Atypical hemolytic uremic syndrome in pregnancy and hellp-syndrome. Differential diagnosis


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Abstract

It is known that the development of atypical hemolytic-uremic syndrome (aHUS) may be associated with pregnancy, which triggers this hereditary disease of the complement system. During pregnancy, however, another life-threatening obstetric complication of pre-eclampsia - HELLP-syndrome - may develop. It also represents a thrombotic microangiopathy (ТМА). Often, there are difficulties in the differential diagnosis of HELLP-syndrome and aHUS during pregnancy and the postpartum period. The author attempts to understand the pathogenesis of the two conditions and to provide criteria for differential diagnosis. There is no doubt that in the case of aHUS, the alternative pathway of complement activation is involved. But in pre-eclampsia and HELLP-syndrome, activation of the complement system is not the only and main pathogenetic pathway of the syndrome. However, the involvement of these pathways in both syndromes produces similar clinical manifestations of aHUS and HELLP-syndrome. One argument in favor of differential diagnosis is the fact that the HELLP-syndrome after birth in most cases is completely leveled, whereas aHUS in most cases manifests within the first 3 months after childbirth. However, the greatest difficulties arise in situations when aHUS develops in the third trimester and causes liver disease, resembling the clinical manifestations of HELLP-syndrome, and when HELLP-syndrome occurs in postpartum period, or having arisen during pregnancy is not stopped in the postpartum period. The process of differential diagnosis can also be difficult in cases of liver damage in aHUS. Therapy of aHUS associated with pregnancy using eculizumab allows effectively control the disease in these patients.

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M. M Batjushin

Email: nephr-roon@rambler.ru

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