in the classical descriptions of the disease there is a predominant lesion of the Central nervous system, as well as the genetic form of the disease.
ISSN:0040-3660
2309-5342
DOI:10.26442/00403660.2019.07.000131