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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| Format: | Article |
| Language: | Russian |
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Kemerovo State Medical University
2019-03-01
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| 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. |
| format | Article |
| id | doaj-art-269a01e1483f4e02a99cdfba8e90fb2a |
| institution | Kabale University |
| issn | 2500-0764 2542-0941 |
| language | Russian |
| publishDate | 2019-03-01 |
| publisher | Kemerovo State Medical University |
| record_format | Article |
| 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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