Open AccessATYPICAL HEMOLYTIC-UREMIC SYNDROME IN GENERAL AND IN PREGNANCY
Author(s) -
А. В. Новиков
Publication year - 2018
Publication title -
arhiv akušerstva i ginekologii im. v.f. snegireva
Language(s) - English
Resource type - Journals
eISSN - 2687-1386
pISSN - 2313-8726
DOI - 10.18821/2313-8726-2018-5-3-132-139
Subject(s) - atypical hemolytic uremic syndrome , thrombotic microangiopathy , medicine , eculizumab , pregnancy , disease , rare disease , alternative complement pathway , genetic testing , eclampsia , intensive care medicine , preeclampsia , complement system , pediatrics , immunology , genetics , antibody , biology
Atypical hemolytic-uremic syndrome (aHUS) is an ultra-rare (orphan) disease, a form of thrombotic microangiopathy, which arises from a disturbance of the activation of an alternative complement pathway. Pregnancy is a frequent trigger for the onset of obstetric aHUS. Against the background of the disease in pregnant women, there is a high risk of developing pre-eclampsia, acute renal damage and consequently maternal mortality. In the world over the past 5 years, the number of confirmed cases of aHUS has increased. However, this is due not so much to the increase in the occurence of the disease as to the improvement in the methods of its diagnosis. The genetic nature of the aHUS dictates the need to create modern sensitive tests for the study of the complement system: measurement of the plasma concentration of factor H and factor I, C3, C4, genetic screening of regulatory genes, and others. It is also necessary to develop new drugs that, along with Eculizumabum (Soliris®, Alexion Pharmaceuticals, Cheshire, CT, USA) would be used in the therapy of aHUS.