ESPN 51th Annual Meeting

ESPN 2018


 
Extrarenal manifestations of atypical hemolytic-uremic syndrome in children
KHADIZHA EMIROVA 3 GALINA GENERALOVA 1 TATIANA ABASEEVA 2 TATIANA PANKRATENKO 2 OLGA ORLOVA 3 ALEXANDR MUZUROV 4 EVGENIIA TOLSTOVA 3 SOFIYA MSTISLAVSKAYA 3

1- CHILDREN ST. VLADIMIR HOSPITAL
2- MOSCOW REGIONAL RESEARCH CLINICAL INSTITUTE NAMED AFTER M.F.VLADIMIRSKY
3- MOSCOW STATE MEDICAL DENTIST UNIVERSITY NAMED AFTER A.I.EVDOKIMOV
4- RUSSIAN MEDICAL ACADEMY OF POST-GRADUATE EDUCATION
 
Introduction:

To estimate the frequency of extrarenal manifestations of aHUS in children.

Material and methods:

129 patients with aHUS were examined: 57 boys (44.2%), 72 girls (55.8%) (mean age 4.58±3.82 years) from 2000 to 2018.

Results:

19 (14.7%) patients had hemolysis, thrombocytopenia and acute renal failure. Extrarenal manifestations in acute aHUS were detected in 110 (85.3%) patients in the form of lesions of: CNS – in 53 (41.1%), cardiovascular system (CVS)-in 50 (36.5%), lungs – in 34 (26.3%), gastrointestinal tract – in 97 (75.2%), eyes– in 25 (19.4%), skin – in 1 (0.8%). Dysfunction of 2 systems was diagnosed in 32 (29.1%) cases, 3 systems-30 (23.2%), 4 systems – 30 (23.2%), 5 systems-14 (10.9%), 6 systems – 4 (3.1%). CNS damage is clinically manifested as psychomotor agitation (15,1%), hallucinations (1.9 percent), tremor (11,3%), impaired coordination (1,9%), seizures (66%), impaired consciousness (stupor – 22,6%, coma 1 and 9.4%, coma 2 of -7.5%, coma 3 to 5.7%), brain edema (11.3 percent), acute cerebrovascular disease (7.5%) and hemorrhage in the brain (11.3 per cent). The defeat of the CVS was characterized by: edema in the myocardium (10%), decreased ejection fraction (28%), dilatation of the cavities of the heart (72%), hypertrophy of the myocardium (28%), pulmonary hypertension (4%), acute cardiovascular failure (6%), myocardial infarction (4%), arrhythmia (8%), coronaropathy (8%), upper subendocardialnah ischemia (10%), subepicardial ischemia (2%), enlargement of the aorta (8%), violation of diastolic function of the left ventricle (6%). Lung damage was manifested by the development of respiratory distress syndrome (73.5%), pneumonia (23.5%), atelectasis (5.9%), pulmonary hemorrhage (8.8%). On the part of the gastrointestinal tract, abdominal syndrome was observed in 22.7% of cases, cytolysis – 80.4%, biliary sludge – 30%, cholelithiasis – 6.2%, pancreatic necrosis – 1%, ulcer of the upper parts – 5.1%, intestinal paresis – 4.1%, bleeding – 5.1%. Defeat of the eyes was characterized by the development of hemorrhagic angioretinopathia (28%), exudative edema of the optic disc (8%), retinal vascular angiopathy (56%), loss of visual fields (12%). In 1 patient, TMA of the skin was manifested by necrosis of the nail phalanges of both hands and feet. All patients (n=110) had plasma therapy in their debut, 75 (68.2%) patients subsequently used eculizumab (early start of therapy – 53.3%, late start – 46.7%). Complete remission was achieved in 73 (66.4%) of 110, CKD 1-2 stages formed in 19 (17.3%), CKD 3-4 stage – 12 (10.9%), CKD 5 stage – 10 (9%). In the acute period died 6 (46,1%), in the remote-7 (53,9%) children.

Conclusions:

Extrarenal manifestations at the beginning of the disease occur in the majority of patients with aHUS. It could be a manifestation of the disease or secondary phenomena due to volumetric overload, uremic encephalopathy, electrolyte disturbances. The high frequency of extrarenal manifestations emphasizes the need for timely start of targeted therapy.