CLINICAL CASE OF OBSTETRIC ATYPICAL HEMOLYTIC UREMIC SYNDROME

Here we present a clinical case of atypical hemolytic uremic syndrome (aHUS) associated with delivery. Even in timely diagnosed patients with aHUS, case fatality rate reaches 29%. Currently, aHUS is considered as a thrombotic microangiopathy, along with typical hemolytic uremic syndrome, thrombotic...

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Main Authors: SVETLANA CH. Kara-Sal, VADIM G. Mozes, KIRA B. Mozes, ELENA V. Rudaeva, IGOR S. Zakharov, SVETLANA I. Yelgina
Format: Article
Language:Russian
Published: Kemerovo State Medical University 2019-03-01
Series:Фундаментальная и клиническая медицина
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Online Access:https://fcm.kemsmu.ru/jour/article/view/135
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author SVETLANA CH. Kara-Sal
VADIM G. Mozes
KIRA B. Mozes
ELENA V. Rudaeva
IGOR S. Zakharov
SVETLANA I. Yelgina
author_facet SVETLANA CH. Kara-Sal
VADIM G. Mozes
KIRA B. Mozes
ELENA V. Rudaeva
IGOR S. Zakharov
SVETLANA I. Yelgina
author_sort SVETLANA CH. Kara-Sal
collection DOAJ
description Here we present a clinical case of atypical hemolytic uremic syndrome (aHUS) associated with delivery. Even in timely diagnosed patients with aHUS, case fatality rate reaches 29%. Currently, aHUS is considered as a thrombotic microangiopathy, along with typical hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, severe preeclampsia, and HELLP syndrome. There is a considerable genetic predisposition to all abovementioned types of thrombotic microangiopathy; however, additional significant stimulation of the complement system is mandatory for aHUS to occur. In children, it can be triggered by respiratory or gastrointestinal infections, while in women it is typically provoked by pregnancy. High mortality of aHUS is due to its rarity and consequent low awareness, non-specific symptoms at its initiation, and rapid development of multiple organ failure including acute kidney injury due to generalized thrombosis in the microvasculature. Successful treatment of aHUS largely depends on the timeliness of diagnosis, specific therapy with eculizumab in combination with a renal replacement therapy, and rapid delivery according to the protocol on severe pre-eclampsia.
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2542-0941
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publishDate 2019-03-01
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series Фундаментальная и клиническая медицина
spelling doaj-art-269a01e1483f4e02a99cdfba8e90fb2a2025-08-20T03:57:40ZrusKemerovo State Medical UniversityФундаментальная и клиническая медицина2500-07642542-09412019-03-01419199134CLINICAL CASE OF OBSTETRIC ATYPICAL HEMOLYTIC UREMIC SYNDROMESVETLANA CH. Kara-Sal0VADIM G. Mozes1KIRA B. Mozes2ELENA V. Rudaeva3IGOR S. Zakharov4SVETLANA I. Yelgina5Tyva Republic Perinatal CenterKemerovo State Medical UniversityKemerovo State Medical UniversityKemerovo State Medical UniversityKemerovo State Medical UniversityKemerovo State Medical UniversityHere we present a clinical case of atypical hemolytic uremic syndrome (aHUS) associated with delivery. Even in timely diagnosed patients with aHUS, case fatality rate reaches 29%. Currently, aHUS is considered as a thrombotic microangiopathy, along with typical hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, severe preeclampsia, and HELLP syndrome. There is a considerable genetic predisposition to all abovementioned types of thrombotic microangiopathy; however, additional significant stimulation of the complement system is mandatory for aHUS to occur. In children, it can be triggered by respiratory or gastrointestinal infections, while in women it is typically provoked by pregnancy. High mortality of aHUS is due to its rarity and consequent low awareness, non-specific symptoms at its initiation, and rapid development of multiple organ failure including acute kidney injury due to generalized thrombosis in the microvasculature. Successful treatment of aHUS largely depends on the timeliness of diagnosis, specific therapy with eculizumab in combination with a renal replacement therapy, and rapid delivery according to the protocol on severe pre-eclampsia.https://fcm.kemsmu.ru/jour/article/view/135атипичный гемолитико-уремический синдромтромботическая микроангиопатияadamts-13atypical hemolytic uremic syndromethrombotic microangiopathyadamts-13
spellingShingle SVETLANA CH. Kara-Sal
VADIM G. Mozes
KIRA B. Mozes
ELENA V. Rudaeva
IGOR S. Zakharov
SVETLANA I. Yelgina
CLINICAL CASE OF OBSTETRIC ATYPICAL HEMOLYTIC UREMIC SYNDROME
Фундаментальная и клиническая медицина
атипичный гемолитико-уремический синдром
тромботическая микроангиопатия
adamts-13
atypical hemolytic uremic syndrome
thrombotic microangiopathy
adamts-13
title CLINICAL CASE OF OBSTETRIC ATYPICAL HEMOLYTIC UREMIC SYNDROME
title_full CLINICAL CASE OF OBSTETRIC ATYPICAL HEMOLYTIC UREMIC SYNDROME
title_fullStr CLINICAL CASE OF OBSTETRIC ATYPICAL HEMOLYTIC UREMIC SYNDROME
title_full_unstemmed CLINICAL CASE OF OBSTETRIC ATYPICAL HEMOLYTIC UREMIC SYNDROME
title_short CLINICAL CASE OF OBSTETRIC ATYPICAL HEMOLYTIC UREMIC SYNDROME
title_sort clinical case of obstetric atypical hemolytic uremic syndrome
topic атипичный гемолитико-уремический синдром
тромботическая микроангиопатия
adamts-13
atypical hemolytic uremic syndrome
thrombotic microangiopathy
adamts-13
url https://fcm.kemsmu.ru/jour/article/view/135
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AT elenavrudaeva clinicalcaseofobstetricatypicalhemolyticuremicsyndrome
AT igorszakharov clinicalcaseofobstetricatypicalhemolyticuremicsyndrome
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