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

Stolyar, A G; Avdonin, P V; Valamina, I E; Stolyarevich, E S.
Ter Arkh; 91(7): 106-110, 2019 Jul 15.
Artigo em Russo | MEDLINE | ID: mdl-32598743
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.
Selo DaSilva