ESPN 50th Annual Meeting

ESPN 2017


 
CARDIOVASCULAR COMPLICATIONS IN ATYPICAL HAEMOLYTIC URAEMIC SYNDROME IN CHILDREN
KHADIJA EMIROVA 1 EVGENIIA TOLSTOVA 1 OLGA ORLOVA 1 TATIANA PANKRATENKO 2 ALEXANDR MUZUROV 3 TATIANA ABASEEVA 2 ANASTASIA MAKULOVA 4

1- MOSCOW STATE MEDICAL DENTIST UNIVERSITY NAMED AFTER A.I.EVDOKIMOV
2- MOSCOW REGIONAL RESEARCH CLINICAL INSTITUTE NAMED AFTER M.F.VLADIMIRSKY
3- RUSSIAN MEDICAL ACADEMY OF POST-GRADUATE EDUCATION
4- THE RUSSIAN NATIONAL RESEARCH MEDICAL UNIVERSITY NAMED AFTER N.I. PIROGOV
 
Introduction:

 We estimated the incidence of cardiovascular complications (CVC) in children with atypical HUS(aHUS) and their outcomes depending on the type of therapy.

Material and methods:

 104 patients with aHUS were examined at the age of 2.5 months-17 years from 1999to2017. Genetic screening was performed in 17(16.3%) patients, among which in 2 cases CFH mutation was detected, in 1 case - CFI and C3 mutations, in 8 - complement genes polymorphisms. In 8(7,7%) cases anti-CFH antibody HUS was diagnosed. Echulizumab therapy was performed in 65(62.5%) patients.

Results:

 CVC in the acute period of aHUS were detected in 74(71.2%) patients: arterial hypertension(AH) in 52(70.3%), left ventricular dilation(LVD) in 27(36.5%), left atrial dilatation - 3(4.1%), decreasing of ejection fraction(EF) - 15(20.3%), ischemic manifestations and coronaropathy - in 6(8.1%). In 7(9.5%)cases cardiac glycosides were required. Two patients died in the acute period and seven in the remote one. CVC were associated with severity of acute kidney injury(AKI):creatinine and proteinuria were higher than in the group without CVC(439.9±250.4 vs 307.9±280.2μmol/l, 6.3±5.2 vs 1.6[0.3,3.3],p<0.05). Eculizumab was assigned to 49(66.2%) patients with CVC, of whom 22 patients(44.9%) in the acute period of the aHUS, 27(55.1%)-in hematologic remission, but persistent organ damage.Among patients with aHUS manifestation before 2012,CVC were detected more often than in the group of children who fell ill after 2012 because of access to eculizumab:85%vs50% (p <0.05). Among 7 patients with aHUS and CVC who did not receive eculizumab, only 1 with AKI was resolved; 2 children died because of the recurrence of aHUS, in 4 - persistent AH remained. In 10 cases, the intensity of LVD decreased, LF was normalized. Chronic CVC were formed in 48(64.9%) patients with aHUS, 12 of them did not receive eculizumab: AH - in 42(87.5%), LVD in 16(33.3%), LV hypertrophy - 5(10.4%), EF<60%-in4(8.3%), ischemic manifestations - 2(4.2%), ventricular extrasystoles - 2(4.2%), dilatation of the aorta, valve stenosis, compaction of the walls - 7 (14.6%). 3 patients died from the CVC: 2 - from acute cardiovascular failure, 1 - rupture of aortic aneurysm.

Conclusions:

 CVC are frequent extrarenal manifestation of the aHUS. The high frequency of CVC chronization in children with aHUS emphasizes the need for timely targeted therapy. The differentiation between CVC caused by TMA and secondary phenomena due to volume overload and/or hypertension is important, but not always clearly.