Акушерский атипичный гемолитико-уремический синдром (аГУС) является одной из причин развития острого повреждения почек (ОПП) и может определять прогноз как у матери, так и у ребенка. Цель исследования. Анализ особенностей клинической картины и течения акушерского аГУС, манифестировавшего ОПП. Материал и методы. С 2011 по 2017 г. наблюдались 45 пациенток в возрасте от 16 до 42 лет, у которых аГУС развился во время беременности или непосредственно после родов. Результаты и обсуждение. У всех пациенток отмечалось развитие ОПП (уровень креатинина сыворотки – 521,5±388,0 мкмоль/л), при этом у 93,3% женщин тромботическая микроангиопатия носила системный характер с развитием полиорганной недостаточности (ПОН). Среднее число пораженных органов составило 3,7±1,2. У всех пациенток развитию аГУС предшествовали акушерские осложнения, оперативные вмешательства, инфекция и т. д. В исходе аГУС у 53,4% женщин отмечено полное восстановление функции почек, у 11,1% развилась хроническая болезнь почек 4–5-й стадий, у 35,5% – диализ-зависимая терминальная почечная недостаточность. Летальность среди всех пациенток составила 23,9%, перинатальная смертность – 32,6%. При раннем старте терапии Экулизумабом (в течение 1–2 нед от дебюта аГУС), по сравнению с началом терапии позже 3 нед, шансы благоприятного исхода для матери увеличиваются в 5,33 раза, а шансы полностью восстановить функцию почек – в 48,7 раза. Заключение. Акушерский аГУС характеризуется развитием ОПП в 100% случаев. У подавляющего большинства пациенток акушерский аГУС протекает с развитием ПОН. Своевременная диагностика аГУС и незамедлительное начало терапии экулизумабом позволяют не только спасти жизнь пациенткам с аГУС, но и полностью восстановить их здоровье.
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.
1. Bentata Y, Housni B, Mimouni A, Azzouzi A, Abouqal R. Acute kidney injury related to pregnancy in developing countries: etiology and risk factors in an intensive care unit. J Nephrol. 2012;25:764-75.
doi: 10.5301/jn.5000058
2. Nwoko R, Plecas D, Garovic VD. Acute kidney injury in the pregnant patient. Clin Nephrol. 2012;78:478-86. doi: 10.5414/CN107323
3. Van Hook JW. Acute kidney injury during pregnancy. Clin Obstet Gynecol. 2014;57:851-61. doi: 10.1097/GRF.0000000000000069
4. Gopalakrishnan N, Dhanapriya J, Muthukumar P, Sakthirajan R, Dineshkumar T, Thirumurugan S, Balasubramaniyan T. Acute kidney injury in pregnancy – a single center experience. Renal Failure. 2015;37(9):1476-80. doi: 10.3109/0886022X.2015.1074493
5. Godara SM, Kute VB, Trivedi HL, Vanikar AV, Shah PR, Gumber MR, et al. Clinical profile and outcome of acute kidney injury related to pregnancy in developing countries: a single-center study from India. Saudi J Kidney Dis Transpl. 2014;25:906-11. doi: 10.4103/1319-2442.135215
6. Hildebrand AM, Liu K, Shariff SZ, Ray JG, Sontrop JM, Clark WF, et al. Characteristics and outcomes of AKI treated with dialysis during pregnancy and the postpartum period. J Am Soc Nephrol. 2015;26:3085-91. doi: 10.1681/ASN.2014100954
7. Patel ML, Sachan R, Radheshyam SP. Acute renal failure in pregnancy: tertiary centre experience from north Indian population. Niger Med J. 2013;54:191-5. doi: 10.4103/0300-1652.114586
8. Tsai HM. A Mechanistic Approach to the Diagnosis and Management of Atypical Hemolytic Uremic Syndrome. Transfus Med Rev. 2014 Oct;28(4):187-97.
9. Fakhouri F, Fremeaux-Bacchi V. Does hemolytic uremic syndrome differ from thrombotic thrombocytopenic purpura. Nat Clin Pract Nephrol. 2007;3:679-87.
10. Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. N Engl J Med. 2009;361:1676-87.
11. Козловская Н.Л., Коротчаева Ю.В., Боброва Л.А., Шилов Е.М. Акушерский атипичный гемолитико-уремический синдром: первый российский опыт диагностики и лечения. Нефрология. 2016;20(2):68-81 [Kozlovskaya NL, Korotchaeva YV, Bobrova LA, Shilov EM. Obstetric atypical hemolytic uremic syndrome: the first russian experience of diagnosis and treatment. Nefrologiya = Nephrology (Saint-Petersburg). 2016;20(2):68-80 (In Russ.)].
12. Fakhouri F, Vercel C, Frémeaux-Bacchi V. Obstetric nephrology: AKI and thrombotic microangiopathies in pregnancy. Clin J Am Soc Nephrol. 2012 Dec;7(12):2100-6.
13. Huerta A, Arjona E, Portoles J, Lopez-Sanchez P, Rabasco C,
Espinosa M, Cavero T, Blasco M, Cao M, Manrique J, Cabello-Chavez V, Suñer M, Heras M, Fulladosa X, Belmar L, Sempere A, Peralta C, Castillo L, Arnau A, Praga M, Rodriguez de Cordoba S. A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome. Kidney Int. 2018 Feb;93(2):450-9. doi: 10.1016/j.kint. 2017.06.022
14. Коротчаева Ю.В., Козловская Н.Л., Демьянова К.А., Боброва Л.А., Шаталов П.А., Коростин Д.О., Ильинский В.В., Борисевич Д.И., Красненко А.Ю. Генетические аспекты акушерского атипичного гемолитико-уремического синдрома. Клиническая нефрология. 2017;(1):12-7 [Korotchaeva YV, Kozlovskaya NL, Demyanova KA, Bobrova LA, Shatalov PA, Korostin DO, Linsky VV, Borisevich DI, Krasnenko AU. Genetic aspects of obstetric atypical hemolytic uremic syndrome. Klinicheskaya Nefrologiya = Clinical Nephrology. 2017;(1):12-7 (In Russ.)].
15. Gopalakrishnan N, Dhanapriya J, Muthukumar P, Sakthirajan R, Dineshkumar T, Thirumurugan S, Balasubramaniyan T. Acute kidney injury in pregnancy-a single center experience. Ren Fail. 2015;37(9): 1476-80. doi: 10.3109/0886022X.2015.1074493
16. Bentata Y, Housni B, Mimouni A, Azzouzi A, Abouqal R. Acute kidney injury related to pregnancy in developing countries: etiology and risk factors in an intensive care unit. J Nephrol. 2012;25:764-75.
doi: 10.5301/jn.5000058
________________________________________________
1. Bentata Y, Housni B, Mimouni A, Azzouzi A, Abouqal R. Acute kidney injury related to pregnancy in developing countries: etiology and risk factors in an intensive care unit. J Nephrol. 2012;25:764-75.
doi: 10.5301/jn.5000058
2. Nwoko R, Plecas D, Garovic VD. Acute kidney injury in the pregnant patient. Clin Nephrol. 2012;78:478-86. doi: 10.5414/CN107323
3. Van Hook JW. Acute kidney injury during pregnancy. Clin Obstet Gynecol. 2014;57:851-61. doi: 10.1097/GRF.0000000000000069
4. Gopalakrishnan N, Dhanapriya J, Muthukumar P, Sakthirajan R, Dineshkumar T, Thirumurugan S, Balasubramaniyan T. Acute kidney injury in pregnancy – a single center experience. Renal Failure. 2015;37(9):1476-80. doi: 10.3109/0886022X.2015.1074493
5. Godara SM, Kute VB, Trivedi HL, Vanikar AV, Shah PR, Gumber MR, et al. Clinical profile and outcome of acute kidney injury related to pregnancy in developing countries: a single-center study from India. Saudi J Kidney Dis Transpl. 2014;25:906-11. doi: 10.4103/1319-2442.135215
6. Hildebrand AM, Liu K, Shariff SZ, Ray JG, Sontrop JM, Clark WF, et al. Characteristics and outcomes of AKI treated with dialysis during pregnancy and the postpartum period. J Am Soc Nephrol. 2015;26:3085-91. doi: 10.1681/ASN.2014100954
7. Patel ML, Sachan R, Radheshyam SP. Acute renal failure in pregnancy: tertiary centre experience from north Indian population. Niger Med J. 2013;54:191-5. doi: 10.4103/0300-1652.114586
8. Tsai HM. A Mechanistic Approach to the Diagnosis and Management of Atypical Hemolytic Uremic Syndrome. Transfus Med Rev. 2014 Oct;28(4):187-97.
9. Fakhouri F, Fremeaux-Bacchi V. Does hemolytic uremic syndrome differ from thrombotic thrombocytopenic purpura. Nat Clin Pract Nephrol. 2007;3:679-87.
10. Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. N Engl J Med. 2009;361:1676-87.
11. [Kozlovskaya NL, Korotchaeva YV, Bobrova LA, Shilov EM. Obstetric atypical hemolytic uremic syndrome: the first russian experience of diagnosis and treatment. Nefrologiya = Nephrology (Saint-Petersburg). 2016;20(2):68-80 (In Russ.)].
12. Fakhouri F, Vercel C, Frémeaux-Bacchi V. Obstetric nephrology: AKI and thrombotic microangiopathies in pregnancy. Clin J Am Soc Nephrol. 2012 Dec;7(12):2100-6.
13. Huerta A, Arjona E, Portoles J, Lopez-Sanchez P, Rabasco C,
Espinosa M, Cavero T, Blasco M, Cao M, Manrique J, Cabello-Chavez V, Suñer M, Heras M, Fulladosa X, Belmar L, Sempere A, Peralta C, Castillo L, Arnau A, Praga M, Rodriguez de Cordoba S. A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome. Kidney Int. 2018 Feb;93(2):450-9. doi: 10.1016/j.kint. 2017.06.022
14. [Korotchaeva YV, Kozlovskaya NL, Demyanova KA, Bobrova LA, Shatalov PA, Korostin DO, Linsky VV, Borisevich DI, Krasnenko AU. Genetic aspects of obstetric atypical hemolytic uremic syndrome. Klinicheskaya Nefrologiya = Clinical Nephrology. 2017;(1):12-7 (In Russ.)].
15. Gopalakrishnan N, Dhanapriya J, Muthukumar P, Sakthirajan R, Dineshkumar T, Thirumurugan S, Balasubramaniyan T. Acute kidney injury in pregnancy-a single center experience. Ren Fail. 2015;37(9): 1476-80. doi: 10.3109/0886022X.2015.1074493
16. Bentata Y, Housni B, Mimouni A, Azzouzi A, Abouqal R. Acute kidney injury related to pregnancy in developing countries: etiology and risk factors in an intensive care unit. J Nephrol. 2012;25:764-75.
doi: 10.5301/jn.5000058
1 ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет), Москва, Россия;
2 ГБУЗ МО «Московский областной научно-исследовательский клинический институт им. М.Ф. Владимирского», Москва, Россия
1 I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Moscow, Russia;
2 M.F. Vladimirsky Moscow Regional Research Clinical Institute, Moscow, Russia