Various phenotypes of postpartum atypical hemolytic uremic syndrome: the role of genetic testing in determining prognosis. Case report

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Abstract

We report a case of atypical hemolytic uremic syndrome (aHUS) that occurred after childbirth in a patient with a history of numerous recurrent episodes of TMA with nephrotic proteinuria and impaired renal function. At 33 weeks of the first spontaneous pregnancy, proteinuria up to 0.8 g/l was first registered, at 38 weeks she was hospitalized with proteinuria, reaching a maximum of 13 g/l, she was delivered promptly, after which progressive thrombocytopenia was noted over the next few days (up to 44×109/l) and anemia and severe arterial hypertension, which could not be corrected by several groups of antihypertensive drugs. Initiated plasma therapy had no effect. After exclusion of all other causes of TMA, therapy with eculizumab was initiated, which made it possible to quickly and completely stop the phenomena of TMA. The presented observation demonstrates the successful treatment of recurrent course of aHUS with eculizumab with the achievement of complete recovery of kidney function in a patient with a homozygous mutation in the MCP gene. It is worth noting the importance of genetic research even in those situations where clinically aHUS is beyond doubt.

About the authors

Tatiana V. Kirsanova

Kulakov National Medical Research Center for Obstetrics, Gynecology and Perinatology

Author for correspondence.
Email: a_tatya@mail.ru
ORCID iD: 0000-0002-6125-590X

канд. мед. наук, вед. науч. сотр. ин-та анестезиологии-реаниматологии и трансфузиологии ФГБУ «НМИЦ АГП им. В.И. Кулакова»

Russian Federation, Moscow

Alina I. Balakireva

Lomonosov Moscow State University

Email: a_tatya@mail.ru
ORCID iD: 0000-0003-0362-580X

аспирант фак-та фундаментальной медицины ФГБОУ ВО «МГУ им. М.В. Ломоносова»

Russian Federation, Moscow

Tatiana A. Fedorova

Kulakov National Medical Research Center for Obstetrics, Gynecology and Perinatology

Email: a_tatya@mail.ru
ORCID iD: 0000-0001-6883-4456

д-р мед. наук, проф., зав. отд. трансфузиологии и экстракорпоральной гемокоррекции ФГБУ «НМИЦ АГП им. акад. В.И. Кулакова»

Russian Federation, Moscow

Aleksei V. Pyregov

Kulakov National Medical Research Center for Obstetrics, Gynecology and Perinatology

Email: a_tatya@mail.ru
ORCID iD: 0000-0001-8382-9671

д-р мед. наук, проф., дир. ин-та анестезиологии-реаниматологии и трансфузиологии, зав. каф. анестезиологии и реаниматологии ФГБУ «НМИЦ АГП им. акад. В.И. Кулакова»

Russian Federation, Moscow

Oleg V. Rogachevskiy

Kulakov National Medical Research Center for Obstetrics, Gynecology and Perinatology

Email: a_tatya@mail.ru
ORCID iD: 0000-0001-9847-5765

д-р мед. наук, проф., зав. отд. экстракорпоральных методов лечения и детоксикации ФГБУ «НМИЦ АГП им. акад. В.И. Кулакова»

Russian Federation, Moscow

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