Obstetric-aHUS is a serious condition characterized by multiple organ failure (MOF) and a high mortality rate. Presentation of obstetric-aHUS is preceded by various precipitating factors, suggesting pregnancy complications, and not the pregnancy per se, often induce aHUS in women with a genetic predisposition to its development. A delay in the correct diagnosis and initiation of the most effective treatment can have serious consequences, reinforcing the need to raise awareness of obstetric-aHUS.
There are indications of an AP activation not only in pts with aHUS but in CAPS pts too. We suppose that the activity of factor H is a more sensitive indicator of complement system changes than factor H level. Patients with CAPS and aHUS have similar clinical and laboratory characteristics. However, CAPS is more severe, with the involvement of a larger number of vascular beds. Perhaps this is due to the double damaging effects on the endothelium ― of antiphospholipid antibodies (aPL) and activated complement. So we hypothesize that CAPS can be called aPL-mediated TMA in pts with a complement system defect.
Obstetric atypical hemolytic uremic syndrome (aHUS) is one of the reasons for the development of acute kidney injury (AKI) and can determine the prognosis of both mother and child. Aim. Analysis of clinical manifestations, course and outcomes of obstetric aHUS. Materials and methods. 45 patients with aHUS development during pregnancy or immediately after childbirth were observed between 2011 and 2017, age from 16 to 42 years. Results and discussion. All patients had AKI (serum creatinine 521,5±388,0 µmol/l, oliguria or anuria that required initiation of hemodialysis). 93.3% pts had extrarenal manifestations of TMA with the development of multiple organ failure (MOF). The mean number of damage organs was 3,7±1,2. In all patients, the development of aHUS was preceded by obstetric complications, surgery, infection, etc. In the outcome: 53.4% women showed complete recovery of renal function, 11.1% developed CKD 4-5 stages, 35.5% had dialysis-dependent end-stage renal failure (ESDR). Maternal mortality was 23.9%. Perinatal mortality was 32.6%. The early start of eculizumab treatment (within 1-2 weeks from the onset of aHUS), compared with therapy start after 3 weeks, increased the chances of favorable outcome for mother in 5.33 times, and the chances for normalization of renal function in 48.7 times. Conclusion. Obstetric aHUS is characterized by the development of AKI in 100% of cases. In most patients, the obstetric aHUS occurs with the development of MOF. Timely diagnosis of aHUS and immediate treatment by eculizumab allows not only to save the life of patients, but also completely restore their health.
Результаты. Различные документально подтвержденные признаки поражения сердца выявлены у 6 (13%) больных (5 -с диагнозом АГУС, 1 -с КАФС). У 5 пациентов вовлечение миокарда развилось в дебюте заболевания в рамках полиор-ганного поражения. Заключение. Представлены наблюдения поражения сердца при ТМА разного генеза. Точная частота вовлечения миокарда и его прогностическое значение у пациентов с ТМА до настоящего времени неизвестны. Ключевые слова: тромботические микроангиопатии, атипичный гемолитико-уремический синдром, катастрофический антифосфолипидный синдром, некоронарогенный инфаркт миокарда, сердечная недостаточность.Aim. To describe cardiac involvement in patients with acute thrombotic microangiopathy (TMA). Materials and methods. The case histories of 46 patients with proven TMA, including 17 patients diagnosed with atypical hemolytic uremic syndrome (aHUS) and 29 patients with catastrophic antiphospholipid syndrome (CAPS), were analyzed. Results. Different documentarily verified signs of cardiac involvement were revealed in 6 (13%) patients (5 and 1 patients diagnosed as having aHUS and CAPS, respectively). Five patients developed myocardial involvement at disease onset in the presence of multiple organ dysfunction. Conclusion. Cases of cardiac involvement in TMA of various genesis are presented. The exact incidence of myocardial involvement and its prognostic value are unknown so far. Key words: thrombotic microangiopathies, atypical hemolytic uremic syndrome, catastrophic antiphospholipid syndrome, noncoronarogenic myocardial infarction, heart failure.АГ -артериальная гипертония АГУС -атипичный гемолитико-уремический синдром АД -артериальное давление АФС -антифосфолипидный синдром ГД -гемодиализ ГУС -гемолитико-уремический синдром ИВЛ -искусственная вентиляция легких ИМ -инфаркт миокарда КАФС -катастрофический антифосфолипидный синдром КГ -коронарография КРС -кардиоренальный синдром ЛДГ -лактатдегидрогеназа ЛЖ -левый желудочек МЖП -межжелудочковая перегородка МНО -международное нормализованное отношение ОПН -острая почечная недостаточность ОПП -острое повреждение почек ОРИТ -отделение реанимации и интенсивной терапии ОЭКТ -однофотонная эмиссионная компьютерная томо-графия ПВВГДФ -продленная веновенозная гемодиафильтрация ПН -почечная недостаточность СДЛА -систолическое давление в легочной артерии СЗП -свежезамороженная плазма СКВ -системная красная волчанка СКФ -скорость клубочковой фильтрации СН -сердечная недостаточность ТМА -тромботические микроангиопатии ТТП -тромботическая тромбоцитопеническая пурпура ЦНС -центральная нервная система ЭК -эндотелиальные клетки ЭКГ -электрокардиограмма ЭхоКГ -эхокардиография ADAMTS-13 -a disintegrin and metalloprotease with thrombospondin-1-like domains, member 13 (металлопротеиназа AD-AMTS-13) VWF -фактор Виллебранда β 2 -ГП -β 2 -гликопротеин Тромботические микроангиопатии (ТМА) -гетеро-генная группа заболеваний, объединенных общностью гистологической картины и клинических проявлений при различии патогенетических звеньев. Основными симпто-мами ТМА служат тромбоцитоп...
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